Ten years experience in surgical management of congenital lung malformations: A prospective, cross sectional study

Abstract

Background: Congenital lung malformations are very rare lung lesions caused by abnormal lung development occurring at different stages of intrauterine life. They are a spectrum of congenital malformations involving the trachea-bronchial tree, pulmonary parenchyma, and the blood vessels. They are a family of pulmonary lesions that include congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations, bronchogenic cysts (BC), and congenital lobar emphysema (CLE). The aim of the study is to determine the pattern of congenital malformation of the lungs and the outcomes of treatment in Lagos. Methods: This is a prospective study of all patients with congenital lung malformations referred to our center in the period between January 2011 and December 2020. All pediatric cases were diagnosed by the pediatricians in our center and confirmed with chest computed tomography (CT) scan. The adult cases were equally confirmed with a chest CT scan. The lesions encountered in this study include BC, CPAM, bronchopulmonary sequestrations, lung hypoplasia (LH), CLE, and pulmonary arterio-venous malformations. Data collected include biodata, type of malformation, mode of management (surgery), and postoperative outcome and were analyzed as percentages and mean. Results: A total of 18 patients who were diagnosed with congenital lung malformations were recruited into this study. The congenital malformations included CLE, CPAM, BC, lung sequestration, LH, and arterio-venous malformation of the lung. Children accounted for 78% (13) of the population, while the adult population was 28%.(5) The neonates constituted 22% (4) with a mean age of 7.5 ± 5.1, whilst the older children made up 50%, with a mean age of 18.8 ± 10.7 months. The mean age of the adult population was 39 ± 15 years. Seventeen (94.41) had surgery, whilst one opted for continued medical surveillance. Two patients died from postoperative respiratory failure. Conclusion: Congenital lung malformations, though rare, are a heterogeneous group of diseases with presentation, ranging from neonatal respiratory distress to asymptomatic presentation in the adult. The mainstay of management remains surgery with a reasonably good outcome.