JBR 2010-3

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Volume 93 Page 117-170 May-June

Bimonthly

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2010

DIAGNOSTIC AND INTERVENTIONAL IMAGING, RELATED IMAGING SCIENCES, AND CONTINUING EDUCATION

ORGANE DE LA SOCIETE ROYALE BELGE DE RADIOLOGIE (SRBR) ORGAAN VAN DE KONINKLIJKE BELGISCHE VERENIGING VOOR RADIOLOGIE (KBVR)

JBR-BTR ♦ 93/3 ♦ 2010 Journal Belge de ♦ Belgisch Tijdschrift voor ♦ RADIOLOGIE

Founded in 1907 A bimonthly journal devoted to diagnostic and interventional imaging, related imaging sciences, and continuing education Contents

The following section consists of short case reports published through the courtesy of the Editors of “Radiological Documents” and supported by Codali-Guerbet and Guerbet The edition – text and figures – is also available on RBRS website at http:www.rbrs.org

SHORT REPORTS Amiodarone pneumonitis T.A. Bley, C.J. François . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118 Congenitally absent right coronary artery in adult T.A. Bley, Ch. J. François . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120 Multiple inflammatory pseudotumors of the liver and spleen E. Bruyeer, K. Ramboer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 122 Sacciform varix of right pulmonary vein T. Couvreur, B. Ghaye . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Intestinal acute graft versus host disease E. Danse, C. Dragean, C. Rovani . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126 Typical imaging features in Hunter’s syndrome L. De Cocker Jr, P.J. Hofkens, V. Van den Bergh, J. Proost, B. Caerts, G. De Vriendt . . . . . . . . . . . . . . . . . . . . . . 128 Acute respiratory symptoms in Mounier-Kuhn syndrome P.A. de Jong, J.K. Vette, L. Meiss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 Idiopathic bone cyst of the mandible S. Dekeyzer, F.M. Vanhoenacker, K. Chapelle . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132 Abdominal and thoracic tuberculosis in a HIV positive patient D. Duterloo, S.T. Bodewitz . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134 Postintubation membranous tracheal rupture B. Grayet, B. Duysinx, B. Ghaye . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 136 Symptomatic brain capillary telangiectasia B. Heremans, G. Wilms, G. Marchal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138 Portohepatic shunt in a patient without cirrhosis A. Kucuk, S. Poturoglu, O. Gurbuz, Y. Savas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140 Small bowel obstruction due to bezoar in jejunal diverticulosis Y. Maréchal, E. Danse, H. Paterson, A. Kartheuser . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Mass in the back as manifestation of neurofibromatosis type 2 A.S. Michel L. van den Hauwe, H. Degryse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144 Metastases in the breast from disseminating melanoma A. Oehlers-Wetzel, C. Meijers, R. den Toom, G. Risseeuw . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 146 Probable cerebral amyloid angiopathy B. Pilet, O. Özsarlak, C. Venstermans, P.M. Parizel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148 Polyarteritis nodosa of the spleen M. Smets, B. Claikens, P. Van Wettere, G. Lambrecht . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Glomus tumor of the stomach K. Van den Bergh, P. Lefere . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152 Pneumocystis carinii pneumonia in a HIV-positive patient A.H.M. van Heese, G.A. Risseeuw . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154 Ruptured renal angiomyolipoma F. Van Hoorn, M. de Win, M. Meier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156

Left pulmonary artery agenesis B.J. Van Kelckhoven, A.P.G. Van Gils . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158 Pneumatosis coli and incarcerated inguinal hernia A. Van Landeghem, B. Arys, N. Peters, K. Verstraete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160 Abdominal mass due to crossed renal ectopia and fusion E.J. Vandervliet, F.M. Vanhoenacker, P.M. Parizel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Rhinolith of the nasal septum E.J. Vandervliet, F.M. Vanhoenacker, J. Van Haesendonck, P.M. Parizel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 Lipoma arborescens of the knee T. Weits, B.H. Tania . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166 Abstract of papers for full membership at the Royal Belgian Society of Radiology . . . . . . . . . . . . . . . . . . . . . . . . 168 Forthcoming Courses and Meetings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Classified services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170 Instructions to Authors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ii Subscribers information . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . cii Advertising index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ciii

Important

Dear Readers, For reasons beyond our control (failure of the postal service), we cannot guarantee that all the corrections mentioned on the galleyproof were duly introduced in the final text.

Please accept our apologies.

The terms used for indexation of subjects were developed by the Radiological Society of North America (RSNA) over a period of years. Their use here is by permission of the RSNA. The terms may not be used in any other index, print or electronic, except by specific permission of RSNA. ◆◆ Indexed in Index Medicus and in Zentralblatt Radiologie. Evaluated for Medline User, EMBASE and CANCERNET. Abstracted in Excerpta Medica Journals. ◆

JBR–BTR, 2010, 93: 118-119.

AMIODARONE PNEUMONITIS T.A. Bley1,2, C.J. François1 Key-word: Lung, effects of drugs on

Background: A 65-year-old male patient presented with progressive shortness of breath over the past 3 months, recently even at rest. He neither complained about fever nor about chest pain.

The laboratory findings were normal with only a slight increase in leucocytes.

A significant restrictive ventilation disorder was noted on plethysmography. His past medical history included three vessel coronary artery disease, arterial hypertension with hypertensive cardiomyopathy and atrial flutter. Carcinoma of the urinary bladder and prostate cancer were diagnosed and successfully treated 10 and 12 years earlier, respectively.

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1. Department of Radiology, University of Wisconsin–Madison, Madison, Wisconsin, USA and 2. Department of Diagnostic Radiology and Medical Physics, University Hospital Freiburg, Germany

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AMIODARONE PNEUMONITIS — BLEY et al

Work-up Chest X-ray (Fig. 1, A: AP view and B: lateral view) shows asymmetrical reticular opacities, mainly in the right lower and middle lobe. Please note volume loss of the right lung as depicted by elevation of the diaphragm and mediastinal shift to the right.

Chest CT (Fig. 2) (lung window settings) demonstrates thickening of the bronchial walls and interand intralobular septae as well as ground glass opacities and peripherally located areas of consolidation. Bilateral pleural effusions are present, greater on the right.

Chest radiographs after six months following cessation of amiodarone and after corticosteroid treatment (Fig. 3, A: AP view and B: lateral view) shows complete remission of the initial findings.

The reticular pattern with patchy opacities that was seen initially has resolved entirely.

Radiological diagnosis Given the history of this patient, which also included the use of amiodarone, drug induced lung toxicity, amiodarone pneumonitis was suspected.

The diagnosis was validated by trans-bronchial biopsy. Amiodarone treatment was stopped and treatment with corticosteroid treatment was initiated. Differential diagnosis of amiodarone pneumonitis include: ventricular dysfunction, infectious, eosinophilic, or organizing pneumonia, pulmonary infarction, exogenous lipoid pneumonia, bronchoalveolar carcinoma and lymphoma.

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for several months and that is often accompanied by malaise, nonproductive cough, and pleuritic chest pain. Elevated erythrocytes sedimentation rate and leucocytosis are commonly found.

Amiodarone pneumonitis manifests in the pulmonary interstitium or in the alveolar space.

Diuresis helps to distinguish amiodarone pneumonia from interstitial pulmonary edema. To confidently establish a specific diagnosis, a lung biopsy of significant size may be required, the recognized diagnostic gold standard.

On chest radiographs and CT scan, focal, patchy or diffuse opacities may be found bilaterally, typically peripheral in location. These opacities may have high attenuation on non-contrast-enhanced CT as amiodarone gets incorporated into type II pneumocytes.

Amiodarone pneumonitis may result in amiodarone induced pulmonary fibrosis, which develops in approximately 5-7% of patients with amiodarone pneumonitis. Amiodarone induced pulmonary fibrosis is an irreversible condition with limited or short term response to corticosteroid treatment and an adverse outcome. Mortality among hospitalized patients with amiodarone pneumonitis is high with approximately 30%. Chest radiographs and pulmonary function tests are recommended in every patient to detect onset of potential amiodarone pneumonitis. Follow-up studies are warranted within the first two years on a regular basis, especially in patients who are at greater risk to develop amiodarone pneumonitis, e.g. patients with poor lung function, COPD, pulmonary emphysema or previous pulmonary surgery.

Discussion Bibliography Amiodarone is a class III anti-arrhythmic drug used for treatment of refractory cardiac tachyarrhythmias.

It accumulates in the liver and lung, and may lead to potentially fatal pulmonary toxicity in 5% of the patients.

Amiodarone pneumonitis was first described in 1980. Its prevalence in patients treated with amiodarone reached up to 15%. With higher age and higher dosage of amiodarone, the risk of amiodarone pneumonitis increases. Due to its long tissue half life, both onset of lung toxicity and clearing following cessation may take several months.

However, pulmonary toxicity may begin after a few days of amiodarone usage or more than a decade after initiation of treatment. Patients typically present with progressive dyspnea that may have lasted

1. Camus P., Marin W.J., Rosenow E.C.: Amiodarone pulmonary toxicity. Clin Chest Med, 2004, 25: 6575.

2. Kuhlman J.E., Teigen C., Ren H., et al.: Amiodarone pulmonary toxicity: CT findings in symptomatic patients. Radiology, 1990, 177: 12125.

3. Polverosi R., Zanellato E., Doroldi C.: Thoracic radiography and high resolution computerized tomography in the diagnosis of pulmonary disorders caused by amiodarone. Radiol Med, 1996, 92: 58-62.

4. Rotmensch H.H., Liron M., Tupilsky M., et al.: Possible association of pneumonitis with amiodarone therapy. Am Heart J, 1980, 100: 412-413.

JBR–BTR, 2010, 93: 120-121.

CONGENITALLY ABSENT RIGHT CORONARY ARTERY IN ADULT T.A. Bley1,2, Ch. J. François1 Key-word: Coronary arteries, abnormalities

Background: A 44-year-old male patient with atrial fibrillation and cardiac insufficiency (NYHA IV) presented for evaluation for heart transplantation. He was referred for cardiac CT for assessment of potential restrictive cardiomyopathy and evaluation of his right ventricle. Cardiac CT was performed utilizing a dual-source CT scanner. ECG-adapted tube current was used to reduce radiation exposure. No pericardial calcifications were noted. The right atrium and the inferior vena cava were dilated with 82 x 58 mm and 47 mm diameter, respectively. There was no evidence of coronary artery disease.

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Department of 1. Radiology, University of Wisconsin, Madison, Wisconsin, USA and Department of 2. Diagnostic Radiology and Medical Physics, University Hospital Freiburg, Germany

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CONGENITALLY ABSENT RIGHT CORONARY ARTERY IN ADULT — BLEY et al

Work-up Transversal thin maximum intensity projection (thin MIP) of contrast-enhanced coronary CT (Fig. 1) demonstrates normal caliber of the left anterior descending artery and proximal left circumflex artery, and small caliber of the distal LCx in the right atrioventricular grove. The right coronary artery is missing.

3D volume rendered technique (VRT) of contrastenhanced cardiac CT (Fig. 2) shows normal course of the LAD. The LCx is elongated and bends around the heart into the right atrioventricular grove, a vascular territory that normally is supplied by the right coronary artery. Please note the regular diameter of the proximal LCx as compared to the small lumen of the distal LCx within the right atrioventricular grove.

VRT (Fig. 3A) and inverted angio view of the same 3D data set (Fig. 3B) display the coronary artery tree. The left circumflex artery is elongated and reaches into the territory of the absent right coronary artery. No coronary artery stenosis is present. (LAD = left anterior descending artery, LCx = left circumflex artery, RV = right ventricle, LV = left ventricle, RA = right atrium, LA = left atrium).

Radiological diagnosis The CT-findings reveal an explicitly rare coronary artery anomaly consisting of entirely absent right coronary artery. Instead, the left circumflex artery (LCx) is elongated and has a course around the apex of the heart into the right atrioventricular grove.

The lumen of the LCx and its marginal branches is patent. The left main and the left anterior descending artery (LAD) with its diagonal branches are normal.

Discussion Coronary artery anomalies are a relatively rare finding. However, with increasing use of non invasive imaging modalities such as MRI and CT depiction of coronary anomalies becomes more frequent and the incidence is reported to be as high as 5.6%.

An ectopic RCA, arising from right sinus or the left sinus are the most common coronary anomalies with an incidence of 1.13% and 0.92%, respectively.

In a large study of 1950 angiograms an absent left main artery and left circumflex artery arising from

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the right sinus were the next most common coronary anomalies with an incidence of 0.67% each.

Absence of the right coronary artery is an explicitly rare finding. In a large analysis of 58 023 coronary angiographies only two cases (0.0034%) of absent RCA were detected. However, its true incidence in the general population is unknown.

The clinical presentation of coronary artery anomalies may vary from entirely asymptomatic patients to exertional angina, myocardial ischemia or even sudden cardiac death. Coronary artery anomalies with an interarterial course, i.e. between the pulmonary artery and the ascending aorta, are thought to be most hemodynamically significant.

Conventional coronary angiography may display the presence of a coronary anomaly. However, for defining the ostial origin and exact course of anomalous coronary arteries multidetector ECGgated CTA has been shown to be superior to angiography. Coronary MR-angiography (MRA) may also be used to noninvasively identify coronary artery anomalies, without the use of ionizing radiation or contrast agent. Both CTA and MRA are noninvasive diagnostic modalities that eliminate the risks inherent in invasive angiography while covering an entire volume of interest in high spatial and temporal resolution.

This report illuminates the benefit of noninvasive coronary CTA for assessment of coronary anomalies in a rare case of an absent right coronary artery with an elongated left circumflex artery.

Coronary artery and pericardial disease could be excluded and the exact course of the anomalous left circumflex could readily be revealed.

Bibliography 1. Angelini P.: Coronary Artery Anomalies: An Entity in Search of an Identity. Circulation, 2007, 115: 1296-1305.

2. Goel M.K., Oezatik M.A., Kunt A., et al.: Coronary anomalies in adult patients. Med Sci Monit, 2002, 8: CR 636-641.

3. Kim S.Y., Seo J.B., D.O., K.-H., et al.: Coronary Artery Anomalies: Classification and ECG-gated Multi-Detector Row CT-Findings with Angiographic Correlation. RadioGraphics, 2006, 26: 317-334.

