Bakari A A, Gali B M, Ibrahim A G, Nggada H A, Ali N, Dogo D, Abubakar A M
Department of Surgery, College of Medical Sciences, University of Maiduguri, Nigeria.
Niger J Clin Pract. 2011 Apr-Jun;14(2):249-52. doi: 10.4103/1119-3077.84032.
Congenital aganglionic mega colon (Hirschsprung's disease) is a motor disorder in the gut, due to a defect in the craniocaudal migration of the neuroblast originating from the neural crest that occurs during the first twelve weeks of gestation, causing a functional intestinal obstruction, with its attendant complications, in infants. Despite modern pediatric practice, with emphasis on early diagnosis, Hirschsprung's disease is seen in adults in regions where perinatal care is limited. We report two cases of Nigerian adults with longstanding, recurrent constipation, getting relieved by laxatives and herbal enemata, and then presented to our Emergency Department with a history of progressive abdominal distention, colicky pain, occasional vomiting, and weight loss. Per rectal examination revealed a gripping sensation in the rectum, 10 cm from the anal verge, with rectal fecal load. Barium enema showed a grossly distended proximal large colon, with high fecal retention, with the transition zone at the middle one-third of the rectum. Due to difficulty in bowel preparation of these patients, emergency laparotomy was done. The first case had a diverting sigmoid colostomy and later had a low anterior resection. The second case had a one-stage procedure. Histology of both the cases showed aganglionosis of the stenotic segment and a normal distal rectum. Both patients had complete resolution of the symptoms, without complications, in a three-year follow-up. The related literatures were reviewed. Hirschsprung's disease should be considered in adults patient presenting with chronic constipation. Low anterior resection of the rectum would be a surgical option for the treatment of short and zonal segment of adult Hirschsprung's disease.
先天性无神经节巨结肠（赫希施普龙病）是一种肠道运动障碍性疾病，病因是在妊娠头十二周期间起源于神经嵴的神经母细胞在头尾方向迁移过程中出现缺陷，导致婴儿出现功能性肠梗阻及其伴随并发症。尽管现代儿科诊疗强调早期诊断，但在围产期护理有限的地区，赫希施普龙病在成人中也有发现。我们报告两例尼日利亚成年患者，他们长期反复便秘，使用泻药和草药灌肠可缓解，随后因进行性腹胀、绞痛、偶尔呕吐和体重减轻的病史就诊于我们的急诊科。经直肠检查发现距肛门边缘10厘米处的直肠有紧握感，直肠内有粪便。钡剂灌肠显示近端大肠明显扩张，粪便潴留严重，移行区位于直肠中三分之一处。由于这些患者肠道准备困难，遂进行了急诊剖腹手术。第一例患者行乙状结肠转流造口术，后来行低位前切除术。第二例患者行一期手术。两例患者的组织学检查均显示狭窄段无神经节，远端直肠正常。在三年的随访中，两名患者的症状均完全缓解，无并发症发生。我们对相关文献进行了综述。对于出现慢性便秘的成年患者，应考虑赫希施普龙病。直肠低位前切除术可作为治疗成人赫希施普龙病短节段和局限性节段的手术选择。
