Percutaneous Intrauterine Laser Ablation of the Abnormal Vessel in Pulmonary Sequestration With Hydrops at 29 Weeks' Gestation

Abstract

Congenital pulmonary sequestration (PS) is a rare malformation consisting of a nonfunctioning bronchopulmonary mass separated from the tracheobronchial tree, which receives an arterial blood supply directly from the systemic circulation. 1 The key feature for diagnosing fetal PS is the identification of a hyperechoic pulmonary mass with an abnormal blood supply. 2 ' 3 The systemic artery from the aorta to the fetal lung mass can be detected by 2-dimensional (2D) color power Doppler imaging 2,3 ; however, 3-dimensional (3D) power Doppler ultrasonography may be even more sensitive. 4 Distinguishing PS from a type III congenital cystic adenomatoid malformation is of interest for prenatal counseling of the parents and for planning postnatal treatment. 5 Although nonimmune hydrops fetalis rarely occurs in cases of PS, it is associated with a high rate of perinatal mortality and severe respiratory insufficiency in the neonate.6'7 Different strategies of in utero treatment have been proposed, such as thoracoamniotic shunting of pleural effusion, 8,9 alcohol ablation of the vascular pedicle, 10 and open fetal surgery. 11 However, prenatal treatment of such cases remains controversial. Another therapeutic option is reported here, which consists of promoting ablation of the abnormal systemic artery from the aorta to the PS at 29 weeks' gestation. In this case, the diagnosis was made by 2D color Doppler ultrasonography. Three-dimensional power Doppler imaging revealed that the abnormal arterial pedicle was in close proximity to the pulmonary vein, leading to a vascular shunt phenomenon and hydrops fetalis. The therapeutic option described appears to act directly on the physiopathologic process of PS, providing an interesting option for such cases.