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CASEREPORTS Ci l t / Vol ume: 7S a y ı / I s s ue:2 Yı l / Yea r : 2018

H e mo p t i z i i l eB a ş v u r a nY e t i ş k i nB i r H a s t a d aS a ğP u l mo n e r A r t e r P r o k s i ma l i n d eK e s i n t i P r o x i ma l I n t e r r u p t i o no f R i g h t P u l mo n a r yA r t e r yi na nA d u l t P a t i e n t P r e s e n t i n gwi t hH e mo p t y s i s

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İ l o p r o s t v eB o s e n t a nK o mb i n a s y o nT e d a v i s i U y g u l a n a nB i r S j ö g r e nS e n d r o muİ l i ş k i l i P u l mo n e r H i p e r t a n s i y o nO l g u s u

AC a s eo f S j ö g r e n ’ s S y n d r o me R e l a t e dP u l mo n a r yA r t e r i a l H y p e r t e n s i o nT r e a t e dwi t hI l o p r o s t a n dB o s e n t a nC o mb i n a t i o nT h e r a p y A y ş eB a h a , B e r k a yE k i c i , N a l a nO g a n , E v r i mE y l e mA k p ı n a r

T r a v ma t i kP u l mo n e r A r t e r D i s e k s i y o n u : O l g uS u n u mu T r a u ma t i c P u l mo n a r yA r t e r yD i s s e c t i o n : AC a s eR e p o r t S a n i y eG ö k n i l Ç a l ı k , Mu s t a f aÇ a l ı k , A t i l l aC a n , H ı d ı r E s me

G ö ğ ü s D u v a r ı Y e r l e ş i ml i N o n H o d g k i nL e n f o ma O l g u s u Ac a s e o f N o n H o d g k i n ' s L y mp h o ma o nC h e s t Wa l l L o c a t i o n Mu h a r r e mÇ a k ma k , A k ı nE r a s l a nB a l c ı , S i y a mi A y d ı n , S u n aP o l a t o ğ l u

K o s t a l H i d a t i kK i s t : N a d i r B i r O l g uS u n u mu C o s t a l H y d a t i dC y s t : AR a r eC a s eR e p o r t

F e r d a n eMe l i k eD u r a n , Mu s t a f aÇ a l ı k , S a n i y eG ö k n i l Ç a l ı k , N u r i D ü z g ü n , H ı d ı r E s me

RESPI RATORYCASEREPORTS Yı l / Yea r : 2018 Ci l t / Vol ume7 S a y ı / I s s ue: 2

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Ul us l ar ar as ı Bi l i ms e l Danı ş maKur ul u/I nt er na t i ona l Adv i s or yBoa r d Ba ş k a n/Cha i r ma n: Ha y a t i Bi l gi ç( T ÜRKİ YE ) Andr ewMi l l er( ABD) Ant oni oAnz uet o( ABD) E l a mi nM. E l a mi n( ABD) Met i nAy t ek i n( ABD) Mour a dT opor s i a n( ABD) MügeAk pı na rE l ç i ( GRE NADA) Ra j endr aPr a s a dT a k ha r( HI NDI S T AN) Ri c ha r dL i ght( ABD) S t ef a noNa v a( İ T AL YA) S ept i mi uMur gu( ABD)

Ul us al Bi l i ms e l Danı ş maKur ul u/S c i ent icAdv i s or yBoa r d

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A. E s r aKuntUz a s l a n( Bur s a ) Abdur r a hma nS eny i gi t( Di y a r ba k i r ) Adem Güngör( Ank a r a ) AhmetAk k a y a( I s pa r t a ) AhmetE r ba y c u( I z mi r ) AhmetHa mdi I l ga z l i ( Koc a el i ) AhmetI l v a n( Mer s i n) AhmetS a mi Ba y r a m( Bur s a ) AhmetUr s a v a s( Bur s a ) Ak i fT ur na( I s t a nbul ) Ak i nYi l di z ha n( I s t a nbul ) Al i Ac a r( I s t a nbul ) Al i Ar i c a n( Mer s i n) Al i Çel i k( Ank a r a ) Al i Kut l u( I s t a nbul ) Al i Ni ha tAnna k k a y a( Düz c e) Al i Öz dül ger( Mer s i n) Apt ul l a hHa hol u( I s t a nbul ) Ar z uE r t ür k( Ank a r a ) At t i l aS a y gi ( I s t a nbul ) Ay da nurE k i c i ( Ki r i k k a l e) Ay di nÇi l eda g( Ank a r a ) Ay l i nBa ba l i k( I s t a nbul ) Ay s eF üs unKa l pa k l i ogl u( Ki r i k k a l e) Ay s eT a naAs l a n( Ank a r a ) Ay s egül S ent ür k( Ank a r a ) Ay t enPa muk ç u( Bol u) Ba ha rKur t( Bol u) Ba ha rUl uba s( Mer s i n) Ba nuE r i sGül ba y( Ank a r a ) Ba y k a l T ül ek( Kony a ) Bena nÇa gl a y a n( I s t a nbul ) Ber naE r enKömür c üogl u( I z mi r ) Bi l geha nS a v a sÖz( Ank a r a ) BügeÖz( I s t a nbul ) Bül entAl t i ns oy( Zongul da k ) Bül entAr ma n( I s t a nbul ) Bül entKoç er( Ank a r a ) Bül entT ut l uogl u( I s t a nbul ) Ça ga t a yT ez el ( I s t a nbul ) Ça gl a rÇuha da r ogl u( I s t a nbul ) Ca ns el At i nk a y aOz t ur k( I s t a nbul ) Ca nt ur kT a s c i ( Ank a r a ) Cengi zÖz ge( Mer s i n) CenkKi r a k l i ( I z mi r ) Cüney tKur ul ( Ank a r a ) Da neE di ger( Bur s a ) Deni zKök s a l ( Ank a r a ) Di l a v erDemi r el ( I s t a nbul ) Di l a v erT a s( I s t a nbul ) Di l ekYi l ma z ba y ha n( I s t a nbul ) E geGül eçBa l ba y( Düz c e) E l i fS en( Ank a r a ) E l i fYi l ma z el Uç a r( E r z ur um)

E mel Cey l a n( Ay di n) E r doga nÇet i nk a y a( I s t a nbul ) E r ha nAy a n( Mer s i n) E r s i nDemi r er( I s t a nbul ) E s enKi y a n( I s t a nbul ) F a t maF i s ek ç i ( Deni z l i ) F a t maS emaOy ma k( Ka y s er i ) F er ha nÖz s ek er( I s t a nbul ) F i genDev ec i ( E l a z i g) F i k r etKa na t( Kony a ) F ua tE r el ( Ba l i k es i r ) F undaÖz t una( T r a bz on) F üs unYi l di z( Koc a el i ) Gündeni zAl t ı a y( E di r ne) Ha k a nAy t a n( T ok a t ) Ha k a nÇer mi k( Ank a r a ) Ha k k ı Ul ut a ş( Ma l a t y a ) Ha s a nÇa y l a k( Ank a r a ) Ha s a nT ür üt( Ka hr a ma nma r a ş ) Ha t i c eS el i mogl uS en( Di y a r ba k i r ) Ha t i c eT ür k er( I s t a nbul ) Hur i y eBer kT a k i r( I s t a nbul ) I br a hi m Ak k ur t( S I VAS ) I br a hi m Ar daYi l ma z( Mer s i n) I l ga zDogus oy( I s t a nbul ) I l k nurE gec eBa s y i gi t( Koc a el i ) I nc i Gül mez( Ka y s er i ) I s ma i l S a v a s( Ank a r a ) Kur t ul usAk s u( Ank a r a ) L ev entAl pa y( I s t a nbul ) L ev entCa ns ev er( I s t a nbul ) L ev entDa l a r( I s t a nbul ) L ev entE l bey l i ( Ga z i a nt ep) L ev entGör enek( I s t a nbul ) L ey l aS a gl a m( E r z ur um) M. Kut l uÇel enk( Ank a r a ) Medi haGönençOr t a k öy l ü( I s t a nbul ) MehmetGenc er( S a nl i ur f a ) MehmetI nc eda y i ( I s t a nbul ) MehmetOguzKök s el ( Mer s i n) Mel i hKa pt a nogl u( S i v a s ) Mel i k eYüc eege( Ank a r a ) Mer a l Gül ha n( Ank a r a ) Muk a dderÇa l i k ogl u( Mer s i n) Mus t a f aE r el el ( I s t a nbul ) Mus t a f aÖz t ür k( Ank a r a ) Mus t a f aYuk s el ( I s t a nbul ) Nur et t i nYi y i t( I s t a nbul ) Nur i T ut a r( Ka y s er i ) OguzUz un( S a ms un) ÖmerAr a z( E r z ur um) ÖnerBa l ba y( Düz c e) ÖnerDi k ens oy( Ga z i a nt ep) Os ma nNur i Ha t i pogl u( E di r ne)

Os ma nS ener( Ank a r a ) Öz l em S el ç ukS önmez( Ank a r a ) Per i Ar ba k( Düz c e) Pi na rÇel i k( Ma ni s a ) Ra bi aAr pa c i ( Mer s i n) Ra ma z a nDemi r( Ka y s er i ) Ra ma z a nGen( Mer s i n) S a i tKa r a k ur t( I s t a nbul ) S a l i hE mr i ( Ank a r a ) S a l i hT opç u( Koc a el i ) S eda tDemi r c a n( Ank a r a ) S ef aL ev entÖz s a hi n( S i v a s ) S er ha tÇel i k el ( T ok a t ) S er i rÖz k a nAk t ogu( I z mi r ) S er v etKa y ha n( I s t a nbul ) S ez a i Çubuk( Ank a r a ) S i bel At i sNa y c i ( Mer s i n) S i bel Öz k ur t( Deni z l i ) S ua tDoga nc i ( Ank a r a ) T a l a tKi l i ç( Ma l a t y a ) T a ns uUl uk a v a kÇi f t ç i ( Ank a r a ) T a y f unÇa l i s k a n( I s t a nbul ) T evkKa pl a n( Ank a r a ) T ül i nKuy uc u( I s t a nbul ) T ur gutI s i t ma ngi l ( I s t a nbul ) UgurGönül l ü( Ank a r a ) Ül k üY. T ur a y( Ank a r a ) Ümi tT ür s en( Mer s i n) Vey s el Yi l ma z( I s t a nbul ) Vol k a nBa y s ungur( I s t a nbul ) Ya k upCa ni t ez( Bur s a ) Yur da nurE r doga n( Ank a r a ) Za f erÇa l i s k a ner( Ank a r a ) Za f erKüç ük oda c i ( I s t a nbul ) Zuha l Ka r a k ur t( I s t a nbul )

Respir Case Rep 2018;7(2): 54-58 DOI: 10.5505/respircase.2018.06978

OLGU SUNUMU

CASE REPORT

Proximal Interruption of Right Pulmonary Artery in an Adult Patient Presenting with Hemoptysis Hemoptizi ile Başvuran Yetişkin Bir Hastada Sağ Pulmoner Arter Proksimalinde Kesinti

RESPIRATORY CASE REPORTS

Mustafa Belal Hafeez Chaudhry1, Kumail Khandwala1, Wasim Ahmed Memon1, Taha Sheikh2, Tanveer Ul Haq1, Muhammad Arif Saeed1

Abstract

Özet

A 42 year old lady presented with dyspnea and 3 episodes of hemoptysis since 1 day. Her chest radiograph revealed mild peripheral fibrosis & volume loss in right lung, subsequent HRCT confirmed the findings with additional diagnosis of absence of the right main pulmonary artery. This was further confirmed on digital subtraction angiography and the right lung was solely supplied by the systemic circulation. The conventional angiography did not demonstrate any active extravasation or blush to suggest active bleeder. Empirical embolization was not attempted due to a risk of pulmonary infarction. Patient was managed symptomatically and conservatively.This condition is important to recognize in the list of differential diagnoses for pulmonary artery abnormalities and any acquired causes of pulmonary vasculature obstruction must be ruled out on imaging modalities.

Kırk iki yaşında kadın hasta, önceki günden beri 3 kez hemoptizi atağı ve dispne yakınması ile başvurdu. Akciğer grafisinde sağ akciğerde volüm kaybı ve hafif periferal fibrozis vardı. Yüksek rezolüsyonlu bilgisayarlı tomografide bu bulgulara ilave olarak sağ pulmoner arterin olmadığı belirlendi. Bu bilgi djijtal subtraction anjiografi ile doğrulandı ve sağ akciğerin sadece sistemik dolaşımdan beslendiği görüldü. Konvansiyonel angiografide herhangi bir ekstra damarlanma veya aktif kanama odağı görülmedi. Pulmoner enfarkt riski nedeniyle ampirik embolizasyon yapılmadı. Hasta semptomatik ve konservatif olarak tedavi edildi. Pulmoner arter anomalilerinin ayırcı tanısında bu durum önemli olup görüntüleme yöntemleri ile pulmoner damarsal obstrüksiyonların nedenleri araştırılmalıdır.

Key words: Proximal Interruption of Pulmonary Artery (PIPA), High Resolution Computed Tomography (HRCT), Digital Subtraction Angiography (DSA).

Proximal Interruption of the pulmonary artery (PIPA) is an uncommon vascular developmental anomaly with a prevalence of 1 in 200,000 and a median age of diagnosis at 14 years (1). It is more commonly found with other cardiovascular anomalies; the prognosis is poor in such cases. PIPA is impor-

Anahtar Sözcükler: Pulmoner arter kesintisi, yüksek çözünürlüklü bilgisayarlı tomografi, dijital anjiografi.

tant to recognize in the differential diagnosis of pulmonary artery anomalies, since there are no consistent guidelines regarding the management. In asymptomatic patients, some clinicians adopt conservative management with close follow-up. Others seek to restore a physiological pulmonary

Department of Radiology, Aga Khan University, Karachi, Pakistan Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan

Aga Khan Üniversitesi, Radyoloji Bölümü, Karachi, Pakistan Dow Sağlık Bilimleri Üniversitesi, Tıp Fakültesi, Karachi, Pakistan

Submitted (Başvuru tarihi): 07.11.2017 Accepted (Kabul tarihi): 15.01.2018 Correspondence (İletişim): Kumail Khandwala, Department of Radiology, Aga Khan University, Karachi, Pakistan e-mail: kumail.khandwala@gmail.com

Respiratory Case Reports

functionality via early revascularization of the interrupted pulmonary artery and embolization of any ruptured collateral vessels if symptoms warrant it. This report is a description of a case of a previously asymptomatic middle-aged woman with right-sided PIPA, who had an insignificant past history and presented with dyspnea and episodes of painless hemoptysis.

right hemidiaphragm. The heart and the mediastinum were shifted toward the ipsilateral right side. The left lung was relatively hyperinflated. The right hilum was not well visualized. There was some pleural thickening in the right lung apex, and fine reticular shadowing with a suggestion of rib notching was observed (Figure 1).

CASE A 42-year-old woman presented at the emergency department (ED) with dyspnea and 3 episodes of hemoptysis (approximately half a cup in volume) since the previous day. There was no associated chest pain, fever, night sweats, or weight loss. Her medical history was only remarkable for hypertension, which was controlled with losartan (angiotensin receptor blocker). She had similar asymptomatic episodes of hemoptysis 4 years earlier, for which no detailed work-up was done. There was a family history of diabetes mellitus and gastrointestinal malignancy. The patient did not provide any history of tuberculosis contact. On physical examination, the patient’s vital signs were stable and a systemic examination was unremarkable. Normal air entry and heart sounds were heard on chest auscultation. A routine laboratory workup was performed through the ED. Her baseline hemoglobin level, at the time of first episode of hemoptysis, was 12.2 g/dL (11.1-14.5) which dropped to 10.1 g/dL (11.1-14.5) over the period of 2 days. A decrease in hematocrit level from 38.7% to 34.4% (35.4-42.0) was also noted. The total leucocyte count was normal: 9.6 x109/L (4.0-10.0), platelet count was normal: 323 x109/L (150-400), peripheral film showed normochromic and normocytic red blood cells, prothrombin time was of 10.5 seconds (9.1-13.1) with an international normalization ratio of 1.0, and the activated partial thromboplastin time was 23.9 seconds (22.934.5). A local ear, nose, throat examination and bronchoscopy were performed, and did not reveal any active site of bleeding. Sputum and bronco-alveolar lavage (BAL) specimens were sent for Gram-stain and bacterial cultures, which turned out to be negative. A smear for fungal hyphae, and an acid-fast bacilli (AFB) smear along with AFB culture were also negative for both specimens. Mycobacterium tuberculosis complex was not detected with a GeneXpert MTB/RIF assay (Cephaid, Sunnyvale, CA, USA) of specimens of both sputum and BAL. A posteroanterior chest radiograph revealed volume loss within the right hemithorax with slight elevation of the Cilt - Vol. 7 Sayı - No. 2

Figure 1: The right hilum is not visualized with right lung volume loss and slightly elevated right hemidiaphragm. Some pleural thickening (arrow heads) on right side with fine reticular shadowing and suggestion of rib notching. Ipsilateral right sided mediastinal shift with engorged left hilum (white arrows)

Subsequently, a high resolution computed tomographic (HRCT) scan of chest was obtained during the patient’s stay in the hospital to delineate the suspected pulmonary pathology. A hypoplastic right lung with volume loss, septal fibrosis, honeycombing, and subpleural cysts, predominantly in the base, were observed. Compensatory marked hyperinflation of the left lung was noted, which also slightly herniated toward the right side. Mediastinal window settings revealed the presence of the prominent left main pulmonary artery and its divisions; however, the complete absence of the right main pulmonary artery was noted (Figure 2). Further characterization of the pulmonary vasculature was not possible as it was an unenhanced scan. The radiologist advised further evaluation and management with digital subtraction angiography (DSA) of the pulmonary and bronchial arteries. Subsequent DSA confirmed the HRCT findings of right-sided proximal Interruption of the pulmonary artery (PIPA) (Figure 3a). The main pulmonary artery and left pulmonary artery appeared dilated. The origin of the right pulmonary artery was not identified. This was followed by an angiogram of the right subclavian artery and bronchial arteries,

Proximal Interruption of Right Pulmonary Artery in an Adult Patient Presenting with Hemoptysis | Chaudhry et al.

which revealed numerous collaterals predominantly arising from the lateral thoracic, internal mammary artery and the thyrocervical trunk supplying the right lung (Figures 3 b-d). Empirical embolization of the arteries supplying right upper lobe was not performed due to a presumed risk of pulmonary infarction/necrosis. The patient was given symptomatic treatment and started on tranexamic acid and cefpodoxime and remained stable with no further episodes of hemoptysis. She was discharged and was followed up as an outpatient for 3 months without any active complaint.

Figure 2: Lung Window (A, B, C) shows hypoplastic right lung with volume loss, septal fibrosis, minimal honeycombing and subpleural cysts predominantly in the base. Compensatory hyperinflation of the left lung with slight right sided herniation. Mediastinal Window (D, E, F) reveals complete absence of the right main pulmonary artery with prominent left main pulmonary artery and its divisions

Figure 3: Native Conventional Pulmonary Angiogram (A) demonstrating proximal interruption of right branch of pulmonary artery with dilated main pulmonary artery trunk and left branch. DSA of the right subclavian artery (B, C) and bronchial arteries (C) revealing numerous collaterals arising from the lateral thoracic, internal mammary artery and thyrocervical trunk supplying the right lung

DISCUSSION PIPA, formerly known as unilateral absence of pulmonary artery, is an uncommon vascular developmental anomaly (2,3). It has a prevalence of 1 in 200,000, with a median age of diagnosis of an isolated form of interruption of the pulmonary artery at 14 years (range: 0.1 to 58 years) (1). In a review by Pool et al. (4), it was found as an isolated

finding in about one-third of the cases; it is more commonly found associated with other cardiovascular abnormalities. Left-sided PIPA is frequently associated with right-sided aortic arch and other congenital cardiovascular anomalies, such as septal defects, transposition of the great arteries, congenitally corrected transposition, vascular intramural pathologies (coarctation of the aorta, subvalvular aortic stenosis, pulmonary stenosis), or combinations and syndromes (Tetralogy of Fallot, Taussig-Bing anomaly, coarctation, scimitar syndrome) (1,5). However, right-sided PIPA is more often (63%) associated with a normal left-sided aortic arch (1). No genetic markers have been linked with any of these disorders. PIPA can potentially be caused by any involution of the proximal sixth aortic arch during the ďŹ rst 16 weeks of intrauterine development. This aberrancy may result in termination of the extrapulmonary portion of the affected pulmonary artery within 1 cm of its origin. However, the intrapulmonary vessels are usually patent and normal in distribution (5). In that case, intrapulmonary arteries derive their blood supply from other systemic collateral vessels, namely, the intercostal, internal mammary, subclavian, and innominate arteries, and from the bronchial artery system. These transpleural and bronchial vessels interconnect extensively with the intrapulmonary vessels via microvascular communications (6). In a review of the literature performed by Harkel et al. (1), the general pattern observed for PIPA was on the side opposite to the aortic arch. PIPA should be differentiated from similar conditions, especially in the presence of a small hemithorax, which may obviate reconsidering the diagnosis. Hypogenetic lung syndrome is characterized by a linear shadow representing the anomalous draining vein (scimitar sign); however, the normal bronchial branching pattern of PIPA differentiates it from hypogenetic lung syndrome. A small lung may also appear hyperlucent, creating potential confusion with Swyer-James syndrome (post-infectious obliterative bronchiolitis), which also demonstrates trapped air on radiography during expiration secondary to bronchiectasis (6). The normal caliber of the pulmonary vessels with normal architecture differentiates this condition from PIPA. In the differential diagnosis of acquired causes of pulmonary vasculature obstruction, conditions like Takayasu arteritis, chronic pulmonary thromboembolism, and mediastinal ďŹ brosis must be ruled out. The clinical symptomatology of PIPA varies, depending on the extent of anomalies and associated defects. The symptomatology may include shortness of breath, recurwww.respircase.com

Respiratory Case Reports

rent pulmonary infections (37%), dyspnea, limited exercise tolerance (40%), hemoptysis (20%), (1) or other symptoms of pulmonary hypertension or heart failure (5,7). Hemoptysis occurs secondary to the rupture of hypertrophied collateral vessels between the pulmonary and systemic vasculature (8). All symptoms aside, pulmonary hypertension alone, with a reported incidence of 19% to 44%, (1,4) is the most important determinant of long-term survival prognosis, followed by hemoptysis. The overall mortality is reported as 7% per year after diagnosis (1). Most congenital anomalies of the pulmonary arteries in adults are found incidentally on chest radiographs or CT scans. The most consistent findings on chest radiography include an ipsilateral hypoplastic hilum, varying degrees of pulmonary hypoplasia (hypolucent and containing small and fewer alveoli, secondary to low oxygen tension), and an ipsilateral shift of the heart and mediastinum toward the affected side (9). This is accompanied by a prominent contralateral hilum (owing to the entire right ventricular output going preferentially toward the uninterrupted pulmonary artery) and consequent compensatory hyperinflation of the contralateral lung (5). The mediastinal portion of the affected pulmonary artery may terminate within 1 cm of its origin or may not be visible on a CT scan (9). Magnetic resonance imaging may be of greater advantage compared with other modalities to delineate the vascular anatomy in addition to identifying associated congenital cardiac abnormalities and limiting ionizing radiation exposure in any age group. The collateral transpleural arteries may appear perpendicular to the pleural surface as multiple linear opacities, and a characteristic serrated pleural and subpleural parenchymal thickening may be delineated in a soft tissue window because of the enlarged intercostal collaterals due to direct anastomosis of transpleural collateral vessels with peripheral branches of the pulmonary artery. Owing to hypoxic vasoconstriction, a mosaic pattern may be observed secondary to underperfusion in the affected lung, and overperfusion may be seen in the unaffected lung. This may be best seen with HRCT, as in our case. Other signs of collateral arteries, such as rib notching, may be identified when the intercostal arteries are involved, in addition to increased subpleural interstitial lung marking. The CT scan is essential to map out a normal bronchial branching pattern (9). There are no consistent guidelines regarding the management of isolated interruption of the pulmonary artery. In asymptomatic patients, some clinicians adopt conCilt - Vol. 7 Sayı - No. 2

servative management with close follow-up. Others seek to restore physiological pulmonary functionality via early revascularization of the interrupted pulmonary artery and embolization of any ruptured collateral vessels, if symptoms warrant it (5,7,8,10).

CONCLUSION PIPA is an uncommon vascular condition. It may be an isolated finding, or more commonly, it is seen with other cardiovascular anomalies. Depending on the extent of any associated anomalies, patients with this condition who survive into adulthood can present with pulmonary hypertension, hemoptysis, recurrent respiratory infections, dyspnea, etc., or may remain asymptomatic. This condition is important to recognize in the list of differential diagnoses for pulmonary artery abnormalities, and any acquired causes of pulmonary vasculature obstruction must be ruled out with imaging modalities.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - M.B.H.C., K.K., W.A.M., T.S., T.U.H., M.A.S.; Planning and Design - M.B.H.C., K.K., W.A.M., T.S., T.U.H., M.A.S.; Supervision - M.B.H.C., K.K., W.A.M., T.S., T.U.H., M.A.S.; Funding - W.A.M., T.U.H.; Materials - T.U.H., M.A.S.; Data Collection and/or Processing M.B.H.C., T.S., K.K.; Analysis and/or Interpretation M.B.H.C., K.K.; Literature Review - K.K., T.S.; Writing M.B.H.C., T.S., K.K.; Critical Review - M.B.H.C., K.K.

YAZAR KATKILARI Fikir - M.B.H.C., K.K., W.A.M., T.S., T.U.H., M.A.S.; Tasarım ve Dizayn - M.B.H.C., K.K., W.A.M., T.S., T.U.H., M.A.S.; Denetleme - M.B.H.C., K.K., W.A.M., T.S., T.U.H., M.A.S.; Kaynaklar - W.A.M., T.U.H.; Malzemeler - T.U.H., M.A.S.; Veri Toplama ve/veya İşleme M.B.H.C., T.S., K.K.; Analiz ve/veya Yorum - M.B.H.C., K.K.; Literatür Taraması - K.K., T.S.; Yazıyı Yazan M.B.H.C., T.S., K.K.; Eleştirel İnceleme - M.B.H.C., K.K.

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Proximal Interruption of Right Pulmonary Artery in an Adult Patient Presenting with Hemoptysis | Chaudhry et al.

Fraentzel O. Ein Fall von abnormer Communication der: Aorta mit der Arteria pulmonalis. Virchows Archiv 1868; 1:420–6. [CrossRef]

Shakibi JG, Rastan H, Nazarian I, Paydar M, Aryanpour I, Siassi B. Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair. Jpn Heart J 1978; 19:439-51.

Pool PE, Vogel JH, Blount SG Jr. Congenital unilateral absence of the pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 1962; 10:70632. Castañer E, Gallardo X, Rimola J, Pallardó Y, Mata JM, Perendreu J, et al. Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics 2006; 26:349-71. [CrossRef] Morgan PW, Foley DW, Erickson SJ. Proximal interruption of a main pulmonary artery with transpleural collateral vessels: CT and MR appearances. J Comput Assist Tomogr 1991; 15:311– 3. [CrossRef]

Welch K, Hanley F, Johnston T, Cailes C, Shah MJ. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg 2005; 79:1399-402. [CrossRef]

Reñé M, Sans J, Dominguez J, Sancho C, Valldeperas J. Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol 1995; 18:251–4.

Ryu DS, Spirn PW, Trotman-Dickenson B, Hunsaker A, Jung SM, Park MS, et al. HRCT findings of proximal interruption of the right pulmonary artery. J Thorac Imaging 2004; 19:171–5. [CrossRef]

10. Liu B, Monroe EJ, Kogut MJ. Proximal interruption of the pulmonary artery: Transcatheter embolization for emergent management of massive hemoptysis. Radiol Case Rep 2015; 8:865. [CrossRef]

www.respircase.com

Respir Case Rep 2018;7(2):59-62 DOI: 10.5505/respircase.2018.78557

OLGU SUNUMU

CASE REPORT

A Case of Sjögren’s Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy İloprost ve Bosentan Kombinasyon Tedavisi Uygulanan Bir Sjögren Sendromu İlişkili Pulmoner Hipertansiyon Olgusu

RESPIRATORY CASE REPORTS

Ayşe Baha1, Berkay Ekici2, Nalan Ogan1, Evrim Eylem Akpınar1

Abstract

Özet

Sjögren’s syndrome (SS), which is characterized by lymphocytic infiltration of the exocrine glands, is the second most common multisystem autoimmune disease after rheumatoid arthritis. Pulmonary involvement ranges from 9% to 20% and it usually occurs as an airway disease or interstitial lung disease. Pulmonary arterial hypertension (PAH) is a very rare condition in SS. In SS-associated PAH, the functional class of patients is usually World Health Organization (WHO) Class III/IV and there is no specific treatment regimen. In addition to nonspecific medical treatment for PAH, treatment of the underlying disease and supportive therapy are important. The aim of this manuscript was to present a case of SS-related PAH (SRP) followed under bosentan and iloprost combination therapy.

Sjögren Sendromu (SS) ekzokrin glandların lenfositik infiltrasyonu ile karakterize, romatoid artritten sonra ikinci en sık görülen multisitem otoimmün hastalıktır. Pulmoner tutulum %9-20 sıklıkta bildirilmiştir ve genellikle hava yolu hastalığı ya da interstisyel akciğer hastalığı şeklinde görülür. Pulmoner Arteriyel Hipertansiyon (PAH) gelişimi SS’da çok nadir bir durumdur. SS ilişkili PAH’da hastalar genellikle fonksiyonel sınıf WHO-III/IV’dür ve spesifik bir tedavi rejimi yoktur. PAH’ın nonspesifik tedavisine ek olarak altta yatan hastalığın tedavisi önemlidir. Bu yazıdaki amacımız iloprost ve bosentan kombinasyon tedavisi altında izlediğimiz bir SS ilişkili PAH (SİP) olgusunu takdim etmektir.

Key words: Sjögren Syndrome, Pulmonary Hypertension, Combination Therapy, Iloprost, Bosentan.

Pulmonary manifestations of Sjören’s Syndrome (SS) include airway disease, pleuritis, interstitial lung disease, and lymphoma. Although pulmonary arterial hypertension (PAH) may complicate SS, this scenario is uncommon (1). PAH is more frequently

Department of Pulmonary Medicine, Ufuk University Faculty of Medicine, Ankara, Turkey 2 Department of Cardiology, Ufuk University Faculty of Medicine, Ankara, Turkey

Anahtar Sözcükler: Sjögren Sendromu, Pulmoner Hipertansiyon, Kombinasyon Tedavisi, İloprost, Bosentan.

associated with other connective tissue diseases, particularly scleroderma. Only a few cases of PAH associated with SS have been reported in the literature (1) and our case is the first case of S related PAH (SRP) reported from Turkey.

Ufuk Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Ankara 2 Ufuk Üniversitesi Tıp Fakültesi, Kardiyoloji Anabilim Dalı, Ankara

Submitted (Başvuru tarihi): 09.11.2017 Accepted (Kabul tarihi): 11.01.2018 Correspondence (İletişim): Ayşe Baha, Department of Pulmonary Medicine, Ufuk University Faculty of Medicine, Ankara, Turkey e-mail: dr_aysedemir@hotmail.com

Respiratory Case Reports

CASE A 64-year-old male who had history of smoking (40 pack-years) and who had been followed for chronic obstructive pulmonary disease (COPD) for 4 years was admitted to the clinic with a complaint of progressive dyspnea. One year before his admission to the clinic, the patient had undergone an inferior vena cava filter placement procedure for a recurrent venous thromboembolism under warfarin treatment. Despite both COPD and rivaroxaban treatment, the patient had experienced progressive dyspnea. Echocardiography performed 1 year earlier had indicated a systolic pulmonary artery pressure (PAPsys) of 75 mmHg; following admission to our clinic, it was measured as 110 mmHg. The patient then underwent right heart catheterization. Following administration of a local anesthetic, the right internal jugular vein was cannulated using the Judkins technique. The mean pulmonary artery wedge (mPCWP) was 10 mmHg, the mean PA pressure (mPAP) was 53 mmHg, the systolic pressure (SRVP) was 73 mmHg, and the diastolic RV pressure (DRVP) was 8 mmHg, and the mean RA pressure (mRAP) was 5 mmHg. Adenosine given at an infusion rate of 50 mcg/kg/minute did not change the mean PAP and vasoreactivity was found to be negative. Lung ventilationperfusion scintigraphy revealed a low probability for pulmonary thromboembolism. Thorax computed tomography angiography (CTA) did not show any filling defect indicating a thrombus, but the diameter of the main pulmonary artery was greater than the aorta (Figure 1). A high-resolution CT revealed lower zone cystic bronchiectasis (Figure 2). Although the patient did not have xerostomia or xerophthalmia, he had high levels of antinuclear-antibody (ANA) and anti-Sjögren's-syndrome-related antigen A (anti-SSA antibodies). A Schirmer test was positive. A biopsy revealed lymphocytic infiltration of the minor salivary gland, suggesting the diagnosis of Sjögren’s syndrome (SS). Hydroxychloroquine and iloprost treatments were started for SS and associated pulmonary arterial hypertension (PAH). The patient’s dyspnea worsened after 5 months of iloprost treatment and his WHO functional status increased from III to IV. A 6-minute walking test result decreased from 230 meters to 75 meters. A pulmonary function test performed 1 year previously revealed FEV1=920 cc (32%), FVC=1590 cc (39%), FEV1/FVC=57%, Diffusing capacity of the lungs for carbon monoxide (DLCO) was not measured. A new pulmonary function test revealed a mixed pattern and the DLCO was determined to be very low (FVC=1480 cc [36%], FEV1=800 cc [25%], FEV1/FVC: 54%, DLCO: 18%). A Cilt - Vol. 7 Sayı - No. 2

thorax CTA revealed no thrombus. PAPsys was measured as 110 mmHg on a repeat echocardiogram. Bosentan was added to the iloprost treatment. PAPsys was measured as 64 mmHg at the first month of combination therapy. The patient is now in clinically stable condition and is being followed up with combination therapy for PAH and hydroxychloroquine therapy for SS.

