Imperial Journal of Interdisciplinary Research (IJIR) Vol-3, Issue-2, 2017 ISSN: 2454-1362, http://www.onlinejournal.in
Case Report: Congenital Cystic Adenomatoid Malformation of an Entire Lung in a 17-YearOld Girl Coexisting With VSD. Dr. Bssem Sahsah Mohammed, Dr. Byan Ahmed Altorbak, Dr. Ghadeer Saleh Alenzi & Ziad alhomidan Abstract: Congenital cystic adenomatoid malformations (CCAM) ,also known as congenital pulmonary airway malformation, is a rare ,noninherited developmental abnormality of lung with incidence of about 1 in 30,000. It arise from excessive unsystematic proliferation of tubular bronchial structures that forms non-working cystic lesion of abnormal lung tissue. We present an unusual case of a 17-year-old girl whom presented with congenital cystic adenomatoid malformation of an entire lung and VSD. Keywords: Congenital malformation, CCAM.
cystic
No other medical conditions or diseases. No family history of diseases or congenital anomalies. On Examination: Patient looked ill, was conscious and oriented Chest examination: Decreased air entry on the left side Rt lung Crepitation S1+S2 with pan systolic murmur.
adenomatoid
Introduction Congenital cystic adenomatoid malformations is rare with incidence of about 1 in 30,000, it is a nonhereditary anomaly of lung structural and functional development so that there is adenomatoid proliferation of cysts, rather than normal tissues,it was classified into five types by Stocker in 2002.CCAM is usually manifests in the first 2 years of lifein respiratory distress or by repeated respiratory infection later on. We present arare case of a 17-year-old girl with CHD whom presented withshortness of breath and right side chest pain,later on to be diagnosed with congenital cystic adenomatoid malformation of an entire lung.
Case Report A 17-year-old girl presented to ER with a history of shortness of breath and right side intermittent chest pain that increase with respiration and movement since 6 days ago with intermittent cough, no hemoptysis. She came from a low-income family and has VSD, had past history of repeated similar conditions in the past.
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Abdominal examination: Soft , lax no organomegaly. Vital signs: Temperature: 36.4 °C Pulse=103 /minute Respiratory Rate=22/minute. BP=134/60 mm hg On X ray Hyper inflated right lung White left lung left meditational shift On CT Hypo plastic left lung with multiple macro cysts And will indentified mass in the upper right lung Course of treatment: Page 900
Imperial Journal of Interdisciplinary Research (IJIR) Vol-3, Issue-2, 2017 ISSN: 2454-1362, http://www.onlinejournal.in O2 supplementary 4 L/ min Mask Perfalgan 1G IV Tab Lasix 20 mg OD Amoxicillin tab every 8 hours
Creatinine=38.18 (low) Total bilirubin=32.5(high) Creatine kinase MB= 27(high) Phosphourous=2.276(high) Potassium=3.3 Glucose =6.11
The patient developed septic shock and type 1 respiratory failure and arrested she did not respond to resuscitation according to ACLS protocol and was announced died after 3 days since admission to ICU. Autopsy could not be perform.
Discussion
Injection Zinforo dose 760 mg
Congenital cystic adenomatoid malformation is a rare hematoma of the lung due to faulty embryonic development and proliferation of
Every 8 hours Steam inhalation She was admitted for 7 days into medical ward. After improvement discharged. However, she deteriorated 2 days later, developed acute respiratory failure, and had to be intubated then shifted to ICU. First day:
Second day:
CBC (ICU) Third day:
WBC=10.19 RBCs=9.18 HGB=16.3 PLT=294
WBC=11.9 RBCs=7.91 HGB=14.5 PLT=236
WBC=12.42 RBCs=8.61 HGB=15.3 PLT=283
Tubular bronchial structures, often affecting one lung lobe and both lungs equally. CCAM is usually discovered prenatallyon prenatal ultrasound or in neonatal period presenting with respiratory distress due to surrounding structures compression. Rarely is it discovered in adulthood with repeated respiratory infections. CCAM can predisposes to the development of neoplasm, such as rhabdomyosarcoma and pneumothorax. Congenital cystic adenomatoid malformation was first described by Ch'in and Tang in 1949; although it is named congenital cystic adenomatoid malformations only 3 out of the 5 types have cysts. In 2002 Stocker classified it into 5 types :
Other tests: Blood urea nitrogen=1.3 (low) Types
Frequency
Prognosis
Type 0
can involve a whole lung it appears small and solid with cartilage, smooth muscle, intertwined by abundant mesenchymal tissues.
