Grisaru D, Achiron R, Lipitz S, Yahav J, Hegesh J, Rotstein Z
Department of Obstetrics and Gynecology, Chaim Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University, Israel.
Ultrasound Obstet Gynecol. 1996 Aug;8(2):131-3. doi: 10.1046/j.1469-0705.1996.08020131.x.
Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs solitus, due to right pulmonary hypoplasia. The prenatal sonographic findings of this abnormality have not been previously described. We describe early sonographic findings in a fetus that included isolated cardiac dextroposition with normal abdominal situs, hydramnios and mild narrowing of the right pulmonary artery. Postnatally, the infant developed heart failure, and a right hypoplastic lung was found. Catheterization revealed collateral supply to the right lung arising from the descending aorta and anomalous drainage of right pulmonary veins to the inferior vena cava. The diagnosis of scimitar syndrome was therefore established. A coil embolization of arterial collaterals to the right lung was performed. Scimitar syndrome should be considered in a fetus with the sonographic findings of a right shift of the mediastinal structures, an intact diaphragm and narrow right pulmonary artery. This may allow early neonatal stabilization and treatment.
弯刀综合征是一种罕见的先天性异常，由于右肺发育不全，在正常位中出现继发性右位心。此前尚未描述过这种异常的产前超声检查结果。我们描述了一名胎儿的早期超声检查结果，包括孤立性心脏右位伴正常腹部位置、羊水过多和右肺动脉轻度狭窄。出生后，婴儿出现心力衰竭，并发现右肺发育不全。心导管检查显示右肺有来自降主动脉的侧支供血以及右肺静脉向下腔静脉的异常引流。因此确诊为弯刀综合征。对右肺的动脉侧支进行了弹簧圈栓塞。对于超声检查发现纵隔结构右移、膈肌完整且右肺动脉狭窄的胎儿，应考虑弯刀综合征。这可能有助于新生儿早期稳定和治疗。
