Soussan Haitam, Jabi Rachid, Ouryemchi Mouad, Haddadi Zakaria, Bouziane Mohammed
General Surgery, Mohammed VI University Hospital/Faculty of Medicine and Pharmacy, Laboratory of Anatomy, Microsurgery and Surgery Experimental and Medical Simulation, Mohammed First University of Oujda, Oujda, MAR.
Radiology, Mohammed VI University Hospital, Oujda, MAR.
Cureus. 2021 Oct 4;13(10):e18484. doi: 10.7759/cureus.18484. eCollection 2021 Oct.
Hirschsprung's disease (HD) in adults is rare, occurring before the age of five years in 90% of cases. It is characterized by the absence of ganglion cells in a colorectal segment, resulting in functional obstruction and an upstream colonic dilatation. HD should be considered in front of any history of chronic constipation. The diagnosis is based on a combination of clinical, manometric, radiological, and histological findings. Surgery is the basis of the treatment and consists of the resection of the aganglionic segment, followed by restoration of continuity between the two healthy segments. We report here the case of a 20-year-old man who presented to the ER with an occlusive syndrome, which initially required a loop colostomy for decompression. History, clinical presentation, and radiological findings were suggestive of HD, but additional diagnostic methods including manometry and biopsy were employed but proved negative. Given the available data, the patient underwent a colectomy with a latero-terminal ileorectal anastomosis. Histological findings of the surgical specimen confirmed the diagnosis of HD
成人先天性巨结肠病（HD）较为罕见，90%的病例在5岁前发病。其特征是结直肠段无神经节细胞，导致功能性梗阻及近端结肠扩张。对于任何慢性便秘病史的患者都应考虑HD。诊断基于临床、测压、放射学及组织学检查结果的综合判断。手术是治疗的基础，包括切除无神经节细胞段，然后恢复两个健康肠段之间的连续性。我们在此报告一例20岁男性患者，因肠梗阻综合征就诊于急诊室，最初需要行袢式结肠造口术减压。病史、临床表现及放射学检查结果提示HD，但包括测压和活检在内的其他诊断方法均为阴性。根据现有资料，该患者接受了结肠切除术及回肠直肠侧端吻合术。手术标本的组织学检查结果确诊为HD