. Figure 1: The diameter of the main pulmonary artery was greater than the diameter of the aorta (thorax computed tomography angiography, mediastinal section)

Figure 2: Bronchiectasis can be seen in the lower zones of a highresolution computed tomography image (parenchymal section)

DISCUSSION SS is characterized by lymphocytic infiltration of the exocrine glands, and is the second most common multisystem autoimmune disease after rheumatoid arthritis (1). In SS, while middle-aged women are most often affected, men may also develop SS, typically after the age of 65 years. Almost all SS-related PAH (SRP) cases reported in the literature are female patients (2-6). Our patient is a rare male example. Systemic manifestations of SS are vasculitis and involvement of the lungs, kidneys, and central nervous system. One of the findings in patients with SS is PAH, but it is rare (1). Only 32 cases of SRP had been reported in the

A Case of Sjögren’s Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy | Baha et al.

literature as of 2007. After 2007, patients with SS were screened for PAH and 1 case series was reported (2). The incidence of SRP is unknown. In a recently published review it was reported that the incidence of PAH in patients with SS is rare (1). In recent years, studies have shown that connective tissue disease (CTD)-related PAH is the second leading cause of PAH (25% of all cases). A study from China reported that the most common underlying disease among CTD-related PAH patients was systemic lupus erythematosus (49%), followed by SS (16%), and scleroderma (6%) (7). In another study, it was reported that SRP accounts for almost 1% of cases of CTDrelated PAH (8). Surprisingly, our patient did not have symptoms of SS, although he had ANA and anti-SSA antibody positivity. In the literature, there is only 1 case similar to ours reporting SRP without clinical manifestations despite serological positivity (4). The pathogenesis of PAH in SS is not clear. However, it is thought that it results from vasculitis with prolonged vasospasm followed by structural vessel remodeling, eventually leading to irreversible thrombotic obstruction of pulmonary arterioles (9). A study indicated that systemic vasculopathy, B-cell activation, and autoimmunity are involved in the pathogenesis of SRP, supporting the vital role of immunosuppressants for the treatment of PAH in SS (2). Zhao et al. (5) reported that endothelial damage, immune complex accumulation, necrotizing vasculitis, and imbalances of endothelium-derived vasoactive molecules are also involved in the pathogenesis of SRP. The gold standard treatment strategy for SRP has yet to be found because of rarity of the disease. These cases are being treated like other CTD-related PAH cases (2). There are many drugs targeting the underlying pathophysiological mechanisms of PAH. In addition to PAH-specific therapy, supportive treatment, as well as the treatment of the underlying disorder, is also important. There is a report of 1 patient with SRP who was treated with long-term epoprostenol therapy (3). Launay et al. (2) examined 28 SRP cases that were reported before 2007. According to this review, 8 patients received standard PAH therapy (bosentan: 3 cases, epoprostenol: 2 cases, prostacyclin: 1 case, and 1 patient’s therapy was not indicated) as first line therapy, and 3 patients received calcium channel blockers. Our case is the first report of the administration of iloprost to treat SRP. The patient’s symptoms and functional status deteriorated with iloprost; therefore, bosentan was added to the therapy. The clinical status of the patient is now stable with this combina-

tion therapy. Our patient is the first case of SRP treatment with the combination of iloprost and bosentan. This case is the second reported SRP patient with ANA and anti-SSA positivity and an absence of classic SS symptoms of dry mouth and dry eye. Our case also suggests that combination therapy with iloprost and bosentan may be an alternative treatment strategy for SRP patients. Unfortunately, no specific treatment has yet been recommended for SRP. However, we suggest that supportive treatment is also crucial in these patients, as well as treatment of the underlying disease.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - A.B., B.E., N.O., E.E.A.; Planning and Design - A.B., B.E., N.O., E.E.A.; Supervision - A.B., B.E., N.O., E.E.A.; Funding -; Materials - B.E., A.B.; Data Collection and/or Processing - B.E., A.B., N.O.; Analysis and/or Interpretation - A.B., B.E.; Literature Review - A.B., N.O., B.E.; Writing - A.B., B.E., E.E.A.; Critical Review - N.O., E.E.A.

YAZAR KATKILARI Fikir - A.B., B.E., N.O., E.E.A.; Tasarım ve Dizayn - A.B., B.E., N.O., E.E.A.; Denetleme - A.B., B.E., N.O., E.E.A.; Kaynaklar -; Malzemeler - B.E., A.B.; Veri Toplama ve/veya İşleme - B.E., A.B., N.O.; Analiz ve/veya Yorum A.B., B.E.; Literatür Taraması - A.B., N.O., B.E.; Yazıyı Yazan - A.B., B.E., E.E.A.; Eleştirel İnceleme - N.O., E.E.A.

REFERENCES 1.

Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjögren’s Syndrome. Eur Respir Rev 2016; 25:110–23. [CrossRef]

Launay D, Hachulla E, Hatron PY, Jais X, Simonneau G, Humbert M. Pulmonary arterial hypertension: a rare complication of primary Sjogren Syndrome report of 9 new cases and review of the literature. Medicine (Baltimore) 2007; 86:299-315.

Fujita T1, Tanabe N, Kasahara Y, Sugiura T, Sakao S, Tatsumi K. Withdrawal of epoprostenol therapy in a patient with pulmonary hypertension associated with Sjögren’s syndrome. Intern Med 2014; 53:2237-40.

www.respircase.com

Respiratory Case Reports

Guerreso K, Conner EA. Possible role of anti-SSA/Ro antibodies in the pathogenesis of pulmonary hypertension. Respir Med Case Rep 2016; 17:47-9. [CrossRef]

Zhao J, Wang Q, Liu Y, Tian Z, Guo X, Wang H, et al. Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China. Int J Cardiol 2017; 236:432–7. [CrossRef]

Hwang JA, Yang TH, Lee JY, Koo DW, Choi IS, Cho SY, et al. Severe pulmonary hypertension in primary Sjögren’s syndrome. Korean Circ J 2013; 43:504-7. [CrossRef]

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YJ Hao, X Jiang, W Zhou, Wang Y, Gao L, Wang Y et al. Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients. Eur Respir J 2014; 44:963–72. [CrossRef]

Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009; 179:151-7. [CrossRef]

Kokosi M, Riemer EC, Highland KB. Pulmonary involvement in Sjögren syndrome. Clin Chest Med 2010; 31:489-500. [CrossRef]

Respir Case Rep 2018;7(2):63-65 DOI: 10.5505/respircase.2018.60352

OLGU SUNUMU

CASE REPORT

Traumatic Pulmonary Artery Dissection: A Case Report Travmatik Pulmoner Arter Diseksiyonu: Olgu Sunumu

RESPIRATORY CASE REPORTS

Saniye Göknil Çalık1, Mustafa Çalık2, Atilla Can2, Hıdır Esme2

Abstract

Özet

Pulmonary artery dissection (PAD) is a rare condition that is usually observed in cases with underlying pulmonary arterial hypertension. However, there are several factors related to the etiology of the disease. Presently described is a case of PAD that developed secondary to trauma. A 42-year-old man presented with left chest pain after a fall from a height. His past medical history and physical examination were unremarkable except for pain in the left hemithorax. He had no other complaints. All routine laboratory blood tests were normal. A radiological examination revealed left hilar enlargement and lower lobe PAD. Medical treatment was prescribed. The general condition of the patient was good and as there were no other symptoms, a conservative approach was pursued. In this case, there was pulmonary hypertension, and it is thought that the trauma led to PAD. This is extremely rare in the literature.

Pulmoner arter disseksiyonu nadir bir durumdur, ancak genellikle pulmoner arteriyel hipertansiyonu olan vakalarda görülür. Bununla birlikte, hastalığın etiyolojisinde farklı faktörlerin bir bağlantısı vardır. Biz travmaya bağlı gelişen pulmoner arter diseksiyonlu olguyu sunduk. Kırk iki yaşındaki erkek, yüksek düşme ve sol göğüs ağrısı nedeni ile hastanemiz acil servisine başvurdu. Anamnez ve fizik muayenesinde sol hemitoraks üzerinde ağrı dışında bir özellik yoktu. Başka hiçbir şikâyeti yoktu. Tüm rutin laboratuvar kan testleri normaldi. Radyolojik incelemesinde sol hiler genişleme ve alt lob pulmoner arter disseksiyonu tespit edildi. Tıbbi tedavi verildi. Genel durumu iyi ve semptomsuz olarak konservatif tedaviyle taburcu edildi. Bizim olgumuzda, var olan pulmoner hipertansiyona eklenen travmanın pulmoner arter diseksiyonuna neden olduğunu düşünüyoruz. Bu durum literatürde son derece nadirdir.

Key words: Trauma, pulmonary artery, dissection.

Anahtar Sözcükler: Travma, pulmoner arter, diseksiyon.

Pulmonary artery dissection (PAD) was first reported by Helmbrecht in 1842 (1). The condition generally presents as a cardiogenic shock or sudden death because the dissection proceeds swiftly;

Emergency and First Aid Program, Vocational School of Health Services KTO Karatay University, Konya, Turkey 2 Department of Thoracic Surgery, Health Sciences University, Konya Training and Research Hospital, Konya, Turkey

it is therefore usually diagnosed in post-mortem examinations (2). Recent improvements in imaging techniques have resulted in earlier diagnoses (1). In this case, PAD developed secondary to trauma.

KTO Karatay Üniversitesi Sağlık Hizmetleri Meslek Yüksek Okulu, İlk ve Acil Yardım Programı, Konya 2 Sağlık Bilimleri Üniversitesi Konya Eğitim ve Araştırma Hastanesi, Göğüs Cerrahisi Kliniği, Konya

Submitted (Başvuru tarihi): 28.08.2017 Accepted (Kabul tarihi): 13.10.2017 Correspondence (İletişim): Mustafa Çalık, Department of Thoracic Surgery, Health Sciences University, Konya Training and Research Hospital, Konya, Turkey e-mail: drmcalik@hotmail.com

Respiratory Case Reports

CASE A 42-year-old man presented with left chest pain that developed following a fall from a height. The pain had gradually worsened and was exacerbated by movement of the left hemithorax. His medical history did not indicate any lung disease or pulmonary hypertension. A physical examination was unremarkable except for pain in the left hemithorax. Respiratory sounds were slightly decreased in the left hemithorax. His routine laboratory blood test results, including complete blood count, erythrocyte sedimentation rate, tumor markers, lactate dehydrogenase, serum electrolytes, and kidney and liver function tests were all within the normal ranges. On an X-ray (Figure 1), left hilar enlargement was detected. A contrast-enhanced thoracic CT (Figure 2) image revealed that the enlarged hilar structure was the pulmonary artery. Left lower lobe pulmonary artery (PA) dissection confirmed it. Seconddegree tricuspid insufficiency was detected in an echocardiographic evaluation. PA pressure was measured at 100 mmHg. Examination was negative for Behcet's disease and the patient had no history of syphilis. The general condition of the patient was good and had no other symptoms, so a conservative approach was pursued. Medical treatment was prescribed, he was discharged without any complaints, and followed up for 6 months without any sign of problem. Written informed consent was obtained from the patient who participated in this study.

. Figure 1: Distention in the bilateral hilar region causing a large area of opacity and tubular opacity of the enlarged lobar branch of the pulmonary artery branch in the superposing sight with the left lower thoracic heart can be seen (white arrow)

Cilt - Vol. 7 SayÄą - No. 2

Figure 2: Thorax computed tomography cut-off: A view of a true lumenlike, crescent-shaped dissection that looks like a false lumen thrombosis in the lobar and segmental pulmonary arteries leading to the left lower lobe of the lung (white arrows)

DISCUSSION Pulmonary artery dissection (PAD) is an extremely rare and potentially fatal disease usually diagnosed in a postmortem examination. PAD is typically a complication of chronic pulmonary hypertension causing a PA aneurysm. Congenital heart anomalies causing prolonged high pulmonary artery flow rates and pulmonary hypertension have been associated with PAD. Generally, there is a patent ductus arteriosus in cases of congenital cardiac pathologies. PAD may also occur in patients without pulmonary hypertension. (1) Other rare causes include chronic inflammation of the PA, right heart endocarditis, amyloidosis, trauma, and severe atherosclerosis (3). Despite the frequent association between chronic obstructive pulmonary disease and pulmonary hypertension, PAD has rarely been reported in this group of patients (1). The presence of multiple arterial aneurysms reported on both the systemic and pulmonary vascular tree has been associated with congenital musculoskeletal abnormalities (4). We do not know the exact etiology of PAD in our patient. The main PA is involved in 80% of PAD cases. However, isolated cases with right or left pulmonary artery involvement have also been seen. Localized small dissections are unusual. Since rupture causes cardiogenic shock and sudden death, diagnosis is rare in the living, and PAD is frequently only detected in an autopsy (5). Khattar et al. (6) stated that only 8 (12.6%) of 63 cases were diagnosed with PAD while alive, and that 34 (53.9%) of the cases had congenital heart disease. PAD usually occurs in patients with medial degeneration and PA dilatation due to a chronic increase in PA pressure (7). PAD is observed, most often, at the site of maximal dilatation of the PA. The false lumen tends to rupture rather than propagate distally or develop a re-entry site (8). The evolution is usually rupture of the vessel with blood flowing into the mediastinum, pericardium, or lung, because there is no exit from the false lumen (9). With increasing survival for patients with PA hypertension, complications such as PAD

Traumatic Pulmonary Artery Dissection: A Case Report | Çalık et al.

may become more common (10). In patients with pulmonary hypertension, new chest pain, acute chest pain, or cardiogenic shock should raise the suspicion of PAD, which can result in sudden death (9).

Turkvatan A, Altinsoy D, Akgul A, Olçer T, Cumhur T. Pulmonary artery dissection diagnosed by multidetector computed tomographic angiography: case report. Turkiye Klinikleri Cardiovascular Sciences 2009; 21:292-6.

Bhatia V, Sharma S, Panda P, Sood RG. Role of multidetector computed tomography (MDCT) in diagnosis of pulmonary artery dissection: a aare but fatal entity. Ann Acad Med Singapore 2014; 43:64-5.

Lentini S, Zito C, David A, Gaeta R. Congenital musculoskeletal abnormalities associated with aortic, pulmonary and iliac aneurysms. Cardiol J 2010; 17:412-4.

Yaman M, Arslan U, Ateş AH, Aksakal A. Pulmonary arterial dissection in a post-partum patient with patent ductus arteriosus: Case report and review of the literature. World J Cardiol 2015; 7:101-3. [CrossRef]

Khattar RS, Fox DJ, Alty JE, Arora A. Pulmonary artery dissection: an emerging cardiovascular complication in surviving patients with chronic pulmonary hypertension. Heart 2005; 91:142-5. [CrossRef]

Inayama Y, Nakatani Y, Kitamura H. Pulmonary artery dissection in patients without underlying pulmonary hypertension. Histopathology 2001; 38:435-42. [CrossRef]

Senbaklavaci Ö, Kaneko Y, Bartunek A, Brunner C, Kurkciyan E, Wunderbaldinger P, et al. Rupture and dissection in pulmonary artery aneurysms: incidence, cause, and treatment-review and case report. J Thorac Cardiovasc Surg 2001; 121:1006-8. [CrossRef]

Corrêa Rde A, Silva LC, Rezende CJ, Bernardes RC, Prata TA, Silva HL. Pulmonary hypertension and pulmonary artery dissection. J Bras Pneumol 2013; 39:238-41.

CONCLUSION In our case, there was pulmonary hypertension and we believe that trauma caused the pulmonary artery dissection. This condition is extremely rare in the literature.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - S.G.Ç., M.Ç., A.C., H.E.; Planning and Design - S.G.Ç., M.Ç., A.C., H.E.; Supervision - S.G.Ç., M.Ç., A.C., H.E.; Funding - S.G.Ç., M.Ç., A.C., H.E.; Materials - S.G.Ç., M.Ç., A.C., H.E.; Data Collection and/or Processing - S.G.Ç., M.Ç.; Analysis and/or Interpretation - S.G.Ç., M.Ç.; Literature Review - S.G.Ç., M.Ç.; Writing - S.G.Ç., M.Ç., A.C.; Critical Review S.G.Ç., M.Ç., A.C., H.E.

YAZAR KATKILARI Fikir - S.G.Ç., M.Ç., A.C., H.E.; Tasarım ve Dizayn S.G.Ç., M.Ç., A.C., H.E.; Denetleme - S.G.Ç., M.Ç., A.C., H.E.; Kaynaklar - S.G.Ç., M.Ç., A.C., H.E.; Malzemeler - S.G.Ç., M.Ç., A.C., H.E.; Veri Toplama ve/veya İşleme - S.G.Ç., M.Ç.; Analiz ve/veya Yorum S.G.Ç., M.Ç.; Literatür Taraması - S.G.Ç., M.Ç.; Yazıyı Yazan - S.G.Ç., M.Ç., A.C.; Eleştirel İnceleme - S.G.Ç., M.Ç., A.C., H.E.

10. Cook R, Duarte AG, Boor P, Daniel C. Chronic pulmonary artery dissection associated with pulmonary arterial hypertension. Pulm Circ 2013; 3:692-5. [CrossRef]

REFERENCES 1.

Perrotta S, Lentini S. Pulmonary artery dissection. J Card Surg 2015; 30:442-7. [CrossRef]

www.respircase.com

Respir Case Rep 2018;7(2):66-70 DOI: 10.5505/respircase.2018.36025

OLGU SUNUMU

CASE REPORT

Göğüs Duvarı Yerleşimli Non-Hodgkin Lenfoma Olgusu A Case of Non-Hodgkin's Lymphoma on Chest Wall Location

RESPIRATORY CASE REPORTS

Muharrem Çakmak, Akın Eraslan Balcı, Siyami Aydın, Suna Polatoğlu

Özet

Abstract

Non-Hodgkin lenfomada torasik tutulum ya mediyastinal-hiler lenfadenopatiler ya da akciğer parankimi tutulumu şeklindedir. Non-Hodgkin lenfomada izole göğüs duvarı tutulumu nadirdir. Burada, sol anterosuperior yerleşimli, enfekte kitle lezyonu nedeniyle kliniğimize müracaat eden, eksizyon ve rekonstrüksiyonu yapılan, Non-Hodgkin lenfomalı olguyu paylaşmayı amaçladık.

Thoracic involvement in non-Hodgkin's lymphoma is generally seen as mediastinal-hilar lymphadenopathy or with pulmonary parenchymal involvement. Isolated chest wall involvement in non-Hodgkin's lymphoma is rare. Described here is the case of a patient with nonHodgkin's lymphoma who had undergone excision and reconstruction and presented at the clinic with an infected mass lesion with a left anterosuperior location.

Anahtar Sözcükler: Göğüs duvarı, Non-Hodgkin lenfoma, rekonstrüksiyon.

Göğüs duvarı tümörleri vücudun bütün primer tümörlerinin %2’sini, toraksa ait malignitelerin %5’ini oluşturur (1). Non-Hodgkin lenfomada (NHL) intratorasik tutulum, mediyastinal, hiler lenfadenopatiler ya da akciğer parankimi tutulumu şeklindedir. Göğüs duvarı tutulumunun en yaygın şekli, internal mamarian lenf nodu tutulumu ile beraber görülen göğüs ön duvarına direkt yayılımdır (2). NHL’da izole göğüs duvarı tutulumu nadirdir. En çok büyük hücreli lenfomada görülür. Çalışmamızda, sol anterosuperior göğüs duvarı yerleşimli, nadir görülen non-Hodgkin lenfoma olgusunu paylaşmayı amaçladık.

Fırat Üniversitesi Tıp Fakültesi, Göğüs Cerrahisi Kliniği, Elazığ

Key words: Chest wall, Non-Hodgkin lymphoma, reconstruction.

OLGU Yirmi beş yaşında erkek hasta, sol anterosuperior bölgede son iki yıldır giderek büyüyen, ağrılı, pürülan vasıflı akıntıları olan kitle şikayetiyle kliniğimize başvurdu. Yapılan muayenesinde, sol pektoral bölgede yer yer nekrotik alanlar içeren, yaygın kızarıklık gösteren, seropürülan akıntılı, yaklaşık 10x7 cm boyutlarında sert kitle lezyonu görüldü (Şekil 1). Kitlenin göğüs duvarındaki sınırlarını görmek için toraks BT istendi. Toraks BT’de sınırları düzensiz, kemik dokuyu invaze etmeyen kitle lezyonu tespit edildi (Şekil 2a ve b). Özgeçmişinde böbrek transplantasyonu hikayesi olan hastanın, soygeçmişinde annede

Department of Chest Surgery, Fırat University Faculty of Medicine, Elazıg, Turkey

Başvuru tarihi (Submitted): 24.10.2017 Kabul tarihi (Accepted): 21.11.2017 İletişim (Correspondence): Muharrem Çakmak, Fırat Üniversitesi Tıp Fakültesi, Göğüs Cerrahisi Kliniği, Elazığ e-mail: drcakmak@gmail.com

Respiratory Case Reports

lenfoma hastalığı olduğu öğrenildi. Pürülan akıntıdan ve dokudan alınan örneklerin, mikrobiyolojik ve patolojik incelemeleri negatifti. Laboratuvar değerleri de normal olan hastaya operasyon planlandı. Ameliyat sırasında kitlenin kas fasyasına uzandığı, fakat kas ve kemiğe invazyon yapmadığı görüldü. Sağlam dokuyu kapsayacak şekilde geniş eksizyon uygulandı (Şekil 3a ve b). Ameliyat sonrası oluşan cilt defekti, gluteal bölgeden alınan deri grefti ile kapatıldı (Şekil 4). Patoloji sonucu diffüz büyük B-hücreli lenfoma olarak bildirildi, cerrahi sınırlar ise negatifti (Şekil 5a, b, c, d). Hastaya cerrahiden 20 gün sonra 4 kür kemoterapi verildi. Dört aydır takip edilen olguda nüks ya da herhangi bir komplikasyon görülmedi.

alan, akut lenfoblastik lösemiden kemik iliği tutulumuna göre ayrılan bir lenfoma tipidir (4).

Şekil 2a ve b: Tomografi kesitleri, aksiyel (a), sagital (b).

Şekil 1: Göğüs duvarındaki kitle.

TARTIŞMA Lenfoma, T ve B lenfositleri ile doğal öldürücü hücrelerden köken alan klonal neoplazmlardır. Hodgkin Lenfoma (HL) ve Non-Hodgkin Lenfoma (NHL) olarak 2’ye ayrılır. Son sınıflamada, NHL, B lenfositlerden, T lenfositlerden ve doğal öldürücü hücrelerden köken alanlar olmak üzere farklı gruplar altında toplanmıştır. Mediyastinal tümörlerin yetişkinlerde %20, çocuklarda ise %50’si mediyastinal lenfomadır (3). Primer mediyastinal B hücreli lenfoma (PML), timik medüller B hücrelerinden köken alan, klinik olarak agresif B hücreli lenfoma tipidir. Büyük mediyastinal kitleye neden olan Lenfoblastik lenfoma (LL) ise, T hücrelerinden köken

Cilt - Vol. 7 Sayı - No. 2

Olguların yaklaşık %90’ı NHL'dır. NHL; erkeklerde 1,5 kat daha fazladır, HL'de bu oran eşittir. PML, üç ve dördüncü dekadda ve kadınlarda daha sık rastlanır. Plevra, perikard, akciğer ve göğüs duvarı tutulumu yapan kitledir. LL, ikinci ve 7’nci dekatlarda sık görülür (5). Erkeklerde sık rastlanan büyük mediyastinal kitle şeklindedir. Olgumuz da, göğüs duvarı tutulumu gösteren PML olgusu idi. Torasik yerleşimli lenfomada, göğüs duvarı invazyonu %2-5 oranında görülür. Press ve ark. (6) tarafından göğüs duvarı lenfoması %1.6 oranında bildirilirken, bir diğer çalışmada bu oran %4.4 olarak bildirilmiştir (1). En sık tutulan bölge, ön toraks duvarının pektoral ve subpektoral bölgeleridir (2,7). Birkaç olguda, plevra, kaburga ve sternumdan kaynaklanan ve cilt tutulumu yapan lenfoma olgusu bildirilmiştir (8-11). Hsu ve ark. (1) serilerinde primer göğüs duvarı NHL oranını %4,45 olarak bildirmiş-

Göğüs Duvarı Yerleşimli Non-Hodgkin Lenfoma Olgusu | Çakmak et al.

tir. Olgumuzda tutulum yeri literatürle uyumlu olarak sol pektoral bölge idi.

mine yol açtığı bildirilmektedir (12). Olgumuzda ise organ transplantasyon hikayesi mevcuttu.

Şekil 4: Greft ile rekonstrüksiyon.

Şekil 3a ve b: İntraoperatif kitle (a), eksize edilmiş kitle (b).

Lenfoma gelişiminde çeşitli faktörler rol oynar; immünsüpresyon, çeşitli enfeksiyonlar, çevresel maruziyetler önemli yer oluşturur. Konjenital immünsüpresyon durumlarındaki kişilerde hayatları boyunca herhangi bir malignite gelişme oranı %25 olup bunların yaklaşık yarısını NHL oluşturmaktadır. Kazanılmış immün yetmezlik sendromu (AIDS), organ transplantasyonu gibi edinsel immünsüpresyon durumlarında ise Epstein-Barr virüsü (EBV) pozitifliğinin diffüz büyük B hücreli lenfoma ve Burkitt lenfoma gelişi-

NHL’de hastalar, yaygın ağrısız LAP ile başvururlar. Sistemik semptomlar fazla görülmez. Kesin tanı, histopatolojik değerlendirme ile konur. HL genellikle servikal ya da supraklavikuler bölgede gelişen ağrısız LAP ile ortaya çıkar. Hastaların %50’sinde mediyastinal kitle mevcut olup asemptomatik olabileceği gibi retrosternal ağrı, dispne, vena kava süperior sendromu gözlenebilir. Hastaların %25’inde, B semptomları denilen ateş, gece terlemesi, son 6 ayda vücut ağırlığının %10’undan daha fazla kilo kaybı gibi sistemik semptomlar gözlenir (13). Olgumuzda, büyüyen, ağrılı, pürülan vasıflı akıntılı kitle dışında şikâyet yoktu. Toraks duvarında lokalize tümörün yayılımını saptamada ve verilen tedaviye cevabın değerlendirilmesinde BT en değerli radyolojik tetkiktir. Bazı olgularda manyetik rezonans (MR) incelemesi de hastalığın derecesi hakkında ilave bilgi sağlayabilir (6). Olgumuzda, yayılımı saptamak için bilgisayarlı tomografi kullanıldı. Enfeksiyona bağlı pürülan akıntı nedeniyle Pozitron Emisyon Tomografisi (PET) düşünülmedi. Diğer lenfoma tiplerinde olduğu gibi kesin tanı histopatolojik olarak konur. BT eşliğinde yapılan transtorasik ince iğne aspirasyon biyopsisi (TİİAB) ile hızlı tanı koymak mümkündür. Ancak HL ve NHL da sık gözlenen fibrozis nedeniyle TİİAB ile alınan örnekler yetersiz kalabilmekte ve doku tanısına ihtiyaç duyulmaktadır. Ama LL’de fibrozis olmadığından tanı koymada ve tedaviye erken başlamawww.respircase.com

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da TİİAB yeterlidir. Eğer TİİAB ile sonuç alınamaz ve doku biyopsisi gerekir ise mediyastinoskopi, mediyastinotomi, torakoskopi, torakotomi ve sternotomi ile tanıya gidilebilir (7). Olgumuzda, kitleden gönderilen örneklerin, mikrobiyolojik ve patolojik incelemeleri negatifti.

Göğüs duvarı lenfomalı hastalarda cerrahi tedavinin yeri net değildir. Hastalar genellikle kemoterapi ya da radyoterapi ile tedavi edilir. Hodgson ve ark. (14) evre I-II lenfomalı 324 hastayı, kemoterapi ve lokal radyoterapi ile tedavi ettiklerini, fakat bu hastaların, kısa yaşam beklentili, lokal kontrolün mümkün olmadığı hastalar olarak bildirmişlerdir. Buna karşın, Ryan ve ark. (15) cerrahi tedavi uyguladıkları göğüs duvarı lenfomalı hastalarda, ortalama sağkalım süresini 49 ay olarak bildirmişlerdir. Bazı otörler, primer göğüs duvarı lenfomalarında eksizyonel biyopsiyi önerirken, çoğu otör, güvenli sınırlar şartıyla rezeksiyonun yapılması gerektiğini bildirirler (16). Olgumuzde kuvvetli malignite şüphesi, enfekte akıntı, ağrı gibi bulgular cerrahi gerekliliğini ön plana çıkarmakta idi. Sonuç olarak, göğüs duvarı tümörlerinin başarılı tedavisi; erken tanı, agresif cerrahi rezeksiyon ve göğüs duvarının tamiridir. Lenfoma gibi kemoterapiye hassas tümörlerde göğüs duvarı tutulumunda cerrahi tedavi ve sonrası radyoterapi, kemoterapi ya da multimodal tedavi başarı oranını artırmaktadır.

ÇIKAR ÇATIŞMASI Bu makalede herhangi bir çıkar çatışması bildirilmemiştir.

YAZAR KATKILARI Fikir - M.Ç., A.E.B., S.A., S.P.; Tasarım ve Dizayn - M.Ç., A.E.B., S.A., S.P.; Denetleme - M.Ç., A.E.B., S.A., S.P.; Kaynaklar - M.Ç., A.E.B.; Malzemeler - ; Veri Toplama ve/veya İşleme - S.A., S.P., M.Ç.; Analiz ve/veya Yorum M.Ç., A.E.B.; Literatür Taraması - M.Ç., A.E.B., S.P.; Yazıyı Yazan - M.Ç.; Eleştirel İnceleme - M.Ç., S.A.

KAYNAKLAR

Şekil 5a, b, c ve d: Kas liflerini yararak ilerleyen, küçük yuvarlak hücrelerden oluşan atipik lenfoid imfiltrasyon (HEx100) (a), Lenfoid hücrelerde LCA ile pozitif boyanma (immünperoksidazx400) (b), Lenfoid hücrelerde B hücre belirleyicisi, CD20 ile pozitif boyanma (immünperoksidazx400) (c), Lenfoid hücrelerde B hücre belirleyicisi, CD79a ile pozitif boyanma (immünperoksidazx400) (d).

Cilt - Vol. 7 Sayı - No. 2

Hsu PK, Hsu HS, Li AF, Wang LS, Huang BS, Huang MH, et al. Non-Hodgkin’s lymphoma presenting as a large chest wall mass. Ann Thorac Surg 2006; 81:1214-8. [CrossRef]

North LB, Libshitz HI, Lorigan JG. Thoracic lymphoma. Radiol Clin North Am 1990; 28:745-62.

Swerdlow SH, Campo E, Harris NL. WHO Classifıcation of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008.

Addis BJ, Isaacson PG. Large cell lymphoma of the mediastinum: a B-cell tumour of probable thymic origin. Histopathology 1986; 10:379-90. [CrossRef]

American Cancer Society. Cancer Fact & Figures. Atlanta: American Cancer Society; 2013.

Göğüs Duvarı Yerleşimli Non-Hodgkin Lenfoma Olgusu | Çakmak et al.

Press GA, Glazer HS, Wasserman TH, Aronberg DJ, Lee JK, Sagel SS. Thoracic wall involvement by Hodgkin disease and non-Hodgkin lymphoma: CT evaluation. Radiology 1985, 157:195–8.

Cho CS, Blank N, Castellino RA. Computerized tomography evaluation of chest wall involvement in lymphoma. Cancer 1985; 55:1892-4. [CrossRef]

Tori M, Fujii Y, Minami M, Ohsawa M, Aozasa K, Matsuda H. Hodgkin’s disease of the chest wall: report of a case. Surg Today 1998; 28:853-6.

Hirai S, Hamanaka Y, Mitsui N, Morifuji K, Sutoh M. Primary malignant lymphoma arising in the pleura without preceding long-standing pyothorax. Ann Thorac Cardiovasc Surg 2004; 10:297-300.

10. Lones MA, Sanger W, Perkins SL, Medeiros LJ. Anaplastic large cell lymphoma arising in bone: report of a case of the monomorphic variant with the t(2;5)(p23;q35) translocation. Arch Pathol Lab Med 2000; 124:1339-43.

11. Faries PL, D’Ayala M, Santos GH. Primary immunoblastic B-cell lymphoma of sternum. J Thorac Cardiovasc Surg 1997; 114:684-5. [CrossRef] 12. Quinlan SC, Pfeiffer RM, Morton LM, Engels EA. Risk factors for early-onset and late-onset post-transplant lymphoproliferative disorder in kidney recipients in the United States. Am J Hematol 2011; 86:206-9. [CrossRef] 13. Shankland KR, Armitage JO, Hancock BW. Non-Hodgkin lymphoma. Lancet 2012; 380:848-57. [CrossRef] 14. Hodgson DC, Tsang RW, Pintilie M, Sun A, Wells W, Crump M, et al. Impact of chest wall and lung invasion on outcome of stage I-II Hodgkin’s lymphoma after combined modality therapy. Int J Radiat Oncol Biol Phys 2003; 57:1374-81. [CrossRef] 15. Ryan MB, McMurtrey MJ, Roth JA. Current management of chest-wall tumors. Surg Clin North Am 1989; 69:1061-80. [CrossRef] 16. Incarbone M, Pastorino U. Surgical treatment of chest wall tumors. World J Surg 2001; 25:218-30. [CrossRef]

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Respir Case Rep 2018;7(2):71-74 DOI: 10.5505/respircase.2018.26213

OLGU SUNUMU

CASE REPORT

Costal Hydatid Cyst: A Rare Case Report Kostal Hidatik Kist: Nadir Bir Olgu Sunumu

RESPIRATORY CASE REPORTS

Ferdane Melike Duran1, Mustafa Çalık1, Saniye Göknil Çalık2, Nuri Düzgün1, Hıdır Esme1

Abstract

Özet

Although human beings have known of hydatid cysts for 2000 years, it is still an important public health problem even today, especially in areas where it is endemic. A 41-year-old male patient consulted the clinic with left back pain. A thorax computed tomography image showed a cystic, expanding mass in the 9th and 10th ribs and the vertebral transverse process, with apparent destruction. Costal hydatid cyst accounts for less than 1% of all cases. There were 38 cases reported in 1978 and 60 in 2010. Although it is endemic, there are few cases reported in our country. An unusual localization of hydatid cyst may lead to an incorrect or late diagnosis. Hydatid cyst should definitely be kept in mind in the differential diagnosis of all lesions, especially in areas where it is endemic.

İnsanoğlu hidatik kisti iki bin yıldır bilmesine rağmen özellikle endemik olarak bulunduğu bölgelerde bugün bile önemli bir halk sağlığı problemidir. Kırk bir yaşındaki erkek hasta sol sırt ağrısı ile kliniğimize danışıldı. Toraks BT incelemesinde 9. ve 10. kaburgalarda ve vertebra transvers procesinde kistik genişleme ve destrüksiyon gösteren kitle tespit edildi. Kostal hidatik kist tüm vakaların %1’den azını teşkil etmektedir. Literatürde, 1978 yılına kadar bildirilen olgu 38 iken 2010 yılında bu sayı 60 olmuştur. Endemik olmasına rağmen ülkemizden bildirilen olgu sayısı da birkaç tane ile sınırlıdır. Kostal hidatik kist nadir ve sıra dışı lokalizasyonlara yerleşimi nedeniyle ayrıcı tanıda kolaylıkla atlanabilir; yanlış veya geç tanı konulmasına neden olabilir. Hidatik kist özellikle endemik olarak bulunduğu bölgelerde tüm lezyonların ayırıcı tanısında mutlaka akılda tutulmalıdır.

Key words: Unusual localization, rib, bone, hydatid, cyst.

Hydatid disease is usually caused by the cestode Echinococcus granulosus (E. granulosus), for which humans are an intermediate host. It has been recognized since the time of Hippocrates. Cystic echinococcosis can be caused by infection with E. granulosus, E. alveolaris, E. multilocularis, or E. vogeli. It is endemic in agricultural regions and where animal breeding is prevalent, such as eastern and southern Europe, North Africa, the Middle East, the Far East, South and Central America, and the former Soviet republics, where environmental health and preventive medicine 1

Department of Thoracic Surgery, Health Sciences University, Konya Training and Research Hospital, Konya, Turkey 2 Emergency and First Aid Program, Vocational School of Health Services KTO Karatay University, Konya, Turkey

Anahtar Sözcükler: Olağan dışı lokalizasyon, kaburga, kemik, hidatik, kist.

services are not insufficient (1). It has spread worldwide as a result of travel from these areas (2). Nearly all hydatid cysts are found in the liver or the lungs (1). Only 0.5% to 2% of all hydatid cyst cases demonstrate attachment to bone, and half of these are in the vertebrae (3). Costal residence of a hydatid cyst is extremely rare. In the literature, costal hydatid cyst has been reported in approximately 60 adult patients (4). Presently described is the case of a patient with a costal hydatid cyst, a presentation that is very unusual.

