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一例采用全肠外营养及肠道延长术治疗的消失性腹裂合并短肠综合征病例。

A case of vanishing gastroschisis with short bowel syndrome treated by total parenteral nutrition and intestinal lengthening procedures.

作者信息

Guanà Riccardo, Marocco Lucia, Garofalo Salvatore, Zambaiti Elisa, Pane Alessandro, Scottoni Federico, Fusaro Fabio, Perucca Giulia, Gennari Fabrizio

机构信息

Pediatric Surgery Unit, University Hospital of Health and Science, Regina Margherita Children's Hospital, Turin, Italy.

Division of Pediatric General, Thoracic, Neonatal & Minimally Invasive Surgery, Regina Margherita Children's Hospital, Turin, Italy.

出版信息

SAGE Open Med Case Rep. 2023 Mar 4;11:2050313X231157490. doi: 10.1177/2050313X231157490. eCollection 2023.

Abstract

The diagnosis of vanishing gastroschisis is made when in the presence of a full-thickness intrauterine abdominal wall defect the eviscerated loops are incarcerated in the fascial interruption. Four types of vanishing gastroschisis are described (A-D). We report on the case of a newborn with a vanishing gastroschisis-D. Gastroschisis was diagnosed at the 19th week of gestation, confirmed at the 30th, when the herniated loops previously visible to the right of the funiculus were no longer visualized. At the 32nd week, delivery was induced. The neonate weighed 1600 g, and the abdomen was distended, free from skin defects. On surgical exploration, the jejunum was 13 cm in length, with a blind ending. The post-atretic intestine measured 22 cm. A jejunostomy and a colostomy were built. The child received total parenteral nutrition for 13 months due to short bowel syndrome and was then subjected to intestinal lengthening procedure when she was 18 months old. Vanishing gastroschisis is a rare entity with a worse prognosis of the "classic" gastroschisis.

摘要

隐匿性腹裂的诊断标准是,在存在全层子宫内腹壁缺损的情况下,脱出的肠袢被嵌顿于筋膜中断处。隐匿性腹裂可分为四种类型(A - D型)。我们报告了一例隐匿性腹裂D型的新生儿病例。妊娠第19周时诊断为腹裂,第30周时得到确认,此前在脐带右侧可见的疝出肠袢此时已不可见。第32周时引产。新生儿体重1600克,腹部膨隆,无皮肤缺损。手术探查发现,空肠长13厘米,呈盲端。闭锁后肠段长22厘米。进行了空肠造口术和结肠造口术。由于短肠综合征,患儿接受了13个月的全胃肠外营养,18个月大时接受了肠延长手术。隐匿性腹裂是一种罕见的疾病,其预后比“经典”腹裂更差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f5dc/9989415/8fae329bb96e/10.1177_2050313X231157490-fig1.jpg

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