Abnormal layering of muscularis propria as a cause of chronic intestinal pseudo-obstruction:A case report and literature review  

作　　者：Napat Angkathunyakul Suporn Treepongkaruna Sani Molagool Nichanan Ruangwattanapaisarn 

机构地区：[1]Department of Pathology, Ramathibodi Hospital, Mahidol University [2]Department of Pediatrics, Ramathibodi Hospital, Mahidol University [3]Department of Surgery, Ramathibodi Hospital,Mahidol University [4]Department of Diagnostic and Therapeutic Radiology, Ramathibodi Hospital, Mahidol University

出　　处：《World Journal of Gastroenterology》2015年第22期7059-7064,共6页世界胃肠病学杂志（英文版）

基　　金：Supported by Faculty of Medicine,Ramathibodi Hospital,Mahidol University,Thailand

摘　　要：Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly,and absence of the 2nd to 4th middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support.He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly,and absence of the 2nd to 4th middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support.He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.

关 键 词：Abnormal layering of muscularis propria BRACHYDACTYLY Chronic intestinal pseudo-obstruction Serosal muscularization Short small bowel Visceral myopathy 

分 类 号：R725.7[医药卫生—儿科]