Sağlık Bilimleri Üniversitesi, Konya Eğitim ve Araştırma Hastanesi, Göğüs Cerrahisi Kliniği, Konya 2 KTO Karatay Üniversitesi Sağlık Hizmetleri Meslek Yüksek Okulu, İlk ve Acil Yardım Programı, Konya

Submitted (Başvuru tarihi): 02.05.2017 Accepted (Kabul tarihi): 12.12.2017 Correspondence (İletişim): Mustafa Çalık, Department of Thoracic Surgery, Health Sciences University, Konya Training and Research Hospital, Konya, Turkey e-mail: drmcalik@hotmail.com

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CASE

DISCUSSION

A 41-year-old male patient presented at the clinic with left back pain. No anomaly was found in a physical examination or laboratory tests. His medical history included an operation for left lung hydatid cyst 20 years previously. A thorax computed tomography (CT) examination revealed a cystic mass 75x40x70 mm in size in the vertebra transverse process and in the left 9th and 10th ribs, with visible destruction (Figure 1). After all diagnostic tests were completed, he underwent surgery. A left-sided posterolateral thoracotomy was performed. An incision was made to the left hemithorax. The thorax was accessed and the cohesions were removed with obtuse and sharp dissection. A smooth-margined, 75x40x70-mm, lobulated lesion was identified adjacent to the T10 vertebra. The mass was protruding into the hemithorax and the adjacent soft tissue. It was also observed that the mass was a degenerated hydatid cyst when it was separated from the surrounding structures. Multiple germinative membranes were removed. The related area was resected with a 2cm en bloc surgical border. The vertebra corpus and spinal cord were intact; no additional surgery was required for the vertebral corpus. The chest wall was closed primarily without any grafting. No perioperative complications developed. The diagnosis was verified with surgical exploration and histopathological evaluation. The patient was discharged on the postoperative sixth day without any complaint. Albendazole treatment of 10 mg/kg/day was administered to the patient for 3 months. In 24 months of follow-up, he had no further recurrence or complications. Written informed consent was obtained from patient who participated in this study.

Although human beings have known of hydatid disease for 2000 years, it has been possible to understand the physiopathology and life cycle of the cyst only in the last 2 centuries. Despite being an ancient concern, it remains an important public health problem, especially in areas where it is endemic (1). Currently, there is virtually no country where hydatid cyst is not present due to increased travel and migration from endemic areas (1,2). The eggs can remain viable for months in pastures, gardens, fomites, sand, or water, and may be consumed in foods such as vegetables, fruits, herbs, or contaminated water. While primarily a parasite of dogs, humans can accidentally become a host by swallowing the cystic form of the parasite. Cysts are not destroyed by the defense mechanisms of the human organism. Cysts formed in the duodenum may be distributed to any part of the body via the vena porta through hematogenous or lymphogenous routes. The liver and lungs, where hydatid cysts are most frequently resident, act as mechanical filters. This is why 90% of hydatid disease cases occur in these organs (1). The remaining 10% are found in tissues and organs, such as the spleen, pancreas, gallbladder, suprarenal gland, pelvis, seminal vesicle, heart, bone, breast, kidney, thyroid gland, and muscles with soft tissue (5). Although many hypotheses have been suggested for these rare localizations, their pathogenesis is not yet completely understood. The theory of shunt flow is perhaps the most accepted. Bone involvement is quite interesting when compared with other rare locations. Most often, localization is in compact bone containing calcium, which is porous, in contrast with soft tissue. It has been demonstrated by Dew (3) that hydatid cyst embryos localize in bone due to high vascularity, and invade other parts of bone and surrounding tissues. Diagnosis can be made by evaluating the clinical, radiological, and laboratory data, as well as the anamnesis. The findings and symptoms of hydatid disease depend on the organ involved, localization in the organ and tissue, the effect on the adjacent tissue, rupture or complications, secondary infections, and immunological reactions. The most frequently seen early symptom is pain (1,3). Left back pain was the initial complaint in our patient who was in compliance with literature. The medical history of this patient included an operation performed on the same side of the lungs 20 years earlier. Radiological monitoring methods are the first and most significant options. Laboratory tests have a limited effect in diagnosis. As in our case, views of hydatid cysts in rare localizations may

Figure 1: A three-dimensional computed tomography scan showing an expanding cystic mass in the 9th and10th ribs with destruction of the vertebral transverse process, seen in the (A) anterior, (B) posterior, and (C) anterior view with soft tissue (white arrow) Cilt - Vol. 7 SayÄą - No. 2

Costal Hydatid Cyst: A Rare Case Report | Duran et al.

lead to diagnostic problems, as they can be confused with benign or malign tumors, abscess, and other cysts. The preoperative diagnosis of our patient was neurogenic cyst. Costal hydatid cysts are differentiated from other bone hydatid cysts based on destruction in the rib or bone matrix and the rupture risk for surrounding tissues. The most current treatment for bone hydatid cyst is still mostly based on the surgeon's opinion and experience, and typically moderate-to-poor quality evidence. Although the "best treatment" of bone hydatid cyst is still a matter of debate, management of these cases can be quite challenging due to the important problem of recurrence. There are numerous patient series in the literature describing hydatid cyst disease, whereas reports of recurrence and its frequency are limited to a few. In general, recurrence is seen at varying rates, beginning from the third month after the first operation to 20 years later (6). The highest recurrence rates were findings in 40% of the vertebrae (7), 17% of all bones (8), and 11.5% of the whole body. The World Health Organization (WHO) has no clear definition of relapse, recurrence, or re-infection of hydatid cyst. However, parameters have been defined in different series in the literature: primary hydatid cyst involvement except the liver and lung, liver cyst hydatid, difficult surgical site, multiple abdominal cysts, primary cyst larger than 7 to 10 cm, and stage I and II hydatid cysts as graded by the WHO (6). In a study evaluating 721 cases of osseous echinococcosis, recurrence was detected in 124 (17%) patients within 2 years. All recurrences occurred at the same bone site and no new bone lesions were detected. In theory, the parasite may remain dormant in previously cured cysts causing no further harm. It may be considered a persistent infection or remission (8). Twenty years prior, our patient underwent surgery for a hydatid cyst in the lung parenchyma on the same side. A hydatid cyst grows 1 to 5 mm per year in the bone (7). Therefore, it would take some 15 years for a single cyst to reach a size of 75 mm. There had been no change in the socioeconomic level or location of our patient over the previous 20 years and the same conditions were present that could lead to infection. Worldwide, the geographical distribution of the disease has not changed since 1930. Many authors have found similar distribution models, although there are now fewer reported cases (8). We accepted the patient as a case of primary hydatid cyst because of its size and due to the absence of other organ involvement, such as the liver, and 20 years having passed since the first operation.

Hypothetically, hydatid cyst is an eradicable disease; however, this has only been accomplished in small, developed island countries, like Iceland, New Zealand, Tasmania, Cyprus, and the Falkland Islands, as well as in limited areas in Argentina and Chile. In undeveloped or developing countries, such as the Mediterranean basin and the Middle East, animals retain their importance as sources of food, transportation, labor, and security. This makes control and eradication of the disease challenging, given the environmental conditions, hard-to-change cultural aspects, and human behaviors. It does not seem that this is likely to change in the near future in endemic areas. Therefore, the cheapest and the easiest approach are to work to prevent hydatid cyst from infecting humans (7). There is a difference between costal and other bone hydatid cysts, due to the risk of cyst rupture due to damage to a rib or the bone matrix and the adjacent tissues. Costal hydatid cyst represents less than 1% of all cases, yet while there were 38 cases reported in 1978, the total was 60 in 2010. Though it is endemic in Turkey, very few cases are reported in our country (8,9). When possible, the ideal treatment to avoid radical surgical resection and recurrence is anthelmintic treatment, though the treatment period is still a matter of debate (5,6,8).

CONCLUSION Costal hydatid cyst is infrequently seen, even in areas like Turkey, where it is endemic. It can be easily neglected in a differential diagnosis due to its rarity, and particularly in unusual localizations, it may result in an incorrect or delayed diagnosis. Hydatid cyst should be kept in mind in the differential diagnosis of all lesions, especially in areas where it is endemic.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Planning and Design - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Supervision - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Funding F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Materials - M.Ç., S.G.Ç.; Data Collection and/or Processing - M.Ç., S.G.Ç.; Analysis and/or Interpretation - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Literature Review - M.Ç., S.G.Ç.; Writing - M.Ç., S.G.Ç.; Critical Review - F.M.D., M.Ç., S.G.Ç., N.D., H.E.

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Respiratory Case Reports

YAZAR KATKILARI Fikir - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Tasarım ve Dizayn - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Denetleme F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Kaynaklar - F.M.D., M.Ç., S.G.Ç., N.D., H.E. Malzemeler - M.Ç., S.G.Ç.; Veri Toplama ve/veya İşleme - M.Ç., S.G.Ç.; Analiz ve/veya Yorum - F.M.D., M.Ç., S.G.Ç., N.D., H.E.; Literatür Taraması - M.Ç., S.G.Ç.; Yazıyı Yazan - M.Ç., S.G.Ç.; Eleştirel İnceleme - F.M.D., M.Ç., S.G.Ç., N.D., H.E.

Demir HA, Demir S, Emir S, Kacar A, Tiryaki T. Primary hydatid cyst of the rib mimicking chest wall tumor: a case report. J Pediatr Surg 2010; 45:2247-9. [CrossRef]

Safioleas M, Nikiteas N, Stamatakos M, Safioleas C, Manti CH, Revenas C, et al. Echinococcal cyst of the subcutaneous tissue: a rare case report. Parasitol Int 2008; 57:236-8. [CrossRef]

Velasco-Tirado V, Romero-Alegría Á, Belhassen-García M, Alonso-Sardón M, Esteban-Velasco C, López-Bernús A, et Al. Recurrence of cystic echinococcosis in an endemic area: a retrospective study. BMC Infect Dis 2017; 17:455. [CrossRef]

Çalık, M., Çalık, S.G., Esme, H. Vertebral hydatid disease: White cancer. Respir Case Rep 2017; 6:103-6. [CrossRef]

Steinmetz S, Racloz G, Stern R, Dominguez D, Al-Mayahi M, Schibler M, et al. Treatment challenges associated with bone echinococcosis. J Antimicrob Chemother 2014; 69:821-6. [CrossRef]

Karaoğlanoğlu N, Gorguner M, Eroglu A. Hydatid disease of rib. Ann Thorac Surg 2001; 71:372-3. [CrossRef]

REFERENCES 1.

Calik SG, Calik M, Yesildag M, Esme H. Intramuscular hydatid cyst report of an unusual case. J Acad Emerg Med Case Rep 2016; 7:61-3. [CrossRef]

Budke CM, Deplazes P, Torgerson PR. Global socioeconomic impact of cystic echinococcosis. Emerg Infect Dis. 2006; 12:296-303. [CrossRef]

Kaloostian PE, Gokaslan ZL. Spinal hydatid disease: a multidisciplinary pathology. World Neurosurg 2015; 83:52-3. [CrossRef]

Cilt - Vol. 7 Sayı - No. 2

Respir Case Rep 2018;7(2):75-78 DOI: 10.5505/respircase.2018.04900

OLGU SUNUMU

CASE REPORT

Video-Assisted Thoracoscopic Removal of a Mysterious Foreign Body Causing Pneumothorax Pnömotoraksa Neden Olan Gizemli Yabancı Cismin Video-Yardımlı Torakoskopik Cerrahi ile Çıkarılması

RESPIRATORY CASE REPORTS

Serkan Yazgan, Banu Yoldaş, Soner Gürsoy

Abstract

Özet

The removal of foreign bodies from the pleural cavity via video-assisted thoracoscopic surgery (VATS) has seldom been reported in the literature. This is a description of the case of a 31-year-old female patient who presented with secondary pneumothorax due to a foreign body. The patient did not have any information about the foreign body. The metallic object was successfully removed with VATS, and it was discovered that the object was a broken injection needle. This inexplicable circumstance issue was referred to the legal department of the hospital. Videothoracoscopic removal is the safest procedure for intrapleural foreign objects.

Plevral kaviteden videotorakoskopik olarak yabancı cisim çıkarılması literatürde oldukça nadirdir. Bu sunuda etiyolojisi aydınlatılamamış olan intraplevral yabancı cisme bağlı pnömotoraks olgusu ve tedavisine yer verilmiştir. Radyolojik olarak sol plevrada ve karaciğerde metalik objeler görülen, 31 yaşındaki bayan hasta, bu yabancı cisimler hakkında herhangi bir bilgiye sahip değildi. Metalik obje video-yardımlı torakoskopi (VATS) ile başarılı biçimde çıkarıldı ve kırılmış bir enjektör iğnesi olduğu anlaşıldı. Bu açıklanamayan durum hastanenin hukuk birimine bildirildi. Plevral aralıkta saptanan yabancı cisimlerin çıkarılmasında, VATS’ın güvenle uygulanabileceğinin altını çizmek isteriz.

Key words: Foreign body, needle, pneumothorax, video-assisted thoracic surgery (VATS).

Removal of foreign bodies from the pleural cavity using VATS has seldom been reported in the literature (1,2). Thoracic surgeons mostly use VATS or thoracotomy under general anesthesia for foreign body removal from the chest wall or the pleural cavity (1-4). However, there is no consensus with regard to treatment (1). Nonetheless, foreign bodies in the pleural cavity should be removed when possible (1,2). Otherwise, pneumothorax or empyema may develop. In this report, the case of a Department of Thoracic Surgery, University of Health Sciences, Dr. Suat Seren Chest Diseases and Thoracic Surgery, Medical Practice and Research Center, İstanbul, Turkey

Anahtar Sözcükler: Yabancı cisim, iğne, pnömotoraks, video-yardımlı torakoskopik cerrahi (VATS).

needle found in the pleural cavity in which the patient had no history of such foreign body penetration is described.

CASE A 31-year-old woman with secondary pneumothorax was referred to our department. There was no significant disorder in her history. Her laboratory tests were normal. Chest radiography showed a Sağlık Bilimleri Üniversitesi, Dr. Suat Seren Göğüs Hastalıkları ve Göğüs Cerrahisi Cerrahisi SUAM, Göğüs Cerrahisi Bölümü, İstanbul

Submitted (Başvuru tarihi): 31.12.2017 Accepted (Kabul tarihi): 31.01.2018 Correspondence (İletişim): Serkan Yazgan, Department of Thoracic Surgery, University of Health Sciences, Dr. Suat Seren Chest Diseases and Thoracic Surgery, Medical Practice and Research Center, İstanbul, Turkey e-mail: serkanyazgan@gmail.com

Respiratory Case Reports

large left pneumothorax and a foreign body in the left upper hemithorax (Figure 1a). The patient had no history of foreign body aspiration. Physical examination did not reveal any entrance site through the skin. No subcutaneous erythema was detected. A chest tube was inserted immediately to treat the pneumothorax and the radiograph was repeated some hours later. When this chest radiography was carefully examined, other foreign bodies were also observed in the patientâ&#x20AC;&#x2122;s abdomen (Figure 1b). Computerized tomography demonstrated metallic objects (2 needles) in the liver and a needle in the left infraclavicular area in the pleural cavity extending from the chest wall (Figure 2).

Figure 2: Coronal computed tomography scan showing a needle (red arrow) in the left upper hemithorax and another needle (red arrow) in the liver

The patient agreed to undergo video-assisted thoracoscopic surgery (VATS) to remove the pleural cavity foreign body. VATS provided clear visualization of the needle penetrating the parietal pleura (Figure 3a). The needle was removed using endoscopic grasping forceps, and it was determined to be a broken injection needle (Figure 3b). Chest tube drainage was maintained until the second day after the operation. There was no evidence of pneumothorax on a follow-up chest radiograph. A general surgery consultation was conducted regarding the foreign bodies in the abdomen. No surgery was planned for the needles in the liver. No psychiatric illness was detected following a psychiatric consultation. The removed needle was delivered to the legal department of the hospital. The patient was discharged on the third day after the operation.

DISCUSSION

Figure 1a and b: Chest radiograph demonstrating left-sided pneumothorax and a needle (red arrow) located between the fourth and fifth posterior ribs in the left hemithorax (a). Chest radiograph showing the chest tube and expansive left lung, as well as 2 needles in the region of the lumbar vertebra (b) Cilt - Vol. 7 SayÄą - No. 2

An intrapleural foreign body causing pneumothorax is exceedingly rare and few cases have been documented in the literature. Previous studies indicate that the etiology is most commonly secondary to iatrogenic injury, or an intentional traumatic or accidental event (1). Brodsky et al. (5) reported the first thoracoscopic removal of foreign bodies from the pleural space with single lung ventilation in 1981. In recent years, thoracic surgeons have begun to use VATS and better, technically advanced videoendoscopy instruments to remove foreign bodies in the pleural space with (3,6,7).

Video-Assisted Thoracoscopic Removal of a Mysterious Foreign Body Causing Pneumothorax | Yazgan et al.

CONCLUSION It is rare for a needle to pass into the pleural space. Complications, such as pneumothorax, empyema, or vascular injury, should be considered and should be eliminated when possible (8). VATS removal is the safest procedure for intrapleural foreign objects or those embedded in the chest wall.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - S.Y., B.Y., S.G.; Planning and Design - S.Y., B.Y., S.G.; Supervision - S.Y., B.Y., S.G.; Funding - S.Y., B.Y.; Materials - S.Y., B.Y.; Data Collection and/or Processing - S.Y.; Analysis and/or Interpretation - S.Y.; Literature Review - S.Y.; Writing - S.Y., B.Y.; Critical Review - S.Y., B.Y., S.G.

YAZAR KATKILARI

Figure 3a and b: Video-assisted thoracoscopic view of a needle (black arrow) penetrating the left upper hemithorax and the parietal pleura (a). The removed broken injection needle (b)

Foreign bodies entering the pleural space are categorized as the result of inhalation or passing through the chest wall from the exterior (7). Some foreign bodies in the pleural space may be incidentally detected radiographically, or they may be detected due to complications, such as secondary pneumothorax. In our case, first, we determined a large left pneumothorax, and then we realized that there were several foreign bodies present. Additional workups led to the discovery of 3 foreign bodies in total: one in the pleural space extending from the chest wall, and 2 in the liver. These shiny metal objects looked like needles. The patient underwent surgery and we removed the pleural cavity foreign body via VATS. When we examined the surgically removed foreign body, we realized that it was a broken injection needle. It was interesting, because neither our patient nor her family could provide any clue as to how the needles might have come to be in the pleural space and the liver. The patient had no psychiatric history or history of needle ingestion or aspiration. It was both interesting and suspicious. Since we thought there might be an unlawful aspect to the incident, the hospital legal department was informed.

Fikir - S.Y., B.Y., S.G.; Tasarım ve Dizayn - S.Y., B.Y., S.G.; Denetleme - S.Y., B.Y., S.G.; Kaynaklar - S.Y., B.Y.; Malzemeler - S.Y., B.Y.; Veri Toplama ve/veya İşleme S.Y.; Analiz ve/veya Yorum - S.Y.; Literatür Taraması S.Y.; Yazıyı Yazan - S.Y., B.Y.; Eleştirel İnceleme - S.Y., B.Y., S.G.

REFERENCES 1.

Weissberg D, Weissberg-Kasav D. Foreign bodies in pleura and chest wall. Ann Thorac Surg 2008; 86:95861. [CrossRef]

Tie ST, Wong JL, Kannan SK, Rahman JA. Pleuroscopic retrieval of a sewing needle from the pleural cavity under conscious sedation by a chest physician. J Bronchology Interv Pulmonol 2012; 19:246-8. [CrossRef]

von Riedenauer WB, Baker MK, Brewer RJ. Video-assisted thoracoscopic removal of migratory acupuncture needle causing pneumothorax. Chest 2007; 131:899-901. [CrossRef]

Urschel JD, Miller JD, Bennett WF. Self-inflicted pneumothoraces. Ann Thorac Surg 2001; 72:280-1. [CrossRef]

Brodsky JB, Welti RS, Mark JB. Thoracoscopy for retrieval of intrathoracic foreign bodies. Anesthesiology 1981; 54:91-2.

6. Yu PS, Chan HH, Lau RW, Capilli FG, Underwood MJ, Wan IY. Penetrating thoracic injury with retained foreign body: can video-assisted thoracic surgery take up the leading role in acute management? J Thorac Dis 2016; 8:2247-51. [CrossRef] www.respircase.com

Respiratory Case Reports

Ekeke C, Noble S, Merritt RE. Management of an intrapleural foreign body and empyema with video-assisted thoracoscopy. J Thorac Dis 2016; 8:2241-3. [CrossRef]

Cilt - Vol. 7 Sayı - No. 2

Apilioğulları B, Yoldaş B, Esme H, Bekçi TT. An unusual self-inserted foreign body in the mediastinum and its management: a case report. Turk Gogus Kalp Dama 2013; 3:810-2. [CrossRef]

Respir Case Rep 2018;7(2):79-81 DOI: 10.5505/respircase.2018.37980

OLGU SUNUMU

CASE REPORT

Primary Pleural Liposarcoma: A Case Report Primer Plevral Liposarkoma: Olgu Sunumu

RESPIRATORY CASE REPORTS

Ayman Ahmed1, Hüseyin Melek1, Ulviye Yalçınkaya2, Ahmet Sami Bayram1

Abstract

Özet

Primary pleural liposarcoma is a rare tumor; only a few cases have been reported in the literature. Presently described is the case of a 77-year-old female with a right pleural-based tumor misinterpreted as loculated pleural effusion, who was suffering from chronic right-sided chest pain. Radical resection of the tumor was performed and revealed a welldifferentiated pleural liposarcoma. This case is discussed with a review of the literature.

Primer plevral liposarkoma literatürde birkaç olguluk seriler olarak tanımlanan nadir görülen bir tümördür. Biz bu yazımızda, sağ plevral tabanlı olup ilk değerlendirmede plevral efüzyon olarak yorumlanan ve sağ göğüs duvarı ağrısı olan 77 yaşında bayan hastamızı sunduk. Kitleye radikal cerrahi rezeksiyon yapıldıktan sonra tanı iyi difensiye plevral liposarkoma geldi. Yazımızda olguyu literatür eşliğinde tartıştık.

Key words: Pleural liposarcoma, loculated pleural effusion, primary pleural tumor.

Primary pleural neoplasms are uncommon; they represent 10% of pleural tumors. The pleura is more commonly involved with a secondary or metastatic neoplasm from the lung or breast, lymphoma, and ovary or gastric carcinoma. Mesothelioma is the most frequent primary pleural tumor, accounting for some 90% (1,2). Primary pleural liposarcoma (PPL) is derived from primitive mesenchymal tissue. There are fewer than 20 cases of PPL described in the literature (3).

CASE A 77-year-old female patient, presented with a 3year history of dull, right-sided chest pain, a nonproductive cough, and progressive shortness of breath. She was a nonsmoker, but she had a history of exposure to asbestos. A clinical examination 1

Department of Thoracic Surgery, Uludağ University Faculty of Medicine, Bursa, Turkey 2 Department of Pathology, Uludağ University Faculty of Medicine, Bursa, Turkey

Anahtar Sözcükler: Plevral liposarkom, loküle plevral efüzyon, primer plevral tümör.

was unremarkable apart from diminished air entry in the right lower chest. A chest X-ray with frontal projection illustrated homogenous, right mid and lower zone opacity with well circumscribed margins (Figure 1a), and a computed tomography (CT) scan of the chest with contrast revealed a well circumscribed soft tissue mass with lobulated margins in the right lower pleural space compressing the right lower lobe (Figures 1c and d) that was suggestive of a fatty tissue mass/loculated effusion. An attempt to perform ultrasound-guided thoracentesis failed. A right posterolateral thoracotomy was performed and revealed a large, well-encapsulated fatty mass, 16×14 cm in size, compressing the right lower lobe, resting on the diaphragm, and arising from the lateral chest wall parietal pleura. The mass 1

Uludağ Üniversitesi Tıp Fakültesi,Göğüs Cerrahisi Anabilim Dalı, Bursa 2 Uludağ Üniversitesi Tıp Fakültesi, Patoloji Anabilim Dalı, Bursa

Submitted (Başvuru tarihi): 11.08.2017 Accepted (Kabul tarihi): 26.10.2017 Correspondence (İletişim): Ayman Ahmed, Department of Thoracic Surgery, Uludağ University Faculty of Medicine, Bursa, Turkey e-mail: aymnuh@gmail.com

Respiratory Case Reports

was dissected free from the surrounding tissue and extirpated (Figure 2). A parietal pleurectomy at the tumor base was done to ensure adequate tumor excision. The postoperative course was uneventful (Figure 1b). Microscopic sections of the tumor showed atypical mononuclear, multinuclear lipoblastic cells of various sizes with hyperchromatic nuclei, and areas of sclerosis with fibrous tissue bands. No necrosis was present. Histopathology examination confirmed the diagnosis of a welldifferentiated variant of pleural liposarcoma (Figure 3) with a negative surgical margin. The management plan was discussed by the hospital tumor board, and the decision was made not to pursue adjuvant radiotherapy or chemotherapy, considering the patientâ&#x20AC;&#x2122;s age, tumor histology, and the negative surgical margin. The patient was followed up regularly for 2 years and no local recurrence or distant metastasis was observed.

pleomorphic, dedifferentiated, and well-differentiated types. The myxoid subtype is the most common variant. Poor disease-free survival and Inferior overall-survival have been observed in the dedifferentiated, pleomorphic, and myxoid types (4,5). There is a great range of biological behavior among these subtypes, from well-differentiated liposarcoma with a low metastatic potential to the high-risk round cell or pleomorphic types, which tend to be higher grade and are associated with a high rate of distant metastases (6).

Figure 3a, b, c and d: Histopathology of the excised tumor. Marked variation in adipocyte size, delicate arborizing vasculature, and atypical hyperchromatic stromal cells (black arrow) H&E x100 (a); Atypical hyperchromatic stromal cells (black arrow) H&E x200 (b); Nuclear staining pattern of MDM2, (c); Nuclear staining pattern of CDK4 (d)

Figure 1a, b, c and d: Preoperative chest X-ray (a), postoperative chest X-ray (b), and preoperative chest computed tomography images (c and d)

Figure 2: Gross pathological specimen

DISCUSSION Primary pleural liposarcoma (PPL) is thought to be derived from residual nests of primitive mesenchymal cells in the pleural cavity. Histological subtypes include the myxoid, Cilt - Vol. 7 SayÄą - No. 2

PPL is a slow-growing tumor with a low potential for invasion. The main presenting symptoms are due to the effects of pressure on surrounding structures, and nonspecific symptoms, including a cough, chest pain, and shortness of breath. Sometimes the tumor is discovered incidentally during routine chest radiography (3). Among 16 documented cases of patients between the ages of 19 and 80 years (mean of 50 years), PPL was more frequent in men (11 men and 5 women) (3). Macroscopically, PPL is nodular, lobular, and has a gelatinous appearance. A series of 4 patients reported by Okby and Travis (7), described tumors with a largest diameter of 16 to 20 cm and weighing up to 1.868 g. Accurate diagnosis relies on careful interpretation of a radiological finding on a chest radiography and CT, with particular attention paid to tumor location, density, and relationship to the lungs, chest wall and mediastinum (7). The tumor size, histological type, and radical surgery are the factors that influence the prognosis of PPL (5). Enzinger and Winslow (8) reported that the 5-year survival rate of patients with well-differentiated and myxoid forms

Primary Pleural Liposarcoma: A Case Report | Ahmed et al.

exceeded 75%, whereas in the round cell and pleomorphic varieties, it was only about 20%. Radical surgery followed by adjuvant radiotherapy may benefit patients with PPL (7). In our case, as the patient was elderly, had a poor performance status, a favorable histological subtype (well differentiated), and a negative margin, no adjuvant radiotherapy was given. In conclusion, patients with well-differentiated pleural liposarcoma causing localized symptoms can be successfully managed with radical resection and tumor free margins without adjuvant radiation therapy. Our patient was doing well and was symptom-free without any recurrence or distant metastasis for 24 months after following this treatment plan.

CONFLICTS OF INTEREST

REFERENCES 1.

Bonomo L, Feragalli B, Sacco R, Merlino B, Storto ML. Malignant pleural disease. Eur J Radiol 2000; 34:98118. [CrossRef]

Gebhard S, Coindre JM, Michels JJ, Terrier P, Bertrand G, Trassard M, et al. Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: a study from the French Federation of Cancer Centers Sarcoma Group.Am J SurgPathol 2002; 26:601-16. [CrossRef]

Carrillo B JA, Navarrete C, López Arias MA, Peláez M. Primary pleural liposarcoma, pleomorphic variant. J Thorac Dis 2014; 6:E166-8. [CrossRef]

Granville L, Laga AC, Allen TC, Dishop M, Roggli VL, Churg A, et al. Review and update of uncommon primary pleural tumors: a practical approach to diagnosis. Arch Pathol Lab Med 2005; 129:1428-43.

Chen M, Yang J, Zhu L, Zhou C, Zhao H. Primary intrathoracic liposarcoma: a clinicopathologic study and prognostic analysis of 23 cases. J Cardiothorac Surg 2014; 9:119. [CrossRef]

Ghadimi MP, Liu P, Peng T, Bolshakov S, Young ED, Torres KE, et al. Pleomorphic liposarcoma: clinical observations and molecular variables. Cancer 2011; 117:5359-69. [CrossRef]

Okby NT, Travis WD. Liposarcoma of the pleural cavity: clinical and pathologic features of 4 cases with a review of the literature. Arch Pathol Lab Med 2000; 124:699703.

Enzinger FM, Winslow DJ. Liposarcoma, a study of 103 cases. Virchows Arch Pathol Anat Physiol Klin Med 1962; 335:367-88.

None declared.

AUTHOR CONTRIBUTIONS Concept - A.A., H.M., U.Y., A.S.B.; Planning and Design - A.A., H.M., U.Y., A.S.B.; Supervision - A.A., H.M., U.Y., A.S.B.; Funding - A.S.B.; Materials - U.Y.; Data Collection and/or Processing - A.A.; Analysis and/or Interpretation - A.A., A.S.B.; Literature Review - H.M.; Writing A.A.; Critical Review - A.A., A.S.B.

YAZAR KATKILARI Fikir - A.A., H.M., U.Y., A.S.B.; Tasarım ve Dizayn - A.A., H.M., U.Y., A.S.B.; Denetleme - A.A., H.M., U.Y., A.S.B.; Kaynaklar - A.S.B., M.Ç.; Malzemeler - U.Y.; Veri Toplama ve/veya İşleme - A.A.; Analiz ve/veya Yorum - A.A., A.S.B.; Literatür Taraması - H.M.; Yazıyı Yazan - A.A.; Eleştirel İnceleme - A.A., A.S.B.

www.respircase.com

Respir Case Rep 2018;7(2):82-85 DOI: 10.5505/respircase.2018.49379

OLGU SUNUMU

CASE REPORT

Pneumocystis Pneumonia with Atypical Presentation in an HIV Seronegative Patient with Systemic Lupus Erythematosus during Steroid Therapy Steroid Tedavisi Alan HIV Seronegatif Sistemik Lupus Eritematozisli bir Hastada Atipik Görünümlü Pnömosistis Pnömonisi

RESPIRATORY CASE REPORTS

Gina Amanda, Dianiati Kusumo Sutoyo

Abstract

Özet

Pneumocystis pneumonia (PCP) is common among HIV patients, but it is rare in patients with autoimmune diseases such as systemic lupus erythematosus (SLE). Some of the risk factors related to PCP in SLE patients include a high steroid dose, greater disease activity, renal involvement, and lower lymphocyte and CD4+ counts. Described herein is the case of a 23year-old female with SLE who was treated with highdose steroid therapy. She was admitted to the clinic with a dry cough and a prolonged fever persisting since the tapering of the steroid dose. High-resolution computed tomography of the thorax revealed intraseptal thickening, subpleural nodules, and enlargement of the 4L, 6, and 7 lymph nodes. A laboratory examination of an induced sputum sample using polymerase chain reaction was positive for Pneumocystis jirovecii. Trimethoprim / sulfamethoxazole was administered for 14 days and clinical improvement was observed.

Pnömosistis pnömonisi (PP) HIV'li hastalarda sık görülürken sistemik lupus eritematozisli (SLE) gibi otoimmün hastalığı olanlarda nadirdir. Yüksek doz steriod kullanımı, ağır hastalık, böbrek tutulumu, lenfosit ve CD4+sayısında düşüklük gibi bazı nedenler SLE hastalarında PP için risk oluşturabilmektedir. Burada, yüksek doz steroid tedavi alan SLE'li 23 yaşındaki kadın olgu sunulmuştur. Steroid dozu azaltıldığı sırada kuru öksürük ve devam eden ateş yakınmaları ile kliniğe yatırıldı. Yüksek çözünürlüklü toraks tomografisinde, intraseptal kalınlaşmalar, subplevral nodüller ve 4L, 6 ve 7 nolu lenf nodlarında büyüme saptandı. İndükte balgam örneğinin PCR ile yapılan incelemesinde Pneumocystis jirovecii pozitif bulundu. Trimetoprim / Sülfametaksazol tedavisi 14 gün uygulandı ve klinik iyileşme gözlendi. Anahtar Sözcükler: CD lenfosit, pnömositis pnömonisi, sistemik lupus eritematozis.

Key words: CD4 lymphocyte, pneumocystis pneumonia, systemic lupus erythematosus.

Pneumocystis pneumonia (PCP) is defined as an opportunistic infection caused by Pneumocystis jirovecii (formerly known as Pneumocystis carinii). PCP is common among HIV patients, but the incidence in non-HIV immunocompromised patients,

including those with connective tissue disease, is increasing (1,2). Chen et al. (3) found 69 (26.1%) patients with autoimmune diseases who were diagnosed with PCP over a 10-year period. PCP infection in systemic lupus erythematosus (SLE)

Department of Pulmonology and Respiratory Medicine Faculty of Endonezya Üniversitesi Tıp Fakültesi, Persahabatan Hastanesi, Medicine Universitas Indonesia, Persahabatan Hospital, Jakarta, Göğüs Hastalıkları Anabilim Dalı, Endonezya Indonesia Submitted (Başvuru tarihi): 30.11.2017 Accepted (Kabul tarihi): 22.01.2018 Correspondence (İletişim): Gina Amanda, Department of Pulmonology and Respiratory Medicine Faculty of Medicine Universitas Indonesia, Persahabatan Hospital, Jakarta, Indonesia e-mail: gina_amanda@ymail.com

Respiratory Case Reports

patients is uncommon, but it may be fatal (4). The incidence of PCP in SLE was reported as 0.58% in Taiwan and 2.4% in Thailand (5,6). Risk factors such as greater disease activity, a high steroid dose, renal involvement, and lower lymphocyte and CD4+ counts are related to PCP in SLE (7). Presently described is a case of PCP with atypical presentation in a patient with SLE that developed during steroid therapy.

mocystis jirovecii. The patient was diagnosed with PCP and treated with trimethoprim/sulfamethoxazole (TMPSMX) for 14 days. After treatment, a marked improvement in clinical features was observed.

