Congenital extralobar pulmonary sequestration supplied by pulmonary artery: a case report / 中华围产医学杂志

ABSTRACT

We report the clinical features of a case of congenital extralobar pulmonary sequestration in the left upper lobe supplied by the pulmonary artery. Prenatal ultrasound examination at 24 weeks of gestation revealed a high echogenic and uniform density mass in the fetal left thoracic cavity with the congenital pulmonary airway malformation volume vatio (CVR) of 1.16, which was supplied by pulmonary arterial vessels. MRI examination at 27 weeks indicated that the left lung volume increased to about 48.52 ml, while the right lung volume was about 8.56 ml giving the total lung volume of 57.08 ml. The congenital pulmonary airway malformation in the left upper lobe was suspected to be congenital bronchial atresia (CBA) or congenital lobar overinflation (CLO). The baby boy was born through vaginal delivery assisted by forceps at 38 +1 weeks without neonatal asphyxia. Postnatal CT and MRI were both indicated suspicious bronchial atresia in the left upper lobe. Bronchofibroscopy on postnatal day 2 excluded CBA or CLO and extralobar pulmonary sequestration was considered. Thoracoscopic surgery was performed due to continuous shortness of breath after birth, despite two-week conservative treatment including oxygenation, invasive and non-invasive mechanical ventilation,etc, and congenital extralobar sequestration was diagnosed. Blood supply from the left pulmonary artery was observed at the base of abnormal lung tissue. Resection of the pathogenic tissue of the left lung was performed thoracoscopically. The boy recovered and was discharged after the operation. Pulmonary sequestration was confirmed by histopathology.