Surgical management of congenital pulmonary malformations after the first decade of life

Abstract

Background: Most congenital pulmonary malformations are discovered early in life, but some are diagnosed in adulthood. We evaluated patients treated surgically after the first decade of life.

Methods: All patients who underwent surgical treatment for a congenital pulmonary malformation diagnosed after 10 years of age at a single institution from 1997 to 2012 were evaluated for presenting symptoms, surgical management, perioperative outcomes, and symptom resolution.

Results: Twenty-two patients met the inclusion criteria. The most common malformations were pulmonary sequestration (n = 12, 55%), congenital cystic adenomatoid malformation (n = 2, 9%), and bronchial agenesis (n = 2, 9%). The median age at diagnosis was 36 years (range, 10-66 years). The most common presenting symptoms were dyspnea (n = 6, 27%) and hemoptysis (n = 4; 18%); 4 (18%) asymptomatic patients received diagnoses. The median duration of symptoms before operation was 14 months. An emergency room visit or hospitalization occurred in 11 patients (50%) before their referral for surgical evaluation. The surgical approach was thoracotomy for 7 patients (32%) and thoracoscopy for 15 patients (68%). All vascular anomalies requiring a pneumonectomy (n = 3, 14%) were done by a thoracotomy, and 83% (10/12) of pulmonary sequestrations were treated thoracoscopically. The median hospital stay was 3 days. There were no perioperative deaths, and minor morbidity occurred in 4 patients (18%). Complete resolution of symptoms after operation occurred in 94% (16/17) of patients, with a median follow-up time of 3 weeks.

Conclusions: Early surgical management of congenital pulmonary malformations found after the first decade of life is recommended to control symptoms and avoid hospitalizations. Most adult pulmonary sequestrations can be treated with minimally invasive techniques.