Congenital bronchopulmonary malformations. Diagnostic and therapeutic considerations.

Congenital bronchopulmonary malformations are uncommon but potentially life-threatening anomalies of infants and children. Between 1970 and 1988, 45 patients from birth to 13 years of age (23 boys and 22 girls) underwent evaluation and treatment for bronchopulmonary malformations. Thirty-seven had solitary lesions: bronchogenic cyst (n = 13), cystic adenomatoid malformation (n = 9), congenital lobar emphysema (n = 6), pulmonary sequestration (n = 6), arteriovenous malformation (n = 2), and bronchial atresia (n = 1). Eight additional patients had two simultaneous abnormalities and three patients had congenital diaphragmatic hernias. Twenty-one patients had respiratory symptoms, which were severe in seven. Twelve had pulmonary infection and 10 patients were completely free of symptoms. Plain chest roentgenogram was the only diagnostic imaging performed in 11 patients. Thirteen patients underwent computed tomographic scan, but in only four was it essential for diagnosis. Prenatal ultrasonography in three patients demonstrated cystic adenomatoid malformation in two, with one false negative study. Postnatally, ultrasonography was also useful in establishing the diagnoses of cystic adenomatoid malformation and pulmonary sequestration. Thoracotomy with excision of the lesion by lobectomy or pneumonectomy resulted in survival of 42 patients (93%). Three deaths in neonates were due to pulmonary hypoplasia and hypertension. Two of them had concomitant diaphragmatic hernia; the other had a cystic adenomatoid malformation and died despite the use of postoperative extracorporeal membrane oxygenation. These data demonstrate that congenital bronchopulmonary malformations usually can be diagnosed by plain chest x-ray films. Ancillary studies such as ultrasonography or computed tomography may occasionally be necessary. Combinations of the different types of bronchopulmonary malformations occurred frequently. All lesions, including symptomatic lesions in neonates, can be managed surgically soon after diagnosis.