Congenital bronchopulmonary malformations: a single-center experience and a review of literature

Ann Thorac Med. 2008 Oct;3(4):135-9. doi: 10.4103/1817-1737.43080.


Purpose: To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature.

Materials and methods: We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.8 months).

Results: Out of 25 patients, 18 (72%) were male and 7 (28%) were female. Age of patients ranged from 1 day to 11 years. The histopathological diagnosis was congenital cystic adenomatoid malformations [CCAM; n = 14 (56%)], congenital lobar emphysema [CLE; n = 5 (20%)], pulmonary sequestrations [PS; n = 3 (12%)], and bronchogenic cysts [BC; n = 3 (12%)]. Antenatal diagnosis was available in only 2 (8%) patients. The common presenting symptoms were respiratory distress and chest infections. Lobectomy was the procedure of choice. Mortality was 16% (n = 4; M: F = 3: 1). Two patients died because of overwhelming sepsis, one from compromised cardiac function, and one from aspiration which might possibly have been prevented.

Conclusion: Patients with progressive respiratory distress due to these anomalies may require urgent surgical intervention regardless of age. The surgical outcome is favorable, with manageable complications. Plain x-ray chest and CT of thorax are usually sufficient for diagnosis and planning of treatment. Pathological diagnosis may differ from the imaging diagnosis. Mortality is found to be more in neonates. Apart from initial stabilization, resection of lesion and careful postoperative care is necessary to reduce mortality and morbidity.

Keywords: BC; CCAM; CLE; PS; congenital bronchopulmonary malformations; lobectomy.