Congenital lung malformations--antenatal and postnatal evaluation and management

We reviewed our institutional experience with pulmonary resection for congenital bronchopulmonary malformations and analysed the management and outcome of pregnancies with a prenatal diagnosis of congenital lung malformations. Between January 1993 and December 2003, 31 patients underwent evaluation and pulmonary resection for bronchopulmonary malformations. Common clinical presentations were respiratory distress (9), respiratory infections/pneumonias (22), and dyspnoea (9). Diagnostic modalities included chest radiography, CT scan (22), MRI scan (7), arteriography (1), and bronchoscopy (5). There were 13 congenital cystic adenomatoid malformations (CCAM), six pulmonary sequestrations, three bronchogenic cysts, and nine congenital lobar emphysemas (CLE). Fifteen patients who underwent resection were diagnosed by antenatal ultrasound. No foetus had hydrops or associated malformations. No pregnancy was terminated. There was no foetal demise. Regression of the sonographic appearance was observed in six cases. Amniotic puncture was required for hydramnios in three cases. Eight emergency resections were performed (CCAM 4; CLE 3; Bronchogenic cyst 1). Surgical procedures included 24 lobectomies, one right middle lobectomy with a wedge resection of the right lower lobe, one completion right lower lobectomy, four sequestrectomies, one mediastinal mass excision and one wedge resection for a bronchogenic cyst. There were no deaths. Postoperative complications included: persistent air leak (n=2; one requiring completion lobectomy) and pneumothorax (1). Persistent mild symptoms were present in five patients, at long-term follow-up. Congenital cystic adenomatoid malformation and congenital lobar emphysema were the commonest congenital anomalies. Congenital lung malformations are increasingly diagnosed antenatally, sometimes necessitating emergent surgical resection. The natural history is variable. All infants with a prenatal diagnosis require postnatal evaluation. Patients should be evaluated for associated disorders. The presence of mass effects is an indication for therapeutic decompression. The risk of pulmonary compression, infection and malignant degeneration makes resection imperative, even in asymptomatic patients. Lobectomy is the procedure of choice, is well tolerated, and leads to excellent outcomes.