Lung malformation
 
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Question :
Mukul Aggarwal, Vikrant Sood
Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi

Address for Correspondence: Dr Vikrant Sood, Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi. Email: drvickyster@gmail.com

Spot diagnosis
Expert Opinion :
Bronchogenic cyst. It represents second most common {7-20 percent} subtype of foregut cysts found in middle mediastinum {1}. It arises as a result of abnormal budding of the lung diverticulum. There are 5 groups on the basis of topography - paratracheal, carinal, hilar, para-esophageal and miscellaneous. {2} Symptomatic patients usually present with symptoms related to cyst infection or compression of adjacent structures. In infants it presents with respiratory distress. It may even present with superior vena cava syndrome and pneumothorax {3}. More than 50 percent of those mediastinal cysts are at or above carina {4}. Signs of airway compression- cough, wheeze, dyspnea, and respiratory distress may be present with features of secondary infection. Four types of presentation are seen namely- asymptomatic, general symptoms {fever}, respiratory symptoms and other symptoms-dysphagia {4}. Differentials may include congenital lobar emphysema, cystadenomatous malformation. Chest CT scan is the preferred modality for imaging. Treatment involves surgical resection. In newborns with asymptomatic bronchogenic cysts, surgical intervention is recommended at age 3-6 months, allowing for compensatory lung growth. Classical surgical excision with thoracotomy is preferred for bronchogenic cysts adherent to surrounding tissues. VATS is useful in elective patients. Palliative procedures such as puncture and aspiration are considered if complete resection not possible. Most common complications are compression and pulmonary infection. Postoperative outcomes are excellent.

References:
1. Shanti CM, Klein MD. Cystic lung disease. Semin Pediatr Surg. 2008` 17: 2-8
2. Maier HC. Bronchogenic cysts of mediastinum. Ann Surg 1948` 127: 476-502
3. Aktogu S, Yuncu G, Halilçolar H, Ermete S, Buduneli T. Bronchogenic cysts: clinicopathological presentation and treatment. Eur Respir J. 1996` 9: 2017-2021
4. Ribet ME, Copin MC, Gosselin BH. Bronchogenic cysts of the lung. Ann Thorac Surg. 1996` 61: 1636-1640
5. Limaïem F, Ayadi-Kaddour A, Djilani H, Kilani T, El Mezni F. Pulmonary and mediastinal bronchogenic cysts: a clinicopathologic study of 33 cases. Lung. 2008` 186: 55-61

E-published: November 2010 Vol 7 Issue 11 Art # 71

Correct Answers : yes  9%

Last Shown : Nov 2010
 
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