Congenital short-bowel;: A case study and review of the literature
Congenital short-bowel;: A case study and review of the literature
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DOI：
10.1055/s-2008-1072255
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发表时间：
1999-08-01
影响因子：
1.8
通讯作者：
Höllwarth, ME
中科院分区：
文献类型：
--
作者：
Schalamon, J;Schober, PH;Höllwarth, ME
先天性短肠（CSB）在小儿外科中是一种罕见的疾病。我们介绍了一名新生男婴的病例，其小肠总长度为 47 厘米，有旋转不良和胃食管反流，在撰写本报告时年龄为 19 个月；主要治疗：步骤为拉德手术、胃底折叠术和长期肠外营养。我们认为，肠道生理性疝入体腔的缺失可能会损害正常的肠道生长和旋转，并导致先天性短肠。对文献中报告的所有病例进行审查后发现，死亡率高达 88%。限制因素似乎是短小肠蠕动减少，导致功能性梗阻和肝衰竭。
A congenital short bowel (CSB) is a rare entity in pediatric surgery. We present the case of a newborn boy with a total small intestinal length of 47 cm, malrotation and gastroesophageal reflux, who is 19 months old at the time of this report; Main treatment: steps were Ladd's procedure, a fundoplication and long-term parenteral nutrition. We suggest that missing physiological herniation of the gut into the coelomic cavity may impair normal intestinal growth and rotation and lead to congenital short bowel. Review of all cases reported in the literature shows a considerable mortality of 88%. The limiting factor seems to be reduced motility of the short small bowel causing functional obstruction and liver failure.