Congenital pulmonary malformations: clinical, radiological and treatment features

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Published Jan 29, 2013
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Vol. 91 No. 1 (2013): Issue Jan 2013

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Rabiaa Ben Abdallah
Habib Bouthour
Youssef Hellal
Mohamed Riadh Ben Malek
Youssef Gharbi
Nejib Kaabar

Abstract

Background: Congenital broncho-pulmonary malformations (CBM) are rare, essentially presented by congenital lobar emphysema, bronchogenic cysts, pulmonary sequestrations and cystic adenomatoid malformations. The diagnosis can be in prenatal. In postnatal, symptoms are variable. Radiological investigations lead to diagnosis in all cases.
Aim: To study the principal clinic, radiologic and therapeutic of the congenital broncho-pulmonary malformations through ten cases.
Methods: Retrospective study of 10 cases of congenital bronchopulmonary malformations diagnosed between 2003 and 2010 in our institution.
Results: The mean ages at the time of diagnosis is 2months (4 days to 16months). The sex ratio is 1. The symptoms consisted of recurrent pneumonia in 4cases, respiratory distress in 2cases,bronchiolite in 2 cases and 2cases of antenatal diagnosis. All patients have a chest X-ray, night patients have a chest computerized tomography and one patient has a bronchial endoscopy. Ten cases of BPM have been investigated: five congenitals lobar emphysema, tow pulmonary sequestrations, tow cystic adénomatoid malformation and one bronchogenic cyst. Eight patients required surgical treatment involving pneumonectomy (1case), lobectomy (5 cases),segmentectomy (1 case) and in 1 case the pulmonary sequestration was treated by ligature of the anomalous artery with pulmonary resection. The histopathological examination confirmed the diagnosis in all cases. The postoperative period was uneventful in 8 cases with a mean of follow-up of 2 years (5 months to 5years). Tow patient died after surgical treatment.
Conclusion: The diagnosis of BPM malformations can be clinical, confirmed by radiological investigations. The improvement in prenatal ultrasound diagnosis modified the management strategy. The treatment varies frome attitude conservatrice to pneumonectomy.

Keywords:

Broncho-pulmonary malformations -lobectomy-pneumonectomy

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