CASE A 23-year-old female presented at the pulmonary infection clinic of the hospital with a prolonged fever and nonproductive cough. Three months earlier, she had been hospitalized with the manifestations of acute decompensated heart failure accompanied by pleural effusion, nephritis, arthritis, and anemia. Laboratory results included a positive anti-nuclear antibody (9.0) and ds-DNA (>200 U/mL) test result. The level of C4 was low (5.2; normal: 15-57 mg/dL) and the level of C3 was normal (100; normal: 83-193 mg/dL). She had been diagnosed with a severe flare-up of SLE. She was hospitalized for a month and stayed in the intensive cardiovascular care unit for 2 weeks. She was initially treated with an intravenous corticosteroid, which was later replaced with an oral steroid. She was still taking the tapering dose of the oral steroid at the time of presentation to the clinic. There was no history of pulmonary tuberculosis (TB) treatment. She had never smoked and there was no exposure to occupational hazards. A physical examination revealed moderately good general condition, including vital signs of a blood pressure of 100/70 mmHg on an anti-hypertension drug, a heart rate of 87/minute, a respiratory rate of 20/minute, and oxygen saturation of 98% in room air. Moon face was present; however, there was no enlargement of the neck or supraclavicular lymph nodes. Enlargement of the cardiac border was observed on chest percussion; but a pulmonary examination was normal. A blood hemogram revealed leukocytosis (17.150/ÎźL) with 15.9% lymphocytes. An HIV test was negative. The acid fast bacilli culture testing for Mycobacterium tuberculosis and a GeneXpert MTB/RIF assay (Cephaid, Sunnyvale, CA, USA) of sputum was negative. The sputum culture was negative for microorganisms. Highresolution computed tomography (HRCT) of the thorax was performed and indicated intraseptal thickening (Figure 1), subpleural nodules (Figure 2), and enlargement of the 4L, 6, and 7 lymph nodes. A polymerase chain reaction (PCR) test of induced sputum was positive for PneuCilt - Vol. 7 SayÄą - No. 2

Figure 1: High-resolution computed tomography of the thorax showed intraseptal thickening (arrow)

Figure 2: High-resolution computed tomography of the thorax revealed subpleural nodules (arrow)

DISCUSSION Pneumocystis jirovecii is an atypical fungus exhibiting pulmonary tropism, though it is an alveolar pathogen, and only disseminates very rarely. It is also a host-specific organism, which means that P. jirovecii only infects humans and cannot infect other mammalians (1,2). The sources of PCP infection are the environment, an asymptomatic carrier, and active PCP patients. It may be transmitted via an airborne route, either in the general population or in a hospital environment (1,8,9). In our case, the patient had been hospitalized for a month before she was diagnosed with PCP, so that may be the potential source of PCP transmission in this case. The use of high-dose steroids and immunosuppressive therapy are included as risk factors for PCP infection in SLE patients (4,7,8). Corticosteroids may decrease CD4+ counts, thus it may play a role in the development of PCP even when used in low or moderate doses. When the dose of steroid was tapered or it was discontinued sud-

Pneumocystis Pneumonia with Atypical Presentation in an HIV Seronegative Patient with Systemic Lupus Erythematosus during Steroid Therapy | Amanda et al.

denly, it led to immune reconstitution inflammatory syndrome (IRIS). In this condition, an excessive inflammatory response against P. jirovecii may injure the lung, causing the appearance of clinical symptoms. It also explains why PCP often occurs in cases of rheumatic disease when the CD4+ count has improved (>200/ÎźL) (2,9). Our patient had consumed a high dose of oral steroid and the manifestations of PCP occurred after the steroid dose had been tapered for several days. The clinical features of PCP in non-HIV immunesuppressed patients, such as connective tissue disease, are mild and non-specific in the early stages. Oxygen saturation measured by pulse oximeter may be normal at rest and chest X-ray findings may be almost normal. However, PCP in HIV-negative patients may cause fulminant respiratory failure with a high mortality index (8). HRCT of the thorax can help in PCP diagnosis. Ground glass opacity is a typical finding of PCP on HRCT, particularly in the upper lobes. Nevertheless, an atypical presentation, including asymmetrical infiltrates, subpleural nodules, cavitation, lymphadenopathy, cyst, pleural effusion, or pneumothorax may be seen on HRCT images of PCP patients (2,10). Roux et al. (11) found atypical HRCT patterns in 14% cases of PCP in HIV-negative patients in France. Several laboratory examinations may be used to detect P. jirovecii, such as microscopic and molecular tests of induced sputum, bronchoalveolar lavage fluids (BALF), or a lung tissue specimen. Microscopic examination using light microscopy can detect this parasite, and especially mature cysts, with staining methods such as toluidine blue O, methanol-Giemsa, or Gomori-Grocottâ&#x20AC;&#x2122;s methenamine silver nitrate. These staining methods have a high sensitivity, but a low specificity for the detection of P. jirovecii in BALF smears. Molecular detection using both conventional and real-time PCR was developed for the diagnosis of PCP. This method has a high sensitivity and specificity for the detection of P. jirovecii in most specimen sources, including induced sputum, BALF, nasopharyngeal aspirate, oropharyngeal wash, and lung biopsy. A PCR assay is also recommended for a diagnosis of PCP in an HIVnegative patient. In fact, a positive result from a PCR test may be seen in PCP patients or a Pneumocystis colonization, but it may be distinguished by clinical, radiological, and laboratory assessment (1,2). In our patient, mild, non-specific symptoms appeared in the early stage. Since our country has a high incidence of pulmonary TB, TB diagnostic procedures were performed. The result of

these tests was negative, so we could exclude TB infection in this patient. The PCP diagnosis was established based on the use of a steroid as a risk factor for PCP, the HRCT scan features, and the positive P. jirovecii result in the induced sputum sample using the PCR method. The recommended drug of choice and first-line therapy treatment for PCP is TMP-SMX. Alternative regimens, such as atovaquone, clindamycin plus primaquine, intravenous pentamidine, or TMP plus dapsone may be administered if there is a contraindication or the patient cannot tolerate TMP-SMX (Table 1). The clinician may also choose the drugs used to treat PCP based on the severity of the disease. The duration of PCP treatment in HIV-positive patients is 21 days. In contrast, in patients without HIV infection, the duration of treatment is 14 days (2). SLE patients who undergo a TMP-SMX regimen should have close monitoring, since it may trigger a lupus flare (12). Sulfa drugs may also provoke an allergic reaction in SLE patients and the frequency is higher in this group than in the normal population (13). Our patient had been administered a TMP-SMX regimen for 2 weeks. She had experienced nausea, but tolerated this adverse reaction. Unlike the guidelines for PCP prophylaxis among HIVpositive patients, there is no published guideline for chemoprophylaxis of PCP in HIV-negative patients who are treated with immunosuppressive therapy (14). Chemoprophylaxis may be suggested in a patient who receives at least 20 mg of prednisone per day for at least 1 month, but it may expose the patient to an adverse reaction to these drugs. A CD4+ cell count of less than 200 cells/mm3 after 1 month of immunosuppressive therapy is another alternative indication to provide PCP prophylaxis in this group. The CD4+ cell count should be monitored in patients who receive more than 15 mg prednisolone or equivalent per day, a corticosteroid for more than 3 months, or with a total lymphocyte count of less than 600 cell/mm3 (2,14). The timing of the start of prophylaxis therapy as well as the duration and the timing of the discontinuation of therapy may need further investigation in this group. Some case reports have revealed that PCP infection still occurred in autoimmune patients after the immunosuppressive agents were discontinued though they had PCP prophylaxis during treatment with immunosuppressive drugs. Furthermore, the incidence of PCP among patients with rheumatic diseases is dissimilar. PCP in SLE patients is rare, but its incidence is high at dermatomyositis patient. This evidence is also another consider-

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Respiratory Case Reports

Tsai MJ, Chou CW, Lin FC, Chang SC. Pneumocystis jiroveci pneumonia in patients with systemic lupus erythematosus after rituximab therapy. Lupus 2012; 21:914-8. [CrossRef]

Weng CT, Liu MF, Weng MY, Lee NY, Wang MC, Lin WC, et al. Pneumocystis jirovecii pneumonia in systemic lupus erythematosus from southern Taiwan. J Clin Rheumatol 2013; 19:252-8. [CrossRef]

Vananuvat P, Suwannalai P, Sungkanuparph S, Limsuwan T, Ngamjanyaporn P, Janwityanujit S. Primary prophylaxis for Pneumocystis jirovecii pneumonia in patients with connective tissue diseases. Semin Arthritis Rheum 2011; 41:497-502. [CrossRef]

Lertnawapan R, Totemchokchyakarn K, Nantiruj K, Janwityanujit S. Risk factors of Pneumocystis jeroveci pneumonia in patients with systemic lupus erythematosus. Rheumatol Int 2009; 29:491-6. [CrossRef]

Concept - G.A., D.K.S.; Planning and Design - G.A., D.K.S.; Supervision - G.A., D.K.S.; Funding - ; Materials ; Data Collection and/or Processing - D.K.S., G.A.; Analysis and/or Interpretation - D.K.S.; Literature Review G.A., D.K.S.; Writing - G.A.; Critical Review - D.K.S.

Mori S, Sugimoto M. Pneumocystis jirovecii Pneumonia in Rheumatoid Arthritis Patients: Risks and Prophylaxis Recommendations. Clin Med Insights Circ Respir Pulm Med 2015; 9(Suppl 1):29-40. [CrossRef]

Mori S, Sugimoto M. Pneumocystis jirovecii infection: an emerging threat to patients with rheumatoid arthritis. Rheumatology (Oxford) 2012; 51:2120-30. [CrossRef]

YAZAR KATKILARI

10. Kanne JP, Yandow DR, Meyer CA. Pneumocystis jiroveci pneumonia: high-resolution CT findings in patients with and without HIV infection. AJR Am J Roentgenol 2012; 198:W555-61. [CrossRef]

ation in the administration of chemoprophylaxis to patients with rheumatic disease (12). In summary, PCP in SLE patients who receive long -term steroid therapy is unusual. The symptoms may be mild in the early stages, but it may be potentially life-threatening with severe infection. Diagnostic procedures, such as radiological and laboratory investigations, should be performed in cases of suspected PCP infection. The treatment for PCP infection may be selected based on the severity of disease and patient’s tolerance. The clinician should be aware of the co-incidence of PCP in patients with rheumatic disease who receive immunosuppressive therapy, since the incidence is increasing and it is more fatal than PCP in HIV-positive patients.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS

Fikir - G.A., D.K.S.; Tasarım ve Dizayn - G.A., D.K.S.; Denetleme - G.A., D.K.S.; Kaynaklar - ; Malzemeler - ; Veri Toplama ve/veya İşleme - D.K.S., G.A.; Analiz ve/veya Yorum - D.K.S.; Literatür Taraması - G.A., D.K.S.; Yazıyı Yazan - G.A.; Eleştirel İnceleme - D.K.S.

REFERENCES 1.

Thomas CF Jr, Limper AH. Pneumocystis pneumonia. N Engl J Med 2004; 350:2487-98. [CrossRef]

Calderon EJ, Gutierrez-Rivero S, Durand-Joly I, Dei-Cas E. Pneumocystis infection in humans: diagnosis and treatment. Expert Rev Anti Infect Ther 2010; 8:683-701. [CrossRef]

Chen M, Tian X, Qin F, Zhou J, Liu J, Wang M, et al. Pneumocystis pneumonia in patients with autoimmune diseases: a retrospective study focused on clinical characteristics and prognostic factors related to death. PLoS One 2015; 10:e0139144. [CrossRef]

Cilt - Vol. 7 Sayı - No. 2

11. Roux A, Canet E, Valade S, Gangneux-Robert F, Hamane S, Lafabrie A, et al. Pneumocystis jirovecii pneumonia in patients with or without AIDS, France. Emerg Infect Dis 2014; 20:1490-7. [CrossRef] 12. Suryaprasad A, Stone JH. When is it safe to stop Pneumocystis jiroveci pneumonia prophylaxis? Insights from three cases complicating autoimmune diseases. Arthritis Rheum 2008; 59:1034-9. [CrossRef] 13. Aceves-Avila FJ, Benites-Godinez V. Drug allergies may be more frequent in systemic lupus erythematosus than in rheumatoid arthritis. J Clin Rheumatol 2008; 14:261-3. [CrossRef] 14. Anevlavis S, Kaltsas K, Bouros D. Prophylaxis for pneumocystis pneumonia (PCP) in non-HIV infected patients. Pneumon 2012; 25:348-50.

Respir Case Rep 2018;7(2):86-89 DOI: 10.5505/respircase.2018.72792

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CASE REPORT

A Rare Cause of Massive Pulmonary Hemorrhage: Invasive Actinomycosis Masif Pulmoner Hemorajinin Nadir bir Nedeni: İnvaziv Aktinomikoz

RESPIRATORY CASE REPORTS

Bilge Yılmaz Kara1, Mehmet Fatih İnecikli2, Melek Memoğlu1, Recep Bedir4, Uğur Kostakoğlu3, Songül Özyurt1, Gökçen Sevilgen5, Şule Batçık6, Ünal Şahin1

Abstract

Özet

Pulmonary actinomycosis is a severe clinical condition that may cause death if unrecognized. It may occur in patients who were previously healthy or may develop in patients with chronic immunosuppressant conditions. Presently described is a rare case of massive pulmonary hemorrhage with a related angioinvasive Actinomyces infection. A 52-year-old formerly immunocompetent man was admitted to the hospital due to blood-streaked sputum. A computed tomography image of the thorax taken after the patient’s clinical status suddenly worsened revealed total collapse of the left lung. No tumoral lesion was observed, but extensive necrosis of the mucosa of both main bronchi with massive hemorrhage in the left main bronchus was noticed in an urgent bronchoscopy performed in the intensive care unit. A histopathological examination of the mucosal punch biopsy demonstrated aggregates of filamentous Grampositive organisms indicating Actinomyces infection.

Pulmoner aktinomikoz geç tanı konulduğunda ölümle sonuçlanabilecek bir durumdur. Daha çok immün yetmezliği olan bireylerde beklendiği gibi immünkompetan kişilerde de görülebileceği unutulmamalıdır. Bu yazıda invaziv pulmoner aktinomikoza bağlı masif pulmoner hemoraji gelişen nadir bir olgu sunulmuştur. Elli iki yaşında bilinen immünsupresif durumu olmayan erkek hasta ağızdan balgamla karışık kan gelmesi nedeni ile yatırıldı. Elektif bronkoskopi planlanan hastanın genel durumunda ani kötüleşme nedeni ile çekilen toraks bilgisayarlı tomografisinde sol akciğerde başvuruda olmayan total atelektazi saptandı. Yoğun bakım ünitesinde yapılan acil bronkoskopide sol ana broşta masif hemoraji ve her iki ana bronş mukozasında yaygın nekrotik görünüm izlendi. Alınan mukozal biyopsinin patolojik incelemesinde aktinomikozla uyumlu gram pozitif filamentöz bakteri agregatları izlendi. Bronşiyal arter kateterizasyonunda ekstravazasyon saptanan artere

Department of Pulmonology, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey 2 Department of Radiology, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey 3 Department of Infectious Diseases, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey 4 Department of Pathology, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey 5 Department of Thoracic Surgery, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey 6 Department of Anethesiology and Reanimation, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey

Recep Tayyip Erdoğan Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Rize 2 Recep Tayyip Erdoğan Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, Rize 3 Recep Tayyip Erdoğan Üniversitesi Tıp Fakültesi, Klinik Mikrobiyoloji ve Enfeksiyon Hastalıkları Ana Bilim Dalı, Rize 4 Recep Tayyip Erdoğan Üniversitesi Tıp Fakültesi, Patoloji Anabilim Dalı, Rize 5 Recep Tayyip Erdoğan Üniversitesi Tıp Fakültesi, Göğüs Cerrahisi Anabilim Dalı, Rize 6 Recep Tayyip Erdoğan Üniversitesi Tıp Fakültesi, Anestezi ve Reanimasyon Anabilim Dalı, Rize

Submitted (Başvuru tarihi): 03.10.2017 Accepted (Kabul tarihi): 29.12.2017 Correspondence (İletişim): Bilge Yılmaz Kara, Department of Pulmonology, Recep Tayyip Erdogan University Faculty of Medicine, Rize, Turkey e-mail: drbilgeyilmaz@hotmail.com

Respiratory Case Reports

Selective embolization of a branch of the feeding artery was successful to control the hemorrhage, but necrotizing pneumonia emerged and the patient could not be discharged from the intensive care unit. He was later lost despite 40 days of treatment with intravenous penicillin-G plus antifungal therapy. Pulmonary angioinvasive infections like actinomycosis must be kept in mind in the absence of bronchial carcinoma or other frequently encountered diseases in cases of massive pulmonary hemorrhage.

embolizasyon işlemi uygulandı. Ancak yerine gelişen bilateral nekrotizan pnömoni nedeni ile hastanın yoğun bakım takibine devam edildi. Hasta IV penisilin G ve anti fungal tedaviye rağmen 40 günlük takip sonrasında kaybedildi. Masif pulmoner hemoraji olgularında sıklıkla kanama yapan hastalıklar ve tümoral lezyonların yokluğunda ayırıcı tanıda aktinomikoz gibi anjioinvaziv enfeksiyonlar düşünülmelidir. Anahtar Sözcükler: Aktinomikoz, masif pulmoner hemoraji, nekrotizan pnömoni.

Key words: Actinomycosis, massive pulmonary hemorrhage, necrotising pneumonia.

Actinomycosis is a chronic granulomatous infection of the lung parenchyma caused by Actinomyces species. These bacteria form aggregates of Gram-positive, branching, ﬁlamentous bacilli, usually found in hard, macroscopic grains of pus called “sulfur granules.” Actinomyces spp. naturally reside on the mucosal surfaces of healthy individuals. They gain access to deeper tissues via trauma, surgical procedures, prosthetic devices, or foreign bodies, and manage to overcome the mucosal barrier and the immune system (1). This report is a description of a case of pulmonary actinomycosis with massive pulmonary hemorrhage and fatal necrotizing pneumonia.

decreased to 44%. An urgent CT revealed total collapse of the left lung (Figure 2). A therapeutic bronchoscopy was performed and the active hemorrhage in the left main stem bronchus was terminated. Selective ventilation of the right lung with a double-lumen endotracheal tube provided adequate oxygenation, and arterial oxygen saturation of 96% was achieved. Selective cannulation and embolization of a branch of the truncus thyrocervicalis, which was responsible for the bleeding, was performed.

CASE A 52-year-old male was admitted to the hospital for blood-streaked sputum, which had been present for several days. He had no history of a prediagnosed medical condition. He was a heavy smoker and habitual drinker. The patient was afebrile, with a heart rate of 78 beats/minute, a respiratory rate of 22 breaths/minute, and a blood pressure of 110/70 mmHg on initial examination. His arterial oxygen saturation was 93% while breathing room air. A general physical examination revealed cachexia and poor oral hygiene. Cardiovascular examination was normal. Thoracic auscultation on both sides of the lungs was normal, with no added breath sounds. Examinations of other organ systems were normal. His leucocyte count was 10,500 cells/µL with a neutrophil predominance. The level of acute phase reactants was not elevated. A computed tomography (CT) scan of the chest revealed a ground-glass opacity in the left upper lobe, indicating hemorrhage (Figure 1). Prior to bronchoscopy, the patient experienced sudden chest pain and was transferred to the intensive care unit due to acute respiratory failure. His oxygen saturation Cilt - Vol. 7 Sayı - No. 2

Figure 1: CT sections showing a groud glass opacity indicating hemorrhage in the left upper lobe

Figure 2: CT sections indicating total collapse of the left lung

A second diagnostic bronchoscopy revealed no tumoral lesion but extensive necrosis of the distal trachea and the main bronchi mucosa (Figure 3). A histopathological examination of the mucosal punch biopsy sample demonstrated aggregates of filamentous Gram-positive organisms, indicating actinomycosis (Figure 4). Microbiological culture isolates failed to identify the bacteriological species because the specimens were not incubated in anaerobic conditions. Bronchial lavage fluid was sent to the laboratory for both direct microscopic investigation

A Rare Cause of Massive Pulmonary Hemorrhage: Invasive Actinomycosis | YÄąlmaz Kara et al.

and mycobacterial culture. In addition, polymerase chain reaction analysis for mycobacteria was performed. There was no bacterial growth on LĂśwenstein-Jensen medium after incubation for 45 days.

mimic tuberculosis (2) or lung cancer (3). Although individual papers describe infection of almost all tissues of human body, pulmonary actinomycosis represents approximately 15% of all cases (4). The most common clinical symptoms of pulmonary actinomycosis are chest pain, a productive cough, and hemoptysis. Subacute presentation of constitutional symptoms, like weight loss, fatigue, or fever, may also concur. In our case, the ground glass opacity in the left upper lobe, smoking history, and massive hemorrhage first suggested bronchogenic carcinoma in the differential diagnosis.

Figure 3: Bronchoscopic sections showing extensive necrosis of bronchial mucosa

Figure 5: Bilateral consolidations showing necrotising pneumonia during treatment with penisillin G

Figure 4: Aggregates of filamentous gram-positive organisms surrounded by inflammatory cells indicating Actinomycosis

Serum immunoglobulin levels were tested for HIV infection and tumor markers, and serum and urine analysis was examined for multiple myeloma. The departments of internal medicine and hematology were consulted. Further invasive investigations, such as a bone marrow biopsy, could not be performed due to the poor health status of the patient. Despite intravenous administration of penicillin-G, necrotizing pneumonia developed in the opposite lung (Figure 5). Antifungal treatment was added for possible coexisting fungal infection. Surgery was not considered due to diffuse infiltrates in both lung fields. The patient was lost after 40 days of treatment in the hospital. Written informed consent was obtained from the parents of the patient for this case presentation.

DISCUSSION Actinomycosis is a rare cause of pulmonary infection and can be difficult to diagnose because its presentation may

Actinomyces infections may affect individuals of all age groups, even children, but the most commonly affected group is middle-aged males. Comorbid conditions like diabetes mellitus, smoking or alcohol abuse, poor oral hygiene or periodontitis, gastrectomy, foreign body aspiration like a fish bone, prosthetic devices like a bronchial stent, and healed pulmonary tuberculosis have been reported in the literature, but immunocompetent patients are more commonly affected unexpectedly. Alcoholics with poor oral hygiene, as in our case, are more susceptible to suppurative infections due to aspiration of oral flora, including Actinomyces spp. (5). A history of choking must be investigated when no other reason seems to be responsible for the condition. The most common radiological findings are a mass, nodule, or consolidation on a chest CT (6). Almost all cases need pathological confirmation of actinomycosis from lung tissue. In a review article, invasive procedures were performed in 23 patients, of whom 14 underwent a wedge resection or lobectomy, 5 underwent a percutaneous transthoracic lung biopsy, and a bronchial biopsy was performed on 4 (7). Anaerobic cultures are required if there is a suspicion of Actinomyces infection, and collections must be made properly in order to identify the pathogens. In our case, the material collection and transport was not appropriate for anaerobic agents, so microbiological isolation of the Actinomyces species was not possible. www.respircase.com

Respiratory Case Reports

Although Actinomyces spp. are susceptible to several antibiotics the most commonly used agent is parenteral penicillin-G, followed by the oral form. The average duration of treatment is 4 to 5 months (8). Diagnosis or treatment of pulmonary actinomycosis usually requires surgical intervention (9). Although surgery seems to be the best treatment modality (10), less invasive techniques, like embolization of the related artery in selected cases with hemorrhage, might be favorable due to fewer complications. Surgery was not the treatment of choice in the present case because the infection had affected many areas of the lung.

CONCLUSION Although rare, pulmonary Actinomyces infection can be life threatening, especially in cases with massive hemorrhage. It must be kept in mind in the differential diagnosis of massive or non-massive hemoptysis or pulmonary hemorrhage, especially in the absence of a tumor or other common causes of bleeding.

CONFLICTS OF INTEREST None declared.

Malzemeler - ; Veri Toplama ve/veya İşleme - M.M., Ş.B.; Analiz ve/veya Yorum - B.Y.K., S.Ö.; Literatür Taraması B.Y.K., U.K.; Yazıyı Yazan - B.Y.K.; Eleştirel İnceleme Ü.Ş., S.Ö.

REFERENCES 1.

Könönen E, Wade WG. Actinomyces and related organisms in human infections. Clin Microbiol Rev 2015; 28:419-42. [CrossRef]

Varshney MK, Trikha V, Khan SA. Actinomycosis or tuberculosis? A diagnostic dilemma. Scand J Infect Dis 2006; 38:378-81. [CrossRef]

Schweigert M, Dubecz A, Beron M, Ofner D, Stein HJ. Pulmonary infections imitating lung cancer: clinical presentation and therapeutical approach. Ir J Med Sci 2013; 182:73-80. [CrossRef]

Russo TA. Agents of actinomycosis. In: Mandell GL, ed. Principles and practice of infectious disease, 5th ed. Philadelphia: Elsevier, Churchill Livingstone, 1995:26452654.

Marra A, Hillejan L, Ukena D. Management of lung abscess. Zentralbl Chir 2015; 140:S47-53. [CrossRef]

Baik JJ, Lee GL, Yoo CG, Han SK, Shim YS, Kim YW. Pulmonary actinomycosis in Korea. Respirology 1999; 4: 31–5. [CrossRef]

Sun XF, Wang P, Liu HR, Shi JH. A Retrospective study of pulmonary actinomycosis in a single institution in China. Chin Med J 2015; 128:1607-10. [CrossRef]

Zhang M, Zhang X-Y, Chen Y-B. Primary pulmonary actinomycosis: a retrospective analyses of 145 cases in mainland China. Int J Tuberc Lung Dis 2017; 21:825-31. [CrossRef]

Endo S, Murayama F, Yamaguchi T, Yamamoto S, Otani S, Saito N, et al. Surgical considerations for pulmonary actinomycosis. Ann Thorac Surg 2002; 74:185-90. [CrossRef]

AUTHOR CONTRIBUTIONS Concept - B.Y.K., M.F.İ., M.M., R.B., U.K., S.Ö., G.S., Ş.B., Ü.Ş.; Planning and Design - B.Y.K., M.F.İ., M.M., R.B., U.K., S.Ö., G.S., Ş.B., Ü.Ş.; Supervision - B.Y.K., M.F.İ., M.M., R.B., U.K., S.Ö., G.S., Ş.B., Ü.Ş.; Funding M.F.İ., R.B.; Materials -; Data Collection and/or Processing - M.M., Ş.B.; Analysis and/or Interpretation B.Y.K., S.Ö.; Literature Review - B.Y.K., U.K., B.Y.; Writing - B.Y.K.; Critical Review - Ü.Ş., S.Ö.

YAZAR KATKILARI Fikir - B.Y.K., M.F.İ., M.M., R.B., U.K., S.Ö., G.S., Ş.B., Ü.Ş.; Tasarım ve Dizayn - B.Y.K., M.F.İ., M.M., R.B., U.K., S.Ö., G.S., Ş.B., Ü.Ş.; Denetleme - B.Y.K., M.F.İ., M.M., R.B., U.K., S.Ö., G.S., Ş.B., Ü.Ş.; Kaynaklar - M.F.İ., R.B.;

Cilt - Vol. 7 Sayı - No. 2

10. Rizzi A, Rocco G, Della Pona C, Robustellini M, Rossi G, Massera F, et al. Pulmonary actinomycosis: surgical considerations. Monaldi Arch Chest Dis 1996; 51:369-72.

Respir Case Rep 2018;7(2):90-96 DOI: 10.5505/respircase.2018.82787

OLGU SUNUMU

CASE REPORT

Tuberculous Lymphadenitis of Hilar Lymph Nodes as a Cause of Right Middle Lobe Syndrome: A Case Report Sağ Orta Lob Sendromuna Neden Olan Hiler Lenf Nodu Tüberküloz Lenfadeniti: Olgu Sunumu

RESPIRATORY CASE REPORTS

Shital Patil1, Mazhar Mirza2

Abstract

Özet

Right middle lobe syndrome (RMLS) is a rare but important clinical entity that is characterized by recurrent or chronic collapse of the middle lobe of the right lung, but which may also involve the lingula of the left lung. In this case report, a 52-year-old female presented with typical constitutional symptoms of tropical disease like cough, fever, and shortness of breath. Chest radiology documented RMLS and bronchoscopy was key to the evaluation of this case, as a sputum examination was inconclusive. Transbronchial needle aspiration (TBNA) of the lymph node and bronchoalveolar lavage (BAL) specimens were tested and Mycobacterium tuberculosis (MTB) of the hilar lymph nodes on the right side was confirmed using the GeneXpert MTB/RIF assay. Four antituberculosis treatment drugs were initiated and maintained for a total of 8 months with steroids as an adjunct in tapering dosages for 4 weeks. Complete clinical and radiological recovery was documented and confirmed bronchoscopically. A high index of suspicion is important when managing RMLS cases and all possible measures should be taken to confirm diagnosis.

Orta lob sendromu (OLS) nadir görülen ve sağ akciğer orta lobda bazen de sol akciğer lingulada tekrarlayan veya kronik kollaps ile karakterize önemli bir klinik tablodur. Burada, öksürük, ateş, nefes darlığı gibi tropikal bir hastalığın tipik semptomları olan 52 yaşındaki bir kadın olgu sunulmaktadır. Balgam örnekleri yetersiz olduğu için grafideki OLS'yi belirlemek amacıyla bronkoskopik inceleme yapıldı. Transbronşial lenf nodu iğne aspirasyonu ve bronkoalveoler lavaj alındı. M. tuberculosis, lenf bezinde GeneXpert MTB/RIF assay ile saptandı. Dört haftada azaltılan dozlarda steroid ile beraber dörtlü antitüberküloz tedavi başlandı ve 8 ay sürdürüldü. Tam bir klinik ve radyolojik iyileşme görüldü ve bronkoskopik olarak da iyileşme saptandı. OLS için yüksek şüphe duyulan olgularda tanı için tüm tetkikler yapılmalıdır. Anahtar Sözcükler: OLS (Orta lob sendromu), tüberküloz lenfadenit, bronkoskopi, BAL, Gene Xpert MTB/RIF.

Key words: RMLS (Right middle lobe syndrome), tuberculous lymphadenitis, Bronchoscopy, BAL, Gene Xpert MTB/RIF.

Department of Pulmonary Medicine, MIMSR Medical College, Latur, Maharashtra, India 2 Department of Internal Medicine, MIMSR Medical College, Latur, Maharashtra, India

MIMSR Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Latur, Maharaştra, Hindistan 2 MIMSR Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Latur, Maharaştra, Hindistan

Submitted (Başvuru tarihi): 23.04.2017 Accepted (Kabul tarihi): 02.10.2017 Correspondence (İletişim): Shital Patil, Department of Pulmonary Medicine, MIMSR Medical College, Latur, Maharashtra, India e-mail: drsvpatil1980@gmail.com

Respiratory Case Reports

In 1948, Graham et al. (1) reported 12 patients with atelectasis and nontuberculous pneumonitis of the middle lobe of the right lung, which Effler and Ervin (2) subsequently termed middle lobe syndrome (MLS). There is limited literature on MLS, likely due to the multiple etiologies, varied presentation, and lack of a consistent clinical definition (3,4). The most common definition of MLS is recurrent or chronic collapse of the middle lobe of the right lung. Although originally described as occurring only in the middle lobe, it can involve the lingula as well, which is sometimes called lingula syndrome (5). Pulmonary symptoms are common in MLS, but some patients are asymptomatic and the disease is only detected with chest radiography (6). The epidemiology of MLS is not well described. It occurs in children and adults of both sexes, in both primary and tertiary care settings (7,8). In a community-based study, out of the 30,588 persons who underwent annual mini chest roentgenography, 0.17% had MLS diagnosed with chest X-ray films (4). The incidence was significantly higher in persons over 50 years of age than in those under 50 years of age (0.26% vs. 0.02%), and was higher in females than in males (0.20% vs. 0.11%) (9).

sides were normal. Bilateral coarse crackles and wheeze were also detected on auscultation. The hematological parameters were: hemoglobin level of 7.2 gm/dL; white blood cell count of 15600/mm3; platelet count of 5.6 lacs; peripheral smear indicated hypochromic, microcytic anemia; blood sugar level (random) of 118 mg/dL; normal liver function and kidney function tests; and HIV Tri-Dot test was non-reactive. Sputum smear examinations for acid-fast bacillus (AFB) stain done on 3 occasions (2 consecutive early morning samples and 1 spot specimen) were negative. A chest X-ray was performed in the posteroanterior (PA) view and revealed triangular opacity in the right mid-lower zone in the paracardiac area obliterating the cardiac border on the right side with an obvious silhouette sign secondary to collapse with consolidation of the right middle lobe. Evidence of a shift of a major fissure upward and a minor fissure downward are direct signs of right middle lobe collapse, also seen clearly in the chest X-ray film. Homogeneous parenchymal infiltrates were seen in the right upper and lower zones. The cardiac border on the left side was not clearly demarcated and was blurred due to opacification in the lingular segment, i.e., the silhouette sign was also positive on the left side (Figure 1).

CASE A 52-year-old female, a housekeeper by occupation, and a non-addict, presented at the MIMSR medical college outdoor clinic of pulmonary medicine with complaints of a cough ongoing for 8 weeks. It was initially dry and continuous, but sputum developed in 2 weeks. The sputum was whitish-yellow originally, but became greenishyellow and increased in volume over 6 weeks, without hemoptysis. She also reported shortness of breath that was grade I (SJRQ) at first, and progressed to grade IV over a period of 6 weeks. A fever had also been present for 6 weeks that was initially low grade and intermittent with minimal chills and without rigors, but which increased in frequency and duration with a predominant surge in the late evening and overnight. The patient was hospitalized in an indoor unit and a thorough medical examination and evaluation was performed and do cumented. Pallor was present, without cyanosis or clubbing, and there was no generalized lymphadenopathy. A respiratory system examination revealed decreased breath sounds in the mammary and infra-axillary area on the right side, and in the mammary and infrascapular area on the left side, while the rest of the lung fields over both

Cilt - Vol. 7 SayÄą - No. 2

Figure 1: Triangular opacity in the right, mid-lower zone of the paracardiac area can be seen obscuring the cardiac border on the right side with an obvious silhouette sign, secondary to collapse, with consolidation of the right middle lobe

The treatment protocol administered in the hospital included parenteral administration of amoxicillin/clavulanic acid 3 times and a single 500 mg dose of oral macrolide azithromycin.

Tuberculous Lymphadenitis of Hilar Lymph Nodes as a Cause of Right Middle Lobe Syndrome: A Case Report | Patil et al.

Fiberoptic bronchoscopy was used to determine the exact cause of the right middle lobe collapse. A Fujinon EPX 201H (Fujifilm Holdings Corp., Tokyo, Japan) system is routinely used in our bronchoscopy unit for all interventional diagnostic procedures, such as bronchial wash, bronchial brush, transbronchial needle aspiration (TBNA) cytology, forceps biopsy, and transbronchial lung biopsy, and we also have access to trained expertise in histopathology. A bronchoscopy image showed an edematous, partially blocked lumen of the right middle lobe. No endobronchial growth or intraluminal mucosal abnormality was observed. There was an intraluminal bulge seen at the opening of the right middle lobe leading to the blockage. The other openings of the right lower lobe were without any gross visible abnormality (Figure 2). A transbronchial lung biopsy (TBLB) was performed by passing forceps through the middle lobe bronchus lumen and 3 TBLB specimens were collected. A conventional TBNA was performed by passing a TBNA needle across the right middle lobe bronchus opening at the superior area where a bulge was visible. Bronchial wash fluid was also collected before and after procedures like TBLB and TBNA by instilling 80 mL of saline. One bronchoalveolar lavage (BAL) aliquot was used for an AFB smear, a second for cytology for malignant cells, and the third for BAL GeneXpert MTB/RIF analysis. The results of BAL cytology and smear for AFB were inconclusive. The TBLB and TBNA specimens were sent for histopathology and cytology evaluation and were confirmed as a chronic inflammatory pathology without any clear evidence of tuberculosis or malignancy.

The results of the TBNA specimen with BAL sent for GeneXpert MTB/RIF analysis were positive for the Mycobacterium tuberculosis (MTB) genome with no detectable resistance to the rifampicin (RIF) gene (rpoB). The 4-drug anti-tuberculosis treatment (ATT) composed of isoniazid, RIF, ethambutol, and pyrazinamide, was administered daily for 2 months, followed by isoniazid and RIF for 4 months, to complete 6 months standardized chemotherapy. Steroid adjunct therapy of prednisolone 15 mg daily for 2 weeks, 10 mg daily for 1 week, and 5 mg daily for 1 week was provided to avoid residual fibrosis, bronchostenosis, and stricture. The patient was discharged home after ATT with strict advice to maintain the regimen of medicines as prescribed and follow-up to assess liver function for any side effects and to perform a chest X-ray after 1 month for radiological response assessment. In Figure 3, a decrease in opacification of the bilateral lung fields, a decrease in the density and opacification of the paracardiac triangular area on the right side, and similar findings on the left side of the paracardiac area can be seen.

Figure 3: Image demonstrating a decrease in opacification in the bilateral lung fields, a decrease in paracardiac triangular opacity on the right side, particularly the density and size of opacification, and similar findings in the left paracardiac area (after 1 month of therapy)

Figure 2: Bronchoscopy image showing edematous, partially blocked lumen of the right middle lobe

A satisfactory clinical response in form of decreased cough, fever, and shortness of breath was observed in 4 weeks; the radiological response was delayed and took 5 months for near total response. In Figure 4, a near total resolution of the radiological abnormalities on both sides is visible, especially the triangular opacity of the right www.respircase.com

Respiratory Case Reports

middle lobe collapse. Minimal parenchymal infiltrates were present in the right paracardiac area. The ATT was extended from 6 months to 8 months total duration to document complete radiological resolution. Complete radiological response was observed after 8 months of therapy. Bronchoscopy was repeated at the conclusion of the ATT, and a complete response without any residual fibrosis or stricture was documented.

