CONGENITAL-MALFORMATIONS OF THE LUNG AND MEDIASTINUM - A QUARTER-CENTURY OF EXPERIENCE FROM A SINGLE INSTITUTION

Congenital malformations of the lung are rare and vary widely in their presentation and severity. The authors reviewed 25 years of experienc e with specific reference to diagnosis, treatment, and outcome. From J uly 1970 to June 1995, 70 patients were diagnosed with congenital malf ormations of the lung, which included sequestration (n = 20), foregut anomalies (n = 20), congenital lobar emphysema (CLE; n = 10), congenit al cystic adenomatoid malformation (CCAM; n = 5), benign lung cysts (n = 6), lung aplasia/hypoplasia (n = 4), and other miscellaneous disord ers (n = 5). All patients with pulmonary hypoplasia presented at birth . With the exception of one patient, infants with CCAM and CLE present ed before 5 months of age. All other patients presented from birth to 16 years of age. A prenatal diagnosis was accurate in two patients. Al though prompt surgical management is the rule, the exceptions were chi ldren with bilateral lung involvement. Corrective surgery was delayed in some patients in whom extended respiratory support was required or in whom the delay led to an increase in pulmonary reserve. Extracorpor eal membrane oxygenation (ECMO) was used in two patients pre- and post operatively to manage persistent pulmonary hypertension. This review, representing the largest series of congenital lung lesions, showed tha t (1) prenatal diagnosis is useful but generally does not change the o utcome; (2) computerized tomography (CT) is the optimum postnatal diag nostic imaging modality if chest radiography is not definitive; (3) EC MO can be an adjunct in treating associated pulmonary hypertension; (4 ) pulmonary resection (lobectomy) is the surgical procedure of choice for most lesions; (5) expected survival is good; and (6) pulmonary hyp ertension is the most common cause of mortality. Copyright (C) 1997 by W.B. Saunders Company