巨膀胱-小结肠-肠蠕动不良综合征2例报告及文献复习  

Report of two cases of macrocy-sticmicrocolon-intestinal hypoperistalsis syndrome and literature review

作　　者：杨文强 张宁[2] 程伶熙 罗艳梅 杨星海 YANG Wenqiang;ZHANG Ning;CHENG Lingxi;LUO Yanmei;YANG Xinghai(School of Medicine,Wuhan University of Science and Technology,Hubei Province,Wuhan 430065,China;Department of Pediatric Surgery,Maternal and Child Hospital of Hubei Province,Tongji Medical College,Huazhong University of Science and Technology,Hubei Province,Wuhan 430070,China)

机构地区：[1]武汉科技大学医学院,湖北武汉430065 [2]华中科技大学同济医学院附属湖北省妇幼保健院小儿外科,湖北武汉430070

出　　处：《中国医药导报》2023年第32期184-187,共4页China Medical Herald

基　　金：湖北省卫生健康委员会科研项目(WJ2021M256)。

摘　　要：巨膀胱-小结肠-肠蠕动不良综合征(MMIHS)是一种罕见的假性肠梗阻综合征,临床表现为非机械性梗阻所致的膀胱巨大、结肠细小,胃肠道动力障碍等,新生儿期即可发病。本文报道2例MMIHS患者,1例为1 d龄新生儿,以腹胀为主要临床表现,通过影像学检查及组织学检查确诊为MMIHS。患儿虽进行胃造瘘术、抗感染、静脉营养等治疗,但未能取得良好的效果,最终因多器官功能衰竭死亡。另1例通过产前MRI诊断为MMIHS,建议其终止妊娠,引产后经病理检查证实。本文通过对患者临床表现、诊断及治疗进行报道,并回顾相关文献,以期提高对该疾病的认识,减少误诊或漏诊。Megacystismicrocolon-intestinal hypoperistalsis syndrome(MMIHS)is a rare pseudoileus syndrome,the clinical manifestations are large bladder,small colons,and gastrointestinal motility disorders caused by non-mechanical obstruction,which can occur in the neonatal period.This paper reports two cases of MMIHS,one case was a one day-old newborn.Abdominal distension was the main clinical manifestation,and the diagnosis of MMIHS was confirmed by imaging and histological examination.Although the patient received gastrostomy,anti-infection,intravenous nutrition,and other treatment,but did not achieve a good effect,and eventually died due to multiple organ failure.The other case was diagnosed as MMIHS by prenatal MRI and was recommended to terminate pregnancy,which was confirmed by pathological examination after induction of labor.In this paper,the clinical manifestations,diagnosis,and treatment of the patients were reported,and the relevant literatures were reviewed in order to improve the understanding of the disease and reduce misdiagnosis or missed diagnosis.

关 键 词：巨膀胱 小结肠 肠梗阻 新生儿 病例报告 

分 类 号：R445[医药卫生—影像医学与核医学]