PULMONARY SEQUESTRATION - 17-YEAR EXPERIENCE AT UCLA

Pulmonary sequestration is a complex anomaly involving the pulmonary p arenchyma and its vascularity. From 1975 to 1992, 10 cases have been t reated at the UCLA Medical Center. The ages of the seven females and t hree males were bimodal, the median age of the seven children was 29 d ays (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 distres s and another was born at 29 weeks gestation. Symptoms included: recur rent pneumonia (5), respiratory distress (5), hemoptysis (2), strider (1), and plueritis (1). Chest radiographs were consistent with sequest ration in seven patients and diaphragmatic hernia in another, but inco rrectly diagnosed one diaphragmatic eventration and one pulmonary vari x. Computed tomographs suggested sequestration in five patients, but m istakenly interpreted a pulmonary varix in one case. Angiography, ultr asonography, and magnetic resonance imaging were infrequent investigat ive studies and yielded variable results. All patients underwent thora cotomy and lobectomy without morbidity or mortality. Five had intralob ar and five had extralobar sequestration. All adults had intralobar se questration. Two had aberrant subdiaphragmatic arterial vessels. Eight had vessels originating from the descending thoracic aorta. Nine pati ents 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 c hest 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 va scular connections.