4. Manghat N.E., Morgan-Hughes G.J., Marshall A.J., et al.: Multidetector row computed tomography: imaging congenital coronary artery anomalies in adults. Heart, 2005, 91: 1515-1522.

JBR–BTR, 2010, 93: 122-123.

MULTIPLE INFLAMMATORY PSEUDOTUMORS OF THE LIVER AND SPLEEN E. Bruyeer, K. Ramboer1 Key-word: Liver neoplasms, diagnosis

Background: A 43-year-old woman presented with periods of fever of 4 weeks duration, epigastric pain and significant loss of weight (16 kg). Laboratory findings revealed elevated C-reactive protein and slightly elevated liver function tests. She had a history of gastric banding 7 years before, which has been removed 8 weeks before. This surgery was complicated by local abscess formation.

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1. Department of Radiology, AZ St-Lucas, Brugge, Belgium

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MULTIPLE INFLAMMATORY PSEUDOTUMORS OF THE LIVER AND SPLEEN — BRUYEER et al

Work-up Ultrasonography of the upper abdomen (Fig. 1) (A: sagittal section through the liver and B: sagittal section through the spleen) reveals multiple hypoechogenic, ill-defined nodules in liver and spleen.

Contrast-enhanced CT scan of the upper abdomen (Fig. 2) demonstrates moderately enhancing multiple lesions in liver and spleen indicating a hypovascular aspect. The lesions show a slow centrifugal pattern of enhancement, as shown on the scans obtained during arterial phase (A), venous phase (B) and delayed phase (C).

On follow-up contrast-enhanced CT scan of the upper abdomen, 6 weeks later, following administration of antibiotics (Fig. 3) a spectacular decline of the number and size of the lesions of the liver and the spleen is noticed.

Radiological diagnosis Screening revealed no primary malignant tumor.

Percutaneous biopsy was performed. Histopathological examination revealed the diagnosis of inflammatory pseudotumor of liver and spleen.

Based on the clinical and radiological findings, the differential diagnosis included metastasis and multiple inflammatory pseudotumors.

Discussion Inflammatory pseudotumor is a benign mass with fibroblastic proliferation and infiltration of chronic inflammatory cells, with a predominance of plasma cells, histiocytes, lymphocytes and some eosinophils.

The lesion can occur in nearly every site of the body, but most commonly involves lung and orbit.

Inflammatory pseudotumor of the liver and spleen is extremely rare and mostly presents as a large, solitary mass, although multiple tumors have been described. The etiology remains unclear.

Symptoms related to inflammatory pseudotumor of the liver include fever, epigastric pain, vomiting, general malaise and weight loss.

Leucocytosis and C-reactive protein elevation have frequently been found. Liver function tests may be normal or enzyme levels may be slightly elevated.

Radiological findings of inflammatory pseudotumor are non specific and variable. On ultrasonography the lesions appear as heterogeneous and hypo-, hyper- or isoechogenic mass lesions.

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On CT, the masses present as ill-defined, hypoattenuating lesions. Following administration of contrast medium, only moderate degree and a variety of patterns of enhancement have been noted. No characteristic enhancement pattern was apparent. On CT scans during delayed phase the masses frequently exhibit hypo-attenuating areas with iso- or hyperattenuating thickened periphery.

The radiographic appearance depends on differing ratios of cellular infiltration to fibrosis.

In the presented case, no significant fibrosis was seen on pathologic examination, and there was a proliferation of myofibroblast-like cells. The areas with hypoechogenicity and low attenuation on CT scan corresponded to predominantly cellular infiltration. Delayed peripheral enhancement can be explained by accumulation of contrast material in the extravascular space.

In summary the radiological findings are not characteristic for inflammatory pseudotumor of the liver.

Therefore percutaneous biopsy should be performed.

The differential diagnosis includes liver abscess, metastasis, peripheral cholangiocarcinoma and hepatocellular carcinoma. In the presented case, however, the combination of lesions in liver and spleen excludes a primary liver tumor.

Inflammatory pseudotumor of the liver should be included in the differential diagnosis in patients with hepatic masses. Because the mass can regress spontaneously or be managed conservatively, awareness of this condition and recognition of its manifestation may guide to perform a liver biopsy and avoid unnecessary surgery.

Bibliography 1. Das Narla L., Newman B., Spottswood S., et al.: Inflammatory pseudotumor. Radiographics, 2003, 23: 719-729.

2. Fukuya T., Honda H., Matsumata T., et al.: Diagnosis of inflammatory pseudotumor of the liver: Value of CT. AJR, 1994, 163: 1087-1091.

3. Nam K.-J., Kang H., Lim J.-H., et al.: Inflammatory pseudotumor of the liver: CT and sonographic findings. AJR, 1996, 167: 485-487.

4. Yoon K.-H., Ha K., Lee J., et al.: Inflammatory pseudotumor of the liver in patients with recurrent pyogenic cholangitis: CT-histopathologic correlation. Radiology, 1999, 211: 373-379.

JBR–BTR, 2010, 93: 124-215.

SACCIFORM VARIX OF RIGHT PULMONARY VEIN T. Couvreur, B. Ghaye1 Key-word: Varices

Background: An 83-year-old woman was admitted initially for evaluation of cardiac failure, secondary to persistent atrial fibrillation. She presented with increasing dyspnea on exertion and oedema of lower limb for some days.

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1. Department of Medical Imaging, University Hospital Sart-Tilman, Domaine Universitaire du Sart-Tilman Liège, Belgium

SACCIFORM VARIX OF RIGHT PULMONARY VEIN — COUVREUR et al

Work-up Chest radiography (Fig. 1) shows on the PA view (A) and profile view (B), besides cardiomegaly, a right paracardiac rounded opacity of 15 mm is seen.

Contrast-enhanced CT scan of the thorax (Fig. 2) includes a transverse section (A), a reformatted image in the coronal plane (B) and a reformatted image in the sagittal plane (C). All images reveal a sacciform vascular structure measuring 18 mm in diameter, that expands from the inferior root of the right inferior pulmonary vein.

Contrast-enhanced CT scan with volume rendering (Fig. 3) clearly shows the sacciform vascular structure, arising from the right inferior pulmonary vein.

Radiological diagnosis Based on CT-findings, the diagnosis of sacciform pulmonary varix originating from the inferior root of the right inferior pulmonary vein is made.

Discussion Congenital anomalies of the pulmonary veins presenting with abnormal diameter can be classified in stenosis, atresia and varices. Pulmonary varix (PV) is defined as a localized enlargement or an aneurysmal dilatation of a segment of a pulmonary vein that drains normally into the left atrium. The first case of a PV was reported by Puchlet in 1843, as a fortuitous finding in a newborn autopsy. This anomaly is rare as only 71 cases have been reported up to 1988. There is no gender, nor age predilection. PV is generally considered to be congenital, but mitral valvular disease was noted in 26-33%. Other congenital diseases can coexist, including hypoplastic pulmonary artery, anomalous pulmonary venous drainage, patent ductus arteriosus, and atrial or ventricular septal defect.

Symptoms are unusual, but rare cases of dyspnea or hemoptysis have been reported. Two fatal spontaneous ruptures into pleural cavity have been reported. Another patient died from a varix communication with a bronchus. Two cases of systemic embolism consecutive to in situ thrombosis in a pulmonary varix have been observed.

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According to imaging, PV can be morphologically classified in saccular, tortuous and confluent types.

These varices are preferentially situated in the right lower lobe (60%), followed by the left upper lobe (17%), the right upper lobe (8%), the right middle lobe (4%) and the left lower lobe (4%). This lesion is a fortuitous finding at chest x-ray and appears as a mass with smooth, well-defined, and sometimes lobulated margins. Differential diagnosis is similar as for all coin lesions, including bronchogenic carcinoma, pulmonary arteriovenous fistula, granulomatous disease, etc. CT scan and MRI provide the correct diagnosis. Spiral CT is now defined as the method of choice in the evaluation of congenital anomalies of the pulmonary venous system. This method is noninvasive and can also estimate other eventual congenital malformations in a single examination.

Bartram and Strickland have described the five angiographic criteria necessary to diagnose a PV: normal arterial phase, opacification of the varix at the pulmonary venous phase, direct drainage into the left atrium, delayed emptying, and anomaly located in the proximal portion of the pulmonary vein.

According to literature, a follow-up with chest xray is necessary and sufficient to monitor the size of the varix. The size of the varix does not increase when there is no pulmonary venous hypertension, while an acute increase in size indicates an elevation of the left atrial pressure and then surgical treatment must be considered. Radiological disappearance of the pulmonary varix has been reported in 4 cases and reduction of the size in 1 case, after prosthetic valvular replacement.

Bibliography 1. Andrikakos P., Gallis P., Christopoulos A., et al.: An asymptomatic pulmonary hilar mass: a case report. Acta Radiol, 2004, 45: 516-518.

2. Asayama J., Shiguma R., Katsume H., et al.: Pulmonary varix. Angiology, 1984, 35: 735-739.

3. Bartram C., Strickland B.: Pulmonary varices. Br J Radiol, 1971, 44: 927-935.

4. Uyama T., Monden Y., Harada K., et al.: Pulmonary varices: a case report and review of the literature. Jpn J Surg, 1988, 18: 359-362.

JBR–BTR, 2010, 93: 126-127.

INTESTINAL ACUTE GRAFT VERSUS HOST DISEASE E. Danse, C. Dragean, C. Rovani1 Key-word: Grafts

Background: We present a case of a 23-year-old man having drug addiction. He is managed by the department of hematology for an idiopathic medullar aplasia. Two months before his admission, he was treated with bone marrow transplantation. During follow-up, we noted a nonspecific recent cutaneous lesion. He was admitted for acute digestive symptoms including anorexia, nausea, vomiting and abdominal pain.

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1. Department of Radiology, UniversitĂŠ Catholique de Louvain, St Luc University Hospital, Brussels, Belgium

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INTESTINAL ACUTE GRAFT VERSUS HOST DISEASE — DANSE et al

Work-up Contrast-enhanced CT scan view of the upper abdomen (Fig. 1) shows on section at the level of the upper abdomen enhancement of the mucosal layer of the jejunal loops and wall thickening of the left colon.

CT scan, sections at the level of the pelvis (Fig. 2), demonstrates mild thickening of the small bowel loops with increased enhancement of the mucosal layer of the intestine.

Abnormal visualization of the small vessels of the mesenteric root, so-called an engorgement of the vasa-recta giving the “the comb sign”, is also observed.

Radiological diagnosis Based on the radiological findings and the clinical story, the diagnosis of acute graft versus host disease of the bowel was suggested and was furtherly confirmed with endoscopic and histologic data.

Discussion The acute graft versus host disease (GVH) is a disease due to reaction of T-lymphocytes of the graft against the host. The mature lymphocytes of the graft are presenting a cytotoxic action on target organs of the receiver , including skin, liver and intestine. Concerning the intestine, the basic lesions are glandular necrosis of the cryptic epithelium and lymphocytic infiltration.This condition is mainly reported in the follow-up of bone marrow transplantation (within the 100 first days), more frequently in allogenic transplantations. It has a mortality of 15%. The usual clinical findings are abdominal cramps, diarrhea, fever, nausea and vomiting. The differential diagnoses to consider are gastro-intestinal infections, neutropenic colitis and pseudomembranous colitis. Other conditions are the intestinal consequences of chemotherapy and/or radiotherapy.

The prognosis of this condition is depending of its early diagnosis. The treatment is based on immunosuppressive medications and diet, with a resolution in 75% of the cases. The imaging findings can be visible along the entire intestinal tract.

The most frequently affected sites are the oesophagus, small bowel (ileum) and the colon. Imaging changes include thickening of the bowel wall, dilatation, abnormal enhancement of the mucosa

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and/or serosa, and engorgement of the vasa recta.

On CT, the thickening is usually mild (5 mm), with small bowel thickening noted in 100%, colonic wall thickening in 59%, gastric or oesophagal thickening in 9%. The increased enhancement of the intestinal layers is noted on the mucosal layer in 54% and the serosal enhancement in 31%. A bowel distension is noted in 23%. The engorgement of the vasa recta (“comb sign”) is visible in 91% and the stranding of mesenteric fat is present in 73%.

The list of CT abnormalities observed in the larger group of neutropenic patients with intestinal complications is large. Neutropenic enterocolitis is the first complication observed on CT in 70% of these patients. Colic wall changes are combined with small bowel abnormalities. Pneumatosis can be noted in the inflamed intestinal wall.

The second intestinal complication is a Clostridium Difficile colitis, noted in 18% of the cases, with an increased wall thickening of the colon (12 mm) combined with wall nodularity, ascitis and mesenteric stranding. GVH is the third intestinal complication, noted in 9% of the case. The location of the bowel disorder is more frequently the small bowel followed by the colon. The imaging findings include mucosal enhancement, wall thickening (~ 5 mm), and small bowel dilatation. Other intestinal disorders in neutropenic patients are cytomegalovirus colitis and ischemic bowel changes.

As a take home message, when CT is required in neutropenic patients having acute abdominal pain, CT can help to suggest GVH intestinal disease in a compatible clinical condition (bone marrow transplantation), by showing enhancement of the mucosa and engorgement of the vasa recta.

Bibliography 1. Kalantari B.N., Mortele K.J., Cantisani V., et al.: CT features with pathologic correlation in acute gastrointestinal graft-versus host disease after bone marrow transplantation in adults. AJR, 2003, 181: 1621-1625.

2. Kirkpatrick I.D., Greenberg H.M.: Gastrointestinal complications in the neutropenic patient: characterization and differentiation with abdominal CT. Radiology, 2003, 226: 668-674.

3. Schmit M., Bethge W., Beck R., et al.: CT of gastrointestinal complications associated with hematopoietic stem cell transplantation. AJR, 2008, 190: 712-719.