Figure 4: Image illustrating near-total resolution of radiological abnormalities on both sides, particularly the triangular opacity of the right middle lobe collapse. Minimal parenchymal infiltrates were present in the right paracardiac area (after 6 months of therapy)

DISCUSSION RMLS is defined as recurrent or chronic collapse of the middle lobe of the right lung (10). MLS is divided pathophysiologically into obstructive and non-obstructive types. Obstructive MLS can be caused by endobronchial lesions or extrinsic compression of the right middle lobe bronchus. Tumors, both benign and malignant, have been estimated to account for about one-quarter of cases of MLS3, but it is important to note that many MLS studies have excluded patients with identifiable neoplasms (1,3). Hamartoma can be a benign cause of obstruction, but malignant causes include primary lung cancer and metastases (3,10). The most common cause of extrinsic compression of the right middle lobe bronchus is enlargement of the peribronchial lymph nodes (4). This can be due to granulomatous infections that cause adenopathy, such as histoplasmosis, but other fungal infections and both typical and atypical mycobacterial infections are also well-described etiologies of obstructive MLS (3,11,12). Furthermore, adenopathy due to sarcoidosis Cilt - Vol. 7 SayÄą - No. 2

or lymph node metastases has also been described in obstructive MLS (13). Other less common causes of obstruction of the right middle lobe bronchus include aspirated foreign objects (particularly in children), broncholiths that erode endobronchially from adjacent calcified lymph nodes, inspissated mucus associated with cystic fibrosis or allergic bronchopulmonary aspergillosis, and endoluminal granulomas associated with sarcoidosis (14). In the non-obstructive type of MLS, the right middle lobe bronchus is patent with no demonstrable obstruction on bronchoscopy (7,8,15). This form of MLS is not restricted to the middle lobe and may be found in other lobes, most often the lingula of the left upper lobe (5). The nonobstructive form has also been called peripheral MLS and occurs in the majority of patients with MLS (3,6). Certain anatomical characteristics make the middle lobe and lingula susceptible to transient obstruction as a result of inflammation or edema. This is probably related to the embryological development of the lungs (16). In the early embryonic phase, the smaller left endodermal lung bud is directed more laterally than the caudally located right bud. Thus, the asymmetry of the main bronchi, as present in adults, is established early in lung development. At the next stage of development, the right main lung bud forms 3 further lung buds and the left side only 2, corresponding to the later pulmonary lobes. The narrow diameter and long length of the middle lobar bronchus, combined with an acute take-off angle, create poor conditions for drainage (3,5,8,16). Furthermore, the deep fissures of both the middle lobe and lingula, with only scanty parenchymal bridges, provide barriers to collateral ventilation (5). This can contribute to poor clearance of mucus with peripheral plugging of the small airways (16). The poor collateral ventilation of the middle lobe, especially in patients with complete fissures, and relative anatomical isolation reduce the chance of reinflation once atelectasis has occurred (2,17). Reich and Johnson (18) hypothesized that women were more likely to regard expectoration as socially unacceptable behavior and so they may habitually suppress a voluntary cough, which can lead to an inability to clear secretions (especially from the middle lobe and lingula), resulting in a chronic nidus for inflammation that favors subsequent infection. Pomerantz et al. (19) described most of their patients as slender women with skeletal abnormalities (e.g., pectus excavatum, mild scoliosis, straight back syndrome, mitral valve prolapse). It has also been associated with the continuous use of antitussive drugs.

Tuberculous Lymphadenitis of Hilar Lymph Nodes as a Cause of Right Middle Lobe Syndrome: A Case Report | Patil et al.

MLS is more common in females; the ratio of females to males ranges from 1.5 to 3 in most studies (3,4,6,8). The most common symptoms are chronic or recurrent cough, observed in 30% to 50% of patients (3,7); dyspnea; chest pain; audible wheezing; and fever and chills related to obstructive pneumonia (3,7,15,20). These symptoms are often intermittent and recurrent. There is frequently a history of multiple treatments for recurrent pneumonia or asthma where antibiotics, mucolytics, and bronchodilators have been used (6). Hemoptysis, weight loss, fatigue, and low-grade fever are symptoms that may indicate complications related to suppurative infections (2,4). A physical examination may be completely normal, but loss of breath sounds and vocal fremitus in the right middle lobe may also be apparent (2,6). Chest radiographs may be normal in patients with intermittent or recurrent collapse (6). Most often, however, patients have abnormalities on the chest radiograph that are most apparent in the lateral view (5). The volume loss resulting from collapse of the right middle lobe is seen as a triangle of increased density between the minor fissure and the lower half of the major fissure (5,20). Its apex is at the hilum, and the base is peripherally located toward the pleura. With long-standing collapse, this radiographic density may have a width of only 2 to 3 mm (5). The PA radiograph may also show obfuscation of the right cardiac border (silhouette sign) (5,20). PA chest radiographs of RML atelectasis have been classified into 4 types (21). In Type 1, no abnormalities were detected, or only slight obliteration of the right cardiac border was seen. In Type 2, a triangular opacity was seen with its base on the right cardiac border. In Type 3, a band-like opacity was seen along the right cardiac border. In Type 4, there was a small, vague opacity near the right cardiac border. The visualization of minor fissure and right cardiac border was not taken into consideration in this classification (21). Flexible bronchoscopy (FB) is important in the evaluation of patients with MLS, especially regarding the patency of the right middle lobe bronchus (7,12). In addition to excluding malignancy or other surgically correctable lesions, FB allows for the collection of specimens for diagnosis of infectious causes (22). Studies have shown that bronchoscopy findings are abnormal in up to 40% of cases (7,12). Stenosis of the middle lobe bronchus and ostial tumors is the most commonly encountered abnormality (23). Kim et al. (13) observed that edematous-type endobronchial tuberculosis (EBTB) was much more common than

actively caseating-type EBTB presenting as RMLS. Since the RML bronchus has a narrowed orifice, even a mild edematous change can lead to obstruction of the bronchial lumen. Due to the anatomical character of the right middle lobe, edematous-type EBTB is the most common type of EBTB presenting as RMLS. Most cases of MLS receive treatment that is directed at the underlying cause, and this is true for both the obstructive and non-obstructive form. Patients with nonobstructive MLS usually respond to medical therapy with bronchodilators, mucolytic agents, and antibiotics (15). Antibiotic treatment is usually recommended to attempt to eradicate an infection that can be associated with bronchiectasis (8). However, the role of antibiotic therapy in the treatment of MLS is not well studied (3). Low-dose macrolide therapy can be used, particularly if bronchiectasis is present. Consideration must also be given to unusual infections (atypical mycobacteria, fungi) that may require specific antimicrobial agents (12,13). Systemic corticosteroid administration with ATT has been shown to more rapidly reduce TB symptoms, but its effect in preventing bronchial stenosis in EBTB remains doubtful (24). A prospective study with 34 EBTB patients revealed that systemic steroid treatment had no significant effect on preventing bronchial stenosis (25). There are no data from randomized, controlled studies to determine the proportion of patients that require surgery because conservative treatment has failed. In addition, the role of FB and antibiotics in the management of MLS is constantly evolving. Surgical removal of the middle lobe is reserved for resistant and complex cases of MLS, usually for patients with isolated MLS who do not respond to medical therapy and who have proven obstruction of the middle lobe bronchus (8). In this case report, near total resolution of right middle lobe consolidation and collapse was observed following medical treatment consisting of ATT administered for 8 months and systemic corticosteroids for 4 weeks.

CONCLUSION Although TB is an endemic disease in India, TB of the hilar lymph nodes causing RMLS can be missed due to a variable clinical and radiological presentation. Delay in diagnosis, due to a lack of expertise in chest radiology or clinical suspicion is the most common reason for a poor outcome in TB. Typical triangular opacity of right middle lobe collapse on a chest radiograph mandates bronchoscopy to look for an endobronchial, mucosal, or peribronchial cause of RMLS. Bronchoscopy is less invasive, and is www.respircase.com

Respiratory Case Reports

a rapid and sensitive technique to diagnose RMLS. Although role of bronchoscopy in sputum-negative pulmonary TB is well documented, it is still underutilized in India. It can be used for the diagnosis, treatment, and in the assessment of response to treatment, especially in cases of RMLS. GeneXpert is a rapid, sensitive, and specific assay, not just for the detection of MTB, but also for RIF resistance. It is an alternative to conventional tests that is reliable and can be performed on all bronchoscopic samples, such as BAL and TBNA specimens. GeneXpert should be used in sputum-negative pulmonary TB cases when conventional diagnostic tests are less sensitive and more timeconsuming.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - S.P., M.M.; Planning and Design - S.P., M.M.; Supervision - S.P., M.M.; Funding - S.P.; Materials - S.P.; Data Collection and/or Processing - S.P.; Analysis and/or Interpretation - S.P.; Literature Review - S.P.; Writing S.P.; Critical Review - S.P.

YAZAR KATKILARI Fikir - S.P., M.M.; Tasarım ve Dizayn - S.P., M.M.; Denetleme - S.P., M.M.; Kaynaklar - S.P.; Malzemeler - S.P.; Veri Toplama ve/veya İşleme - S.P.; Analiz ve/veya Yorum - S.P.; Literatür Taraması - S.P.; Yazıyı Yazan - S.P.; Eleştirel İnceleme - S.P.

REFERENCES 1.

Graham EA, Burford TH, Mayer JH. Middle lobe syndrome. Post Grad Med 1948; 4:29-34. [CrossRef]

Effler DB, Ervin JR. The middle lobe syndrome; a review of the anatomic and clinical features. Am Rev Tuberc 1955; 71:775–84.

Bertelsen S, Struve-Christensen E, Aasted A, Sparup J. Isolated middle lobe atelectasis: aetiology, pathogenesis, and treatment of the so-called middle lobe syndrome. Thorax 1980; 35: 449–52. [CrossRef]

Culiner MM. The right middle lobe syndrome, a nonobstructive complex. Dis Chest 1966; 50:57-66. [CrossRef]

Ayed AK. Resection of the right middle lobe and lingula in children for middle lobe/lingula syndrome. Chest 2004; 125:38-42. [CrossRef]

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Fraser R, Pare J, Pare P, Frazer R, Generoux G: Roentgenologic signs in the diagnosis of chest disease; in Fraser RG, et al (eds): Diagnosis of Diseases of the Chest. Philadelphia, Saunders, 1988.

Dees SC, Spock A. Right middle lobe syndrome in children. JAMA 1966; 197:8–14. [CrossRef]

Einarsson JT, Einarsson JG, Isaksson H, Gudbjartsson T, Gudmundsson G. Middle lobe syndrome: a nationwide study on clinicopathological features and surgical treatment. Clin Respir J 2009; 3:77–81. [CrossRef]

Iwata M, Ida M, Takeuchi E, Nakamura Y, Horiguchi T, Sato A. Middle lobe syndrome: incidence and relationship to atypical mycobacterial pulmonary disease. Nihon Kyobu Shikkan Gakkai Zasshi 1996; 34:57-62.

10. Wagner RB, Johnston MR. Middle lobe syndrome. Annal Thorac Surg 1983; 35:679-86. [CrossRef] 11. Bradham RR, Sealy WC, Young WG Jr. Chronic middle lobe infection. Factors responsible for its development. Ann Thorac Surg 1966; 2:612–6. [CrossRef] 12. Kala J, Sahay S, Shah A: Bronchial anthracofibrosis and tuberculosis presenting as a middle lobe syndrome. Prim Care Respir J 2008; 17:51–5. [CrossRef] 13. Kim HC, Kim HS, Lee SJ, Jeong YY, Jeon KN, Lee JD, et al: Endobronchial tuberculosis presenting as right middle lobe syndrome: clinical characteristics and bronchoscopic findings in 22 cases. Yonsei Med J 2008; 49: 615–9. [CrossRef] 14. Oshima M, Maeda H, Furonaka O, Doi M, Nishizaka T, Kuwabara M. Sarcoidosis with multiple organ involvement emerging as Lofgren’s syndrome. Intern Med 2003; 42: 534–7. 15. Gudmundsson G, Gross TJ. Middle lobe syndrome. Am Fam Physician 1996; 53:2547-50. 16. Berrocal T, Madrid C, Novo S, Gutierrez J, Arjonilla A, Gomez-Leon N. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics 2004; 24:e17. [CrossRef] 17. Inners CR, Terry PB, Traystman RJ, Menkes HA. Collateral ventilation and the middle lobe syndrome. Am Rev Respir Dis 1978; 118:305–10. 18. Reich JM, Johnson RE. Mycobacterium avium complex pulmonary infection presenting as isolated lingular or middle lobe pattern. The Lady Windermere syndrome. Chest 1992; 101:1605-9. 19. Pomerantz M, Denton JR, Huitt GA, Brown JM, Powell LA, Iseman MD. Resection of the right middle lobe and lingula for mycobacterial infection. Ann Thorac Surg 1996; 6:990-3. [CrossRef]

Tuberculous Lymphadenitis of Hilar Lymph Nodes as a Cause of Right Middle Lobe Syndrome: A Case Report | Patil et al.

20. McElvein RB, Mayo P. Middle lobe disease. South Med J 1967; 60:1029–32. [CrossRef] 21. Miyazaki A, Ashizawa K, Mori M, Ohtsubo M. Right Middle Lobe Atelectasis: Chest Radiographic and CT Appearances Correlating with the Clinical Features. Acta Med. Nagasaki 2003; 48:159-66. 22. Rock MJ. The diagnostic utility of bronchoalveolar lavage in immunocompetent children with unexplained infiltrates on chest radiograph. Pediatrics 1995; 95:373–7.

23. Erelel M, Yakar F, Yakar A. Endobronchial tuberculosis with lobar obstruction successfully treated by argon plasma coagulation. South Med J 2009; 102:1078–81. [CrossRef] 24. Cisneros JR, Murray KM. Corticosteroids in tuberculosis. Ann Pharmacother 1996; 30:1298-303. [CrossRef] 25. Park IW, Choi BW, Hue SH. Prospective study of corticosteroid as an adjunct in the treatment of endobronchial tuberculosis in adults. Respirology 1997; 2:275-81. [CrossRef]

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Respir Case Rep 2018;7(2):97-100 DOI: 10.5505/respircase.2018.83788

OLGU SUNUMU

CASE REPORT

Stent Migrasyonuna Sekonder Gelişen Trakea-Özofageal Fistül Olgusu Trachea-Esophageal Fistula Case Occuring in the Stent Migration

RESPIRATORY CASE REPORTS

Mehmet Erdem Çakmak1, Hayriye Cankar Dal1, İbrahim Mungan1, Serdar Yamanyar1, Derya Ademoğlu1, Büşra Tezcan2, Dilek Kazancı2, Sema Turan1

Özet

Abstract

Trakea-özofageal fistül, özofagus ve trakea arasında bağlantı olmasıdır. Konjenital veya edinsel olarak oluşabilirler. Erişkinlerde çoğunlukla edinsel şekli görülür. Trakea-özofageal fistül hayatı tehdit eden bir durum olup uygun yöntemle tedavi edilmesi gerekir. Bu olgu sunumunda Ivor Lewis ameliyatı nedeniyle takip edilen hastada özofagusa stent uygulanması sonrası stent migrasyonuna bağlı gelişen trakeözofageal fistül olgusu sunulmuştur.

A tracheal-esophageal fistula (TEF) is a connection between the esophagus and the trachea. It may be congenital or acquired. TEF seen in adults is usually acquired. TEF is a life-threatening condition, and must be treated appropriately. This case report is a description of a case of TEF due to stent migration occurring after an Ivor Lewis operation and esophageal stent implantation.

Anahtar Sözcükler: Trakea-özofageal fistül, stent, migrasyon.

Trakea-özofageal fistül (TÖF), özofagus ve trakea arasında bağlantı olmasıdır. Konjenital veya edinsel olarak oluşabilirler. Erişkinlerde çoğunlukla edinsel TÖF görülür. Özofagus kanseri, akciğer kanseri, lenfoma veya bu hastalıkların tedavisi için uygulanan radyoterapi, penetran boyun ve göğüs travmaları, özofagus rezeksiyonu, larenjektomi, özofagus dilatasyonu ve inhalasyon yaralanmaları için uygulanan entübasyon, özofagusa stent yerleştirilmesi, granülomatöz mediastinal enfeksiyonlar, AIDS, yabancı cisim, servikal operasyonlar ve Zenker divertikülü, edinsel TÖF patolojisinin gelişiminde rol alan etkenlerdir (1).

Türkiye Yüksek İhtisas Hastanesi, Yoğun Bakım Anabilim Dalı, Ankara 2 Türkiye Yüksek İhtisas Hastanesi, Anesteziyoloji ve Reanimasyon Anabilim Dalı, Ankara

Key words: Trachea-esophageal fistula, stent, migration.

Özofajektomi, özellikle rezeksiyonu mümkün olan özofagus kanserlerinde standart tedavi yöntemidir. Cerrahi rezeksiyonlar arasında transhiatal özafajektomi ve Ivor Lewis özofajektomi gibi cerrahi yöntemler yer almaktadır. Hastamızda gerçekleştirilen Ivor Lewis özofajektomi 2 basamaklı bir cerrahi işlem olup laparatomi ile gerçekleştirilen mide serbestleştirilmesi sonrasında torakotomi ile yapılan özofajektomi ve özofagogastrik anastomozu içerir. Özofagus karsinomunda, yüksek dereceli displazide, kostik özofageal yaralanmalarda kullanılabilir (2). Ivor Lewis özofajektomi sonrası postoperatif dönemde nadir de olsa TÖF görülebilir (3).

Department of Critical Care, Türkiye Yüksek İhtisas Hospital, Ankara, Turkey 2 Department of Anaesthesiology and Reanimation, Türkiye Yüksek İhtisas Hospital, Ankara, Turkey

Başvuru tarihi (Submitted): 04.11.2017 Kabul tarihi (Accepted): 05.02.2018 İletişim (Correspondence): Mehmet Erdem Çakmak, Türkiye Yüksek İhtisas Hastanesi, Yoğun Bakım Anabilim Dalı, Ankara e-mail: erdem.cakmak@deu.edu.tr

Respiratory Case Reports

Bu olgu sunumunda Ivor Lewis özofajektomi sonrası anastomoz kaçağı gelişen ve özofagusa stent uygulanması sonrası stent migrasyonuna bağlı gelişen trakeaözofageal fistül olgusu sunulmuştur.

OLGU Altmış bir yaşında erkek hasta, özofagus karsinomu tanısıyla Ivor Lewis özofajektomi ve özofagogastrik anastomoz sonrası hastada postoperatif dokuzuncu günde anastomoz kaçağı gelişmiş. Postoperatif onuncu günde, hastanın özofagusuna tam kaplı metalik stent yerleştirilmiş. Hastada postoperatif takiplerinde dördüncü ayda öksürük, göğüs ağrısı, nefes darlığı gelişmesi üzerine hipoksemik solunum yetmezliği ön tanısıyla cerrahi yoğun bakımda izleme alındı. Yoğun bakım yatışında kalp atım hızı 115/dk, kan basıncı 130/75 mmHg, solunum sayısı 26/dk, ateşi 38,2 °C saptandı. Solunum sistemi fizik muayenesinde hastanın her iki akciğerinde ralleri vardı. Yoğun bakım yatışında APACHE II skoru 16 saptandı. Tam kan sayımında beyaz küre: 13.700/mm3, CRP düzeyi 85 mg/L saptandı. Koagülasyon parametreleri, renal ve hepatik fonksiyon testleri normaldi. Hastanın çekilen akciğer grafisinde sağ diyaframda düzleşme ve sağ kostofrenik sinüste kapalılık izlendi (Şekil 1). Hastaya çekilen toraks bilgisayarlı tomografide trakea alt ucu ve özofagus arasında yaklaşık bir cm ilişki (Şekil 2) ve her iki akciğer alt loblarda buzlu cam alanları ve konsolide alanlar izlendi. Hastaya aspirasyon pnömonisi tanısıyla piperasilin tazobaktam başlandı. Oral alımı kesilerek parenteral beslenmeye başlandı. Hastaya özofagogastroskopi yapıldı. Özofagusa daha önce konulmuş olan stentin proksimal ucunun migrasyona bağlı trakeaya penetre olduğu ve fistül oluşturduğu görüldü. Bu stent çıkarılarak özofagotrakeal fistül hattını geçecek şekilde tekrar tam kaplı metalik özofagus stenti yerleştirildi. Bu stentin distal migrasyonunu önlemek için iki adet klips ile özofagusa tutturuldu. Takiplerinde solunum yetmezliği ve pnömonisi geriledi. Kontrol endoskopisinde stentin yerinde olduğu gözlendi ve fistül saptanmadı (Şekil 3). Hasta şifa ile taburcu edildi. Son yerleştirilen stent sonrası bir yıldır takipte olan hastada komplikasyon saptanmadı.

Şekil 1: Akciğer grafisinde sağ diyaframda düzleşme ve sağ kostofrenik sinüste kapanma.

Şekil 2: Toraks bilgisayarlı tomografide trakea alt ucu ve özofagus arasındaki fistül.

Şekil 3: Tam kaplı özofagus stenti.

Cilt - Vol. 7 Sayı - No. 2

Stent Migrasyonuna Sekonder Gelişen Trakea-Özofageal Fistül Olgusu | Çakmak et al.

TARTIŞMA Trakea-özofageal fistülün en mortal komplikasyonu sekresyonların respiratuar sisteme geçmesi olup bu hastalarda ölümün asıl sebebi fistülün yol açtığı kronik aspirasyon sonucu oluşan pulmoner enfeksiyonlardır. Bu olgularda oral alım yetersiz olup kilo kaybı ve su içerken öksürük atakları olmaktadır (4). TÖF tanısı ve hastalık seyrinin izlenmesi için standart arka-ön akciğer grafisi, bilgisayarlı tomografi, özofagoskopi ve bronkoskopi gereklidir. Direkt grafide mediastinal amfizem, pnömonik infiltrasyon, plevral effüzyon ve bronşiyal invazyona bağlı atelektazi görülebilir. Tomografide fistül lokalizasyonu, tümörün büyüklüğü ve çevre yapılarla ilişkisi değerlendirilebilir. Tomografik olarak özofagus ile trakea arasında anomali tespit edildiğinde özofagoskopi veya bronkoskopiyle değerlendirme yapılmalıdır. Özofagoskopi ve bronkoskopi, fistülün tanısında ve tedavisinde yol gösterici olmaktadır (5). Olgumuzda özofagoskopi hastanın tanısında ve tedavisinde yol gösterici olmuştur. TÖF’ün spontan olarak kapanması nadirdir ve cerrahi olarak kapatılmaları gerekebilir. TÖF küçük ise primer tamir edilir, geniş TÖF’de ise trakeal rezeksiyon gerekebilir ancak opere edilemeyen hastalarda endoskopik olarak takılabilen stentler tercih edilebilir. Bu sayede pulmoner aspirasyonun önüne geçilebilir. Ayrıca uygun benign trakea-özofageal fistüllerde özofagusa stent uygulaması yapılabilir (6,7). TÖF kapatılmasında günümüzün en uygun yaklaşımının stent yerleştirilmesi olmasına rağmen, stent yerleştirilmesinin aynı zamanda TÖF’de etiyolojik bir faktör olduğu akılda tutulmalıdır. Metal stent özofagus içinde normal mukozaya basınç uygulayarak nekroz oluşturabilir. Stent normal mukoza içinde kronik inflamatuvar yanıt oluşturur. Bazen stent içi epitelize olur, bazen de düz yüzey oluşturan kollajenöz bir materyalle kaplanır. Zamanla stent müskülaris propriyaya hatta serozaya ulaşarak fistül oluşumuna zemin hazırlayabilir. Stentin basısına bağlı gelişen özofagus nekrozuna veya stentin migrasyonuna sekonder gelişen TÖF’ün kapatılmasında da yine en etkili yöntem, yeni stent yerleştirilmesi olabilmektedir (5). TÖF’de stent yerleştirilmesi sonrasında yeniden fistül oranı % 0-35 arasında bildirilmiştir. Bu durumda en başarılı sonuç ikinci bir stent yerleştirilmesi ile elde

edilebilmektedir (8,9). Metalik özofagus stentleri plastik stentlere göre daha pahalı olmasına rağmen daha az sıklıkta komplikasyonlara yol açmaktadır. Bu nedenle daha ekonomik bulunmuşlardır (10). Bu tedaviler yetersiz kalırsa ve hastanın durumu uygunsa tecrübeli ellerde cerrahi bypass alternatif olarak önerilmiştir (11). TÖF nadir ama mortaliteye yol açan ciddi bir klinik tablodur. Endoskopik özofageal veya trakeal stent yerleştirilmesi TÖF için iyi bir tedavi seçeneği olarak görülmektedir. Bu olgu sunumunda özofageal stent migrasyonuna bağlı olarak gelişen ve başarılı bir şekilde tedavi edilen trakeaözofageal fistüllü bir olgu ele alınmıştır.

ÇIKAR ÇATIŞMASI Bu makalede herhangi bir çıkar çatışması bildirilmemiştir.

YAZAR KATKILARI Fikir - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Tasarım ve Dizayn - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Denetleme - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Kaynaklar - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Malzemeler - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Veri Toplama ve/veya İşleme M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Analiz ve/veya Yorum - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Literatür Taraması - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Yazıyı Yazan - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.; Eleştirel İnceleme - M.E.Ç., H.C.D., İ.M., S.Y., D.A., B.T., D.K., S.T.

KAYNAKLAR 1.

Grillo HC, Moncure AC, McEnany MT. Repair of inflamatory tracheoesophageal fistula. Ann Thorac Surg 1976; 22:112-9.

Allen MS. Transthoracic resection of the esophagus. In Shields TW, LoCicero J, Reed CE, ed. General Thoracic Surgery, vol 2, 7th ed. Philadelphia: Lippincott Williams and Wilkins; 2009:1752-9.

Buskens CJ, Hulscher JB, Fockens P, Obertop H, van Lanschot JJ. Benign tracheo-neo-esophageal fistulas after subtotal esophagectomy. Ann Thorac Surg 2001; 72:221-4. [CrossRef]

Eroğlu A, Türkyılmaz A, Aydın Y. Özofagus kanserli olgularda fistül ve tedavi algoritması. J Clin Analytic Med 2010: 1-5.

Turkyilmaz A, Aydin Y, Eroglu A, Bilen Y, Karaoglanoglu N. Palliative management of esophagorespiratory fistula www.respircase.com

Respiratory Case Reports

in esophageal malignancy. Surg Laparosc Endosc Percutan Tech. 2009; 19: 364-7. [CrossRef]

Homann N, Noftz MR, Klingenberg-Noftz RD, Ludwig D. Delayed complications after placement of self-expanding stents in malignant esophageal obstruction: treatment strategies and survival rate. Dig Dis Sci 2008; 53:334– 40. [CrossRef]

Couraud L, Ballester MJ, Delaisement C. Acquired tracheoesophageal fistula and its management. Semin Thorac Cardiovasc Surg 1996; 8:392-9.

Yakut M, Çınar K, İdilman R. Malign özofagus darlığı olgusunda multipl stent uygulaması. Endoskopi dergisi 2011; 19:18-9.

10. Giral A. Kalaycı C. Özofagusun kanser dışı hastalıklarında cerrahi tedavi. In: Yüksel M, Ed. Özofagus Hastalıklarının Tıbbi ve Cerrahi Tedavisi, İstanbul;Nobel Tıp; 2002, 309-20.

Wang MQ, Sze DY, Wang ZP, Wang ZQ, Gao YA, Dake MD. Delayed complications after esophageal stent placement for treatment of malignant esophageal obstructions and esophagorespiratory fistulas. J Vasc Interv Radiol 2001; 12:465–74. [CrossRef]

11. Low DE, Kozarek RA. Comparison of conventional and wire mesh expandable prostheses and surgical bypass in patients with malignant esophagorespiratory fistulas. Ann Thorac Surg. 1998; 65:919–23. [CrossRef]

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Respir Case Rep 2018;7(2):101-105 DOI: 10.5505/respircase.2018.05025

OLGU SUNUMU

CASE REPORT

Pneumomediastinum after Endobronchial Ultrasound–Guided Transbronchial Needle Aspiration: A Case Report Endobronşial Ultrason Rehberliğinde Transbronşial İğne Aspirasyonu Sonrası Gelişen Pnömomediasten: Olgu Sunumu

RESPIRATORY CASE REPORTS

Ali Kadri Çırak, Sami Deniz, Dursun Alizoroğlu

Abstract

Özet

Pneumomediastinum is defined as an abnormal accumulation of air within the mediastinum. It occurs most often when increased alveolar pressure leads to alveolar rupture, and less frequently when there is perforation of the tracheobronchial tree. Pressure gradients then allow air to spread via the fascia to the surrounding soft tissues, mediastinum, and/or retroperitoneum. This is a report of a case of pneumomediastinum following endobronchial ultrasound– guided fine needle aspiration (EBUS/TBNA) of the mediastinal lymph nodes, widely regarded as a safe procedure.

Pnömomediasten mediasten içine anormal şekilde havanın birikmesi olarak tanımlanmaktadır. Sıklıkla alveoler basıncın artışı sonucu alveol rüptürü olduğunda ve daha az olarak da trakeobronşial ağacın perforasyonu sonucunda meydana gelmektedir. Basınç farklığı nedeniyle hava, yumuşak dokuları saran fasya boyunca mediasten ve/veya retroperitonal bölgeye ulaşır. Genelde güvenli bir işlem olarak bilinen endobronşial ultrason eşliğinde yapılan mediastinal lenf bezi iğne aspirasyonu sonrası pnömomediasten gelişen olguyu sunuyoruz.

Key words: EBUS/TBNA, complication, pneumomediastinum.

Mediastinal lymph node sampling is a critical step in the staging of lung cancer and in the diagnosis of inflammatory conditions, such as sarcoidosis (1,2). Over the past decade, endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has emerged as a minimally invasive, highly accurate technique for sampling intrathoracic lymph nodes, with a sensitivity of 88% to 93% in distinguishing lymph node metastases from benign conditions (3) It uses an ultrasonic bronchofiberscope with a convex probe (CP-EBUS; XBF-

Health Sciences University İzmir Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital, İzmir, Turkey

Anahtar Sözcükler: EBUS/TBNA, komplikasyon, pneumomediastinum.

UC160F-OL8/BC-UC260F-OL8; Olympus Medical Systems, Tokyo, Japan) that allows for real-time needle aspiration of mediastinal and hilar lymph nodes guided by ultrasound images. EBUS-TBNA was given an EU CE marking in July 2004 and US Food and Drug Administration approval in March 2006 (4). Complications specifically related to EBUS-TBNA are pneumothorax and bleeding (5,6). Complications can be avoided through proper knowledge of the mediastinal anatomy and with bronchoscopy experience (7).

Sağlık Bilimleri Üniversitesi İzmir Dr. Suat Seren Göğüs Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi, İzmir

Submitted (Başvuru tarihi): 02.08.2017 Accepted (Kabul tarihi): 04.10.2017 Correspondence (İletişim): Ali Kadri Çırak, Health Sciences University İzmir Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital, İzmir, Turkey e-mail: alikadri.cirak@saglik.gov.tr

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Respiratory Case Reports

CASE A 68-year-old woman, with a 30-pack-year history of tobacco use, was admitted with a dry cough. Computed tomography (CT) and positron emission tomography scanning of the lungs revealed a left upper lobe nodule (1.3 cm long axis diameter; SUVmax 4.0) and bilateral paratracheal, subcarinal lymph nodes (short axis <1 cm; SUVmax similar to mediastinal vascular tissue) (Figures 13). Bronchoscopy was performed with endobronchial ultrasound-guided fine needle aspiration (EBUS-TBNA) of nodal stations 4R and 7, using a 22-gauge needle. EBUS-TBNA is performed in the bronchoscopy unit of the hospital as an outpatient procedure using convex-probe bronchoscopes (Olympus 7.5 MHz, BF-UC160F and BFUC160F-OL8; Olympus Optical, Tokyo, Japan) with an EU C2000 processor (Olympus Optical, Tokyo, Japan). The scope was inserted through the oral route, in the supine position and under local anesthesia with lidocaine and conscious sedation with intravenous midazolam. Since severe coughing continued during the procedure, samples could only be taken once from lymph node stations 4R and 7.

hours of close monitoring, the crepitus and radiological findings markedly improved. The cytopathology of lymph node stations 4R and 7 was benign. The patient declined to pursue advanced diagnostic procedures.

Figure 2: A pre-procedure CT sacan of the chest

Figure 3: A post-procedure chest x-ray

Figure 1: Pre-procedure chest x-ray

An hour after the procedure the patient complained of chest pain and swelling of the neck and chest wall. A physical examination detected tachycardia and crepitus that extended from the sternum to the neck. A postprocedure chest X-ray and CT scan of the chest revealed extensive pneumomediastinum and subcutaneous emphysema (Figure 4 and 5). She was hemodynamically stable. Thoracic surgery was performed and a small incision was made to relieve the subcutaneous emphysema. After 48 Cilt - Vol. 7 SayÄą - No. 2

Figure 4: A pre-procedure PET-CT scan of the chest

DISCUSSION Pneumomediastinum is defined as an abnormal accumulation of air within the mediastinum (8). It was first de-

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scribed by Hamman in 1939 (9). There are 3 reasons pneumomediastinum may occur: soft tissue infection, mucosal disruption, and spontaneous pneumomediastinum (10). While any one or combination of these mechanisms can lead to an abnormal accumulation of air in the mediastinum, most cases of intraprocedural iatrogenic pneumomediastinum are caused by the Macklin effect (The proposed mechanism is alveolar rupture with air dissecting along the peribronchovascular interstitial sheaths, interlobular septa, visceral pleura, and into the mediastinum) (11) or ventilation-induced barotrauma that causes mucosal disruption and spreading of air to nearby tissues via the fascial planes that connect them (12). Another potential cause of pneumomediastinum following bronchoscopy is blunt airway trauma from the bronchoscope. Additionally, forceful coughing can lead to tracheal microperforation, which can cause pneumomediastinum. Adequate sedation is an important part of EBUSTBNA, since it provides patient comfort and potentially increases the diagnostic yield. Two common types of sedation used in EBUS-TBNA: (1) Deep sedation (DS) or general anesthesia, and (2) moderate sedation (MS) or conscious sedation. DS using propofol has an acceptable safety profile in endoscopic procedures. MS is mostly preferred for procedures that require rapid recovery. A combination of fentanyl and midazolam is commonly used in endoscopic procedures for sedation. Local anesthesia of the airways is achieved by nebulizing 1% or 2% lidocaine and spraying lidocaine in the posterior pharynx (13).

Figure 5: A post procedure CT scan of the chest

The cause of the pneumomediastinum in the present patient was likely a tracheal microperforation due to forceful coughing. Even though nearly all patients are administered adequate anesthesia (midazolam and propofol), because of some contraindications, this patient

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was just given midazolam. As a result, the patient’s cough reflex continued during the operation.

