van Hoeve Karen, Hoffman Ilse, Fusaro Fabio, Pirenne Jacques, Vander Auwera Ann, Dieltjens Anne-Marie, De Hertogh Gert, Monbaliu Diethard, Miserez Marc
a Department of Gastroenterology, Hepatology and Nutrition , University Hospital Gasthuisberg , Leuven , Belgium.
b Fabio Fusaro MD Neonatal Surgery Unit , Bambino Gesù Children's and Research Hospital , Rome , Italy.
Acta Chir Belg. 2016 Dec;116(6):333-339. doi: 10.1080/00015458.2016.1176420. Epub 2016 Aug 1.
Microvillus inclusion disease (MVID) is a known congenital cause of intractable diarrhea resulting in permanent intestinal failure. There is need for a lifelong total parenteral nutrition (TPN) from diagnosis and the prognosis is poor. Most patients die by the second decade of life as a result of complications of parenteral alimentation including liver failure or sepsis. The only available treatment at this moment is a small bowel transplantation. But before that moment, the patients often suffer from a persistent failure to thrive and electrolyte disturbances despite continuous TPN.
We report what we believe is a first case of an extensive small bowel resection in a 5-month-old boy with proven MVID to act as a bridge to (liver-) intestinal transplantation to treat failure to thrive and intractable diarrhea.
An extensive small bowel resection can be done to enhance the chance of survival leading up to the transplantation by managing fluid and electrolyte imbalance. It facilitates medical management of these patients and makes a bowel transplantation possible at a later stage.
微绒毛包涵体病（MVID）是一种已知的导致顽固性腹泻的先天性病因，可导致永久性肠衰竭。自诊断起就需要终身进行全胃肠外营养（TPN），且预后较差。大多数患者在生命的第二个十年因包括肝衰竭或败血症在内的胃肠外营养并发症而死亡。目前唯一可用的治疗方法是小肠移植。但在此之前，尽管持续进行TPN，患者仍常出现持续的生长发育不良和电解质紊乱。
我们报告了我们认为的首例对一名5个月大、经证实患有MVID的男童进行广泛小肠切除的病例，以此作为（肝 -）肠移植的桥梁，以治疗生长发育不良和顽固性腹泻。
通过处理液体和电解质失衡，进行广泛小肠切除可提高移植前的生存几率。这有助于对这些患者进行医疗管理，并使后期进行肠移植成为可能。