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TYPICAL IMAGING FEATURES IN HUNTER’S SYNDROME L. De Cocker Jr1, P.J. Hofkens2, V. Van den Bergh3, J. Proost3, B. Caerts1, G. De Vriendt1 Key-word: Mucopolysaccharidosis

Background: A 17-year-old boy with known Hunter’s syndrome was admitted to the emergency department after an episode of generalized seizures. The episode of seizures was probably related to medication. He had a characteristic coarse facial appearance and macrocrania. There was no mental retardation, dwarfism nor deafness.

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Department of 1. Radioloy, 2. Internal Medicine and 3. Neurology, Turnhoutse Associatie van Ziekenhuizen, Turnhout, Belgium

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TYPICAL IMAGING FEATURES IN HUNTER’S SYNDROME — DE COCKER et al

Work-up CT scan of the brain (Fig. 1) shows on A multiple cyst-like hypodense lesions in the subcortical and deep white matter. There is marked thickening of the calvarium. Section through frontal and temporal lobes (B) shows the same small hypodense lesions in the white matter. Bilateral temporal arachnoid cysts and megacisterna magna can be seen. Note macrocrania and thickening of the skull.

CT scan of the cervical spine, sagittal MPR image (Fig. 2), demonstrates characteristic thickening of the posterior longitudinal ligament in the upper cervical spine.

MRI of the brain (Fig. 3), sagittal T2-weighted MRimage (A), shows cribriform hyperintense lesions are noted in the subcortical and deep white matter, with extensive involvement of the corpus callosum.

The lesions show signal intensities equal to cerebrospinal fluid at all sequences and represent dilated perivascular spaces of the white matter.

Also note megacisterna magna. On coronal FLAIRimage (B), dilated perivascular spaces are seen throughout the white matter. Note signal intensities of these cyst-like lesions equal to cerebrospinal fluid. On axial T2-weighted image (C), bilateral anterior temporal arachnoid cysts are seen as well as megacisterna magna. Cribriform changes in the temporal white matter are obvious.

Radiological diagnosis The numerous cyst-like lesions in the subcortical and deep white matter, with CSFdensity on CT scan and CSF-intensity on MRI, the macrocrania and thickening of the skull and posterior longitudinal ligament in the spine constitute the typical findings of Hunter’s syndrome (mucopolysaccharidosis type II).

Discussion The presented case illustrates the clinical and imaging findings typical of Hunter’s syndrome (mucopolysaccharidosis II). The mucopolysaccharidoses are a hetero-geneous group of inherited lysosomal storage disorders characterized by failure to degrade glycosaminoglycans or mucopolysaccharides. All mucopolysaccharidoses (MPS) are autosomal recessive except Hunter,

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which is X-linked. MPS-patients have a variable degree of phenotype presentation (depending on enzyme dysfunction), ranging from mild to severe.

Our patient has the mild form of Hunter’s syndrome with a typical facial appearance as predominant clinical finding.

The brain findings in patients with MPS are of two types. Dilated perivascular spaces, megacisterna magna, arachnoid cysts and communicating hydrocephalus result from impaired reabsorption of cerebrospinal fluid, presumably due to build-up of glycosaminoglycans in the leptomeninges with arachnoid membrane destruction and a ball-valve effect. Dilated perivascular spaces in patients with MPS were previously falsely believed to be due to macroscopic deposition of mucopolysaccharides.

The presence of multiple dilated perivascular spaces in the corpus callosum is a classic feature of MPS. The other spectrum of findings in Hunter’s syndrome primarily affects the brain parenchyma and consists of white matter T2 hyperintensities and atrophy. The latter findings are not prominent in our patient.

Other characteristic imaging features of Hunter’s syndrome seen in the presented case include macrocrania and thickening of the skull. Thickening of the posterior longitudinal ligament typically occurs in the upper cervical region and is also nicely illustrated in this case.

In conclusion, MPS should be considered when dilated perivascular spaces with predominant involvement of the corpus callosum are observed.

Other findings, such as macrocrania and thickened skull should be looked at to raise confidence in suggesting the diagnosis if it is not already known.

The severe form of Hunter’s syndrome presents in the early years but the mild form might be diagnosed as late as the teenage, with characteristic coarse facial appearance as the only presenting symptom and absence of other typical findings such as deafness and dwarfism.

Bibliography 1. Matheus M.G., Castillo M., Smith J.K., et al.: Brain MRI findings in patients with mucopolysaccharidosis types I and II and mild clinical presentation. Neuroradiology, 2004, 46: 666.

JBR–BTR, 2010, 93: 130-131.

ACUTE RESPIRATORY SYMPTOMS IN MOUNIER-KUHN SYNDROME P.A. de Jong, J.K. Vette, L. Meiss1 Key-word: Trachea, abnormalities

Background: A 73-year-old female patient presented at the emergency room with cough and dyspnea. There was green sputum production and she had fever. She had given up smoking 15 years before. Her medical history was positive for recurrent bronchitis. Physical examination revealed rhonchi, a body temperature of 38.2°C and tachypnea of 35 breaths per minute. Oxygen saturation was 80% without oxygen supply. Chest radiography did not reveal a consolidation.

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1. Department of Radiology, Meander Medical Center, PO Box 1502, 3800 BM, Amersfoort, The Netherlands

ACUTE RESPIRATORY SYMPTOMS IN MOUNIER-KUHN SYNDROME — DE JONG ET AL

Work-up Chest radiograph (Fig. 1) demonstrates on the posterior-anterior (PA) view (A) a large transverse dimension of the trachea (3.2 cm) and left main bronchus (2.4 cm), large lung volumes, irregular diaphragmatic contours. Lateral chest radiograph (B) demonstrates the large trachea (3.7 cm) to a better extent.

Thin-section CT scan of the lungs (Fig. 2) includes four detail views of axial CT images (A) showing the wide trachea and left main bronchus (PA: 2.5 cm) with an abrupt change to more normal dimensions. In addition, there are tracheal diverticulae. Axial thin CT-section (B) demonstrates the large diameters of the trachea (transverse: 3.1 cm, PA: 3.6 cm) and the presence of bronchiectasis.

Radiological diagnosis The radiological diagnosis was made of syndrome of Mounier-Kuhn, with tracheobronchomegaly, tracheal diverticulae and bronchiectasis.

Discussion Tracheobronchomegaly is a rare disorder. It appears to impair mucous clearing and therefore leads to recurrent infections. The best diagnostic clue is a tracheal dilatation. The lateral chest radiograph is usually the most conspicuous. Normal values of the trachea have been published for chest radiography and computed tomography.

On a chest radiograph, normal antero-posterior tracheal diameters (profile views) (mean ± standard deviation) are approximately 17 ± 2 mm and 21 ± 3 mm for females and males respectively.

Diameters of the trachea in the coronal plane (mean ± SD) are 17 ± 2 mm and 20 ± 2 mm for females and males respectively.

The other imaging findings of tracheobronchomegaly or Mounier-Kuhn disease include

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dilatation of the main bronchi, usually with an abrupt change to normal caliber, diverticulae of the trachea and bronchiectasis.

On expiration, a collapse of the trachea can be seen.

Mounier-Kuhn syndrome appears to be autosomal recessive in some families and in some cases the disease is associated with the disease of EhlersDanlos or Marfan. There is also a congenital variant of the disease. Overall, most cases are sporadic.

The disease is usually diagnosed at a younger age than in our patient, between 30-50 years. A strong male predominance has been observed.

In the presented case, the clinical symptoms and the imaging findings were very characteristic for the Mounier-Kuhn Syndrome, with exception for age at presentation and sex.

The diagnosis was made based on the CT scan, although, in retrospect, the chest radiograph also demonstrated the dilatation of the trachea and main bronchi as well. At presentation, the patient was treated as if having a pneumonia based on the clinical findings in the emergency room. The case illustrates the importance of looking carefully at the trachea and main bronchi on both the PA and lateral chest radiograph.

Bibliography 1. Breatnach E., Abbott G.C., Fraser R.G.: Dimensions of the Normal Human Trachea. AJR, 1984, 142: 903-906.

2. Griscom N.T., Wohl M.E.: Dimensions of the Growing Trachea Related to Age and Gender.

AJR, 1986, 146: 233-237.

3. Gurney J.W., et al.: Diagnostic Imaging. In Chest.

First edition. Amirsys Inc, Salt Lake City, Utah, USA. Section I3: 6-9.

4. Jong P.A. de, Long F.R., Wong J.C., et al.: Computed tomographic estimation of lung dimensions throughout the growth period. Eur Respir J, 2006, 27: 261-267.

JBR–BTR, 2010, 93: 132-133.

IDIOPATHIC BONE CYST OF THE MANDIBLE S. Dekeyzer1, F.M. Vanhoenacker1,2, K. Chapelle3 Key-word: Bones, cysts

Background: A 20-year-old man was referred to the department of Maxillofacial Surgery for further work-up of a radiolucent lesion within the left mandible, which was incidentally found on a panoramic radiograph, taken by the dentist. Clinically, all teeth were vital.

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1. Department of Radiology AZ St.-Maarten, Duffel and Mechelen, Belgium, 2. Department of Radiology Antwerp University Hospital, Edegem, Belgium and 3. Department of Maxillofacial Surgery AZ St.-Maarten, Duffel, Belgium

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IDIOPATHIC BONE CYST OF THE MANDIBLET — DEKEYZER et al

Work-up Panoramic radiograph (Fig. 1) shows presence of an ill-defined radiolucent lesion undulating between the roots of teeth 35-37 on the left side of the mandible (arrows).

Dental CT of the mandible (Fig. 2) includees an axial CT (A) on which the lesion appears to expand between the roots of the teeth. There is thinning and slight scalloping of the lingual and buccal cortex (arrows). Sagittal reformatted CT images (B) demonstrates thinning of the lingual and buccal cortex. On coronal reformatted CT images (C), although there is extensive bone involvement around the roots of the teeth, the lamina dura is relatively spared and there is no significant displacement of the teeth (arrows).

Radiological diagnosis Based on the imaging features, the differential diagnosis of an idiopathic bone cyst, odontogenic keratocyst, ameloblastoma or large radicular cyst was made. The age of the patient, and the absence of clinical symptoms are -however- in favor of idiopathic bone cyst.

Histological examination after biopsy of the lesion revealed a hemorrhagic content and the absence of epithelial lining, which is consistent with the diagnosis of idiopathic bone cyst.

Discussion An idiopathic bone cyst or simple bone cyst is a cavity of bone that lacks an epithelial lining, and therefore it cannot be regarded as a true cyst. Other synonyms, such as traumatic or hemorrhagic bone cyst have been used, because some believe that it develops in response to trauma. However, no evidence exists to support a traumatic cause. As the pathogenesis of the lesion is still a matter of debate, the term “idiopathic� bone cyst is preferred.

The lesion is usually discovered in the first 2 decades of life as an asymptomatic lesion, incidentally found on radiographs. The teeth in the affected area are vital. Location within the ramus and posterior mandible is most frequent.

Radiographically, the lesion is radiolucent with variable margins, ranging from well-defined to illdefined borders. The lesion often scallops between the roots of the teeth and may cause thinning and scalloping of the endosteal side of either the buccal or lingual cortex. Despite its extensive expansion, displacement of the teeth is rare and the lamina dura is usually intact or only partly disrupted. This characteristic may be very helpful to distinguish

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the lesion from other radiolucent lesions of the jaws.

Unfortunately, many other radiolucent mandibular lesions may have similar imaging appearances.

The correct diagnosis can be suggested by a combination of different parameters, such as patient age, prevalence, location, border contour and effect on adjacent structures.

A radicular cyst is the most common odontogenic cyst and occurs between the fourth and sixth decade of life. It results from inflammation secondary to caries and has a typical periapical location with welldefined sclerotic borders.

A follicular cyst is typically diagnosed in patients between 30 and 40 years of age, represents a common developmental lesion and is invariably located around the crown of the tooth. Margins are well-demarcated and sclerotic. The lesion is usually larger than a radicular cyst.

An odontogenic keratocyst (OKC) is lined by keratinizing squamous epithelium. Similar to idiopathic bone cyst, OKC is common in the body or ramus of the mandible, but occurs in an older age group and is more destructive. There is often resorption of the lamina dura and teeth displacement.

Ameloblastoma is most common between 20 and 50 years. The predilection site is the molarramus region of the mandible. Most mandibular ameloblastomas are well-defined. Tooth displacement, root resorption, cortical expansion and erosion are common. The internal structure varies from totally radiolucent to mixed with the presence of bony septa.

Although clinical and radiological features can narrow the differential diagnosis, histological confirmation is often mandatory for a definite diagnosis.

Management of an idiopathic bone cyst consists of a conservative opening into the lesion. This usually initiates bleeding and subsequent healing.

Radiographic follow-up is advised.

Bibliography 1. Bernaerts A., Vanhoenacker F.M., Hintjens J., et al.: Tumors and tumor-like lesions of the jaw: radiolucent lesions. JBR-BTR, 2006, 89: 81-90.

2. Dunfee B., Sakai O., Pistey R., et al.: Radiologic and pathologic characteristics of benign and malignant lesions of the mandible. Radiographics, 2006, 26: 1751-1768.

3. Scholl R., Kellett H.M., Neumann D.P., et al.: Cysts and cystic lesions of the mandible: clinical and radiologic-histopathologic review.

Radiographics, 1999, 19: 1107-1124.

JBR–BTR, 2010, 93: 134-135.

ABDOMINAL AND THORACIC TUBERCULOSIS IN A HIV POSITIVE PATIENT D. Duterloo, S.T. Bodewitz1 Key-word: Tuberculosis, gastrointestinal

Background: A 40-year-old Asian man, living in The Netherlands for the last three years, presented with complaints of continuous abdominal pain and bloody diarrhea, which he had since a couple of days. On physical examination, his abdomen was swollen but soft and nontender.

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ABDOMINAL AND THORACIC TUBERCULOSIS IN A HIV POSITIVE PATIENT — DUTERLOO et al

Work-up Radiography of the chest (Fig. 1) shows a small area of infiltration-consolidation at the lower part of the right lung.

Abdominal US (Fig. 2) demonstrates omental thickening and ascites.