Figure 6: Two days after procedure chest x-ray

Often accompanied by chest pain, an acute onset of dyspnea, and swelling of the neck, chest wall, and face, pneumomediastinum can be diagnosed easily on chest Xray films. Radiological features of pneumomediastinum include visualization of subcutaneous emphysema and free mediastinal air as hyperlucent lines enhancing the mediastinal viscera and outlining the lateral heart borders along with superior mediastinal widening. Other signs include a continuous diaphragm sign, a continuous left hemidiaphragm sign, a ring-around-the-artery sign (air surrounding the extrapericardial segment of the right main pulmonary artery and appearing as a lucent ring around the right pulmonary artery), a thymic/spinnaker sail sign (outlining of the thymus due to large pneumomediastinum elevating the thymic lobes), a V sign (confluence of innominate veins outlined in a frontal view), a Naclerio's V sign (air outlining the lateral border of the descending aorta and extending laterally between the parietal pleura and the medial left hemidiaphragm), pneumopericardium, air in the pulmonary ligament, a tubular artery sign, a double bronchial wall sign, and an extrapleural sign. A lateral chest X-ray is also helpful in detecting increased retrosternal air, and increases the rate of detection of pneumomediastinum. Vertical translucent air streaks around the aorta and pulmonary artery are also diagnostic of pneumomediastinum (8,14). Our patient had trouble with chest pain and swelling of the neck and chest wall. The radiological features of our patient included the visualization of subcutaneous emphywww.respircase.com

Respiratory Case Reports

sema, free mediastinal air as hyperlucent lines highlighting the mediastinal viscera and outlining the lateral heart borders, along with superior mediastinal widening. Over the last 2 decades, EBUS has emerged as a highly effective and minimally invasive technique for sampling peribronchial, mediastinal, and lung masses for pathological examination. Although initial studies of EBUSTBNA focused on evaluating diagnostic performance, an impressively low complication rate has also been reported (5) (Table 1). Table 1: Complications following endobronchial ultrasound-guided transbronchial needle aspiration Outcome Complication rate, % (95% confidence interval) Any complication within 24 hours

1.44 (0.87-2.24)

Bleeding requiring intervention

0.2 (0.05-0.7)

Pneumothorax

0.53 (0.21-1.1)

Clinically significant airway injury

0.1 (0.002-0.4)

Sustained hypoxia

0.3 (0.08-0.8)

Hypotension

0.1 (0.002-0.4)

Respiratory failure within 24 hours

0.23 (0.05-0.7)

EBUS-TBNA was performed 11,753 times (3.76/case) at 6115 lymph node stations and lesions (1.92/station or lesion) by Çağlayan at al. (15) Five instances of a serious complication were recorded (0.16%): fever lasting >24 hours, infection of bronchogenic cyst, mediastinal abscess, pericarditis, and pneumomediastinitis with empyema. In another study, EBUS-TBNA was performed using a convex probe in 7345 cases. Complications (except pneumomediastinum) were observed in 90 cases (1.23%; 95% confidence interval: 0.97%-1.48%) in 32 facilities (15.2%). Lymph node station 7 was the most frequently sampled, in 34 patients (43.0%), followed by 4R, in 24 patients (30.4%). The number of samples taken in any lymph node was 1 in 24 cases (57.1%), 2 in 14 cases (33.3%), 3 in 1 case (2.4%), and 4 in 3 cases (7.1%). A 22-gauge needle was used in 30 cases (85.7%) and a 21-gauge needle was used in 5 cases (14.3%) with hemorrhage. There was no information about sampling in 7 cases (16). In our patient, we took samples from lymph node stations 4R and 7 once while using a 22-gauge needle. Procedural training has long been an important component in the specialty of pulmonary medicine. The American Thoracic Society/European Respiratory Society and CHEST guidelines based on the number of procedures Cilt - Vol. 7 Sayı - No. 2

have not been proven adequate to determine competence (40 procedures for EBUS-TBNA). Ideal outcomes might include diagnostic yield, complication rates, and patient tolerance. Some training programs have been moving toward incorporating simulation training and web-based virtual bronchoscopy training. In summary, no validated methods of determining competency exist at this time (17). Before this patient, we had a total of 250 bronchoscopy patients. The management of pneumomediastinum depends on the clinical severity and the underlying etiology. Spontaneous pneumomediastinum has a benign course, resolving on its own. The management of this condition is usually supportive, with reassurance, observation, treatment of underlying condition, and oxygen therapy. In rare cases, surgery may be required to correct pneumomediastinum when caused by severe tracheobronchial disruption. However, occasional deaths from splinting of great vessels and the trachea by mediastinal emphysema have also been reported, demanding careful vigilance of this condition (10). The lack of pneumothorax in our patient, which is often a secondary complication of pneumomediastinum, combined with the lack of any identifiable lesion and hemodynamic stability, allowed for conservative management. In conclusion, we reported a case of iatrogenic pneumomediastinum during EBUS-TBNA. Despite being rare, this is a complication that can occur and must be recognized promptly to avoid delay in treatment if clinically indicated. In addition to a highlighting a rare complication of EBUSTBNA, we also wanted to emphasize the importance of anesthesia.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - A.K.Ç., S.D., D.A.; Planning and Design A.K.Ç., S.D., D.A.; Supervision - A.K.Ç., S.D., D.A.; Funding - A.K.Ç., S.D.; Materials - S.D.; Data Collection and/or Processing - A.K.Ç., S.D., D.A.; Analysis and/or Interpretation - S.D.; Literature Review - D.A.; Writing A.K.Ç.; Critical Review - S.D., D.A.

YAZAR KATKILARI Fikir - A.K.Ç., S.D., D.A.; Tasarım ve Dizayn - A.K.Ç., S.D., D.A.; Denetleme - A.K.Ç., S.D., D.A.; Kaynaklar A.K.Ç., S.D.; Malzemeler - S.D.; Veri Toplama ve/veya İşleme - A.K.Ç., S.D., D.A.; Analiz ve/veya Yorum - S.D.;

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Pneumomediastinum after Endobronchial Ultrasound–Guided Transbronchial Needle Aspiration: A Case Report | Çırak et al.

Literatür Taraması - D.A.; Yazıyı Yazan - A.K.Ç.; Eleştirel İnceleme - S.D., D.A.

Bejvan SM, Godwin JD. Pneumomediastinum: old signs and new signs. AJR Am J Roentgenol 1996; 166:1041–8. [CrossRef]

REFERENCES

Hamman L. Spontaneous mediastinal emphysema. Bull Johns Hopkins Hosp 1939; 64;1-21.

Detterbeck FC, Jantz MA, Wallace M, Vansteenkiste J, Silvestri GA. American College of Chest Physicians. Invasive mediastinal staging of lung cancer: ACCP evidencebased clinical practice guidelines (2nd edition). Chest 2007; 132(suppl 3): 202S-20S.

Wong M, Yasufuku K, Nakajima T, Herth FJ, Sekine Y, Shibuya K, et al. Endobronchial ultrasound: new insight for the diagnosis of sarcoidosis. Eur Respir J 2007; 29:1182-6. [CrossRef]

Adams K, Shah PL, Edmonds L, Lim E. Test performance of endobronchial ultrasound and transbronchial needle aspiration biopsy for mediastinal staging in patients with lung cancer: systematic review and meta-analysis. Thorax 2009; 64:757-2. [CrossRef]

von Bartheld MB, van Breda A, Annema JT Complication rate of endosonography (endobronchial and endoscopic ultrasound): a systematic review. Respiration 2014;87:1– 9. [CrossRef]

Eapen GA, Shah AM, Lei X, Jimenez CA, Morice RC, Yarmus L, et al. Complications, consequences, and practice patterns of endobronchial ultrasound guided transbronchial needle aspiration: results of the AQuIRE registry. Chest 2013; 143:1044–53. [CrossRef]

Yasufuku K, Chiyo M, Sekine Y, Chhajed PN, Shibuya K, Iizasa T, et al. Real-time endobronchial ultrasoundguided transbronchial needle aspiration of mediastinal and hilar lymph nodes. Chest 2004; 126:122–8. [CrossRef]

Bolliger CT, Mathur PN, Beamis JF, Becker HD, Cavaliere S, Colt H, et al. ERS/ATS statement on interventional pulmonology: European Respiratory Society/ American Thoracic Society. Eur Respir J 2002; 19:356–73.

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10. Maunder RJ, Pierson DJ, Hudson LD. Subcutaneous and mediastinal emphysema: pathophysiology, diagnosis, and management. Arch Intern Med 1984; 144:1447–53. [CrossRef] 11. Macklin CC. Transport of air along sheaths of pulmonic blood vessels from alveoli to mediastinum. Arch Intern Med 1939; 64:913–26. [CrossRef] 12. Kumar A, Pontoppidan H, Falke KJ, Wilson RS, Laver MB. Pulmonary barotrauma during mechanical ventilation. Crit Care Med 1973; 1:181–6. [CrossRef] 13. Aswanetmanee P, Limsuwat C, Kabach M, Alraiyes AH, Kheir F. The role of sedation in endobronchial ultrasound-guided transbronchial needle aspiration: systematic review. Endosc Ultrasound 2016; 5:300–6. 14. Beyers JA, Melonas CF. The visible wall of a main bronchus: a new radiological sign of pneumomediastinum. Br J Radiol 1987; 60:877–9. [CrossRef] 15. Çağlayan B, Yılmaz A, Bilaçeroğlu S, Cömert SŞ, Demirci NY, Salepçi B. Complications of convex-probe endobronchial ultrasound-guided transbronchial needle aspiration: a multi-center retrospective study. Respir Care 2016; 61:243–8. [CrossRef] 16. Asano F, Aoe M, Ohsaki Y, Okada Y, Sasada S, Sato S, et al. Complications associated with endobronchial ultrasound-guided transbronchial needle aspiration: a nationwide survey by the Japan Society for Respiratory Endoscopy. Respir Res 2013, 14:50-8. [CrossRef] 17. Ernst A, Wahidi MM, Read CA, Buckley JD, AddrizzoHarris DJ, Shah PL, et al. Adult bronchoscopy training current state and suggestions for the future: CHEST Expert Panel Report. Chest 2015; 148:321-32. [CrossRef]

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Respir Case Rep 2018;7(2):106-109 DOI: 10.5505/respircase.2018.60024

OLGU SUNUMU

CASE REPORT

ACTH Salgılayan Bronşiyal Karsinoidin Neden Olduğu Ektopik Cushing Sendromu Olgusu A Case of Ectopic Cushing Syndrome Caused by ACTH-Releasing Bronchial Carcinoid

RESPIRATORY CASE REPORTS

Burçin Çelik1, Uğur Avcı2, Ayşegül Atmaca2

Özet

Abstract

Küçük hücreli akciğer kanseri ve bronkopulmoner karsinoid tümörler ektopik ACTH salınımının en sık nedenidir ve oldukça nadir bir durumdur. Bu makalede ACTH salgılayan bronşiyal karsinoid tümörünün neden olduğu ektopik Cushing sendromlu olguyu sunduk. Bilinç bulanıklığı ve konuşamama şikayetleri acil servise başvuran 65 yaşında kadın hastada hipoglisemi, hipopotasemi, hiperkortizolemi ve ACTH yüksekliği saptandı. Hastanın hipofiz ve batın MR'da patoloji saptanmadı. Hastada hipofizer MR'ın normal olması ve bilateral inferior petrozal sinüs örneklemesinde santral/periferik ACTH gradientinin 2'nin altında olması ektopik ACTH salınımını düşündürdü. Toraks BT'de sağ üst lob apikal segmentte 12x10 mm subplevral nodül izlendi, PET-BT görüntülemede patolojik FDG tutulumu izlenmedi. Hastaya genel anestezi altında VATS wedge rezeksiyon uygulandı ve histolojik incelemede tipik bronşial karsinoid tanısı kondu. Hasta postoperatif 5. gününde haliyle ile taburcu edildi. Karsinoid tümöre bağlı ektopik Cushing sendromlu olgularda 5 yıllık sağkalım %70 olarak bildirilmekle birlikte 60 yaş üstü hastalarda prognozun daha kötü olduğu rapor edilmektedir. Cerrahi olarak kaynağın ortadan kaldırabilmesi hastanın tedavisindeki en önemli adımdır.

Small cell lung cancer and bronchopulmonary carcinoid tumors are the most common causes of an ectopic release of adrenocorticotropic hormone (ACTH) release. In this article, a patient with Cushing syndrome caused by an ACTH-releasing bronchial carcinoid tumor is presented. A 65-year-old female patient was admitted to the emergency service with the complaints of blurred consciousness and loss of speech, and was diagnosed with hypoglycemia, hypopotassemia, hypercortisolemia, and a high ACTH level. Magnetic resonance images (MRI) of the pituitary gland and the abdomen revealed no pathology. Since the pituitary MRI was normal, and her central/peripheral ACTH gradient was less than 2 in bilateral inferior petrosal sinus sampling, it was considered to be a case of ectopic ACTH release. A thorax computed tomography (CT) revealed a 12x10 mm subpleural nodule at the right upper lobe apical segment. Pathological fludeoxyglucose involvement was not observed in PET–CT imaging. The patient underwent video-assisted thoracoscopic surgery wedge resection under general anesthesia and was diagnosed with typical bronchial carcinoid based on a histological study. She was discharged (in that condition) on the fifth postoperative day. Although the survival rate in patients with ectopic Cushing syndrome caused by carcinoid tumor has been reported to be 70%, the prognosis in patients over 60 is worse. The surgical elimination of the source is the most significant step in treatment.

Anahtar Sözcükler: Akciğer, Cushing sendromu, ektopik, karsinoid tumor.

Key words: Lung, Cushing syndrome, carcinoid tumor, ectopic.

Ondokuz Mayıs Üniversitesi Tıp Fakültesi, Göğüs Cerrahisi Anabilim Dalı, Samsun 2 Ondokuz Mayıs Üniversitesi Tıp Fakültesi, Endokrinoloji Anabilim Dalı, Samsun

Department of Thoracic Surgery, Ondokuz Mayıs University Faculty of Medicine, Samsun, Turkey 2 Department of Endocronology, Ondokuz Mayıs University Faculty of Medicine, Samsun, Turkey

Başvuru tarihi (Submitted): 01.11.2017 Kabul tarihi (Accepted): 15.02.2018 İletişim (Correspondence): Burçin Çelik, Ondokuz Mayıs Üniversitesi Tıp Fakültesi, Göğüs Cerrahisi Anabilim Dalı, Samsun e-mail: cburcin@hotmail.com

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Respiratory Case Reports

Ektopik ACTH salınımı oldukça nadir bir durumdur ve literatürde ilk tanımlanan paraneoplastik endokrin sendrom olup tüm ACTH-bağımlı Cushing sendromu olgularının sadece %10-20'sini oluşturur (1,2). Nöroendokrin tümörler özellikle de bronkopulmoner karsinoid tümörler ve küçük hücreli akciğer kanseri ektopik ACTH salınımına bağlı Cushing sendromunun en sık nedenleridir. Dünya genelinde bronkopulmoner karsinoid tümör oranı 0,22/100000'dir. Ektopik ACTH salınımının kaynağı tespit edildiğinde gereken tedavi odağın cerrahi olarak çıkarılmasıdır (1-3). Bu makalede, ACTH salgılayan bronşiyal karsinoid tümörünün neden olduğu ektopik Cushing sendromlu olguyu sunduk.

OLGU Bilinç bulanıklığı ve konuşamama şikâyetleri ile acil servise başvuran 65 yaşında kadın hastada hipoglisemi, hipopotasemi, hiperkortizolemi ve ACTH yüksekliği saptandı. Özgeçmişinde; hipertansiyon, diyabet, karaciğer yetmezliği ve konjestif kalp yetmezliği tanıları olan hastada bilateral plevral effüzyon mevcuttu. Daha önce histerektomi ve kolesistektomi ameliyatları geçiren hasta 7 paket/yıl sigara kullanıcısıydı. Laboratuvar değerleri; ACTH 177,51 pg/mL, kortizol 63,44 μg/dL, parathormon 68,66 pg/mL, GGT 824 U/L, ALT 98 U/L, AST 93 U/L, ALP 356 U/L, sedimantasyon 31 mm/saat, PO2 45,8 mmHg, PCO2 30,9 mmHg, SatO2 %83, pH 7,57 idi. İki gün Dexametazon süpresyon testi sonrasında kortizol değeri 34,62 µg/dL (Normal: <1,8 µg/dL) olarak ölçüldü. Hipofiz MR'da ve batın MR'da patoloji saptanmadı. Hastada hipofizer MR'ın normal olması ve bilateral inferior petrozal sinüs örneklemesinde santral/periferik ACTH gradientinin CRH öncesi 2'nin altında CRH (kortikotropin releasing hormon) uygulaması sonrasında 3’ün altında olması ektopik ACTH salınımını düşündürdü (Tablo 1). Toraks BT'de aksiyal kesitlerde sağ üst lob apikal segmentte 12x10 mm subplevral nodül izlendi (Şekil 1a ve b). PET-BT görüntülemede tüm vücutta patolojik FDG tutulumu izlenmedi. Sağ akciğer üst lobdaki lezyonun ektopik odak olduğu düşünülerek cerrahi tedavi kararı alındı. Komorbid hastalıklar nedeniyle anesteziyoloji kliniği tarafından preoperatif incelemede ASA III olarak değerlendirilen hastaya genel anestezi altında fleksibl bronkoskopi ve VATS wedge rezeksiyon uygulandı (Şekil 2). Fleksibl bronkoskopide her iki bronşiyal sistem normal olarak izlendi ve endobronşiyal lezyon tespit edilmedi. Sağ üst lob apikal segmentten eksize edilen materyalin histolojik inceleCilt - Vol. 7 Sayı - No. 2

mesinde tipik bronşial karsinoid tanısı kondu. Postoperatif dönemde hastanın serum ACTH değeri 88,3 pg/ml'ye, serum kortizol değeri 24,52 μg/dL'ye geriledi ve 1 mg Dexametazon süpresyon testi sonrasında kortizol değeri 2,24 µg/dL olarak ölçüldü. Genel durumunun düzelen hasta postoperatif 5. gününde salah ile taburcu edildi. Takiplerinde hasta postoperatif 5. ayda karaciğer yetmezliği ve genel durum bozukluğu nedeniyle kaybedildi. Tablo 1: Bilateral inferior petrozal sinüs örneklemesi. ACTH SAĞ SOL PERİFERİK SANTRAL/PERİFERİK (pg/ml) İPS İPS VEN -5.dk 171 154 132 1,29 0.dk

158

153

135

1,17

3.dk

164

176

120

1,36

5.dk

158

153

117

1,35

8.dk

163

166

113

1,44

10.dk

181

179

117

1,54

15.dk

172

171

128

1,34

İPS: inferior petrozal sinüs; ACTH: adrenokortikotropik hormon

Şekil 1a ve b: Toraks BT aksiyel kesitlerde sağ üst lob anterior segmentte 12x10 mm ebadında nodüler lezyon.

TARTIŞMA Cushing sendromu, ilk kez 1932 yılında Harvey Williams Cushing tarafından tanımlanmış olan kortizol hormonunun olağanın üstünde bir düzeyde olduğu durumlarda

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ACTH Salgılayan Bronşiyal Karsinoidin Neden Olduğu Ektopik Cushing Sendromu Olgusu | Çelik et al.

ortaya çıkan belirtiler ve bulguların bütünüdür. Ektopik Cushing sendromu ise tüm Cushing olgularının %1020'sini oluşturur ve ilk olarak Liddle tarafından 1963 yılında tanımlanmıştır (2-5). Ektopik Cushing sendromlu olguların yaklaşık %3-50'si bronşial karsinoid tümörlere bağlıdır. Diğer nedenler; küçük hücreli akciğer kanseri, timik karsinoidler, pankreatik nöroendokrin tümörler, feokromasitoma ve tiroid karsinomlar olup %12-36'sında kaynak tespit edilemez (2,4). Ektopik ACTH sekresyonundaki semptomlar genellikle hiperkortikolizm semptomlarına benzer. En sık; obesite, kilo alma, pleatore, ay yüz, ince cilt ve arteriyal hipertansiyon izlenir. Maligniteye bağlı ektopik Cushing sendromlu hastalarda semptomlar daha ağırdır ve hastalık daha hızlı seyreder, çünkü yüksek serum ACTH değeri ve yüksek kortizol sekresyonu söz konusudur (6).

Şekil 2: Cerrahi eksizyon materyalinde subplevral yerleşimli karsinoid tümör.

Tanı için hipofiz bezi manyetik rezonans görüntüleme ile araştırılmalıdır. Radyolojik olarak patoloji tespit edilmediğinde ektopik Cushing sendromu tanısında bilateral inferior petrozal sinüs örneklemesi kullanılmaktadır. Bu yöntem girişimsel radyoloji yöntemi olup bazal ve kortikotropin salgılatıcı hormon veya desmopressinle uyarılmış durumda periferal ve her iki petrosal sinüsten alınan kan örneklerindeki ACTH düzeylerinin karşılaştırılması esasına dayanır. Eğer işlem esnasında santral ve periferik kan örnekleri arasında santral lehine bir yükseklik varsa santral ACTH salınımını; yoksa ektopik bir odaktan ACTH salınımını düşündürür.

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Diğer tanı yöntemleri; bilgisayarlı tomografi, kolonoskopi, somatostatin reseptör sintigrafisi, PET BT, 68Ga-DOTApeptide PET-BT'dir (2). 18F-FDG PET'in ektopik ACTH sekrete eden odağı tespitinde duyarlılığı %64, pozitif prediktif değeri %53 olarak bildirilmektedir (3). Ektopik Cushing sendromunda genel sağ kalım; 1 yıllık %84, 5 yıllık %70 olarak bildirilmektedir (2). Karsinoid tümöre bağlı ektopik Cushing sendromlu olgularda 5 yıllık sağkalım %70 olarak bildirilmekle birlikte 60 yaş üstü hastalarda prognozun daha kötü olduğu (5 yıllık sağ kalım %52&%81) rapor edilmektedir. En iyi sağ kalım oranı bronşiyal karsinoide bağlı olgularda izlenmekte ve bu oran %84 civarındadır. Uzak metastazlı olgularda prognoz azalmaktadır ve 5 yıllık sağ kalım %61'e kadar gerilemektedir (2). Cerrahi olarak kaynağın ortadan kaldırabilmesi hastanın tedavisindeki en önemli adımdır. Ektopik Cushing sendromlu olguların %40'ında radikal cerrahi tedavi uygulanabilmekte ve %80'inde kür sağlanabilmektedir. Bronşiyal karsinoidli olguların yaklaşık %70'inde küratif cerrahi sağlanabilmekte ve mediastinal lenf nodlarında metastaz bile çıksa radyoterapi gerekmemektedir (2). Literatürdeki en geniş serileri olan Davi ve ark. (2) serisinde 110 olgunun 45 bronşiyal karsinoidli olgu olup bu olguların 37'sine (%82) cerrahi tedavi uygulanmıştı. Olgumuz; cerrahi tedavi sonrası kortizol değerlerinde ve genel durumda düzelme oldu ancak birçok komorbid hastalıklar olması nedeniyle postoperatif 5. ayda kaybedildi. Sonuç olarak, oldukça ender rastlanan ektopik Cushing sendromunda odak tespit edilmeli ve tedavi edilmelidir. Etyolojinin karsinoid tümör olduğu olgularda cerrahi tedavi tercih edilmelidir.

ÇIKAR ÇATIŞMASI Bu makalede herhangi bir çıkar çatışması bildirilmemiştir.

YAZAR KATKILARI Fikir - B.Ç., U.A., A.A.; Tasarım ve Dizayn - B.Ç., U.A., A.A.; Denetleme - B.Ç., U.A., A.A.; Kaynaklar - B.Ç., U.A.; Malzemeler - B.Ç., U.A.; Veri Toplama ve/veya İşleme - B.Ç.; Analiz ve/veya Yorum - B.Ç., A.A.; Literatür Taraması - B.Ç.; Yazıyı Yazan - B.Ç., U.A., A.A.; Eleştirel İnceleme - B.Ç., U.A., A.A.

KAYNAKLAR 1.

Vrkljan M, Vizner B, Zawawi A, Bekic M, Gorecan M, Grbac I. Ectopic ACTH secretion and Cushing's syndrome. Acta Clin Croat 2000; 39:77-81. [CrossRef]

www.respircase.com

Respiratory Case Reports

Davi MV, Cosaro E, Piacentini S, Reimondo G, Albiger N, Arnaldi G, et al. Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study. Eur J Endocrinology 2017; 176:451–59. [CrossRef]

Liddle GW, Island DP, Ney RL. Nicholson WE, Shimizu N. Nonpituitary neoplasms and Cushing’s syndrome. Ectopic “adrenocorticotropin” produced by nonpituitary neoplasms as a cause of Cushing’s syndrome. Arch Intern Med 1963; 111:471–5.

Li WY, Liu XD, Li WN, Dong SY, Qu XH, Gong SL, et al. Paraneoplastic Cushing's syndrome associated with bronchopulmonary carcinoid tumor in youth: A case report and review of the literature. Oncol Lett 2016; 12:69-72. [CrossRef]

Cieszyński L, Berendt-Obołończyk M, Szulc M, Sworczak K. Cushing’s syndrome due to ectopic ACTH secretion. Endokrynol Pol 2016; 67:458-71. [CrossRef]

Zhang HY, Zhao J. Ectopic Cushing syndrome in small cell lung cancer: A case report and literature review. Thorac Cancer 2017; 8:114–7. [CrossRef]

Cilt - Vol. 7 Sayı - No. 2

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Respir Case Rep 2018;7(2):110-113 DOI: 10.5505/respircase.2018.59672

OLGU SUNUMU

CASE REPORT

Endobronşial Metastaz ile Seyreden Kolon Adenokarsinom Olgusu A Case of Endobronchial Metastasis Caused by Colon Adenocarcinoma

RESPIRATORY CASE REPORTS

Saltuğ Buğra Kaya1, Talat Kılıç2, Ayşe Nur Akatlı3, Emine Türkmen Şamdanlı3

Özet

Abstract

Kolon adenokarsinomu nedeniyle takipli 60 yaşında erkek hasta, öksürük ve nefes darlığı şikâyeti ile kliniğimize başvurdu. Hastanın takipleri sırasında çekilen Toraks BT’de sağ hiler bölgede sağ ana bronşa invaze kitle lezyon ve yaygın bilateral nodüler lezyonlar tespit edilmesi üzerine ikincil primer akciğer tümör açısından değerlendirilmek üzere, göğüs hastalıkları polikliniğine yönlendirilmişti. Hastanın yapılan bronkoskopisinde; sağ ana bronştan başlayıp orta lob girişinde yoğunlaşan yaygın endobronşiyal lezyon tespit edildi. Alınan endobronşial biyopsi sonucu immünohistokimyasal çalışmalar ile desteklenerek kolon adenokarsinom metastazı olarak değerlendirildi. Radyolojik görünümü primer akciğer kanserine benzediğinden ve kolon adenokarsinomların endobronşial metastazı nadir olduğundan sunumu uygun görüldü.

A 60-year-old male patient with a diagnosis of colon adenocarcinoma was admitted to the clinic with complaints of a cough and shortness of breath. A thorax computed tomography image of the right main bronchus taken during follow-up of the patient revealed an invasive mass lesion in the right hilar region. The patient was referred to the pulmonology department for evaluation in terms of a primary lung tumor. The patient underwent a bronchoscopy. A widespread endobronchial lesion beginning at the right main bronchus and intensifying at the entrance of the middle lobe was detected. Biopsies were performed in both bronchial trees. The biopsy results confirmed colon adenocarcinoma metastasis, which was supported by immunohistochemical findings. Since the radiological appearance resembled primary lung cancer and endobronchial metastasis of colon adenocarcinoma is rare, the outcome of the case was deemed appropriate.

Anahtar Sözcükler: Endobronşial metastaz, kolon edenokarsinomu, akciğer metastazı.

Key words: Endobronchial metastasis, etoncarsinoma, lung metastasis.

Endobronşiyal lezyonlardan (EBL) alınan biyopsilerin birçok histopatolojik alt tipi mevcut olup en sık görülen primer akciğer karsinomlarıdır. Özellikle skuamoz hücreli ve küçük hücreli akciğer karsinomlarında endobronşiyal lezyon sık görülür. Endobronşiyal tümörlerin sadece% 1,1'i metastatiktir (1,2). Metastatik lezyonlardan en 1

Sağlık Bakanlığı, Kamu Hastaneleri Kurumu, Cizre Devlet Hastanesi, Göğüs Hastalıkları Kliniği, Şırnak 2 İnönü Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Malatya 3 İnönü Üniversitesi Tıp Fakültesi, Patoloji Anabilim Dalı, Malatya

yaygın EBL, kolorektal, meme ve renal karsinomlarda görülür (3). Mide, over, tiroid, uterus, testiküler, nazofarenks, prostat, adrenal karsinomalar, sarkomlar, histositoma ve plazmositomlar gibi çeşitli malignitelerde EBL gözlenir (4-8).

Department of Chest Diseases, Ministry of Health, Public Hospitals Institution, Cizre State Hospital, Şırnak, Turkey 2 Department of Chest Diseases, İnönü University Faculty of Medicine, Malatya, Turkey 3 Department of Pathology, İnönü University Faculty of Medicine, Malatya, Turkey

Başvuru tarihi (Submitted): 23.10.2017 Kabul tarihi (Accepted): 12.12.2017 İletişim (Correspondence): Talat Kılıç, İnönü Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Malatya e-mail: talatkilic2013@gmail.com

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colon

Respiratory Case Reports

OLGU Altmış yaşında erkek hastaya, 3 yıl önce ileus nedeniyle genel cerrahi tarafından acil sağ hemikolektomi operasyonu yapılmış ve patolojik değerlendirme sonrası orta derece diferansiye kolon adenokarsinomu tanısı almıştı. Yaygın lenfovasküler invazyon ve lenf bezlerinde tümör metastazı tespit edilmiş, tümör visseral peritonu perfore etmiş ve perinöral invazyon bulunmaktaydı, cerrahi sınırlar negatif olarak raporlanmıştı. Hasta tanı aldığında evreleme için çekilen Toraks Bilgisayarlı Tomografisinde (BT) buzlu cam dansitesinde nodüler lezyonlar mevcut olup hastaya medikal onkoloji tarafından kemoterapi başlanmıştı. Kemoterapi sonrasında çekilen kontrol Toraks BT’sinde lezyonlarda regresyon tespit edildi (Şekil 1). Tedavi protokolüne tam olarak uyum göstermeyen hasta kendi isteği ile tedavisine yaklaşık 1 yıl ara verdi. Sonrasında kontrole gelen hastanın evreleme için çekilen Toraks BT’sinde bilateral yaygın nodüler lezyonlar tespit edildi ve hasta yeniden kemoterapi programına alındı (Şekil 2). Hasta tedavisine devam ederken kontrol amaçlı yapılan radyolojik tetkiklerinde sağ hiler bölgede lokalize sağ ana bronşa invaze kitle lezyonu tespit edilmesi üzerine ikincil primer tümör açısından göğüs hastalıkları polikliniğine yönlendirilmişti. (Şekil 3).

Nefes darlığı ve kuru kronik öksürük şikâyeti bulunan hastanın fizik muayenesinde solunum sesleri bilateral azalmıştı. Kan tetkiklerinde kreatinin değeri 1,25 mg/dl ölçülmüş olup diğer parametreleri normal aralıkta tespit edildi. Soy geçmiş ve alışkanlıklarında herhangi bir özellik olmayan hastaya PET-BT, Kranial MR çekildi ve bronkoskopi yapıldı. Hastanın Kranial MR’ında herhangi bir özellik tespit edilmedi. PET-BT sinde sol supraklavikular bölgede SUVmax: 5,5 ölçülen hilusu seçilen lenf bezi, her iki akciğerde subsantimetrik çoğu periferik yerleşimli SUVmax:10,7 ölçülen yüzlerce nodüler lezyon, sağ akciğer üst lob santralinde spiküle düzensiz kenarlı, boyutu 5,6x2,7 cm olan SUVmax:20,2 ölçülen konsolide alan, intra-abdominal alanda psoas ve rektus abdominalis kasına invaze 5,4x6,2 cm boyutta SUVmax: 15,8 ölçülen kitle lezyon tespit edildi. PET-BT raporunda sağ akciğerdeki lezyon için bronkoalveoler karsinom? organize pnömoni? metastaz? ön tanıları bulunmaktaydı. Hastanın bronkoskopisinde sağ ana bronştan başlayıp orta lob girişinde yoğunlaşan yaygın endobronşiyal lezyon tespit edildi. Sol bronş sisteminde dağınık nodüller şekilde endobronşiyal lezyon görüldü. Her iki bronş ağacından biyopsiler alındı. Bronkoskopik biyopsinin histopatolojik incelemesinde abortif asiner yapılar yapmış atipik epitelyal hücrelerden oluşan neoplastik gelişim gözlendi. İmmünohistokimyasal olarak tümör hücreleri CDX2 ile pozitif, CK7 ve CK2O ile fokal pozitif boyanırken TTF-1 ile immünreaktivite izlenmedi. Morfolojik bulgular, immünohistokimyasal bulgular eşliğinde kolon adenokarsinom metastazı olarak raporlandı (Şekil 4).

Şekil 1: Kontrol Toraks BT’de lezyonlarda regresyon.

Şekil 3: Toraks BT’de sağ ana bronşa invaze kitle lezyon.

TARTIŞMA

Şekil 2: Toraks BT’de bilateral yaygın nodüler lezyonlar. Cilt - Vol. 7 Sayı - No. 2

Endobronşiyal lezyonlardan alınan biyopsilerin birçok histopatolojik alt tipi mevcut olup en sık görülen primer akciğer karsinomlarıdır. Özellikle skuamoz hücreli ve küçük hücreli akciğer karsinomlarında EBL sık görülür. Endobronşiyal tümörlerin sadece%1,1'imetastatiktir (1,2).

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Endobronşial Metastaz ile Seyreden Kolon Adenokarsinom Olgusu | Kaya et al.

Metastatik lezyonlardan en yaygın EBL, kolorektal, meme ve renal karsinomlarda görülür (3). Mide, over, tiroid, uterus, testiküler, nazofarenks, prostat, adrenal karsinomalar, sarkomlar, histositoma ve plazmositomlar gibi çeşitli malignitelerde EBL gözlemlenir (4-8). Endobronşial metastazı olan 204 hastanın incelendiği çalışmada olguların %30’unda kolorektal kanser metastazı saptanmıştır (9). Primer tümör ile endobronşiyal metastaz arasında geçen zaman ortalama 9 ay ile 5 yıl arasında değişmektedir (10). Kolorektal kansere bağlı EBL olan 24 hastanın retrospektif değerlendirilmesinde, primer tümörün tanısı ile EBL arasında geçen ortanca süre 53 ay olarak bulunmuştur. Bu süre bizim hastamızda yaklaşık 36 aydı. En sık semptomlar hastamızda olduğu gibi dispne, öksürük ve hemoptizidir. EBL saptanmasından itibaren ortalama sağ kalım süresi 14 ay (3-40 ay), primer tümörün saptanmasından itibaren ortalama sağ kalım 70 ay (23-245 ay) olarak bulunmuştur. Hastaların %54’ünde ortalama sağ kalım 5 yıldan uzundur (5). Endobronşiyal metastazı olan hastalarda radyolojik olarak tespit edilen en sık lezyonlar bilateral nodüller, atelektaziler, soliter pulmoner nodül olarak izlenilmektedir. Bizim hastamızda da santral bölgede kitle lezyonu ve bilateral yaygın nodüler lezyonlar mevcuttu. Klinik ve radyolojik bulgu olarak ana bronşların metastatik tutulumu, santral yerleşimli bronkojenik karsinomdan ayırt edilemez (10,11). Torak BT, bronş içi lezyonları her zaman gösterememektedir. Pulmoner metastazları ya da mediastinel lenfadenopatileri ortaya çıkardığı için bütün olgularda radyolojik olarak görüntüleme yapılmalıdır (12). Endobronşiyal lezyonların tanısında en değerli yöntem bronkoskopidir. Metastatik lezyonlardan histopatolojik tanı için biyopsi alınabildiği gibi fırsatçı akciğer enfeksiyonu, kanama veya ilaç reaksiyonu gibi radyolojik olarak metastazı taklit edebilecek durumları da ayırt etmede çok önemlidir. Bronkoskopi ile tanı konma oranı EBL izlenen olgularda %84,2 olup bizim olgumuzda birden çok EBL izlendi. Alınan biyopsi sonucu, immünohistokimyasal bulgular eşliğinde özellikle TTF-1 negatif olması ve CDX2 pozitifliği ile CK20’nin fokal pozitif boyanması nedeniyle kolon adenokarsinomu olarak sonuçlandı. TTF-1 erişkin akciğerinde öncelikle tip II alveolar pnömosit ve silleri olmayan bronşial hücrelerde identifiye edilmiştir. TTF-1, kromozom 14q13 de tek lokuslu bir gende bulunur ve akciğer dokusunda surfaktan proteinleri (A, B, C) ve Klara hücre sekretuvar proteini gen ekspresyonunu regüle eder. TTF-1`in inaktivasyonunun, trakeoözofageal fistüle, pulmoner dallanmanın bozukluğuna ve nadir akciğer hipoplazilerine neden olduğu bildirilmiştir. TTF-1

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ekspresyonu, akciğer ve tiroid kanserlerinde yüksek oranlarda görülmektedir. TTF-1 in özellikle akciğer kökenli adenokarsinomlar ile akciğere metastaz yapan adenokarsinomların ayırıcı tanısında oldukça yüksek sensitivite (%70-94) ve spesifiteye (%100) sahip olduğunu bildiren çalışmalar mevcuttur. Bu nedenle, TTF-1'in immunohistokimyasal uygulaması, akciğer kökenli adenokarsinomların identifikasyonunda oldukça yaygın olarak kullanılmaktadır (13). Bunun yanında CDX2 immunohistokimyasal belirtecinin kolorektal adenokarsinom metastazlarını ayırt etmede kullanılan yararlı bir belirteç olduğu gösterilmiştir (14).

