Pulmonary sequestration: 17-year experience at UCLA

Pulmonary sequestration is a complex anomaly involving the pulmonary parenchyma and its vascularity. From 1975 to 1992, 10 cases have been treated at the UCLA Medical Center. The ages of the seven females and three males were bimodal, the median age of the seven children was 29 days (range 1 day-6 years); it was 32 years (range 28-39) for the three adults. One child was delivered by cesarean section for fetal distress and another was born at 29 weeks gestation. Symptoms included: recurrent pneumonia (5), respiratory distress (5), hemoptysis (2), stridor (1), and pleuritis (1). Chest radiographs were consistent with sequestration in seven patients and diaphragmatic hernia in another, but incorrectly diagnosed one diaphragmatic eventration and one pulmonary varix. Computed tomographs suggested sequestration in five patients, but mistakenly interpreted a pulmonary varix in one case. Angiography, ultrasonography, and magnetic resonance imaging were infrequent investigative studies and yielded variable results. All patients underwent thoracotomy and lobectomy without morbidity or mortality. Five had intralobar and five had extralobar sequestration. All adults had intralobar sequestration. Two had aberrant subdiaphragmatic arterial vessels. Eight had vessels originating from the descending thoracic aorta. Nine patients are asymptomatic at six months to 10 years follow up. The 29-week-old premature infant died 30 days postoperatively due to necrotizing enterocolitis. Pulmonary sequestration remains an uncommon entity. The radiologic investigations that provide the most information are the chest radiography and computed tomography. Definitive diagnosis is made at thoracotomy. Pulmonary sequestrations are resected with excellent results by the trained thoracic surgeon who is aware of the unusual vascular connections.