Jolley Alexandra Frances, Beare Elizabeth Jane, Granger Jeremy, Cord-Udy Catherine Lucy, Muller Peter, Moore Lynette
1 Anatomical Pathology, SA Pathology, Women's and Children's Hospital, North Adelaide, South Australia, Australia.
2 Department of Obstetrics and Gynaecology, Women's and Children's Hospital, North Adelaide, South Australia, Australia.
Pediatr Dev Pathol. 2017 Mar-Apr;20(2):158-162. doi: 10.1177/1093526616683872. Epub 2017 Jan 25.
We present a severe case of vanishing gastroschisis resulting in intrauterine death with post mortem examination findings. Gastroschisis is defined as a full thickness paraumbilical abdominal wall defect associated with evisceration of fetal intestine. It is almost always right-sided. Vanishing gastroschisis is an extremely rare form of gastroschisis that results in short bowel syndrome due to exteriorized bowel disconnected from the lumen of the rest of the bowel proximally as well as distally in association with partial or complete closure of the abdominal wall. This case is only the second published case of vanishing gastroschisis resulting in intrauterine fetal death including post mortem examination findings. It highlights the importance of being aware of this rare form of gastroschisis and provides insights regarding pathogenesis, ultrasound surveillance, and antenatal counseling.
我们呈现了一例严重的消失型腹裂病例，该病例导致宫内死亡，并给出了尸检结果。腹裂被定义为与胎儿肠管脱出相关的全层脐旁腹壁缺损。它几乎总是发生在右侧。消失型腹裂是一种极其罕见的腹裂形式，由于外置肠管在近端和远端均与其余肠管腔断开连接，并伴有腹壁部分或完全闭合，从而导致短肠综合征。该病例是第二例已发表的导致宫内胎儿死亡的消失型腹裂病例，包括尸检结果。它凸显了认识这种罕见腹裂形式的重要性，并提供了有关发病机制、超声监测和产前咨询的见解。