On contrast-enhanced CT scan of the chest (Fig. 3) there is a small area of nodular infiltration (‘tree in bud’ pattern) peripherally in the right lung, possibly due to bronchial spread of infection, as well as mediastinal lymphadenopathy and pleural fluid on the right side.

On contrast-enhanced CT scan of the abdomen (Fig. 4), there is presence of omental thickening, ventrally on the right side, as well as ascites.

Radiological diagnosis Needle aspiration of ascites was performed, demonstrating reactive changes without malignant cells. During his stay in hospital, the patient proved to be HIV-positive. Biopsy of the omental thickening, performed later, showed acid-fast rods.

Based on CT findings, combined with the presence of acid-fast bacteria in the biopsy material (thickened omentum), the diagnosis gastrointestinal tuberculosis in a HIVpositive patient was made.

Discussion Gastrointestinal (GI) tuberculosis (TB) may involve any part of the gut, although the ileum and colon are more frequently affected. The ileocecal region is the most common site of involvement. A minority of patients (20-25%) with GI TB (< 50%) present with abnormal findings on radiograph or CT scan of the chest, consisting of patchy to nodular infiltration. Non-specific symptoms such as weight loss and abdominal pain are present in 8090% of the patients with GI TB. More rare symp-

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toms include malabsorption, diarrhea and hematochezia.

Pathologically, GI TB is characterized by inflammation and fibrosis of the bowel wall and regional lymph nodes. Mucosal ulceration results from necrosis of Peyer patches, lymph follicles, and vascular thrombosis.

At this stage of the disease, changes are reversible and healing without scarring is possible.

As disease progresses, the ulceration becomes confluent and extensive fibrosis develops leading to bowel wall thickening, pseudotumoral mass lesions and ascites.

On CT scan, irregular soft tissue densities in the omentum, low-attenuating masses surrounded by thick solid rims, low-attenuating necrotic (most often mesenteric) lymph nodes, disorganized appearance of soft-tissue densities, high-attenuating ascitic fluid and bowel loops forming poorly defined masses can be seen, together with a multilocular enhancement pattern after intravenous administration of iodinated contrast material.

The differential diagnosis of GI TB includes Crohn’s disease, non-Hodgkin lymphoma, yersiniosis, South American blastomycosis, and anisakiasis. Differentiating Crohn’s disease from TB before treatment initiation is important, as steroid therapy can be catastrophic in patients with undiagnosed TB. Targeted biopsy is currently considered the most rapid and efficient method for this differentiation.

Bibliography 1. Anand M.K.N., Reddy J.J.M., Khan A.N.: Tuberculosis, Gastrointestinal.http://emedicine. medscape.com (last update January 19, 2007).

2. Epstein B.M., Mann J.H.: CT of abdominal tuberculosis. AJR, 1982, 139: 861-866.

3. Herchline T., Amorosa J.K.: Tuberculosis. http://emedicine.medscape.com/ (last update January 20, 2009).

JBR–BTR, 2010, 93: 136-137.

POSTINTUBATION MEMBRANOUS TRACHEAL RUPTURE B. Grayet1, B. Duysinx2, B. Ghaye1

Background: A 41-year-old female underwent general anaesthesia for teeth surgery. No complication occurred during and immediately after surgery. Three days later, she presented to the Emergency Department with retrosternal pain and subcutaneous emphysema at the level of the upper chest and the neck.

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Departments of 1. Medical Imaging and 2. Pneumology, University Hospital of Liège, Liège, Belgium

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Work-up Chest X-ray (Fig. 1) shows parietal emphysema in the upper chest and neck. Pneumomediastinum is also demonstrated, manifesting as gas lucency outlining the inner surface of left mediastinal pleura (arrow).

CT scan of the chest (Fig. 2) shows air dissecting soft tissues of upper chest and neck and a pneumomediastinum extending until the level of the hila (A). Note extrapleural extension of air simulating a pneumothorax (B, arrow). Axial CT scan close to lung apex (C) and coronal thick MPR reformatted image centred on the trachea (D) reveal a large fusiform defect in the posterior wall of the trachea (arrow).

Fiberoptic bronchoscopy (Fig. 3) performed immediately after CT confirms the large defect involving the membranous wall of the trachea (arrows).

Radiological diagnosis Based on the clinical history and on the large tracheal defect seen on CT, which was confirmed at bronchoscopy, the diagnosis of postintubation membranous tracheal rupture was made.

Discussion Tracheal laceration is a rare but potentially devastating complication of endotracheal intubation. Anatomy of the trachea and mechanisms of rupture, including overinflated tube cuff, sudden motion of endotracheal tube and direct tear of the trachea, explain the posterior location of the lesions. Therefore the membranous wall or the junction between tracheal membranous wall and cartilage are almost exclusively involved.

Inadequate tracheal tube size, repeated attempts at a difficult intubation, double lumen tracheal tube and anatomic abnormalities of the trachea increase the risk of rupture. Small female patients also seem to have a predisposing risk.

Clinical symptoms can occur during surgery, immediately after extubation or within the next 12 hours.

The first signs and symptoms of tracheal rupture include subcutaneous emphysema of the upper chest, neck and head, haemoptysis, and dyspnea and cyanosis in severe cases. Sometimes symptoms can be more insidious and short tears having a spontaneously favourable outcome are probably underdiagnosed.

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Radiological signs of tracheal rupture are nonspecific. Chest X-ray usually shows a pneumomediastinum and extensive parietal emphysema.

Rarely, a pneumothorax can be associated, when the laceration has extended to a main bronchus, particularly on the right side. CT confirms these findings and usually shows the site of rupture and its extension. Bronchoscopy is indicated when CT findings are suggestive of tracheal laceration.

Management of tracheal rupture is controversial and the choice between conservative versus surgical treatment is debated. Conservative treatment, include broad spectrum antibiotherapy, antiseptic and anti-inflammatory aerosoltherapy, cutaneous incisions with massages in extensive subcutaneous air and rarely percutaneous mediastinal drainage. It is generally indicated for stable and non-symptomatic patients with a distal tracheal rupture less than 4 cm in length. Surgical treatment is recommended for symptomatic and unstable patients and for laceration of more than 4 cm. It will be performed through a cervicotomy when the laceration is located in the upper two thirds of the trachea and through a thoracotomy for rupture manifesting during a thoracic surgical procedure (immediate repair), and for tears involving the distal trachea, a main bronchus, or both.

Clinical outcome is generally favorable.

Nevertheless complications, including mediastinitis, tension pneumomediastinum or even tension pneumoperitoneum have been reported.

Bibliography 1. Fraipont V., Lambermont B., Ghaye B., et al.: Unusual complication after percutaneous dilatational tracheostomy: pneumoperitoneum with abdominal compartment syndrome. Intensive Care Med, 1999, 25: 1334-1335.

2. Jougon J., Ballester M., Choukroun E., et al.: Conservative Treatment for Postintubation Tracheobronchial Rupture. Ann Thorac Surg, 2000, 69: 216-220.

3. Lancelin C., Chapelier A.R., Fadel E., et al.: Transcervical-Transtracheal Endoluminal Repair of Membranous Tracheal Disruptions. Ann Thorac Surg, 2000, 70: 984-986.

4. Ross H.M., Grant F.J., Wilson R.S., et al.: Nonoperative Management of Tracheal Laceration During Endotracheal Intubation. Ann Thorac Surg, 1997, 63: 240-242.

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SYMPTOMATIC BRAIN CAPILLARY TELANGIECTASIA B. Heremans, G. Wilms, G. Marchal1 Key-word: Telangiectasia

Background: A 35-year-old patient presented to our hospital with persistent tinnitus and drowsiness.

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1. Department of Radiology, UZ Gasthuisberg, KUL, Leuven, Belgium

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SYMPTOMATIC BRAIN CAPILLARY TELANGIECTASIA — HEREMANS et al

Work-up MRI of the brain (Fig. 1) included an axial T2weighted image (A) on which the lesion is hardly visible. On B (axial FLAIR image) a slightly hyperintense, small, rounded solitary lesion is seen centrally in the pons. On the axial gradient echo image (C), the lesion obviously exhibits hypointensity. On the axial T1-weighted image (D), a iso- to slightly hypointense small, rounded solitary lesion is seen centrally in the pons. Gadolinium-enhanced axial and sagittal T1-weighted image (E, F) shows homogeneous contrast enhancement of the lesion.

Radiological diagnosis The imaging findings are consistent with brain capillary telangiectasia.

Discussion Brain capillary telangiectasias are part of the vascular malformations of the brain besides pial arteriovenous malformations, cavernomas and venous angiomas or developmental venous anomalies.

Brain capillary telangiectasias are not uncommon.

They are small vascular lesions (ranging from several mm to 2 cm in diameter), usually located in the pons. Microscopically, they consist of irregular clusters of multiple thin-walled ectatic vascular channels interposed between normal brain parenchyma, without adjacent gliosis or hemosiderin deposition.

The clinical manifestations related to capillary malformations are variable, although typically they are a fortuitous finding on a brain imaging study.

Occasionally they are seen in patients presenting with headaches, confusion, weakness, dizziness, visual changes, vertigo, tinnitus, or seizures, but without clear connection to the symptomatology.

Brain capillary telangiectasias have a characteristic MR-imaging appearance that correlates with their microscopic tissue composition. Lacking hemosiderin, capillary telangiectasias are not hypointense on conventional or fast spin-echo T2weighted images but appear as isointense or hyperintense areas compared with the normal brain parenchyma. In T1-weighted unenhanced images, they exhibit iso- or hypointensity. Because telangiectasias are composed of sacs of stagnant

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blood which haemoglobin presumably partially desaturates to deoxyhaemoglobin, they exhibit susceptibility dephasing on gradient-echo images.

Being vascular lesions, they show mild contrast enhancement, creating a mesh of enhanced structures on a background of unenhanced brain parenchyma.

Nonenhanced CT does not depict capillary telangiectasias. Since they do enhance they can be visible on contrast-enhanced CT, but they may be overlooked due to insufficient enhancement or to artifacts in the posterior fossa. On angiography they are occult similar to cavernomas because of the lack of arteriovenous shunting.

The differential diagnosis of enhancing lesions in the pons includes neoplasms such as astrocytoma, metastasis or lymphoma, subacute infarction, active demyelination, or acute inflammatory processes such as acute disseminated encephalomyelitis, in addition to capillary telangiectasias.

Only the capillary telangiectasias contain blood products, so only these would demonstrate increased susceptibility on GRE-images. Classic capillary telangiectasias can be distinguished from cavernous angiomas on MRI. Cavernomas, which contain hemosiderin-laden macrophages and often are calcified, show marked signal intensity loss on GRE, called the “blooming effect”. Its hallmark is a surrounding hypointense hemosiderin ring with an internal ‘popcorn’ appearance on T2-weighted images.

In conclusion, brain capillary telangiectasias are small, enhancing lesions that are usually located in the pons and that are often undetectable on conventional T1- and T2-weighted images. They lack the ‘hemosiderin rim’ of cavernous angioma and appear hypointense on GRE-images.

Bibliography 1. Lee R.R., Becher M.W., Benson M.L., et al.: Brain Capillary Telangiectasia: MR-Imaging Appearance and Clinicohistopathologic Findings. Radiology, 1997, 205: 797-805.

2. Scaglione C., Salvi F., Riguzzi P., et al.: Symptomatic unruptured capillary telangiectasia of the brain stem: report of three cases and review of the literature. J Neurol Neurosurg Psychiatry, 2001, 71: 390-393.

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PORTOHEPATIC SHUNT IN A PATIENT WITHOUT CIRRHOSIS A. Kucuk1, S. Poturoglu2, O. Gurbuz1, Y. Savas1 Key-word: Aneurysm, splenic

Background: A 65-year-old woman was admitted with complaint of a left gluteal mass. She had a history of congenital hip dislocation and a known splenic enlargement for more than 40 years.

The patient had pancytopenia on admission. Hematocrit level 23.7%, white blood cell count 2.5 x 103/uL, platelet count 69 x 103/uL were measured. Liver function tests were normal. Viral markers such as hepatitis B surface antigen and anti-hepatitis C related to liver disease were negative. There was no known liver biopsy or history of trauma.

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Department of 1. Radiology and 2. Internal Medicine, Haseki Educational and Research Hospital, Istanbul, Turkey

PORTOHEPATIC SHUNT IN A PATIENT WITHOUT CIRRHOSIS â&#x20AC;&#x201D; KUCUK et al

Work-up Contrast-enhanced CT image (Fig. 1) through the splenic hilum (A) shows a huge aneurysm of the splenic vein (arrow) with anteroposterior diameter of 6.5 cm. Almost complete and curvilinear calcification of the wall and also presence of a thrombus anteriorly and laterally (long arrow). On contrastenhanced CT image caudal to previous image (B), there is continuity between the lumen of the aneurysm and the splenic vein. The confluence of splenic and superior mesenteric vein has a diameter of 26 mm (C). Contrast-enhanced CT image through the right liver lobe (D) shows that the main portal vein and right branch of portal vein (arrow) are both dilated aneurysmatically. On contrastenhanced CT image cranial to previous image through the right liver lobe, there is obvious dilatation of the portal vein (white arrow) and hepatic vein (black arrow) and presence of a (portosystemic) shunt between the two veins.

US examination through the splenic hilum (Fig. 2) demonstrates splenic vein aneurysm as an anechoic cystic mass that shows posterior acoustic enhancement. With CD-US examination (not shown), a monophasic venous flow in a hepatopetal direction through the right branch of the portal vein was observed.

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from pancreatitis, traumatic injury to the vessel wall, or abdominal surgery have been reported.

Portosystemic shunts are also extremely rare.

Only a few cases were reported in English literature: a case with calcified portal vein aneurysm and portohepatic venous shunt in a patient with liver cirrhosis by So, a case which was diagnosed with a latent portosystemic encephalopathy during hospitalization by Fulcher, and a third case with intrahepatic aneurysmal portohepatic venous shunt that was treated with embolization by Fanelli.

We report on a non-cirrhotic patient with a calcified splenic vein aneurysm, aneurysmal dilatation of main portal vein and right branch of portal vein associated with spontaneous portohepatic shunt.