Rare
Usually Fatal
Type 1
Cyst(s)2-10 cm in diameter associated with malignancy
≈ 65%
good prognosis
Type 2
Sponge like multiple small cysts (<2 cm) ,usually associated with other systemic anomalies.
≈ 15%
poor prognosis
Type 3
mostly solid associated microscopic adenomatoid cysts
≈5%
poor prognosis can be fatal
Type 4
Large cysts air filled or fluid filled ,associated with pneumothorax
≈ 10%
good prognosis
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Imperial Journal of Interdisciplinary Research (IJIR) Vol-3, Issue-2, 2017 ISSN: 2454-1362, http://www.onlinejournal.in
Early diagnosis and management is crucial for prevention of deterioration of patient condition and prevention of complications. Surgical intervention is the definitive management in symptomatic patient to allow normal tissue expansion and prevention of deterioration of preexisting problems.
malformation in a 5-month-old infant. Lung India 2016;33:208-11
Conclusion
3.Chikkannaiah, Panduranga, Ranjit Kangle, and Manjiri Hawal. "Congenital cystic adenomatoid malformation of lung: Report of two cases with review of literature." Lung India: official organ of
Congenital cystic adenomatoid malformation is a hematoma of the lung without an identifiable cause that could cause problems in fetal period(hydrops and stillbirth),neonatal period(respiratory distress) or rarely later on manifesting in respiratory distress, neoplasm or repeated respiratory infection among others or could remain symptomless . Early identification is important to allow for surgery along other modalities for optimal results.
Footnotes: No sponsor nor support. There was no conflict of Interest.
2.Bolde S, Pudale S, Pandit G, Ruikar K, Ingle SB. Congenital pulmonary airway malformation: A report of two cases. World Journal of Clinical Cases : WJCC. 2015;3(5):470-473. doi:10.12998/wjcc.v3.i5.470.
Indian Chest Society 30.3 (2013): 215. 4.Feng A, Cai H, Sun Q, Zhang Y, Chen L, Meng F. Congenital cystic adenomatoid malformation of lung in adults: 2 rare cases report and review of the literature. Diagnostic Pathology. 2012;7:37. doi:10.1186/1746-1596-7-37. 5.Sfakianaki AK, Copel JA. Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration. Reviews in Obstetrics and Gynecology. 2012;5(2):85-93. 6.Sood, Mangla. "Congenital cystic adenomatoid malformation of the lung: a case report." Journal of Nepal Paediatric Society 31.1 (2011): 64-67. 7.Ch'in KT, Tang MY. Congenital adenomatoid malformation of one lobe of lung with general anasarca. Arch Path. 1949. 48:221-9. 8.Singh, Sudesh, et al. "Congenital Cystic Adenomatoid Malformation (CCAM) of Lung in an Infant: A Case Report from Jammu & Kashmir, Northern India." International Journal of Pediatrics 3.2.2 (2015): 481-484. 9.TAŞTEKİN, Ebru, et al. "Congenital pulmonary airway malformation type 2: a case report with review of the literature." Japanese Journal of Clinical Oncology 44.3 (2014): 278-281. 10.Kwak, Hyun Jung, et al. "Congenital cystic adenomatoid malformation with bronchial atresia in elderly patients." Tuberculosis and respiratory
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