Morino Masaaki, Hoshino Masaya, Musha Ikuma
Department of Pediatric Emergency Medicine, Saitama International Medical Centre, Saitama, Japan.
Pediatr Int. 2013 Aug;55(4):e90-2. doi: 10.1111/ped.12088.
The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn-Werner-Wunderlich syndrome (HWWS) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non-rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high-riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS. The patient underwent a preventative Ladd's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations.
双子宫、阴道半段梗阻和同侧肾缺如的组合代表一种罕见的先天性异常，称为赫林-韦纳-温德利希综合征（HWWS）或阴道半段梗阻和同侧肾异常（OHVIRA）综合征。最近有报道称几种异常与该综合征相关。本例HWWS患者有多种异常：肠旋转不良、腹部大血管异常，包括下腔静脉重复和高位主动脉分叉，以及牙发育不全。HWWS患者中从未有过牙发育不全的报道。该患者接受了预防性的拉德手术和阴道重建。为防止诸如肠旋转不良等伴随异常引发严重并发症，应对HWWS患者进行详细的相关畸形评估。