Şekil 4: Bronkoskopik biyopside asiner dizelenim paternine sahip atipik epitelyal hücrelerden oluşan tümör (H&E, x100) (a), Tümör hücrelerinde immunohistokimyasal olarak TTF-1 negatifliği (x100) (b), Tümöral hücrelerin immunohistokimyasal olarak CDX2 ile pozitif boyanması (x100) (c), Kolektomi spesimeninde kolon mukozası devamlılığındaki adenokarsinom alanları (H&E, x40) (d).

Sonuç olarak, nadir bir olgu olması nedeniyle olgu sunumu olarak tartışılmıştır.

ÇIKAR ÇATIŞMASI Bu makalede herhangi bir çıkar çatışması bildirilmemiştir.

YAZAR KATKILARI Fikir - S.B.K., T.K., A.N.A., E.T.Ş.; Tasarım ve Dizayn S.B.K., T.K., A.N.A., E.T.Ş.; Denetleme - S.B.K., T.K., A.N.A., E.T.Ş.; Kaynaklar - S.B.K., T.K., A.N.A., E.T.Ş.; Malzemeler - S.B.K., T.K., A.N.A., E.T.Ş.; Veri Toplama ve/veya İşleme - S.B.K., T.K.; Analiz ve/veya Yorum S.B.K., T.K.; Literatür Taraması - S.B.K., T.K.; Yazıyı Yazan - S.B.K., T.K.; Eleştirel İnceleme - S.B.K., T.K.

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Respiratory Case Reports

KAYNAKLAR 1.

Salud A, Porcel JM, Rovirosa A, Bellmunt J. Endobronchial metastatic disease: analysis of 32 cases. J Surg Oncol 1996; 62:249–52. [CrossRef] Kreisman H, Wolkove N, Finkelstein HS, Cohen C, Margolese R, Frank H. Breast cancer and thoracic metastases: review of 119 patients. Thorax 1983; 38:175–9. [CrossRef]

Berg HK, Petrelli NJ, Herrera L, Lopez C, Mittelman A. Endobronchial metastasis from colorectal carcinoma. Dis Colon Rectum 1984; 27:745–8. [CrossRef]

Amer E, Guy J, Vaze B. Endobronchial metastasis from renal adenocarcinoma simulating a foreign body. Thorax 1981; 36:183–4. [CrossRef]

Fournel C, Bertoletti L, Nguyen B, Vergnon JM. Endobronchial metastases from colorectal cancers: natural history and role of interventional bronchoscopy. Respiration 2009; 77:63–9. [CrossRef]

Gallivan GJ, Emery RW. Endobronchial metastasis from cancer of the breast. Chest 1978; 74:320. [CrossRef]

Hanyu T, Kanda T, Matsuki A, Hasegawa G, Yajima K, Tsuchida M, et al. Endobronchial metastasis from adenocarcinoma of gastric cardia 7 years after potentially curable resection. World J Gastrointest Surg 2010; 2:270–4. [CrossRef]

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Shen Q, Yao Y, Teng X, Zhou J. Endobronchial metastasis from prostate cancer mimicking primary lung cancer. Intern Med 2010; 49:1613–5. [CrossRef]

Sorensen JB. Endobronchial metastases from extrapulmonary solid tumors. Acta Oncol 2004; 43:73-9. [CrossRef]

10. Katsimbri PP, Bamias TA, Froudarakis EM, Peponis IA, Constantopoulos SH, Pavlidis NA. Endobronchial metastases secondary to solid tumors: report of eight cases and review of the literature. Lung Cancer 2000; 28:163-70. [CrossRef] 11. Diaz G, Jimenez D, Dominguez-Reborias S, Carrillo F, Pérez-Rodríguez E. Yield of bronchoscopy in the diagnosis of neoplasm metastatic to lung. RespirMed 2003; 97:27-9. [CrossRef] 12. Ohno T, Nakayama Y, Kurihara T, Ichikawa H, Tsuda K, Ishida T, et al. Endobronchial metastasis of breast cancer 5 years after breast-conserving therapy. Int J Clin Oncol 2001; 6:101-4. [CrossRef] 13. Nart D, Sarsık B, Doğanavşargil B, Sezak M, Yaman B, Çiriş M ve ark. Primer ve metastatik akciğer tümörlerinin ayırıcı tanısında tiroid transkripsiyon faktör-1 ekspresyonunun önemi ve güvenilirliği. Ege Tıp Dergisi/Ege J Med 2008; 47:171-5. 14. Tanaka S, Saito K, Ito T, Tajima K, Mogi A, Shitara Y, et al. CDX2 as a useful marker of colorectal adenocarcinoma metastases to lung in pre-operative biopsy specimens. Oncol Rep 2007; 18:87-92. [CrossRef]

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Respir Case Rep 2018;7(2):114-117 DOI: 10.5505/respircase.2018.93723

OLGU SUNUMU

CASE REPORT

Bronchioloalveolar Carcinoma in PeutzJeghers Syndrome: A Case Report Peutz-Jeghers Sendromunda Bronkoalveoler Karsinom: Olgu Sunumu

RESPIRATORY CASE REPORTS

Burcu Yormaz, Baykal Tülek, Mecit Süerdem

Abstract

Özet

Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant genetic disease. There is an increased prevalence of cancer in the PJS. A 48-year-old male patient with PJS was admitted to the clinic with bilateral consolidated areas in the thorax observed on a computed tomography image. A bronchoscopy revealed a tumorous lesion in the mucosa in the right upper lobe anterior segment. Pathological analysis of a sample led to a diagnosis of bronchioloalveolar carcinoma.

Peutz-Jeghers sendromu nadir görülen otozomal dominant geçişli herediter bir hastalıktır. PeutzJeghers sendromunda kanser prevalansında artış vardır. Peutz-Jeghers sendromlu 48 yaşında bir erkek hasta bilgisayarlı akciğer tomografisinde bilateral konsolide alanlar ile kliniğimize yatırıldı. Bronkoskopide sağ üst lob anterior segment bronşu içinde tümoral lezyon görüldü. Patolojik tetkikde bronkoalveoler kanser tanısı kondu.

Key words: Peutz-Jeghers syndrome, bronchioloalveolar carcinoma, lung.

Anahtar Sözcükler: Peutz-Jeghers sendromu, bronkoalveoler karsinom, akciğer.

Lung cancer is the leading cause of cancer-related death all over the world. Unfortunately, at first diagnosis, the majority of patients have an advanced stage of the illness. The stage observed upon diagnosis greatly affects survival. Early detection and diagnosis is the key to improving the survival rate of lung cancer patients. In addition, in rare cases, some syndromes may coexist with lung cancer, such as PJS (1,2). PJS is an autosomal dominant inherited disease characterized by intestinal hamartomatous polyps and mucocutaneous pigmentation associated with a serine-threonine kinase 11 (STK11) mutation (3). In 1921, Peutz (4) described a Dutch family with multiple intestinal hamartomatous polyps and characteristic melanin spots on the skin and mucosa. In 1949 Jeghers et al. (5) described 3 more

families with identical lesions seen at the Johns Hopkins Clinic. The original name of Peutz-Jeghers syndrome was first used in 1954 (6). The incidence of the syndrome is estimated to be 1 in 50,000 to 200,000 (7). Mucocutaneous pigmented lesions have been observed in 95% of patients, which may be the first finding of the syndrome. These lesions may be seen at birth. The classic pigmented lesions may be found on the lips, in the mouth, on the nose, in the perianal area, on the fingers, or in the dorsal and volar regions of the hands and feet. These pigmented macules are probably the result of a melanin increase in the basal cells due to an inflammatory block in the melanin migration (8-10). The most important clinical problem in patients with PJS is the mechanical complications that arise

Department of Chest Disease, Selçuk University Faculty of Medicine, Konya, Turkey

Selçuk Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Konya

Submitted (Başvuru tarihi): 28.12.2017 Accepted (Kabul tarihi): 08.02.2018 Correspondence (İletişim): Burcu Yormaz, Department of Chest Disease, Selçuk University Faculty of Medicine, Konya, Turkey e-mail: burcyormaz@gmail.com

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from intestinal polyps. Polyps may be seen in the entire gastrointestinal system. However, they are usually localized in the small intestine and the colon. Other sites include the gall bladder, bronchia, bladder, and the ureter. Gastrointestinal polyps may lead to bleeding, anemia, and abdominal pain.

CASE A 48-year-old male patient presented at the clinic in August 2017 with the complaints of a cough and white foamy phlegm. A physical examination revealed brown spots in the perioral and perinasal area, and blue mucocutaneous lesions were observed in the mouth (Figure 1). His history included 28 pack-years of smoking. The pigmented lesions on his face and in his mouth were present at birth. He had suffered from abdominal pain since childhood, and had undergone colon polypectomy operations a total of 4 times, with the last operation in 2010, with the diagnosis of Peutz-Jeghers syndrome (PJS). His mother also had pigmented lesions in the perioral area. The patientâ&#x20AC;&#x2122;s daughter had undergone 2 polypectomies, and his son had undergone 3 polypectomy operations with the diagnosis of PJS. Thorax tomography revealed consolidated areas containing air bronchograms in the right upper lobe posterior and middle lobe lateral segments, and patchy frosted glass areas in both lungs, which was more obvious on the right, a nodular lesion 17x14 mm in size in the left upper lobe, and common micronodules (Figure 2). A positron emission tomography examination revealed a hypermetabolic mass lesion in the right upper lobe posterior segment. An upper abdominal ultrasonography revealed lesions 32x24 mm in size in the lateral segment of the left lobe of the liver and 37x42 mm in the caudate lobe with a relatively smooth contour, ISO-light compared with the liver parenchyma, and compatible with hyperechoic hemangioma. In the contrastive dynamic magnetic resonance imaging (MRI) of the abdomen, 5 or 6 wellcircumscribed mass lesions 49x41 mm in size with a lobular contour, of which the largest was located in the caudal lobe and near the inferior vena cava, and which were incompatible with hemangioma, were observed in the liver. No pathology was observed in a brain MRI apart from bilateral maxillary sinus, mucosal thickening, and a retention cyst on the left.

Figure 1: Perioral and mucocutaneous lesions

Figure 2: Thorax computed tomography images

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Bronchioloalveolar Carcinoma in Peutz-Jeghers Syndrome: A Case Report | Yormaz et al.

Bronchoscopy revealed a tumorous lesion in the mucosa in the right upper lobe anterior segment carina separation, and a biopsy was performed. In addition, a transbronchial lung biopsy sample was taken from the middle lobe lateral segment. Bronchioloalveolar lung carcinoma was diagnosed based on the pathological examination (Figure 3).

evaluated in 6 trials. Lung cancer was responsible for 15% of cancer cases. Hearle et al. (16) then published a cohort study that involved 419 individuals with PJS. They analyzed the incidence of cancer and found that the STK11 mutation was present in 297 of the patients, and that different organ cancers developed in 23% of the patients. The risk of developing all types of cancer increased after the age of 50 years. In the series of Mehenni et al. (17) with 149 patients, 31 malignancies were detected in patients with STK11-mutation PJS, and none of those patients had lung cancer.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS

Figure 3: Bronchioloalveolar lung carcinoma (H&E; original magnification x100)

DISCUSSION In this case report, we presented a patient with PJS who developed bronchoalveolar carcinoma, which is a rare and unusual type of lung cancer. To the best of our knowledge, this is the first such case published in our country. A total of 143 patients with PJS had been reported as of 2000. However, only 6 of these patients had lung cancer. Epidemiological studies show that there is an elevation in the prevalence of cancer as well as non-malignant gonadal tumors in patients with PJS. The risk of cancer is thought to be increased in PJS due to a hamartomaadenocarcinoma relationship. The presence of adenomatous foci within the polyps of PJS supports this hypothesis. However, cancer is also seen in organs where there are no hamartomas associated with the syndrome (11). It has been suggested that PJS polyps have no potential for malignancy, given the rarity of transformation, and that the polyps are not hamartoma, but only abnormal mucosal prolapse associated with the genetic mutation. (12). Thus, the increased risk of cancer in patients with PJS may be attributed to mucosal instability through the conventional neoplastic pathways. In a case series, Li et al. (13) and Giardiello et al. (14) reported cancer patients at the rate of 28% and 22% cancer patients were reported in their series. Giardiello et al. (15) carried out a meta-analysis of 210 individuals

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Concept - B.Y., B.T., M.S.; Planning and Design - B.Y., B.T., M.S.; Supervision - B.Y., B.T., M.S.; Funding -; Materials -; Data Collection and/or Processing -; Analysis and/or Interpretation -; Literature Review -; Writing -; Critical Review -

YAZAR KATKILARI Fikir - B.Y., B.T., M.S.; Tasarım ve Dizayn - B.Y., B.T., M.S.; Denetleme - B.Y., B.T., M.S.; Kaynaklar -; Malzemeler -; Veri Toplama ve/veya İşleme -; Analiz ve/veya Yorum -; Literatür Taraması -; Yazıyı Yazan -; Eleştirel İnceleme -

REFERENCES 1.

Jemal A, Bray F, Center MM, Ferlay J, Ward E, Forman D. Global cancer statistics. CA Cancer J Clin 2011; 61: pp. 69-90. [CrossRef]

Aaltonen LA, Järvinen H, Gruber SB, Billaud MJR. JassPeutz-Jeghers syndrome, Hamilton SR, Aaltonen LA (Eds.). WHO classification of tumours. Pathology & genetics of tumours of the digestive system. IARC Press, Lyon 2000; pp.74-6.

Hemminki A, Markie D, Tomlinson I, Avizienyte E, Roth S, Loukola A, et al. A serine/threonin kinase gene defective in Peutz-Jeghers syndrome. Nature 1998; 391:184-7.

Peutz JLA. Over een zeer merkwaardige, gecombineerde familiaire polyposis van de slijmvliezen van de tractus intestinalis met die van de neuskeelholte en gepaard met eigenaardige pigmentaties van huid-en slijmvliezen, Nederl Maandschr Geneeesk 10 1921; pp.134-46.

Jeghers H, McKusick VA, Katz KH. Generalized intestinal polyposis and melanin spots of the oral mucosa, lip, and

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Respiratory Case Reports

digits; a syndrome of diagnostic significance. N Engl J Med 1949; 241:1031-6. 6.

Westermann AM, Entius MM, de Baar E, Boor PPC, Koole R MLF, van Velthuysen. Peutz-Jeghers syndrome: 78-year follow-up of the original family. Lancet 1999; 353:1211-5. [CrossRef] Giardiello FM, Trimbath JD. Peutze-Jeghers syndrome and management recommendations. Clin Gastroenterol Hepatol 2006; 4:408-15.

Gruber SB, Entius MM, Petersen GM, Laken SJ, Longo PA, Boyer R, et al. Pathogenesis of adenocarcinomas in Peutz-Jeghers syndrome. Cancer Res, 1998; 58:5267-70.

Holst VA, Finkelstein S, Yousem SA. Bronchioloalveolar adenocarcinoma of lung: monoclonal origin for multifocal disease. Am J Surg Pathol 1998; 22: 1343-50. [CrossRef]

12. Jansen M, de Leng WW, Baas AF, Myoshi H, MathusVliegen L, Taketo MM, et al. Mucosal prolapse in the pathogenesis of Peutze-Jeghers polyposis. Gut 2006; 55:1-5. 13. Li MH, Shen JX, Zhong B, He W, Ma JY. Peutz-Jeghers syndrome complicated with pulmonary mucinous adenocarcinoma: a case report and review of the literature. Zhonghua Jie He He Hu Xi Za Zhi 2011; 34:919-22. 14. Giardiello FM, Welsh SB, Hamilton SH, Offerhaus G.J.A, Gittelsohn AM, Boker SV, et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med 1987; 316:1511-4. [CrossRef] 15. Giardiello FM, Brensinger JD, Tersmette AC, Goodman SN, Petersen GM, Booker SV, et al. Very high risk of cancer in familial Peutze-Jeghers syndrome. Gastroenterology 2000; 119:1447-53.

10. Spigelman AD, Murday V, Phillips RK. Cancer and the Peutz-jeghers syndrome. Gut 1989; 30:1588-90. [CrossRef]

16. Hearle N, Schumacher V, Menko FH, Olschwang S, Boardman LA, Gille JJ, et al. Frequency and spectrum of cancers in the Peutze-Jeghers syndrome. Clin Cancer Res 2006; 12:3209-15.

11. Boardman LA, Thibodeau SN, Schaid DJ, Lindor NM, McDonnell SK, Burgart LJ, et al. Increased risk of cancer in patients with the Peutz-Jeghers syndrome. Ann Int Med 1998; 128:896-9. [CrossRef]

17. Mehenni H, Resta N, Park JG, Miyaki M, Guanti G, Costanza MC. Cancer risks in LKB1 germline mutation carriers. Gut 2006; 55:984-90. [CrossRef]

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Respir Case Rep 2018;7(2):118-122 DOI: 10.5505/respircase.2018.93653

OLGU SUNUMU

CASE REPORT

Sarkoidozda Nadir bir Komplikasyon: Pnömotoraks A Rare Complication in Sarcoidosis: Pneumothorax

RESPIRATORY CASE REPORTS

Zühre Taymaz1, Dursun Alizoroğlu1, Ahmet Emin Erbaycu1, Nur Yücel2

Özet

Abstract

Sarkoidoz seyrinde pnömotoraks nadir bir durumdur ve patogenezde subplevral bleb veya büllerin rüptürü ve subplevral granulomların nekrozu sorumlu tutulmaktadır. Sunulan evre 2 hastada, başlangıç akciğer parankim tutulumu yoğun olup her iki akciğeri tümüyle kapsamaktadır. Sistemik kortikosteroid tedavisine klinik ve radyolojik olarak iyi yanıt alınsa da tedavinin ikinci ayında tek taraflı pnömotoraks izlenmiştir. Tüp torakostomi ve kapalı su altı drenajı uygulanmıştır. Hasta, literatür bilgileri eşliğinde nadir bir sarkoidoz seyri olarak sunulmuştur.

Pneumothorax is a rare entity in the course of sarcoidosis, yet the rupture of subpleural blebs or bullae or the necrosis of subpleural granulomas may be pathogenic agents responsible for spontaneous pneumothorax. In the case presented, there was intense initial pulmonary paranchymal involvement in the bilateral lung zones. Though there was a good clinical and radiological response to systemic corticosteroid therapy, unilateral pneumothorax occurred following 2 months of treatment. A tube thoracostomy and underwater sealed drainage was performed. This case is presented with a discussion of the literature as a rare example of sarcoidosis.

Anahtar Sözcükler: Sarkoidoz, pnömotoraks, kortikosteroid, subplevral granulom.

Key words: Sarcoidosis, pneumothorax, corticosteroid, subpleural granuloma.

Sarkoidoz başlıca lenf bezleri ve akciğeri tutan granülomatöz inflamasyonla karakterize multisistemik bir hastalıktır. Diğer organ ve sistem tutulumları, deri, göz, periferik lenf bezleri, karaciğer, lenf bezleri, dalak, kalp ve sinir sistemidir. Akciğer sıklıkla tutulmakla birlikte radyolojik olarak simetrik, bilateral hiler, üst mediastinal lenfadenopati, retiküler, retikülonodüler, fokal alveolar opasiteler tarzında parankimal infiltratif lezyonlar görülmektedir (1). Hastaların %25’inde sarkoidozun nadir akciğer

bulguları olan kaviter nodül, plevral efüzyon, miçetoma, trakeal ve/veya bronşiyal stenoz, lober atelektazi, bül ve pnömotoraks görülebilmektedir (2). Sarkoidozda pnömotoraks atipik bir tutulumdur ve oldukça nadir olup, sıklığı literatürde %2-4 olarak bildirilmiştir. Sıklıkla üçüncü evre ve sonrasında görülmektedir. Çok nadiren hastalığın ilk bulgusu olarak karşılaşılmaktadır (1,3-7). Sarkoidoz nedeniyle tedavi altında olan bir hastada gelişen pnömotoraks, literatür bilgileri eşliğinde sunulmuştur.

Department of Thoracic Diseases, İzmir Dr. Suat Seren Thoracic Diseases and Surgery Training and Research Hospital, İzmir, Turkey 2 Department of Pathology, İzmir Dr. Suat Seren Thoracic Diseases and Surgery Training and Research Hospital, İzmir, Turkey Başvuru tarihi (Submitted): 21.10.2017 Kabul tarihi (Accepted): 25.12.2017 1

İletişim (Correspondence): Ahmet Emin Erbaycu, İzmir Dr. Suat Seren Göğüs Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Hastalıkları Kliniği, İzmir e-mail: afumetsu67@gmail.com

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OLGU Yirmi altı yaşında, özgeçmişinde bir özellik olmayan, sigara içmemiş olan erkek hasta, altı aydır progresif efor dispnesi ve öksürük şikâyeti ile polikliniğe başvurdu. Solunum muayenesinde her iki akciğerin solunuma katılımı eşit ve solunum sesleri azalmış idi. Akciğer radyogramında her iki akciğerde tüm zonları kaplayan retikülonodüler infiltrasyon bulgusu izlendi (Şekil 1). Toraks bilgisayarlı tomografisin (BT)’de en büyüğü 2 cm olan bilateral hiler ve mediastinal lenfadenopati (LAP) ve her iki akciğer parankiminde yaygın milimetrik nodüler infiltrasyonlar, bronş duvar kalınlaşmaları izlendi (Şekil 2).

testi negatif idi. Endobronşial US ile mediastinal LAP’den transbronşiyal iğne biyopsisi ve aynı seansta orta lobdan bronkoalveoler lavaj (BAL) yapıldı. Mediastinal lenf bezlerine kolayca ulaşıldığı için transbronşiyal akciğer biyopsisi alınmadı. BAL yaymalarında %76 alveolar makrofaj, %15 lenfosit, %9 nötrofil lökosit izlendi. Mediastende 4R ve 7. İstasyonlardan alınan lenf bezi biyopsisi “sarkoidozla uyumlu granülamatöz lenfadenit” olarak raporlandı (Şekil 3). Bronş aspirasyon direkt bakısında asidorezistan basil izlenmedi ve kültürde üreme olmadı, PCR (polimeraz zincir reaksiyonu) negatif idi.

Şekil 3: EBUS biyopsisi lenfoid dokuda epiteloid histiositlerden oluşan granülom (H&EX100).

Şekil 1: Akciğer grafisinde iki taraflı parankimal dansiteler.

Hasta radyolojik ve laboratuvar bulgular eşliğinde evre 2 sarkoidoz olarak değerlendirildi ve metilprednizolon tedavisi başlandı. Tedavinin iki ayı tamamlandığında; yakınmalarında düzelme olduğunu söyleyen hastanın akciğer radyografisinde (Şekil 4) ve toraks BT’de solda pnömotoraks bulgusu saptandı (Şekil 5). Tüp torakostomi ve kapalı sualtı drenajı uygulandı. Akciğeri ekspanse olan hastanın drenajı sonlandırıldı. Sistemik steroid tedavisinin altıncı ayında belirgin klinik ve radyolojik iyileşme izlendi (Şekil 6).

TARTIŞMA

Şekil 2: Toraks BT’de iki taraflı parankimal tutulum.

Biyokimyasal ve diğer rutin kan testleri olağan, romatolojik serolojik testler negatif bulundu. Solunum fonksiyon testinde FEV1: 2,54 L (%55), FVC: 2,88 L (%53), FEV1/FVC: %88, Karbon monoksit difüzyon kapasitesi %42 olarak saptandı. Oda havası oksijen satürasyonu %93 idi. Serum ACE düzeyi: 315 U/L, tüberkülin deri Cilt - Vol. 7 Sayı - No. 2

Ülkemizde sarkoidozun tahmini yıllık insidansı 4/100,000 kişi olarak hesaplanmıştır (8). Sarkoidozda en sık gözlenen torasik tutulumlar hiler, mediastinal, paratrakeal lenfadenopati ve parankim tutulumu olmakla birlikte hava yolları, endobronşiyal, plevral ve pulmoner vasküler tutulum da olabilir. Pnömotoraks ise oldukça nadirdir (%2-4) ve genellikle geç dönem fibrotik ve büllöz hastalığın bir komplikasyonu olarak saptanmaktadır. Yine erken dönemde görülmesi oldukça nadirdir (9).

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Sarkoidozda Nadir bir Komplikasyon: Pnömotoraks | Taymaz et al.

Şekil 4: Tedavinin ikinci ayı kontrolünde tek taraflı pnömotoraks.

Şekil 5: Toraks BT’de pnömotoraks görünümü.

Sarkoidozlu 52 hastayı içeren bir seride iki hastada pnömotoraks tespit edilmiştir ve patogenezinde subplevral bleb veya büllerin rüptürü veya subplevral granülomların nekrozu sorumlu tutulmuştur (10). Bildirilen beş farklı hastada; pnömotoraksın üç genç erkekte tesadüfi oluştuğu, ancak diğer iki siyahi kadın hastada geç dönem fibrotik ve büllöz hastalığın tekrarlayan pnömotoraksa neden olduğu belirtilmiştir (11). Sarkoidozda spontan pnömotoraks tek veya iki taraflı olabilmekte, genellikle de bül rüptürüne sekonder ya da subplevral lokalizasyondaki granülomun nekrozu sonucu ortaya çıkmaktadır. Hemorajik ya da non-hemorajik plevral efüzyon ile birlikte de seyredebilmektedir (1,9). Coşkun ve ark. (12) sistemik kortikosteroid tedavisi sürerken gelişen bilateral pnömotoraks (Omori ve ark. (6) sol üst lobda subplevral bleblerin tespit edildiği, pnömotoraks gelişmiş genç bir erkek sarkoidoz olgusu sunmuşlardır. Pnömotoraks her iki tarafta da görülebilmektedir. Değişik seriler sağ veya sol predominanslığı rapor etmişlerdir (5,7).

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Evre 2 olarak sınıflandırdığımız olgumuzda pnömotoraks erken dönemde değil, tedavi sürecinde gelişmiştir. Hastada radyolojik olarak bül saptanmamış, her iki akciğer parankiminde yaygın milimetrik nodüler infiltrasyonlar görülmüştür. Pnömotoraksa yol açan nedenin subplevral lokalizasyondaki granülomların nekrozu olduğu düşünülmüştür. Komplikasyonsuz tek taraflı pnömotoraks olabileceği gibi fatal düzeyde solunum fonksiyonlarını bozabilecek bilateral pnömotoraks da olabileceği bildirilmiştir. Pnömotoraks, genellikle teşhis edilmiş sarkoidozun bir komplikasyonu olarak görülmekle birlikte, hastalığın ilk belirtisi veya relapsların ilk kanıtı olarak da ortaya çıkmaktadır (3,4,6,7). Sunduğumuz hastada ise tedavinin ikinci ayında, klinik ve radyolojik iyileşmenin gözlemlendiği bir dönemde pnömotoraks karşımıza çıkmıştır.

Şekil 6: Toraks BT’de pnömotoraks görünümü.

Sarkoidozda bül, evre 4 hastalıkta sıklıkla görülen lokalize kistik hava boşluklarından farklı bir patolojidir (13). Sarkoidozda bül oluşum mekanizmaları çok açık olmamakla birlikte üç mekanizma üzerinde durulmaktadır; 1. Endobronşiyal tutuluma bağlı bronş veya bronşiollerin obstrüksiyonu sonucunda ortaya çıkan periferik hava yolu hapsinin özellikle öksürük ataklarına bağlı gerilip rüptürü (14,15), 2. Pulmoner parankim etrafındaki retraksiyon ve kollapsın bül formasyonuna yol açabilmesi (4), 3. Çeşitli inflamatuvar medyatörlere bağlı oluşan inflamatuvar alveolitin doku destüriksiyonuna yol açması (4,5). Büllöz sarkoidoz genellikle 3-4 yıllık semptomu olan hastalarda rapor edilmiş, 21-67 yaş aralığı gibi geniş bir aralıkta görüldüğü, hastaların çoğunluğunda hava yolu obstrüksiyonu gözlendiği ve büllerin üst veya alt loblarda eşit olarak olabileceği seriler bildirilmiştir (5). Bu hastalarda solunum fonksiyonlarının restriktif veya normal olabileceği de bilinmektedir.

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Respiratory Case Reports

Hastamızda bül formasyonu izlenmemiştir. Bunun muhtemel nedenleri hiç sigara içmemiş olması, semptomları başlayalı henüz bir yıl olmaması ve erken evre sarkoidoz olması olarak düşünülmüştür. Hem pnömotoraks hem büllöz değişiklikler sarkoidozda oldukça nadir görülmektedir. Hastalığın tanıda göz ardı edilen formlarının da olduğu akılda tutulmalıdır. Plevral lezyonların görüntülenmesinde yüksek rezolüsyonlu bilgisayarlı tomografi (HRCT)’nin BT’ye üstünlüğü bilinmekle birlikte, ne HRCT ne de patolojik örnekleme plevral ve subplevral tutulumları tereddütsüz ayırt edememektedir. HRCT’nin plevral lezyonları göstermedeki üstünlüğü tedavi ve takip yönetimi açısından yararlıdır (16,17). Pnömotoraks yönetimi çok açık olmamakla birlikte, erken dönem steroid tedavisinin pnömotoraks relapsları için yararlı olacağı ifade edilmiş olsa da, karşıt görüş olarak Froudarakis ve ark. (3) steroidsiz takip ettikleri hastalarında, tekrarlayan pnömotoraks gözlemlememişler, sonuçta steroid kullanımını ileri evre, solunum fonksiyonları kısıtlanmış ve semptomatik hastalara önermişlerdir. Kortikosteroide cevapsız pulmoner fonksiyon bozukluğu ile birlikte olan şiddetli büllöz hastalık durumunda, tüp drenajının fayda sağlamadığı durumlarda ya da sık tekrarlayan pnömotorakslarda torakoskopik büllektomi önerilmektedir. Konservatif tedavi yöntemlerine rağmen tekrarlayan pnömotorakslarda cerrahi tedaviler; plevrektomi, dekortikasyon veya bül wedge rezeksiyonu tercih edilmektedir. (1). Hastamızın tanı anında semptomatik olması, radyolojik lezyonların yoğunluğu, solunum fonksiyonlarının kısıtlılığı ve hipoksi nedeniyle sistemik steroid tedavisine hemen başlanmıştır. Yoğun parankim tutulumuyla birlikte muhtemelen plevra tutulumunun da olması ve belirgin öksürük yakınması, spontan pnömotoraks gelişiminin olası nedenleri olarak düşünülmüştür.

SONUÇ Sarkoidozda pnömotoraks genellikle ileri dönemlerde ortaya çıkmaktadır. Subplevral bleb veya büllerin rüptürü veya subplevral granulomların nekrozu sonucu erken dönemde de karşılaşılabilir. Sigara öyküsü olmayan genç hastalarda da asemptomatik pnömotoraks olabileceği akılda tutulmalıdır.

ÇIKAR ÇATIŞMASI Bu makalede herhangi bir çıkar çatışması bildirilmemiştir.

Fikir - Z.T., D.A., A.E.E., N.Y.; Tasarım ve Dizayn - Z.T., D.A., A.E.E., N.Y.; Denetleme - Z.T., D.A., A.E.E., N.Y.; Kaynaklar - Z.T.; Malzemeler - Z.T.; Veri Toplama ve/veya İşleme - Z.T., N.Y.; Analiz ve/veya Yorum - Z.T., D.A.; Literatür Taraması - A.E.E.; Yazıyı Yazan - Z.T.; Eleştirel İnceleme - A.E.E.

KAYNAKLAR 1.

Rastogi R, Joon P, Gupta Y, Majidwani A, Pratap V, Sharma S. Pneumothorax – a rare presenting sign of sarcoidosis. J Gen Practice 2016; 4:256. [CrossRef]

Verschakelen JA. Sarcoidosis: imaging features. Eur Respir Mon 2005; 32:265-83.

Froudarakis ME, Bouros D, Voloudaki A, Papiris S, Kottakis Y, Constantopoulos SH, et al. Pneumothorax as a first manifestation of sarcoidosis. Chest 1997; 112:278-80. [CrossRef]

Judson MA, Strange C. Bullous sarcoidosis: a report of three cases. Chest 1998; 114:1474-8. [CrossRef]

Jeebun V, Forrest IA. Sarcoidosis: an underrecognised cause for bullous lung disease? Eur Respir J 2009; 34:999-1001. [CrossRef]

Omori H, Asahi H, Irinoda T, Itabashi T, Saito K. Pneumothorax as a presenting manifestation of early sarcoidosis. Jpn J Thorac Cardiovasc Surg 2004; 52:335. [CrossRef]

Liu Y, Dai HP, Xu LL, Li X. Recurrent pneumothorax as a presenting manifestation of active sarcoidosis: a case report and literature review. Chin Med J (Engl) 2010; 123:1615-6.

Musellim B, Kumbasar O, Ongen G, Cetinkaya E, Turker H, Uzaslan E, et al. Epidemiological features of Turkish patients with sarcoidosis. Respir Med 2009; 103:907-12. [CrossRef]

Gomm SA. An unusual presentation of sarcoidosisspontaneous haemopneumothorax. Postgrad Med J 1984; 60: 621-3. [CrossRef]

10. Riley EA. Boeck's sarcoid: a review based upon a clinical study of fifty-two cases. Am Rev Tuberc 1950; 62:231-85. 11. Scadding JG. Sarcoidosis, 1st ed. London: Eyre and Spotiswoode;1967:136. 12. Coşkun F, Ursavaş A, Çetinoğlu ED, Dilektaşlı AG, Uzaslan E. Sarcoidosis complicating with bilateral pneumothorax. Respir Case Rep 2012; 1:55-8. [CrossRef] 13. Manika K, Kioumis I, Zarogoulidis K, Kougioumtzi I, Dryllis G, Pitsiou G, et al. Pneumothorax in sarcoidosis. J Thorac Dis 2014; 6: S466-9. [CrossRef]

YAZAR KATKILARI Cilt - Vol. 7 Sayı - No. 2

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14. Pena CM, Cosgrove DM, Eng P, Kirby T, Rice T, Mehta AC. Bullectomies for bullous sarcoidosis. Cleve Clin J Med 1993; 60:157-60. [CrossRef] 15. Zar HJ, Cole RP. Bullous emphysema occurring in pulmonary sarcoidosis. Respiration 1995; 62:290-3. [CrossRef]

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16. Remy-Jardin M, Remy J, Deffontaines C, Duhamel A. Assessment of diffuse infiltrative lung disease: comparison of conventional CT and high-resolution CT. Radiology 1991; 181:157-62. [CrossRef] 17. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. CT and pathological correlation of pulmonary sarcoidosis. Semin Ultrasound CT MR 1995; 16:361-70. [CrossRef]

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Respir Case Rep 2018;7(2):123-126 DOI: 10.5505/respircase.2018.97659

OLGU SUNUMU

CASE REPORT

Radiographic and Scintigraphic Correlation in a Patient with Pulmonary Alveolar Microlithiasis Pulmoner Alveoler Mikrolitiyazisli bir Hastada Radyografik ve Sintigrafik Korelasyon

RESPIRATORY CASE REPORTS

Arzu Cengiz1, Emel Ceylan2, Can Zafer Karaman3

Abstract

Özet

Pulmonary alveolar microlithiasis (PAM) is a rare, idiopathic lung disease that leads to alveolar deposition of calcium phosphate microliths. Most patients are asymptomatic, but radiological features are quite marked and are nearly pathognomonic. Bone scintigraphy can be useful in the detection of early pulmonary calcifications. The imaging findings of a 65year-old male patient who was diagnosed with PAM are described in this report. He presented with nonspecific chest pain, shortness of breath, wheezing, and a cough ongoing for 4 years. There was fine reticular and nodular infiltration seen on a chest X-ray. Chest computed tomography images revealed bilateral, diffuse, fine, calcified interstitial nodules and nodular septal thickening in the middle and lower lung zones. A whole body bone scintigraphy with technetium-99m methylene diphosphonate (Tc-99m MDP) revealed bilateral, diffuse uptake in the pulmonary parenchyma.

