Extralobar sequestration of the lung in children.

Sixteen patients with extralobar sequestration of the lung are reported on. The age at time of diagnosis varied from 21 days to 12 years (mean 3.6 years). Most of the patients presented with respiratory symptoms, such as dyspnoea, pneumonia, coughing attacks, cyanosis and asthmatic symptoms, but diagnosis was made incidentally in 4. Diagnosis was established by chest X-ray in every case. Preoperative angiography and bronchography was carried out in six cases. All 16 patients underwent thoracotomy for removal of the sequestration. Rudimentary bronchial remnants were found in 6 cases. Extralobar sequestration was associated with 12 additional malformations in 10 of the 16 patients. The clinical picture varies considerably and many of the "typical" features, such as systemic arterial blood supply, left lower lobe localization and diaphragmatic defect, are often absent. Diagnosis and treatment are easy.