There was no evidence of hepatic disease, liver biopsy, nor previous trauma. The patient had pulmonary hypertension on echocardiographic examination which may be a consequence of the portohepatic shunt.

Cranio-caudal diameter of spleen was measured as 25 cm, and pancytopenia of the patient was explained by sequestration through the spleen. The gluteal abscess of the patient was drained by a simple surgical intervention. The patient was informed about findings and risk factors i.e. hepatic encephalopathy related to portosystemic shunt, rupture, or acute thrombosis of aneurysms and called for close follow-up.

Radiological diagnosis Bibliography The findings on CT, US and CD-US, together with the medical history of the patient, suggested the diagnosis of a huge calcified splenic aneurysm, aneurysmal dilatation of portal vein and a spontaneous portohepatic shunt in a hepatopetal direction.

Discussion Although aneurysms of portal vein and splenic vein are uncommon, they are detected more frequently with increasing use of non-invasive imaging modalities. The etiology of aneurysms can be congenital or more commonly acquired.

Acquired causes such as portal hypertension, or chronic hepatic disease, vascular inflammation

1. Fanelli F., Marcelli G., Bezzi M., et al.: Intrahepatic aneurysmal portohepatic venous shunt: embolization with a tissue adhesive solution.

J Endovasc Ther, 2003, 10: 147-153.

2. Fulcher A., Turner M.: Aneurysms of the portal vein and superior mesenteric vein. Abdom Imag, 1997, 22: 287-292.

3. Ozbek S.S., Killi M.R., Pourbagher M., et al.: Portal venous system aneurysms: report of five cases. Ultrasound Med, 1999, 18: 417-422; quiz 423.

4. So N.M., Lam W.W.: Calcified portal vein aneurysm and portohepatic shunt in a patient with liver cirrhosis. Clin Radiol, 2003, 58: 742-744.

JBR–BTR, 2010, 93: 142-143.

SMALL BOWEL OBSTRUCTION DUE TO BEZOAR IN JEJUNAL DIVERTICULOSIS Y. Maréchal1, E. Danse1, H. Paterson2, A. Kartheuser2 Key-word: Intestines, diverticula

Background: An 87-year-old man was admitted urgently with abdominal pain, distension and absolute constipation for 48 hours. A plain abdominal radiograph confirmed small bowel obstruction and blood tests revealed a raised CRP (7 mg/ml) with normal hematology, renal function, liver function and LDH.

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1. Department of Radiology and 2. Colorectal Surgery Unit, Department of Abdominal Surgery and Transplantation, St-Luc University Hospital, Brussels, Belgium

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SMALL BOWEL OBSTRUCTION DUE TO BEZOAR IN JEJUNAL DIVERTICULOSIS — MARÉCHAL et al

Work-up Contrast-enhanced CT scan of the abdomen (Fig. 1) includes a transverse section at the level of the iliac fossae (A) which shows dilated, fluid-filled bowel loops. The point of occlusion in the left iliac fossa is shown. Hypodense non-vascularised intraluminal mass lesion, immediately proximal to the site of bowel size change (arrow). Reformatted image in the coronal plane (MPR-image) (B) demonstrates diverticulae at the mesenteric border of the jejunum. One of these diverticula contains a non-vascularized intra-luminal mass (arrow).

Reformatted image in the coronal plane (MPRimage) (C) demonstrates diverticulae at the mesenteric border of the jejunum (arrow).

Transverse section at mid-abdominal level (D) shows signs of intestinal obstruction. Gas bubbles are seen in the intestinal wall (arrows). On transverse section at mid-abdominal level (pulmonary window setting) (E), bubbles of gas are noted within the bowel wall and outlining the diverticula.

There is no evidence for free peritoneal fluid, nor gas within the superior mesenteric or portal veins (the latter not shown).

Radiological diagnosis Based on the radiological findings, the diagnosis of acute mechanical obstruction secondary to migration of bezoar originating within a large jejunal diverticulum, associated with radiological signs of localized pneumatosis and localized perforation was made. At laparotomy, a mid-jejunal 4 cm bezoar, causing obstruction, was removed by enterotomy. There were no signs of intestinal perforation or ischemia. No bowel resection was required. The subsequent post-operative recovery was uneventful. The final diagnosis was pseudoperforation related to jejunal diverticulosis complicated by small bowel obstruction due to bezoar.

Discussion Jejunal diverticulosis is estimated to occur in 0.02-7.1% of the population and is more frequent in the elderly. It is 7 times more common than ileal diverticulae and associated with diverticulae of the sigmoid colon and duodenum. The cause is unclear, but like in colonic diverticulae, jejunal diverticulae are found at the point of entry of jejunal vessels on the mesenteric border.

Sixty percent of patients are asymptomatic. In 30% ill-defined symptoms of dyspepsia, flatulence

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or abdominal pain are described. In 10% of cases complications requiring surgical intervention occur. These include hemorrhage, perforation, occlusion (bezoars and stricture), inflammation and bacterial overgrowth.

Pneumoperitoneum without perforation, as presented, has been described, and is attributed to the thin wall of the diverticulae functioning as a semipermeable membrane. The finding of encysted gas in the wall of the jejunum due to subserosal dissection has also been described. In our case the presence of gas outside the intestinal wall was interpreted as suspected for intestinal ischemia and localised perforation.

Intestinal occlusion secondary to migration of an intra-diverticular bezoar is an uncommon complication.

Up to now only 39 cases have been reported. If possible, the bezoar may be fragmented and pushed into the caecum for subsequent evacuation. Otherwise enterotomy is required. In the absence of perforation there is no indication to excise the diverticular segment of jejunum.

CT scan is helpful for the identification of bezoar, by showing an intraluminal gas containing mass in cases of phytobezoar.

The presented case illustrates jejunal diverticulosis uncommonly presenting with complications, i.e. obstruction due to impacted bezoar. The radiological finding of intraparietal extra-intestinal gas was in this case ‘benign’, and could be misinterpreted as pneumoperitoneum by bowel perforation. Features that suggest the harmlessness are localization of gas within the wall of diverticulae and absence of gas in the mesenteric or portal veins, in addition to correlation with the clinical context.

Bibliography 1. Harris L.M., Volpe C.M., Doerr R.J.: Small bowel obstruction secondary to enterolith impaction complicating jejunal diverticulosis. Am J Gastroenterol, 1997, 92: 1538-1540.

2. Shanmugan R.P., Shivakumar P.: A rare complication of jejunal diverticulosis. Trop Gastroenterol, 2006, 27: 134-135.

3. Shocket E., Simon S.: Small bowel obstruction due to enterolith (bezoar) formed in a duodenal diverticulum. A case report and review of the literature. Am J Gastroenterol, 1982, 77: 621-624.

4. Wright F.W., Lumsden K.: Recurrent pneumoperitoneum due to jejunal diverticulosis. Clin Radiol, 1975, 26: 327-331.

JBR–BTR, 2010, 93: 144-145.

MASS IN THE BACK AS MANIFESTATION OF NEUROFIBROMATOSIS TYPE 2 A.S. Michel L. van den Hauwe, H. Degryse1 Key-word: Neurofibromatosis

Background: A 13-year-old boy was referred for MR imaging of the thoracolumbar spine to further differentiate a spindle shaped hyporeflective soft tissue mass in the back seen on ultrasonography (not shown) most characteristic for a lipoma.

After a closer look at the medical history we learn that the boy is known since the age of 7 with a problem of paresis with atrophy of the left arm. An earlier MR examination of the thoracic outlet showed bilateral soft tissue masses interpreted as diffuse thickening of the plexus brachialis nerves, thus explaining the patient’s complaints, without however a clear etiology being withheld. One year ago a new small mass was excised from the boy’s lip. Anatomopathological analysis revealed an interesting diagnosis: submucosal neurofibroma. Now putting these at first apparently independent events in a greater context, we decided to also realize a MR examination of the brain.

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1. Department of Medical Imaging, AZ KLINA, Brasschaat, Belgium

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MASS IN THE BACK AS MANIFESTATION OF NEUROFIBROMATOSIS TYPE 2 — MICHEL et al

Work-up MRI of the thoracolumbar spine (Fig. 1) includes a sagittal T2-weighted image (WI) (A) and enhanced T1-WI (B). At the level of the palpable swelling a spindle shaped hypointense, nonenhancing soft tissue mass is recognized. The sagittal T2-WI shows the presence of multiple nodular lesions which are wedged into the roots of the cauda equina (A).

There is a clear contrast enhancement after intravenous Gadolinium injection (B).

MRI of the thoracic outlet (Fig. 2) shows hyperintense lesions on T2- and STIR-weighted images at the level of the apex of both lungs.

MRI of the brain (Fig. 3) consists of an axial and coronal T2-WI (A,C,D) and enhanced T1-WI (B) at the level of the internal auditory canal (IAC) and Meckel’s cave.

There is a bilateral nodular contrast enhancement in the IAC after intravenous Gadolinium injection. Image of bilateral schwannomas originating from the nervus vestibulocochlearis. At the level of Meckel’s cavum 3 enhancing nodules are seen as well as bilateral nervus trigeminus schwannomas.

Radiological diagnosis Based on the pathognomonic findings on MRI of the brain and the clinical characteristics the patient was diagnosed with neurofibromatosis type 2 (NF2).

Discussion Neurofibromatosis type 2 (NF2) is in this case differentiated from neurofibromatosis type 1 (NF1) by the pathognomonic finding of bilateral acoustic neurofibromas on the MRI of the brain. The presence of cutaneous neurofibromas and peripheral nerve sheath tumors predominantly point in the direction of NF1, but the absence of café au lait patches and the visualization of neurofibromas/ schwannomas on the roots of the cauda equina make NF2 a very likely diagnosis.

Neurofibromatosis (NF) is an autosomal dominant disorder that affects bone, nervous system, soft tissue, and skin. At least 8 different clinical phenotypes of neurofibromatosis have been identified.

The most common types are NF1 (von Recklinghausen disease) and NF2. NF1 has a birth incidence of one in 2500. NF2, is a lot rarer with a

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birth incidence of about one in 33.000-40.000. In this case, familial anamnesis for the same clinical entity was negative so the diagnosis of a de novo mutation is plausible.

In literature 50% of the NF2 cases appear to be new germ line somatic mutations.

The National Institute of Health (NIH) Consensus Development Conference formulated following diagnostic criteria for NF2: (1) bilateral acoustic nerve (nVIII) masses seen with appropriate imaging techniques (e.g. CT or MRI) or (2) a first-degree relative with NF2 and either unilateral acoustic nerve mass, or two of the following disorders: neurofibroma, meningeoma, glioma, schwannoma, or juvenile posterior subcapsular lenticular opacity.

NF2 mainly presents in young adults and predominantly features symptoms associated with vestibular schwannomas as hearing loss and vertigo. Complaints due to cranial neuropathies, cataract, cutaneous lesions and peripheral muscle weakness can also exist. On the other hand, in children, the disease presentation is somewhat different. In a large UK database Evans et al observed that in the pediatric onset cases of NF2 significantly less children had hearing loss and vertigo complaints and that relatively more children presented with cutaneous lesions and other neurological deficit.

To define the clinical spectrum of the disease, gadolinium-enhanced MRI of brain and spine with special attention for the cranial nerves is required.

In addition, neurological, dermatological and ocular examinations are advised as well as a systematic follow-up in time. Finally, genetic staging of patient and family should be considered. NF2 is a severely debilitating disease, which is worse with childhood onset, and multidisciplinary management at specialty treatment centres is required.

Bibliography 1. Evans D., et al.: Paediatric presentation of type 2 neurofibromatosis. Arch Dis Child, 1999, 81: 496499.

2. Ferner R.: Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective.

Lancet Neurol, 2007, 6: 340-351.

3. Ruggieri M., et al.: Earliest clinical manifestations and natural history of neurofibromatosis type 2 (NF2) in childhood: a study of 24 patients.

Neuropediatrics, 2005, 36: 21-34.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 146-147.

METASTASES IN THE BREAST FROM DISSEMINATING MELANOMA A. Oehlers-Wetzel1, C. Meijers2, R. den Toom3, G. Risseeuw1 Key-word: Melanoma

Background: A 44-year-old female was referred to our hospital with a palpable mass in the left breast. Upon physical examination, a tumor was detected in both the left and the right breast.

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Department of 1. Radiology and 3. Surgery, Ruwaard van Putten Hospital, Spijkenisse, The Netherlands; 2. Department of Pathology, Pathan, Rotterdam, The Netherlands

METASTASES IN THE BREAST FROM DISSEMINATING MELANOMA â&#x20AC;&#x201D; OEHLERS-WETZEL et al

Work-up Mammography of the breasts (Fig. 1) shows two masses on the craniolateral view of the left breast.

The dominant rounded mass corresponds to the finding during physical examination. Laterocranial to this lesion, there is a second lesion which was not detected during palpation. On the craniolateral view of the right breast, one rounded lesion with illdefined margins, corresponding to the palpable mass, is seen.

Ultrasonography of the breasts (Fig. 2) shows on A (left breast) two lesions. The dominant lesion has a diameter of 3.6 cm and appears inhomogeneous.

The second lesion of 1 cm in diameter has an hypoechoic appearance. In the right breast (B), a hypoechoic 2 cm lesion with ill-defined margins is seen.

CT scan of the thorax and abdomen (Fig. 3) demonstrates nodular opacities in both breasts, suspicious for metastases (A). Metastases are also present in the left pedicle and dorsal elements of the second lumbar vertebra (B).

Microphotographs of the lesions after biopsy (Fig. 4) is representative for all three lesions (A: with Hematoxyllin-eosin stain, B: with Melan-A, a marker for melanoma). Cells with large, irregular and hyperchromatic nuclei are present. The cells are positive for Vimentine, Melan-A and S100.

HMB45 is partially positive. The hormone receptors for estrogen and progesterone are negative.

Radiological diagnosis Pathological examination of the three biopsies taken under ultrasound guidance indicated that the masses in the breasts were metastases of melanoma. This diagnosis was supported by the medical history of the patient. The patient had undergone excision of melanoma (Breslow thickness 2.99 mm) 2 years previously, with a positive sentinel node under the right arm.