Pulmoner alveolar mikrolitiyazis (PAM), alveoler kalsiyum fosfat mikrolitlerinin birikimine yol açan, nedeni bilinmeyen nadir bir hastalıktır. Hastaların çoğu asemptomatiktir, fakat radyolojik özellikleri oldukça gürültülü ve neredeyse patognomoniktir. Kemik sintigrafisi erken pulmoner kalsifikasyonların saptanmasında kullanılabilir. Bu yazıda, PAM tanısı alan 65 yaşında bir erkek hastanın görüntüleme bulguları sunulmuştur. Hasta, dört yıldır devam eden nonspesifik göğüs ağrısı, nefes darlığı, hırıltı ve öksürük şikayetleriyle başvurdu. Akciğer grafisinde ince retiküler ve nodüler infiltrasyon vardı. Toraks bilgisayarlı tomografisi, bilateral orta ve alt zonlarda ince kalsifiye intersitisyel nodüller ve septal kalınlaşma gösterdi. Teknesyum 99m metilen difosfonat (Tc-99m MDP) ile yapılan tüm vücut kemik sintigrafisinde pulmoner parankimde bilateral diffüz tutulum izlendi. Anahtar Sözcükler: Pulmoner alveoler mikrolitiyazis, XRay bilgisayarlı tomografi, kemik sintigrafisi.

Key words: Pulmonary alveolar microlithiasis, X-Ray computed tomography, bone scintigraphy.

Pulmonary alveolar microlithiasis (PAM) is a rare, diffuse lung disease characterized by the extensive intra-alveolar accumulation of calcispherites, which are salts of calcium and phosphate, in the absence of any known disorder of calcium meta1

Department of Nuclear Medicine, Adnan Menderes University Faculty of Medicine, Aydın, Turkey 2 Department of Chest Disease, Adnan Menderes University, Faculty of Medicine, Aydın, Turkey 3 Department of Radiology, Adnan Menderes University, Faculty of Medicine, Aydın, Turkey

bolism. The etiology of PAM remains unclear; however, 50% of cases are familial (1). It has been described in all age groups and the mean age at diagnosis is 35 years. A gender predominance has not been observed (2,3). Although the symptoms 1

Adnan Menderes Üniversitesi Tıp Fakültesi, Nükleer Tıp Anabilim Dalı, Aydın 2 Adnan Menderes Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Aydın 3 Adnan Menderes Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, Aydın

Submitted (Başvuru tarihi): 17.12.2017 Accepted (Kabul tarihi): 18.01.2018 Correspondence (İletişim): Arzu Cengiz, Department of Nuclear Medicine, Adnan Menderes University Faculty of Medicine, Aydın, Turkey e-mail: arzukincengiz@gmail.com

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Respiratory Case Reports

may be unremarkable, radiological features are quite vivid and are nearly pathognomonic. Chest radiographs usually reveal diffuse, bilateral areas of micronodular calcifications that predominate in the middle and lower lung areas. CT scans show numerous, sand-like calcifications throughout the lungs with subpleural and peribronchial distribution. Bone scanning with 99mTcdiphosphonate usually reveals diffuse, intense uptake throughout both lungs (4-6). In this case report, the imaging methods used in the PAM diagnosis of a patient are described.

CASE A 65-year-old man presented with a history of nonspecific chest pain, shortness of breath, wheezing, and a cough ongoing for 4 years. He had substernal burning in the chest that increased with breathing and he reported persistent chest pain on the right chest wall and that his cough increased in the presence of dust. He also complained of fatigue and headache. He had a 25-pack-year smoking history, but he had quit 35 years earlier. He had been suffering from gastric symptoms compatible with gastroesophageal reflux for 25 years, and hypertension for 10 years. As a result of changes on his chest X-ray and thorax computed tomography (CT) images, a lung biopsy had been recommended 10 years ago, but he had declined because of a lack of symptoms at the time. There was no known family history of respiratory diseases. A physical examination revealed normal vital signs with a pulse rate of 76/minute, blood pressure of 135/78 mmHg, and saturation of 96%. There was no sign of dyspnea, cyanosis, edema, or clubbing. A physical examination of the chest was normal, with the exception of minimal crackles in both basal lungs and a wheeze on forced expiration. Blood test findings, including serum calcium concentration, were normal. Pulmonary function tests demonstrated normal findings with forced expiratory volume in the first second (FEV1) was 2.36 (81.2%) liters, the forced vital capacity (FVC) was 3.09 (82.7%), and the ratio of FEV1/FVC was 76.4%. A reversibility test was negative. There was fine reticular and nodular infiltration observed on a chest X-ray, with numerous fine calcifications dispersed to the middle and lower lung zones (Figure 1). The findings were more pronounced in the central areas obscuring the mediastinal borders, but the diaphragmatic outlines were protected.

Cilt - Vol. 7 SayÄą - No. 2

Figure 1: Plain chest radiography showing fine reticular and nodular infiltration with numerous fine calcifications dispersed to the middle and lower lung zones

A chest CT and high resolution computed tomography (HRCT) revealed bilateral, diffuse, randomly distributed, fine, calcified, interstitial nodules and nodular septal thickening in the middle and lower lung zones (Figure 2). Together with the diffuse but centrally located groundglass attenuations, the typical crazy paving pattern was apparent in the mid to lower lung zones. The nodules became more intense in the subpleural areas, close to the diffusely calcified pleural surfaces.

Figure 2: Chest computed tomography image revealing bilateral, diffuse, randomly distributed fine, calcified interstitial nodules and nodular septal thickening in the middle and lower lung zones

Whole body bone scintigraphy was performed after intravenous administration of 20 mCi (740 MBq) technetium99m methylene diphosphonate (Tc-99m MDP). The bone scan revealed diffusely increased Tc-99m MDP uptake in both lungs. In accordance with radiological imaging

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methods, radiotracer uptake was concentrated at the base of the lungs on bone scintigraphy (Figure 3). The patient was diagnosed with pulmonary alveolar microlithiasis (PAM) according to radiological and radionuclide imaging. Because the patient did not agree to an invasive procedure, bronchoscopic examination for histopathological evaluation could not be performed.

Figure 3: Bone scintigraphy showing intense bilateral uptake of technetium-99m methylene diphosphonate in the lungs. Increased uptake was predominantly located in the lower lung regions

DISCUSSION PAM is a rare disease characterized by intra-alveolar calcium deposits. Most patients are asymptomatic and diagnosed incidentally. Calcification in extrapulmonary sites has also been reported, including the pericardium, prostate, and seminal vesicles (7,8). Because PAM appears to be familial in about half of the cases, family members of the patient should be screened using chest radiography after any person in the family has been diagnosed with PAM. Family members of our patient were scanned and none were diagnosed with PAM. While in some cases, the illness has demonstrated slow progression, in other cases, it has worsened over time and lead to pulmonary fibrosis, respiratory failure, and cor-pulmonale. The most common symptoms are dyspnea, cough, chest pain, sporadic hemoptysis, and asthenia (9). In our patient, the major symptoms were a cough

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and nonspecific chest pain. There was no increase in his complaints during follow-up. Imaging findings are highly diagnostic in patients with PAM. Chest radiography usually reveals scattered bilateral micronodular calcifications that have a ‘’sandstorm” appearance. In these patients, small thin-walled subpleural cysts are responsible for the “black pleura” sign seen in the chest radiography. It can be detected more clearly with CT imaging. Tomographic changes are predominant in the inferior and posterior portions of the lungs (4,9,10). On HRCT imaging, the crazy-paving pattern is considered to be very specific and pathognomonic of PAM. This pattern is defined as areas of ground-glass opacity with a thickening of the interposed interlobular septa, which occurs due to the accumulation of calculi in the peripheries of secondary pulmonary lobules (11). Due to the good correlation between the chest CT findings and pathological findings, the diagnosis of PAM can be made on the basis of the typical radiological pattern (9). Bone scintigraphy is an imaging method that may be used to diagnose PAM. A bone scan usually reveals diffuse, intense uptake throughout both lungs, as seen in our patient. Although several mechanisms have been described for this finding, chemical absorption of hydroxyapatite crystals is largely accepted (12). Bone scintigraphy may be useful in the detection of early, small pulmonary calcified nodules that cannot be detected with conventional radiography (6). In conclusion, PAM is a rare disease that has very typical and pathognomonic imaging findings. Bone scintigraphy may be useful, especially in the early phases of the disease when the classic radiographical findings are not yet visible.

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - A.C., E.C., C.Z.K.; Planning and Design - A.C., E.C., C.Z.K.; Supervision - A.C., E.C., C.Z.K.; Funding A.C., E.C., C.Z.K.; Materials - E.C.; Data Collection and/or Processing - A.C.; Analysis and/or Interpretation A.C., E.C., C.Z.K.; Literature Review - A.C., E.C., C.Z.K.; Writing - A.C., E.C., C.Z.K.; Critical Review - A.C., E.C., C.Z.K.

YAZAR KATKILARI Fikir - A.C., E.C., C.Z.K.; Tasarım ve Dizayn - A.C., E.C., C.Z.K.; Denetleme - A.C., E.C., C.Z.K.; Kaynaklar - A.C., E.C., C.Z.K.; Malzemeler - E.C.; Veri Toplama ve/veya www.respircase.com

Respiratory Case Reports

İşleme - A.C.; Analiz ve/veya Yorum - A.C., E.C., C.Z.K.; Literatür Taraması - A.C., E.C., C.Z.K.; Yazıyı Yazan A.C., E.C., C.Z.K.; Eleştirel İnceleme - A.C., E.C., C.Z.K.

Shah M, Joshi JM. Bone scintigraphy in pulmonary alveolar microlithiasis. Indian J Chest Dis Allied Sci 2011; 53:221–3.

Arslan A, Yalin T, Akan H, Belet U. Pulmonary alveolar microlithiasis associated with calcifications in the seminal vesicles. J Belge Radiol 1996; 79:118–9.

Kashyap S, Mohapatra PR. Pulmonary alveolar microlithiasis. Lung India 2013; 30:143-7. [CrossRef]

Ferreira Francisco FA, Pereira e Silva JL, Hochhegger B, Zanetti G, Marchiori E. Pulmonary alveolar microlithiasis. State-of-the-art review. Respir Med 2013; 107:1-9. [CrossRef]

REFERENCES 1.

Sosman MC, Dodd GD, Jones WD, Pillmore GU. The familial occurrence of pulmonary alveolar microlithiasis. Am J Roentgenol Radium Ther Nucl Med 1957; 77: 947–1012.

Castellana G, Lamorgese V. Pulmonary alveolar microlithiasis. World cases and review of the literature. Respiration 2003; 70:549-55. [CrossRef]

Mariotta S, Ricci A, Papale M, De Clementi F, Sposato B, Guidi L, et al. Pulmonary alveolar microlithiasis: report on 576 cases published in the literature. Sarcoidosis Vasc Diffuse Lung Dis 2004; 21:173-81.

Marchiori E, Gonçalves CM, Escuissato DL, Teixeira KI, Rodrigues R, Barreto MM, et al. Pulmonary alveolar microlithiasis: high-resolution computed tomography findings in 10 patients. J Bras Pneumol 2007; 33: 552–7. [CrossRef]

Khaladkar SM, Kondapavuluri SK, Kamal A, Kalra R, Kuber R. Pulmonary alveolar microlithiasis - clinicoradiological dissociation - a case report with radiological review. J Radiol Case Rep 2016; 31:14-21. [CrossRef]

Cilt - Vol. 7 Sayı - No. 2

10. Cluzel P, Grenier P, Bernadac P, Laurent F, Picard JD. Pulmonary alveolar microlithiasis: CT findings. J Comput Assist Tomogr 1991; 15:938-42. [CrossRef] 11. Gasparetto EL, Tazoniero P, Escuissato DL, Marchiori E, Frare E Silva RL, Sakamoto D. Pulmonary alveolar microlithiasis presenting with crazy-paving pattern on high resolution CT. Br J Radiol 2004; 77:974-6. [CrossRef] 12. Fallahi B, Ghafary BM, Fard-Esfahani A, Eftekhari M. Diffuse pulmonary uptake of bone-seeking radiotracer in bone scintigraphy of a rare case of pulmonary alveolar microlithiasis. Indian J Nucl Med 2015; 30:277-9. [CrossRef]

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Respir Case Rep 2018;7(2):127-130 DOI: 10.5505/respircase.2018.10337

OLGU SUNUMU

CASE REPORT

İmmünsüpresif bir Hastada Açıklanamayan Plevral İnfeksiyon: Nocardiozis Unexplained Pleural Infection in an Immunosuppressive Patient: Nocardiosis

RESPIRATORY CASE REPORTS

Gamze Göker1, Ahmet Emin Erbaycu1, Mine Gayaf1, Dursun Alizoroğlu1, Soner Gürsoy2, Mete Demirel3

Özet

Abstract

Plevral Nocardiosis nadir bir plevral tutulumdur ve genellikle akciğer parankim tutulumu ile birliktedir. Sunulan hasta temporal arterit nedeniyle uzun süreli sistemik kortikosteroid tedavisi almıştır. Tedavisi halen sürerken plevral sıvının teşhisi için kliniğimize yönlendirilmiştir. Plevral sıvının kültüründe Nocardia üremesi üzerine trimetoprim-sulfametaksazol tedavisi başlanmıştır. Tedavi ile klinik ve radyolojik iyileşme gözlenmiştir. Plevral Nocardiozis, immünsüprese hastalarda nedeni açıklanamayan plevral sıvıların ayırıcı tanısında akla getirilmelidir.

Pleural involvement in nocardiosis is rare and usually accompanies pulmonary paranchymal involvement. In the case presented here, the patient had been treated with a systemic corticosteroid for a long time for temporal arteritis. He was continuing the treatment when he was referred to our clinic for a diagnosis of the pleural disease. Following a positive pleural culture result for Nocardia, trimethoprim / sulfamethoxazole treatment was initiated. Clinical and radiological improvement was observed with treatment. Pleural nocardiosis should be kept in mind in the differential diagnosis of cases of pleural effusion with an unknown origin, particularly among immunosuppressed patients.

Anahtar Sözcükler: Plevral nocardiozis, plevral sıvı, kortikosteroid, immünsüpresyon.

Key words: Pleural nocardiosis, pleural effusion, corticosteroid, immunsupression.

Plevral boşlukta infeksiyonlar sıklıkla parapnömonik effüzyon ve ampiyem şeklinde görülmektedir. En sık görülen neden tüberküloz ve diğer bakteriyel ajanlardır. Nadiren de olsa fungal, paraziter ve viral ajanlar plevral boşlukta infeksiyona neden olmaktadırlar (1,2).

Nocardiozis; Nocardia cinsinden bakterilerin bazı türlerince oluşturulan lokalize veya sistemik bir infeksiyondur. Nocardia tüm dünyada toprak, organik bitki örtüsü ve suda bulunan aerobik aktinomiçestir. Şimdiye kadar tanımlanan 50 türden 22’si potansiyel patojendir. İnfeksiyon çoğunlukla

1 1

İzmir Dr. Suat Seren Göğüs Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Hastalıkları Kliniği, İzmir 2 İzmir Dr. Suat Seren Göğüs Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Cerrahisi Kliniği, İzmir 3 İzmir Dr. Suat Seren Göğüs Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi, Mikrobiyoloji Bölümü, İzmir

Department of Chest Diseases, İzmir Dr. Suat Seren Training and Research Hospital for Thoracic Medicine and Surgery, İzmir, Turkey 2 Department of Chest Surgery, İzmir Dr. Suat Seren Training and Research Hospital for Thoracic Medicine and Surgery, İzmir, Turkey 3 Department of Microbiology, İzmir Dr. Suat Seren Training and Research Hospital for Thoracic Medicine and Surgery, İzmir, Turkey

Başvuru tarihi (Submitted): 27.10.2017 Kabul tarihi (Accepted): 08.01.2018 İletişim (Correspondence): Ahmet Emin Erbaycu, İzmir Dr. Suat Seren Göğüs Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Hastalıkları Kliniği, İzmir e-mail: afumetsu67@gmail.com

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inhalasyon yoluyla olmaktadır, ancak deri ve subkutan dokuya doğrudan bulaş yoluyla da primer kutanöz nocardiosis gelişebilmektedir (1-3). Pulmoner nokardiozis; nadir ama ciddi fırsatçı infeksiyondur. Genellikle tüberküloz, akciğer kanseri veya apsesini taklit eden subakut ya da kronik nekrotizan pnömoni ile seyretmektedir. Bu hastaların %25’inde plevral efüzyon ve ampiyem şeklinde plevral tutulum ile karşılaşılmaktadır (2,3). Bu yazıda, plevral sıvının ön planda olduğu ve plevral sıvının araştırılması esnasında kültürde üreme sonucunda plevral nocardiozis teşhisi konulan ve başarılı şekilde tedavi edilen bir hasta literatür bilgileri ışığında sunulmuştur.

pürülan sekresyon aspire edildi ve benzer şekilde antrakotik pigmentasyon izlendi. BAL ve bronş aspirasyonu, asidoezistan basil (-), M. Tuberculosis PCR (-) idi ve tüberküloz/mantar kültüründe üreme görülmedi. Bronş aspirasyonu, BAL, post-balgam ve fırça sitolojisi benign idi. BAL hücre sayımına elverişsizdi ve polimorfonükleer lökosit kümeleri izlendi. Balgam kültüründe üreme olmadı. Sağ hemitorakstan yapılan torasentez ile püy vasıflı sıvı alındı. Sitolojisi benign ve nötrofillerden zengindi. Plevra kültüründe gram boyamadaki hücre profili nedeniyle enkübasyon uzatıldı ve modifiye kinyon ile boyama yapıldı. Takipte 4. günden sonra Nocardia species üremesi tespit edildi. Kan aerob ve anaerob kültüründe üreme olmadı.

OLGU Yetmiş beş yaşında erkek hasta, nefes darlığı, sağda sırt ağrısı ve ateş şikâyetleri ile başvurdu. Dış merkezde oral antibiyotik tedavisi verilmiş, şikâyetlerinde gerileme olmamış idi. Özgeçmişinde; hipertansiyon, diyabet, koroner arter hastalığı, geçirilmiş bypass vardı. Hastaya 8 ay önce temporal arterit nedeni ile sistemik kortikosteroid (48 mg/gün metilprednizolon) tedavisi başlanmıştı. Başvurduğunda 12 mg/gün şeklinde tedavisi devam ediyordu. Soygeçmişinde özellik yoktu. Yirmi beş paket yılı sigara öyküsü vardı ve 10 yıldır sigara içmiyordu. Fizik muayenesinde; vital bulguları stabil, dinlemekle sağda bazalde solunum sesleri azalmış idi. Laboratuvarda; glukoz: 267 mg/dl, laktat dehidrogenaz: 429 U/L, üre 75 mg/dl, albümin 3,4 gr/dl, lökosit: 17,4 x10.3/uL, nötrofil: %90,4, arteriyel kan gazında; PO2: 72,1 mmHg, PCO2: 41,5 mmHg, satürasyon %98,9 ölçüldü. Anti HIV 1-2, HbsAg, anti HCV, HbeAg negatif idi. Akciğer grafisinde; sağda heterojen vasıfta dansite ve sinüs kapalılığı saptanmıştır (Şekil 1). Toraks bilgisayarlı tomografisi (BT)’de; sağ hemitoraksta en derin yerinde 6,5 cm ölçülen plevral effüzyon, komşuluklarında akciğer parankiminde distorsiyon oluşturan fibroatelektatik değişiklikler ve fibrotik zeminli yer yer nodüler formasyon oluşturan dansite artışları, üst lobda düzensiz sınırlı konsolidasyon izlendi (Şekil 2). Bronkoskopide endobronşiyal lezyon izlenmedi ve sağ ana bronşta bol pürülan sekresyon aspire edildi. Tüm lob ve segment bronşlarının mukozalarında - en yoğun olarak sağ alt ve üst lob segmentleri ve sol alt lob segmentlerinde olmak üzere - antrakotik pigmentasyon izlendi. Sağ alt lob lateral bazal segment içinden fırça biyopsi alındı ve bronkoalveoler lavaj (BAL) yapıldı. Sol ana bronşta bol Cilt - Vol. 7 Sayı - No. 2

Şekil 1: Akciğer grafisi.

İntravenöz trimetoprim – sulfametaksazol (400/80 mg, 2x1) tedavisi başlandı. Göğüs Cerrahisi Kliniği tarafından kapalı sualtı drenajı uygulandı ve 12 gün sonra sonlandırıldı. Santral sinir sistemi tutulumu açısından beyin BT çekildi, olağan olarak raporlandı. Tedavinin ikinci ayında yapılan kontrolde klinik ve radyolojik iyileşme saptandı (Şekil 3). Göğüs Cerrahisi konsültasyonunda herhangi bir cerrahi işlem düşünülmedi.

TARTIŞMA Nocardia’nın plevral tutulumu özellikle immünsüprese hastalarda akla gelmesi gereken bir durumdur. Plöropulmoner nokardiosizin en sık tespit edilen etkenleri Nocardia brasiliensis ve Nocardia asteroides’dir (1,2,4). Pulmoner nokardiyozisli hastalarda plevral sıvı hemen daima parankimal infiltrasyonla birliktedir. Plevral sıvı seröz ya da püy görünümünde olup kültür pozitif olabilir

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İmmünsüpresif bir Hastada Açıklanamayan Plevral İnfeksiyon: Nocardiozis | Göker et al.

ya da olmayabilir (5). Sunulan hasta plevral sıvının ayırıcı tanısı için kliniğimize yönlendirilmiştir. Toraks BT’de plevral sıvı tümüyle ön planda olsa da sağ üst lobda Nocardiozisin akciğer parankim tutulumu olabilecek bir konsolidasyon mevcuttur. Bu nedenle parankim ve plevranın birlikte tutulumu düşünülmüştür.

rülmektedir. Plevral sıvı bazen tek teşhis aracıdır. Nocardiozis, sıklıkla kortikosteroid tedavi alan ya da sistemik immünsüpresif kişilerde ortaya çıkmaktadır. Örneğin; solid organ nakilleri, diyabet, otoimmün hastalıklar, kronik akciğer hastalığı, kronik granülomatöz hastalık, kronik alkolizm ve HIV infeksiyonu gibi (6). İnhale beklometazon dipropionat kullanırken infeksiyonun görüldüğü olgu da bildirilmiştir (4). Hastamızın öyküsü incelendiğinde temporal arterit teşhisi ile bir yıl önce sistemik kortikosteroid tedavisi başlandığı öğrenilmiştir. Bu zeminde nocardiozisin geliştiği düşünülmüştür.

Şekil 3: Tedavinin ikinci ayında akciğer grafisi.

Şekil 2: Toraks bilgisayarlı tomografi kesitleri.

Plevral nocardia tutulumu ya göğüs duvarından doğrudan yolla ya da akciğer parankim dokusundan geçiş ile gö-

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Plevral sıvının ‘beyin kalp infüzyon kanlı agar’ ve ‘Sabourauds dekstroz agar’ kullanılarak kültüre alınması tanı koydurucudur. Nocardia asteroides yavaş büyüyen bir organizma olduğundan tanı için aerobik kültür en az 2 hafta gözlenmelidir. Klinik örneklerin Gram boyası ile boyanması nocardiozisin erken teşhisinde faydalıdır. Hastaların sadece üçte birinde smear ve kültür pozitifliği birliktedir (1,5). Hızlı ve doğru sonuçlanan güncel moleküler teknikler yaygın kullanılmamakla birlikte artık teşhis sürecinde büyük avantaj sağlamışlardır (7). Teşhis konulan hastada plevral sıvının mikrobiyolojik incelemeleri sırasında, kültürde 4. günde Nocardia üremesi etkeni ayırt etmeyi sağlamıştır. İlginç bir nokta hastadan alınan solunum yolu örneklerinin kültürlerinde (BAL, bronş aspirasyonu) Nocardia ürememesi, plevral sıvıda üreme olmasıdır. Mikrobiyoloji Laboratuvarı’nda BAL rutin pratikte anaerob kültüre alınmamaktadır. Plevral sıvı kültüründe öncelikle gram boyamadaki hücresel profil incelenerek kültür enkübasyonu uzatılmış ve ardından Nocardia üremesi tespit edilmiştir. Sağ üst lobda tespit edilen dansitewww.respircase.com

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nin Nocardia infeksiyonuna bağlı konsolidasyon olduğu ve buradan plevral boşluğa infeksiyonun yayıldığı düşünülmüştür. Plevral nokardiyozisli hastalar sülfonamidler veya uygun alternatif antibiyotiklerle tedavi edilmeli, efüzyona da pnömoni komplikasyonu olarak gelişen plevral efüzyondaki gibi yaklaşılmalıdır (5). Trimetoprim – sulfametaksazol tedavide kullanılan kombinasyondur. Eğer allerji nedeniyle sulfon grubu verilemiyor ise kültürde ilaç direnç paternine göre alternatif bir seçim yapılmaktadır. Nüks eğilimi nedeniyle tedavinin 6-12 ay yapılması önerilmektedir (8). Hastamıza trimetoprim – sulfametaksazol tedavisi başlanmış ve birinci ay kontrolünde belirgin klinik ve radyolojik iyileşme gözlenmiştir.

Taraması - M.G., A.E.E.; Yazıyı Yazan - A.E.E., G.G.; Eleştirel İnceleme - M.D., S.G.

KAYNAKLAR 1.

Saubolle MA, Sussland D. Nocardiosis: Review of clinical and laboratory experience. J Clin Microbiol 2003; 41:4497−501. [CrossRef]

Vohra P, Sharma M, Yadav A, Chaudhary U. Nocardiosis: A review of clinicomicrobiological features. Int J LifeSc Bt Pharm Res 2013; 2:20−8.

Bagali S, Mantur P. Pleural nocardiosis in an immunocompetent patient: A Case Report. J Clin Diagn Res 2016; 10:1-2. [CrossRef]

Gowrinath K, Rao PS, Mohapatra AK, Prakash PY. Pleural nocardiosis. Indian J Chest Dis Allied Sci 2009; 51:169-71.

Feigin DS: Nocardiosis of the lung: chest radiographic findings in 21 cases. Radiology 1986; 159:9-14. [CrossRef]

Yang M, Xu M, Wei W, Gao H, Zhang X, Zhao H, et al. Clinical findings of 40 patients with nocardiosis: A retrospective analysis in a tertiary hospital. Exp Ther Med 2014; 8:25-30. [CrossRef]

Wauters G, Avesani V, Charlier J, Janssens M, Vaneechoutte M, Delmee M. Distribution of nocardia species in clinical samples and their routine rapid identification in the laboratory. J Clin Microbiol 2005; 43:2624-8. [CrossRef]

Wilson JW. Nocardiosis: updates and clinical overview. Mayo Clin Proc 2012; 87:403-07. [CrossRef]

SONUÇ Plevral Nocardiozis çok nadir bir tutulumdur. Nocardiozis çoğunlukla bağışıklığı baskılanmış hastada geliştiğinden bu tanı immünsuprese hastada parankimal infiltrasyon ve plevral effüzyon varlığında düşünülmelidir. Sistemik kortikosteroid alan hastalarda açıklanamayan plevral efüzyon varlığında akla gelmelidir.

ÇIKAR ÇATIŞMASI Bu makalede herhangi bir çıkar çatışması bildirilmemiştir.

YAZAR KATKILARI Fikir - G.G., A.E.E., M.G., D.A., S.G., M.D.; Tasarım ve Dizayn - G.G., A.E.E., M.G., D.A., S.G., M.D.; Denetleme - G.G., A.E.E., M.G., D.A., S.G., M.D.; Kaynaklar G.G.; Malzemeler - G.G., M.D.; Veri Toplama ve/veya İşleme - G.G.; Analiz ve/veya Yorum - M.G.; Literatür

Cilt - Vol. 7 Sayı - No. 2

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Respir Case Rep 2017;7(2):131-133 DOI: 10.5505/respircase.2018.53244

LETTER TO EDITOR

EDİTÖRE MEKTUP

Damage to a Fiberoptic Bronchoscope due to Nasal Septal Deformity

RESPIRATORY CASE REPORTS

Nasal Deviasyona Bağlı Fiberoptik Bronkoskop Hasarı

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To the Editor, A 75-year-old male patient was admitted to the clinic, and it was determined that he had a mass lesion on the left upper lob. A bronchoscopic procedure was planned for the following day in the operating room. Informed consent was obtained from the patient. Topical anesthesia of Cathejell with lidocaine 2% (Montavit Pharmazeutische Fabrik Ges.m.b.H., Absam, Austria) was applied to the oropharynx and both nasal passages. The patient was then placed on continuous cardiac and pulse oximetry monitoring, and supplemental oxygen was applied (generally 3 L/minute by oral cannula), and intravenous access was established. The bronchoscope was passed through the left nostril, and was advanced past the vocal cords, trachea, and right main bronchus without any difficulty. When it was attempted to move the bronchoscope from the right lung to the left lung, friction and resistance was felt on the bronchoscope during withdrawal, but there was no resistance during forward motion. A tube of Cathejell was administered through the nose and the bronchoscope was moved backward and forward. During withdrawal, the friction continued and so lavage was performed and brush and forceps biopsies were taken. When the procedure was completed, withdrawal of the bronchoscope was intended but the friction and resistance increased. One more Cathejell tube was injected alongside the bronchoscope through the nose and the bronchoscope was moved backward and forward again. The resistance continued during withdrawal, and finally the bronchoscope was pulled out by force. There was bleeding from both the mouth and nose. On examination of the bronchoscope, surface tears due to friction were seen (Figure 1). The patient was referred to the Ear, Nose and Throat Clinic because of the bleeding in the operating room and after the necessary intervention was made, the bleeding was stopped, but the

reason for this damage could not be understood. On the following day, endoscopic examination of the nose was performed by members of the Ear, Nose and Throat Clinic and in the left posterior nasal cavity, a horizontal septal spur of nasal deviation obstructing the nasal passage was determined (Figure 2).

Figure 1: Surface tears on the bronchoscope

Figure 2: A horizontal nasal septal deviation obstructing the nasal passage. NS: Nasal septum; IC: Inferior concha

Flexible bronchoscopy is a safe and frequently applied technique for the assessment, diagnosis, and treatment of patients suffering from respiratory disease. The procedure has progressively improved and use has broadened since it was first used in 1968 (1). Flexible bronchoscopy is now accepted as an essential diagnostic and therapeutic instrument in pulmonary diseases (2). Following topical anesthesia

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and sedation, the flexible bronchoscope is usually inserted nasally. The oral route is an alternative; the choice depends upon individual patient characteristics (3). In this case, the nasal route was preferred. Initially, there were no difficulties in the nasal passage. However, when the bronchoscope was withdrawn, friction and resistance were felt. Interestingly, this was only a difficulty in a single direction. In this case, a left-side nasal septal deformity was found. Mladina (4) published a systematic classification of septal deformities in 1987. Nasal septum deformities were described as consisting of 6 basic types. This classification is simple, but very useful for defining septal deformities and is divided into 2 main groups: the first 4 types are vertical and the other 2 are horizontal deformities. In the current case, the nasal septal deformity on the left side was horizontal and resembled type 5. The type 5 deformity, which is known in the literature as a “septal spur,” is typically unilateral and protrudes laterally. The other side of the nasal septum is always normal. This is one of the most frequent deformities found in the general population. The incidence is low during childhood, but increases with age, and in adults, it reaches the greatest frequency (28%). Clinically, people with this type of deformity always have impaired unilateral nasal breathing (5). Unfortunately, the current patient was not questioned about any difficulty in unilateral nasal breathing. Particularly in patients with septal deformities, nasal passage width should be carefully examined before beginning a bronchoscopic procedure. After some time, mucosal edema may increase and nasal passage narrowing may ensue, in which case, if the procedure allows for the extraction, the bronchoscopy must be extracted, or if the procedure does not allow for extraction, the removal of the bronchoscope should not be forced, but the patient should be referred to ear, nose and throat specialists to avoid damage.

Cilt - Vol. 7 Sayı - No. 2

Hasan Kahraman1, Saime Sağıroğlu2, Hüseyin Arpağ1, Nurhan Atilla1, Fulsen Bozkuş1

Department of Chest Disease, Kahramanmaraş Sütçü Imam University, Kahramanmaraş, Turkey 2 Department of Ear Nose Throat Disease, Kahramanmaraş Sütçü Imam University, Kahramanmaraş, Turkey Correspondence (İletişim): Hasan Kahraman, Department of Chest Disease, Kahramanmaraş Sütçü Imam University, Kahramanmaraş, Turkey e-mail: drhasankahraman@hotmail.com

CONFLICTS OF INTEREST None declared.

AUTHOR CONTRIBUTIONS Concept - H.K., S.S., H.A., N.A., F.B.; Planning and Design - H.K., S.S., H.A., N.A., F.B.; Supervision H.K., S.S., H.A., N.A., F.B.; Funding - H.K.; Materials - H.K.; Data Collection and/or Processing - H.K.; Analysis and/or Interpretation - H.K.; Literature Review - H.K.; Writing - H.K.; Critical Review - H.K.

YAZAR KATKILARI Fikir - H.K., S.S., H.A., N.A., F.B.; Tasarım ve Dizayn - H.K., S.S., H.A., N.A., F.B.; Denetleme - H.K., S.S., H.A., N.A., F.B.; Kaynaklar - H.K.; Malzemeler H.K.; Veri Toplama ve/veya İşleme - H.K.; Analiz ve/veya Yorum - H.K.; Literatür Taraması - H.K.; Yazıyı Yazan - H.K.; Eleştirel İnceleme - H.K.

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https://www.uptodate.com/contents/flexiblebronchoscopy-in-adults-overview?source=see_link

Du Rand IA, Blaikley J, Booton R, Chaudhuri N, Gupta V, Khalid S, et al. British Thoracic Society guideline for diagnostic flexible bronchoscopy in adults. Thorax 2013; 68(Suppl1):i1–i44. [CrossRef]

https://www.uptodate.com/contents/flexiblebronchoscopy-in-adults-preparation-proceduraltechnique-andcomplications?source=search_result&search=bronchoscopy&s electedTitle=2~150#H876897868

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Mladina R. The role of maxillar morphology in the development of pathological septal deformities. Rhinology 1987; 25:199–205. [CrossRef]

Mladina R, Skitarelić N, Poje G, Šubarić M. Clinical implications of nasal septal deformities. Balkan Med J 2015; 32:137-46. [CrossRef]

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