The multiple facets of pulmonary sequestration☆

Abstract 

Purpose: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. Methods: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. Results: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. Conclusions: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations. J Pediatr Surg 36:784-790. Copyright © 2001 by W.B. Saunders Company.

Keywords:  Pulmonary sequestration, communicating bronchopulmonary foregut malformation, congenital cystic adenomatoid malformation, bronchogenic cyst, scimitar syndrome, fetal/prenatal diagnosis