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As mentioned, the patient also had bone metastasis in the lumbar spine at the occasion of the current consultation.

Being diagnosed with multiple metastases from melanoma, the patient died after 5 months.

Discussion The female breast is associated with many diseases. From the oncologic point of view, it is not only a host of benign and malignant tumors of breast glandular parenchyma, but can also be a site for metastasis disseminating from various tumors.

The most common primary tumors which metastasize to the breast are lymphoma and malignant melanoma.

Metastasis to the breast from malignant melanoma or other extra-mammary neoplasm is rare. Its incidence ranges from 1.3 to 2.7% of all breast malignancies. However, this complication is being encountered with greater frequency, due to the increasing incidence of malignant melanoma.

Therefore, special attention should be given to breast masses or abnormal imaging study of the breasts presenting in patients with a history of melanoma. Especially for further treatment, the diagnosis of metastasis of melanoma is of great importance, since the finding of metastasis in the breast constitutes a poor prognostic sign.

Close follow-up is recommended to ensure correct management of these patients, in order to avoid unnecessary procedures with no benefit to the patient whose survival is usually less than one year.

Bibliography 1. Bassi F., Gatti G., Mauri E., et al.: A. Breast metastases from cutaneous malignant melanoma.

Breast, 2004, 13: 533-535.

2. Ravdel L., Robinson W., Lewis K., et al.: Metastatic melanoma in the breast: A report of 27 cases. J Surg Oncol, 2006, 94: 101-104.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 148-149.

PROBABLE CEREBRAL AMYLOID ANGIOPATHY B. Pilet1, O. Ă&#x2013;zsarlak1,2, C. Venstermans1, P.M. Parizel1 Key-word: Amyloidosis

Background: A 55-year-old man presented with short-term memory loss, headache, diplopia and visual hallucinations. Higher mental functions were normal. The patient had a previous medical history of seizures, and was under longstanding anti-epileptic treatment. Recently he had been diagnosed with multiple myeloma.

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1. Department of Radiology, Antwerp University Hospital, Edegem, Belgium and 2. Department of Radiology AZ Monica, Deurne, Belgium.

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PROBABLE CEREBRAL AMYLOID ANGIOPATHY — PILET et al

Work-up MRI of the brain, axial gradient-recalled echo FLASH T2*-weighted images (Fig. 1) includes a section at the level of the lateral ventricles (A) and a section through the posterior fossa (B). Both images reveal multiple punctate hypointense lesions, both supra- and infratentorially, with sparing of the deep white matter and basal ganglia.

On axial fat-suppressed turbo spin-echo T2weighted image at the same level as Fig. 1A (C) and axial fat-suppressed turbo FLAIR image at the same level as Fig. 1A (D), the numerous lesions which were easily recognizable on the gradientrecalled echo FLASH T2*-weighted images, are not visible on the T2-weighted nor on the FLAIR images. Note some patchy white matter abnormalities, consistent with mild subcortical arteriosclerotic leukoencephalopathy.

Radiological diagnosis The presence of multiple hypointense foci on T2*-weighted gradient-recalled echo images, indicates hemosiderin deposition in petechial brain hemorrhages. The age and clinical history of the patient, as well as the typical cortical and subcortical location of the microbleeds, meet the Boston criteria for a probable diagnosis of cerebral amyloid angiopathy (CAA). However, cerebral microbleeds can also occur in other disease conditions.

Discussion Cerebral microhemorrhages appear as punctate or ovoid foci of marked signal intensity loss on T2*weighted gradient-recalled echo (GRE) MRI. Compared with turbo FLAIR and turbo spin-echo T2-weighted sequences, the T2*-weighted GRE sequence has a markedly greater sensitivity for local magnetic field inhomogeneities produced by microscopic deposits of hemosiderin. Though microbleeds are in general clinically asymptomatic, they are recognized as a marker of small vessel pathology, most commonly cerebral amyloid angiopathy or hypertensive vasculopathy.

Cerebral amyloid (or ‘congophilic’) angiopathy is a condition characterized by the deposition of ßetaamyloid in cortical, subcortical and leptomeningeal vessels, with sparing of the deep white matter, basal ganglia, and cerebellum. CAA is more frequently found in elderly patients, and is associated with Alzheimer’s disease. CAA manifests radiologically as intracranial hemorrhages (ICH) in a distinctive cortical-subcortical distribu-

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tion, leukoencephalopathy, and/or atrophy. In a normotensive elderly patient who presents with ICH without a history of trauma, CAA should always be considered. Although histopathological demonstration of vascular amyloid is still required for a solid diagnosis of CAA, histological analysis is often not practically feasible. Hence it is important to recognize the imaging findings of CAA. A scoring system (the so-called Boston criteria) can be used to assess the likelihood of CAA on MR examinations of the brain.

Several other conditions must be considered in the differential diagnosis such as subcortical arteriosclerotic leukoencephalopathy, also known as Binswanger’s disease or hypertensive cerebral angiopathy, is characterized by the development of intimal hyperplasia and hyalinosis in deep penetrating brain arterioles as the result of chronic systemic hypertension. Hypertensive bleeds, occurring predominantly in men between 60 to 80 years of age, are the underlying cause for 10% of all clinical strokes. Cerebral microhemorrhages associated with chronic hypertension are more commonly found in the thalamus, basal ganglia, cerebellum, and pons.

Another differential diagnosis is multifocal petechial cerebral hemorrhages that can also be found in diffuse axonal injury, also known as shearing injury. These lesions are commonly found in victims of high-speed motor vehicle accidents.

Their underlying mechanism is a combination of acceleration, deceleration, and rotation, affecting portions of the brain where tissue density differs: gray-white matter junctions, basal ganglia, splenium of the corpus callosum, and dorsal midbrain.

Finally, other unusual causes of cerebral microbleeding includee capillary teleangiectasias, vasculitis, micrometastases, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), and ParryRomberg syndrome.

Bibliography 1. Chao C.P., Kotsenas A.L., Broderick D.F.: Cerebral amyloid angiopathy: CT and MR imaging findings. Radiographics, 2006, 26: 1517-1531.

2. Parizel P.M., Özsarlak Ö., Van Goethem J.W., et al.: Imaging findings in diffuse axonal injury after closed head trauma (pictorial essay). Eur Radiol, 1998, 8: 960-965.

3. Tsushima Y., Aoki J., Endo K.: Brain microhemorrhages detected on T2*-Weighted Gradient-Echo MR Images. AJNR, 2003, 24: 88-96.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 150-151.

POLYARTERITIS NODOSA OF THE SPLEEN M. Smets1, B. Claikens1, P. Van Wettere1, G. Lambrecht2 Key-word: Arteritis

Background: A 64-year-old male presented with pain in his right lower leg and progressive swelling of the right ankle, without history of previous trauma. Further anamnesis revealed fever, anorexia and weight loss over the past few weeks. Laboratory results showed increased inflammatory parameters (CRP 203 mg/l).

A lower extremity ultrasonographic examination ruled out deep venous thrombosis.

A contrast-enhanced CT scan of the abdomen revealed multiple splenic infarctions.

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Departments of 1. Medical Imaging and 2. Internal Medicine, AZ Damiaan, Oostende, Belgium

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POLYARTERITIS NODOSA OF THE SPLEEN â&#x20AC;&#x201D; SMETS et al

Work-up Contrast-enhanced CT scan of the abdomen (Fig. 1) shows splenomegaly with multiple hypodense peripheric lesions with triangular morphology dispersed throughout the spleen, suggestive of splenic infarctions and presence of a cyst in the right liver lobe.

Arteriography of abdominal aorta and splenic artery (Fig. 2). Shows on A (early phase) irregular aspect of the intraparenchymal small arteries of the spleen with multiple areas of stenosis and dilatation, as well as multiple intraparenchymal microaneuryms. Late phase (B) demonstrates heterogenous contrast-enhancement with radiolucent cortical areas, diagnostic for multiple areas of splenic infarction.

Arteriography of the renal and hepatic arteries showed no abnormalities.

Radiological diagnosis Based on the arteriographic finding of multiple intraparenchymal splenic microaneurysms, combined with the clinical presentation of fever and weight loss, the patient was diagnosed with polyarteritis nodosa. Immunosuppressive therapy was started with administration of prednisone.

Discussion Polyarteritis nodosa (PAN) is a progressive inflammatory vasculitis of small- and mediumsized arteries that leads to necrosis and destruction of the wall of vessels. Thus, PAN belongs to the pathologic group of necrotizing vasculitis which also includes other diseases such as Churg-Strauss syndrome, Kawasaki disease and Wegener granulomatosis.

PAN is a rare condition and predominantly affects people in their fourth to sixth decade of life, with the male-to-female ratio being 2-3:1.The exact cause is unknown, but studies suggest that PAN is probably mediated by deposition of immune complexes. Viruses play a role in the pathogenesis in some cases, most notably hepatitis B virus.

Many of the clinical symptoms are related to organ ischemia secondary to arterial branch occlusions.

The kidneys are most frequently affected (in 7080% of cases). Renal failure can occur because of

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multiple infarcts; hypertension can develop from renal artery involvement. Cutaneous manifestations occur in approximately 40% of affected patients and include palpable purpura, livedo reticularis and ischemic ulcers. Peripheral neuropathies (mononeuritis multiplex) are noted in 50% of patients and are often symptomatic early. One half of patients complain of arthralgia or myalgia. Blind muscle biopsies reveal 30-50% arteritis.

Gastro-intestinal involvement is noted in 15% of individuals with PAN, with ischemia occurring most often in the small intestine. The heart, testicles, lung and spleen are rarely involved.

Early diagnosis and treatment of PAN are necessary to prevent serious organ damage. Ten diagnostic criteria of PAN have been classified by the American College of Rheumatology and include weight loss, diffuse myalgias, mononeuropathy and hypertension. A positive angiogram with typical findings is one of the ten criteria. PAN is diagnosed if at least three of the ten criteria are present.

A definitive diagnosis may be made by performing tissue biopsy from a symptomatic organ site.

Arteriography is the preferential imaging technique for diagnosis with the most common findings being multiple saccular intraparenchymal microaneurysms, areas with intermittent smooth tapering and dilatation, and occlusions. The latter can result in organ infarcts which can be detected easily by CT. The main target organs are the kidneys and the liver. The arteriographic findings are not pathognomonic and can be found in any necrotizing vasculitis as well as in some cases of drug abuse.

Without treatment almost all patients die within 2 to 5 years. Immunosuppressive therapy is effective and has been reported to increase 5-year survival rates to 48%.

Bibliography 1. Stanson A.W., Friese J.L., Johnson C.M., et al.: Polyarteritis Nodosa: spectrum of angiographic findings. Radiographics, 2001, 21: 151-159.

2. Jee K.N., Ha H.K., Lee I.J., et al.: Radiologic findings of abdominal polyarteritis nodosa.

Am J Roentgenol, 2000, 174: 1675-1679.

3. Hagspiel K.D., Angle J.F., Spinosa D.J., et al.: Case 13: Polyarteritis nodosa â&#x20AC;&#x201C; Systemic necrotizing vasculitis with involvement of hepatic and superior mesenteric arteries. Radiology, 1999, 212: 359.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 152-153.

GLOMUS TUMOR OF THE STOMACH K. Van den Bergh, P. Lefere1 Key-word: Stomach, neoplasms

Background: A 67-year-old woman presented with acute onset of epigastric discomfort. Clinical history revealed several episodes of nausea during the last 2 years. Laboratory findings were normal. Gastroscopy showed a smooth bulging structure in the antrum with a regular surface.

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1. Department of Radiology, Stedelijk Ziekenhuis Roeselare, Roeselare, Belgium

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GLOMUS TUMOR OF THE STOMACH â&#x20AC;&#x201D; VAN DEN BERGH et al

Work-up Unenhanced CT scan of the upper abdomen (Fig. 1) includees axial (A) and coronal (B) views whichh show a well defined, non-calcified mass of 6 cm in diameter located within the gastric antrum.

The mass has a homogeneous density.

Contrast-enhanced CT scan (Fig. 2) (axial (A) and coronal (B) view) demonstrates an avidly enhancing gastric antral mass following intravenous contrast administration. No fat stranding nor adenopathies are seen.

Radiological diagnosis The woman underwent Billroth II gastric resection. Histopathologic diagnosis revealed glomus tumor of the stomach.

Differential diagnosis included adenocarcinoma, (MALT) lymphoma, mesenchymal tumor, carcinoĂŻd tumor and ectopic pancreatic tissue Discussion A glomus tumor is a mesenchymal tumor that originates from the neuromyoarterial glomus bodies. A neuromyoarterial glomus is an arteriovenous shunt, having a temperature regulating function. A glomus tumor consists of vascular channels lined by normal endothelium, surrounded by glomus cells. Glomustumors may occur anywhere in the body. The most frequent location of a glomus tumor is in the subungual region of the digits. Infrequently, they occur in the viscera, most often in the gastric antrum. These visceral lesions arise in the intramuscular layer and typically occur as a solitary submucosal nodule with or without ulceration. They usually present as an isolated mass of less than 2,5 cm diameter. Metastatic

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disease has been reported in tumors greater than 5 cm. Usually, visceral glomus tumors remain clinically silent. Sometimes they cause epigastric discomfort, infrequently complicated with gastrointestinal bleeding. Gastric glomus tumors mostly appear in the sixth decade of life, with a male to female ratio of 1 / 2,5.

Radiologically, gastric glomus tumors have an extensive differential diagnosis, including GIST, carcinoĂŻd tumor, lymphoma, lymphangioma and adenocarcinoma. Ectopic pancreatic tissue can also be present at the antrum, although it contains small nodules with central umbilication. On unenhanced CT scan, visceral glomus tumors manifest as well-circumscribed submucosal masses with homogeneous density, sometimes with flecks of calcification. They avidly enhance on post-contrast images.

In case of a solitary, hypervascular submucosal tumor of the antrum, gastric glomus tumor should certainly be included in the differential diagnosis.

Correct preoperative diagnosis is difficult.

Therefore, glomus tumors require complete resection for adequate treatment and diagnosis.

