A Case of Extralobar Pulmonary Sequestration Combined With Congenital Diaphragmatic Hernia and Complex Cardiac Anomaly

Abstract

A two-month-old boy, who was prenatally noted as having congenital heart defects and a right supradiaphragmatic mass, was diagnosed after birth as having double outlet right ventricle (DORV) with pulmonary atresia. The supradiaphragmatic mass was unlikely to be malignant on the basis of ultrasonographic findings and tumor markers, and it was initially planned that he undergoes elective surgery. Enhanced computed tomography at one month of age showed an inflow of an aberrant artery from the abdominal aorta into the mass and a prolapse of the right kidney and adrenal gland into the right thoracic cavity. He was diagnosed as having right pulmonary sequestration and right-sided late-presenting congenital diaphragmatic hernia (CDH). Lung surgery and CDH repair were to precede heart surgery because increased pulmonary blood flow and vascular bed development were essential for cardiovascular surgery for DORV. At two months of age, thoracoscopic resection for pulmonary sequestration and diaphragmatic plication were concomitantly performed without complications. There is a controversy over the indication and timing of surgery for pulmonary sequestration, and surgery for late-onset CDH is considered to be elective. The case accompanied by complex cardiac anomaly should be given special consideration, and early definitive surgery is a possible option.