Bibliography 1. Ba-Ssalamah A., et al.: Dedicated Multidetector CT of the Stomach: Spectrum of Diseases. RadioGraphics, 2003, 23: 625.

2. Lorber J., et al.: Glomus tumor of the gastric antrum: Case report. Curr Surg, 2005, 62: 436438.

3. Kim J.K., et al.: Glomus tumor of the stomach: CT findings. Abdom Imag, 2001, 26: 303-305.

4. Park S.H., et al.: Unusual gastric tumors: radiologic-pathologic correlation. Radiographics, 1999, 19: 1435-1446.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 154-155.

PNEUMOCYSTIS CARINII PNEUMONIA IN A HIV-POSITIVE PATIENT A.H.M. van Heese1, G.A. Risseeuw2 Key-word: Pneumonia

Background: A 38-year-old man, without significant medical history, was referred to the hospital with weight loss, fatigue, night sweat, non-productive coughing and aphthae in the mouth.

Physical examination displayed a cachectic man with small lymph nodes palpable in the supraclavicular region. Auscultation of heart, lungs and abdomen displayed no abnormalities.

Blood examination revealed Hb of 5.4 mmol/l, erythrocyte sedimentation rate of 107 mm/h and leukocyte count of 6.0 x 109/l.

Additional blood examination showed a IgA of 4.2 g/l and revealed that the patient was HIV-1 positive, but seronegative for hepatitis B, hepatitis C, lues and tuberculosis.

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Departments of 1. Surgery and Tropical Medicine and 2. Radiology, Ruwaard van Putten Hospital, Spijkenisse, The Netherlands

PNEUMOCYSTIS CARINII PNEUMONIA IN A HIV-POSITIVE PATIENT — HEESE et al

Work-up On conventional chest radiograph (Fig. 1), on the top of the lungs there are cavities of different wallthickness without fluid.

CT scan of the thorax (Fig. 2) includes a section at the level of the top of the lungs (A) on which bilaterally cavitating lesions with septation are visible. Section at hilar level (B) demonstrates little round opacities with central cavitation and areas with interstitial infiltration in both lungs.

Radiological diagnosis Bronchoalveolar lavage was performed. Based on the chest X-ray findings, the CT abnormalities and the bronchoalveolar lavage, Pneumocystis carinii pneumonia was diagnosed in a HIV-positive patient.

Treatment was started with co-trimoxazol and an anti-retroviral medicine.

Discussion Pneumocystis carinii pneumonia (PCP) or P. jiroveci, is the most common opportunistic infection in HIV patients since the 1980’s. PCP is also associated with lymphoreticular malignancies and immunosuppressive therapy.

The symptoms are fever, non-productive cough and progressive dyspnea. Patients may also have weight loss, fatigue and thoracic pain. In 50% of the patients, lung auscultation is normal.

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The classic radiographic abnormalities are a ground-glass pattern, interstitial infiltrates and thin walled cysts or cavities. Atypical appearances include lobar or segmental abnormality, pneumothorax, pleural effusion, lymphadenopathy, nodules, apical consolidation or a normal chest radiograph.

The precise pathogenesis of the abnormalities is unknown but they may be due to tissue necrosis, check-valve obstruction, endobronchial invasion of PCP or result from the cytotoxic effect of HIV on the lungs.

In HIV patients with interstitial infiltrates, ground-glass pattern and cavitation in the lungs, one should consider fungal, mycobacterial or pyogenic infections, tuberculosis or Kaposi’s sarcoma as differential diagnosis.

One has to be aware of the fact that in patients with coughing or dyspnea, with normal lung auscultation and normal chest radiograph, it may be necessary to make a chest CT scan to visualize the presence of abnormalities that will point out a diagnosis, so that treatment can be started.

Bibliography 1. Hartman T.E., Primack S.L., Müller N.L., et al.: Diagnosis of thoracic complications in AIDS: Accuracy of CT. AJR, 1994, 162: 547-553.

2. Klein J.S., Warnock M., Webb W.R., et al.: Cavitating and Noncavitating Granulomas in AIDS patients with Pneumocystis pneumonitis.

AJR, 1989, 152: 753-754.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 156-157.

RUPTURED RENAL ANGIOMYOLIPOMA F. Van Hoorn, M. de Win, M. Meier1 Key-word: Lipoma and lipomatosis

Background: A 59-year-old woman presented with left-sided flank pain of 5 days duration, fever, leucocytosis and microscopic hematuria. Medical history revealed only a medically treated hypertension. Because of a suspected pyelonephritis, her general practitioner prescribed antibiotics. The severe flank pain persisted, so she was referred to the emergency department for further analysis.

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1. Department of Radiology, Academic Medical Center, Amsterdam, The Netherlands

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RUPTURED RENAL ANGIOMYOLIPOMA — VAN HOORN et al

Work-up On ultrasonography of the left kidney (Fig. 1), a hypo-echoic zone surrounding the left kidney suggestive for hematoma is noticed. A hyper-echoic, vascular tumor of 63 mm is seen in the lower pole of the kidney.

Contrast-enhanced CT scan of the abdomen (Fig. 2) shows on the axial section at the level of the kidneys (A) a tumor in the lower pole of the left kidney containing hypodense areas consistent with fat. The reformatted image (MPR) in coronal plane (B) shows the tumor as well as a hypodense area surrounding the left kidney and corresponding to hematoma. The hematoma is contained within Gerota’s fascia.

On angiography of the left kidney before treatment (Fig. 3A), a large enhancing lesion with a nidus in the lower pole is seen. After embolisation with PVA particles and subsequent coiling of a segmental renal artery (B) there is no longer enhancement of the mass, with normal aspect of the remaining parenchyma.

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when a fat containing renal mass is found by ultrasonography. Contrast-enhanced CT scan is the modality of choice to differentiate between benign or malignant tumors.

Although smaller lesions are mostly asymptomatic, AML of more than 4 cm in diameter account for 90% of all symptomatic cases. Patients can present with a diversity of complaints like flank pain, a palpable abdominal mass, hematuria or even shock.

Nowadays, renal sparing therapeutic options like nephron-sparing surgery and transcatheter arterial embolisation have replaced the total nephrectomy.

In patients with large AML with tuberous sclerosis, surgery is a good alternative because of the high recurrence rate.

In tumors that show active bleeding or in the emergency setting, embolotherapy is a safe procedure with good long-term results.

After embolisation, the aspect of the AML on imaging studies will change, reflecting the degradation of the angiomatous component of the mass, leaving a mass with characteristics of fat and muscle tissue, i.e. a myolipoma.

Radiological diagnosis Bibliography The spontaneous, non-traumatic renal hemorrhage to the subcapsular and perirenal space that occurred in our patient is also known as Wünderlich’s syndrome. As in almost all cases, the underlying pathology was an angiomyolipoma. In the presented case, this tumor was treated successfully by transcatheter arterial embolisation and coiling of the feeding arteries.

Discussion Angiomyolipoma (AML) is a benign renal tumor that contains elements of vascular tissue, smooth muscle and fat.

AML can occur as part of a tuberous sclerosis complex, but more often it is found sporadically, predominantly in women between the fourth and seventh decade of life. Diagnosis can be suggested

1. Boorjian S.A., Frank I., Inman B., et al.: The role of partial nephrectomy for the management of sporadic renal angiomyolipoma. Urology, 2007, 70: 1064-1068.

2. Chang Y.H., Wang L.J., huang C.K., et al.: The efficacy and outcomes of urgent superselective transcatheter arterial embolization of patients with ruptured renal angiomyolipomas. J Trauma 2007; 62: 1487-1490. Erratum in: J Trauma, 2007, 63: 1190.

3. Kothary N., Soulen M.C., Clark T.W., et al.: Renal angiomyolipoma: long-term results after arterial embolization. J Vasc Interv Radiol, 2005, 16: 4550.

4. Oesterling J.E., Fishman E.K., Goldman S.M., et al.: The management of renal angiomyolipoma.

J Urol, 1986, 135: 1121-1124.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 158-159.

LEFT PULMONARY ARTERY AGENESIS B.J. Van Kelckhoven, A.P.G. Van Gils1 Key-word: Pulmonary arteries, abnormalities

Background: A 45-year-old woman without medical history presented to the first aid department with shortness of breath and some mild thoracic pain. At first the conventional radiography of the chest was interpreted as normal. D-dimer concentrations were raised and a pulmonary embolism was suspected.

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1. Department of Radiology, HagaZiekenhuis, Den Haag, The Netherlands

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LEFT PULMONARY ARTERY AGENESIS — VAN KELCKHOVEN et al

Work-up Conventional radiography of the chest (PA-view) (Fig. 1) shows a right sided aortic arch, deviation of the trachea to the left, asymmetric lung-volumes, the left lung volume being smaller than the right, and abnormal pulmonary artery branching pattern on the left side. No signs of pulmonary infarction nor alveolar consolidation are noted.

Contrast-enhanced CT scan of the thorax (Fig. 2) included a transverse image at the level of the pulmonary trunk (A) that showed absence of left pulmonary artery, asymmetric lung volumes, and left lung smaller than right. Sagittal image of the left hemithorax (B) demonstrates hypertrophic intercostal arteries arising from descending aorta.

On coronal reformatted images of chest CT (C), normal branching pattern of pulmonary artery on the right, and absence of pulmonary arteries on the left are noted.

MIP-images of contrast-enhanced CT scan of the thorax (Fig. 3) shows on the left lateral view (A) and posterior view (B) a right sided aortic arch and mirror-image branching pattern of cervical vessels.

The first vessel to arise from the aorta is a left sided brachiocephalic artery (innominate artery).

Hereafter the right sided common carotid artery and subclavian artery arise from the aortic arch.

Hypertrophic intercostal arteries arise from the descending aorta. Note the absence of pulmonary arteries on the left side.

Radiological diagnosis Radiological findings are corresponding with absence of left pulmonary artery combined with right mirror-image aortic arch. No congenital heart disease was found in this patient.

Discussion Unilateral absence of the pulmonary artery is a rare congenital condition that can occur on both the left and right sides. Absence of the pulmonary artery at the pulmonary trunk or “unilateral nonconfluent pulmonary artery” which is a more accurate description of the abnormality, causes underdevelopment of the lung tissue. The abnormality may occur as an isolated lesion, but is frequently accompanied by congenital heart disease like tetralogy of Fallot or septal defects.

Findings on physical examination are unremarkable, exception made for decreased breath sounds on the affected side.

On imaging, the underdeveloped lung may give rise to an asymmetric chest appearance with mediastinal shift to the affected side as shown in

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the presented case. Elevation of the hemidiaphragm on the affected side may be seen. Systemic collateral vessels arising from the aorta e.g. bronchial arteries or intercostal arteries provide blood supply to the affected lung. Venous return to the left atrium assured through normal pulmonary veins. Rib notching due to enlarged intercostal arteries can occur.

Approximately 15% of patients are asymptomatic. About 40% of patients have mild symptoms and present with recurrent pulmonary infections and decreased exercise tolerance. The systemic flow to the affected lung causes a left-to right shunt., which can lead to pulmonary hypertension and right ventricular hypertrophy in 25% of patients. About 20% of patients present with haemoptysis due to bronchial artery hypertrophy.

One of the known accompanying congenital conditions in absence of a pulmonary artery is a right aortic arch, a condition that occurs in approximately 0.1% of the population. There are two main types of right aortic arch, classified according to its course. In type 1 a mirror-image branching pattern is seen.

First a left sided brachiocephalic artery (innominate artery) arises from the arch, then a right sided common carotid artery and finally the right sided subclavian artery. In type 2 an aberrant left subclavian artery running from the descending aorta, encircling the oesophagus and trachea is present.

This artery, called a.lusoria, forms a vascular ring encircling the oesophagus and trachea. On radiographs a right sided arch is identified as an abnormal convex right mediastinal contour, absence of the aortic contour on the left side and deviation of the trachea to the left.

In our patient there were no clinical signs of congenital heart disease, and there was no cardiologic history. Besides the absence of the left pulmonary artery and the right mirror-image aortic arch no developmental (heart) conditions were found.

Bibliography 1. Craatz S., Kunzel E., Spanel-Borowski K.: Right sided aortic arch and tetralogy of Fallot in humans-a morphological study of 10 cases.

Cardiovasc Pathol, 2003, 12: 226-232.

2. Griffin N., Mansfield L., Redmond K.C., et al.: Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases. Clin Radiol, 2007, 62: 238-244.

3. Sotomora R.F., Edwards J.E.: Anatomic identification of so-called absent pulmonary artery.

Circulation, 1978, 57: 624-633.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 160-161.

PNEUMATOSIS COLI AND INCARCERATED INGUINAL HERNIA A. Van Landeghem, B. Arys, N. Peters, K. Verstraete1 Key-word: Intestines, cysts

Background: A 53-year-old man was in follow-up for metastasized head and neck cancer. He was in a poor overall condition.

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1. Department of Medical Imaging, University Hospital Ghent, Ghent, Belgium

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PNEUMATOSIS COLI AND INCARCERATED INGUINAL HERNIA— VAN LANDEGHEM et al

Work-up On contrast-enhanced CT scan of the abdomen (Fig. 1), transverse section at the level of the upper abdomen (soft-tissue window) (A), linear to serpiginous air collections within the colonic wall at the splenic flexure are seen (white arrows). On the transverse section (lung window setting at the same level as in A) (B), pneumatosis intestinalis is best observed (black arrows). Transverse section at mid-abdominal level (soft-tissue window) (C) shows retroperitoneal air inclusions in the vicinity of the ascending colon (white arrowheads) suggesting perforation. Multiplanar reconstructed image (MPR) in the coronal plane (D) demonstrates massive incarcerated inguinal herniation with thickening of the wall of the herniated colon (arrows).

Radiological diagnosis Based on the CT findings the diagnosis of pneumatosis coli caused by an incarcerated inguinal herniation was made.

Discussion Pneumatosis intestinalis (PI) is defined as the presence of gas in the bowel wall. PI is a sign, not a disease, and it must be interpreted relative to the patient’s overall clinical condition.

The cause of PI appears to be multifactorial, the exact mechanism is not known. Several theories have been proposed. A bacterial theory proposes that the gas produced in pneumatosis is of bacterial origin.

A mechanical theory suggests that gas enters the bowel wall because of direct trauma or increased pressure. Mechanical disruption is the predisposing cause in pneumatosis associated with obstruction.

A third theory, the mucosal damage theory suggests that some type of mucosal disruption must occur so that gas or bacteria can enter the bowel wall. Mucosal disruption is the primary causative factor of pneumatosis associated with inflammation or ischemia and is also considered a

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major factor in pneumatosis associated with Crohn’s disease.

CT is the most sensitive imaging technique for identification of PI. On CT PI usually appears as a linear or bubbly pattern of gas accumulation within the bowel wall. Viewing CT images with lung windows may facilitate the detection of PI, especially in the colon.

There is a spectrum of diseases that produce this abnormality, ranging from benign to life-threatening. Cystic fibrosis, asthma and COPD are benign pulmonary causes of PI. Systemic diseases and intestinal disorders make up a large number of causes of PI. These include collagen vascular disease such as scleroderma and IBD. Corticosteroid administration is the common cause of medicationinduced PI. Mesenteric ischemia is the most common life-threatening cause of PI. Other life-threatening causes include bowel obstruction, cecal ileus, toxic megacolon.

In PI due to benign causes, the bowel wall is usually normal. The presence of additional findings, such as bowel wall thickening, absent or intense mucosal enhancement, dilated bowel loops, arterial or venous occlusion, ascites and hepatic portal or portomesenteric venous gas increases the possibility of PI due to a life-threatening cause.

Correlation with clinical, physical examination and laboratory test results is the best indicator of whether PI is due to a benign or life-threatening cause.

Bibliography 1. Connor R., Jones B., Fishman E.K., et al.: Pneumatosis intestinalis: role of computed tomography in diagnosis and management.

J Comput Assist Tomogr, 1984, 8: 269-275.

2. Galandiuk S., Fazio V.W.: Pneumatosis cystoides intestinalis: a review of the literature. Dis Colon Rectum, 1986, 29: 358-363.

3. Ho L.M., Paulson E.K., Thompson W.M.: Pneumatosis intestinalis in the adult: benign to lifethreatening causes. AJR, 2007, 188: 1604-1613.

4. Schindera S.T., Triller J., Vock P., et al.: Detection of hepatic portal venous gas: its clinical impact and outcome. Emerg Radiol, 2006, 12: 164-170.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 162-163.

ABDOMINAL MASS DUE TO CROSSED RENAL ECTOPIA AND FUSION E.J. Vandervliet1,2, F.M. Vanhoenacker1,2, P.M. Parizel2 Key-word: Kidney, abnormalities

Background: A 22-year-old woman was referred to the department of Radiology, because of a painless mass at the right hemiabdomen.

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Department of 1. Radiology, AZ St.-Maarten, Duffel and Mechelen, Belgium, and Department of 2. Radiology, Antwerp University Hospital, UZA, University of Antwerp, Edegem, Belgium

ABDOMINAL MASS DUE TO CROSSED RENAL ECTOPIA AND FUSION â&#x20AC;&#x201D; VANDERVLIET et al

Work-up Ultrasonography of the abdomen was performed (Fig. 1) and shows on the longitudinal view a mass extending along the inferior border of the right kidney (arrow).

On axial contrast-enhanced CT-image of the abdomen (Fig. 2) the absence of a kidney in the left retroperitoneal space is noted.

Coronal reformatted contrast-enhanced CTimages of the abdomen (Fig. 3) shows on A a normally located right kidney (arrow) and on B ectopic location of the left kidney, which appears to be fused with the lower pole of the right kidney (curved arrow).

On coronal MIP-image (Fig. 4), two ureters are noted, one originating at the normal, orthotopic right kidney (arrow), and the other originating at the ectopic kidney (curved arrow). The latter ureter crosses the midline and ends distally at the left posterior border of the bladder.

Radiological diagnosis Based on the characteristic imaging features, the diagnosis of crossed fused renal ectopia is made.

Discussion Renal ectopia (RE) is a rare condition in which one or both kidneys are abnormally located in the retroperitoneum due to a developmental anomaly.

In ipsilateral RE the kidney is located on the same side as its ureteral orifice, whereas in crossed RE both kidneys are located in the same body half, albeit with their ureteral orifices normally situated.

In the case of crossed RE many variants exist, with or without fusion of the kidneys. Only very seldom is there a solitary kidney.

Appearing at approximately 5 or 6 weeks of gestation at the level of the S2 vertebra, kidney and ureter develop from the renal blastema and the ureteric bud respectively. The ureteric bud and the blastema fuse and ascend as immature kidneys in the retroperitoneum towards the final location of the kidneys in the retroperitoneal space. Anomalies

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develop due to any combination of growth arrest, arrest of renal ascent and fusion.

In autopsy series the incidence of crossed renal ectopia is estimated as 1 in 2000, 85% of which are fused variants. The male to female ratio is 3 to 2.

An autosomal recessive trait has been suggested and in animals teratogenic agents have been incriminated.

The most common associated anomalies include ectopic ureter, extrarenal calices, urethral valves, anorectal malformations and vertebral anomalies.

If symptomatic at all, the most common symptoms are pain and hematuria due to urolithiasis.

Reflux is a very common finding, resulting in infection and even in end-stage renal insufficiency. A higher incidence of renal cell carcinoma has been described in RE. Plain radiographs may show the absence of normal kidney shadow and an abdominal mass alongside the normal kidney. CT is considered the gold standard in the diagnosis as it offers excellent visualization of the implantation of the ureters and the vascular supply and may differentiate between fused and nonfused variants. Ultrasound is comparable to CT. MRI has no clear role in the diagnosis.

The differential diagnosis includes horseshoe kidney, which is the most common fusion anomaly of the urinary tract, transplanted kidney and acquired renal displacement, for instance due to hepatomegaly.

Therapy is limited to treatment of symptoms and complications.

Bibliography 1. Friedland G.W., de Vries P.: Renal ectopia and fusion. Embryologic Basis. Urology, 1975, 5: 698706.

2. Guarino N., Tadini B., Camardi P., et al.: The incidence of associated urological abnormalities in children with renal ectopia. J Urol, 2004, 172: 1757-1759.

3. Hertz M., Rubinstein Z.J., Shahin N., et al.: Crossed renal ectopia: clinical and radiological findings in 22 cases. Clin Radiol, 1977, 28: 339344.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 164-165.

RHINOLITH OF THE NASAL SEPTUM E.J. Vandervliet1,3, F.M. Vanhoenacker1,3, J. Van Haesendonck2, P.M. Parizel3 Key-word: Foreign bodies, in air and food passages

Background: A 42-year-old woman was referred to the department of Head and Neck Surgery, because of left sided nasal obstruction and foul smell. She underwent an endoscopic investigation of the nasal cavities, during which a brown-grayish mass was noted resting on the floor of the left nasal cavity.

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Department of 1. Radiology and 2. Head and Neck Surgery, AZ St.-Maarten, Duffel and Mechelen, Belgium and 3. Department of Radiology, Antwerp University Hospital, UZA, University of Antwerp, Edegem, Belgium

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RHINOLITH OF THE NASAL SEPTUM â&#x20AC;&#x201D; VANDERVLIET et al

Work-up Axial CT image of the nasal cavities and sinuses (Fig. 1) shows a dense radiopaque structure located centrally in the left nasal cavity.

On coronal MPR-image of the nasal cavities and the maxillary sinuses (Fig. 2), note the deformity of the concha nasalis inferior, which appears to be displaced upwards.

There is a slight deviation of the septum to the right side.

On sagittal MPR- image of the left nasal cavity and the left frontal and sphenoid sinuses (Fig. 3) it appears that the radiopaque structure is located about halfway between the anterior and posterior portion of the nares.

Radiological diagnosis The CT images suggest a rhinolith with associated subtle chronic inflammation of the maxillary sinuses. Endoscopic removal of the radio-opaque fragment revealed a small, metal screw.

Discussion A rhinolith is an uncommon finding, presenting as a hard, dense and usually irregular mass in the nasal cavity. It is most often found on the floor of the nose, halfway between the anterior and posterior portion of the nares. Very rarely, it arises in the frontal or maxillary sinuses. Males and females are equally affected and rhinoliths have been reported in all age groups, most often however in children.

Rhinoliths consist of calcarous encrustations of a nidus that can be endogenous or exogenous in nature.

Endogenous nuclei include dried blood clots or mucus, teeth and (post-traumatic or post-surgical) bony fragments.

Exogenous nuclei seem to be more common and include fruit seeds, beads, buttons, pebbles, cotton, or (as in the current case) a metal screw, usually inserted via the nostrils in childhood and then accidentally forgotten. Sneezing, coughing or vomiting, resulting in regurgitation of material into the nasal cavity, have also been reported as a cause. If present at all, symptoms can be purulent rhinorrhea, unilateral nasal obstruction, local pain, headache, fever, anosmia and odor (because of foul-smelling discharge). Cases have been reported

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of rhinoliths causing secondary chronic sinusitis, erosion of the wall between the nasal cavity and the maxillary sinus, and perforation of the palate.

The pathogenesis remains unclear, but it is suggested that the foreign body incites an inflammatory reaction with deposition of mineral salts. Physical and chemical factors (pH changes, hypersaturation of secretions and enzymatic activities of bacteria) as well as mechanical factors (stasis of nasal secretions and tears and alterations of aerial flow) are thought to contribute to this process. Chemical analysis of the stones may reveal deposits of calcium phosphate and/or magnesium phosphates around a nucleus consisting of magnesium containing whitlockite, but stones consisting of siderite and ferrihydrite with a nucleus of high iron content have also been reported. In all cases the predominant material is anorganic, with organic components mostly deriving from nasal secretions.

Conventional radiography may reveal a radiopaque lesion with a central area of lesser opacity on the floor of the nasal cavity. Several projections taken from different angles to evaluate the precise size and location are often required. CT is the preferred imaging modality, because of its superior sensitivity and specificity for identifying calcifications and foreign bodies in a complex anatomical area such as the paranasal sinuses.

The differential diagnosis includes other disorders, such as calcified polyps, odontoma, osteoma, ossifying fibroma, tori, osteosarcoma, retained roots and impacted teeth.

Treatment consists usually of endoscopic removal under local anaesthesia. In rare cases of firmly adherent stones, an extended surgical approach is mandatory (lateral rhinotomy). When the rhinolith is very large, it can be crushed manually or by using a lithotripter.

Bibliography 1. Barros C.A., Martins R.R., Silva J.B., et al.: Rhinolith: a radiographic finding in a dental clinic. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 2005, 100: 486-490.

2. Hadi U., Ghossaini S., Zaytoun G.: Rhinolithiasis: A forgotten entity. Otolaryngol Head Neck Surg, 2002, 126: 48-51.

3. Keck T., Liener K., Sträter J., et al.: Rhinolith of the nasal septum. Int J Pediatr Otorhinolaryngol, 2000, 53: 225-228.

JBRâ&#x20AC;&#x201C;BTR, 2010, 93: 166-167.

LIPOMA ARBORESCENS OF THE KNEE T. Weits1, B.H. Tania2 Key-word: Lipoma and lipomatosis

Background: A 35-year-old man presented with a progressive painless swelling in the right knee.

Medical history mentioned an arthroscopic partial medial meniscectomy. There was no recent trauma nor fever.

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LIPOMA ARBORESCENS OF THE KNEE â&#x20AC;&#x201D; WEITS et al

Work-up Plain radiograph of the right knee (Fig. 1) includes an antero-posterior view (A) and a lateral view (B) on which no bony abnormalities are visible. There is a slight narrowing of the medial joint space. Intra-articularly, a fatty mass is suspected with distension of the joint capsule and suprapatellar pouch.

MRI of the right knee (Fig. 2) consisted of a sagittal unenhanced T1W image (A), a coronal T2W fat saturation image (B), a sagittal fat saturation image (C) and a coronal fat saturation image (D). A large frond-like, intra-articular fatty mass of synovial origin is visible. There is distension of the joint capsule, including the supra-patellar pouch.

Some degenerative abnormalities are seen in the medial compartment of the knee. Saturation of the synovial mass is visible when the fat saturation technique is used.

Radiological diagnosis Based on the imaging and histopathology findings, the diagnosis of lipoma arborescens was made.

Discussion Lipoma arborescens is a very rare frond-like intra-articular mass, characterized by villous lipomatous proliferation of the synovium, usually involving the knee joint. It forms part of the differential diagnosis for a slowly progressive chronically swollen knee.

Most likely it is an idiopathic, unusual response to chronic synovial irritation and underlying degenerative joint disease.

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The clinical presentation is a painless swelling of the knee and this abnormality is reported in adolescents and adults, with no predilection of gender.

MRI shows a high accuracy in the identification and characterization of lipoma arborescens.

Saturation of the synovial mass on fat saturation techniques is diagnostic evidence.

MRI allows correct evaluation of size and grade, accurate treatment planning (synovectomy) and effective follow-up.

The differential diagnosis consists of the following disorders: synovial lipoma, which occurs usually a single mass in or on Hoffaâ&#x20AC;&#x2122;s fat pad, synovial osteochondromatosis, calcified masses, synovitis, visualized as thickened synovium but without saturation with fat saturation techniques, and loose bodies, visualized as decreased signal structures most often calcified, in some cases with a cortical rim.

Other causes of synovial proliferation should be considered. Saturation of the frondlike synovial masses on fat saturation techniques leads to the diagnosis.

Bibliography 1. Davies A.P., Blewitt N.: Lipoma arborescens of the knee. Knee, 2005, 12: 394-396.

2. Feller J.F., Rishi M., Hughes E.C.: Lipoma arborescens of the knee: MR demonstration.

AJR, 1994, 163: 162-164.

3. Ikushima K., Ueda T., Kudawara I.: Lipoma arborescens of the knee as a possible cause of osteoarthrosis. Orthopedics, 2001, 24: 603-605.

4. Kloen P., Keel S.B., Chandler H.P.: Lipoma arborescens of the knee. J Bone Joint Surg Br, 1998, 80: 298-301.