成人先天性巨结肠的诊治分析

Clinical analysis of diagnosis and treatment of adult Hirschsprung's disease

目的：探讨成人先天性巨结肠的临床特征及诊治经验。方法回顾性分析自2004年1月至2014年1月收治的21例成人先天性巨结肠患者的临床资料。结果21例成人先天性巨结肠患者中男性17例、女性4例，年龄15～52岁；4例有胎便排出延迟病史，11例自幼儿期有间歇性便秘、腹胀史；14例钡剂灌肠可见痉挛段、移行段、扩张段的典型表现，6例行肛门直肠测压均未引出肛门直肠抑制反射，所有患者均经术中及术后病理学检查确诊。所有患者均接受了手术治疗，4例行Soave术，4例行Swenson术，8例行Rehbein术，5例行改良Duhamel术。术后排便功能良好。结论慢性便秘史、钡剂灌肠和肛门直肠测压是成人先天性巨结肠主要诊断依据，经病理学检查确诊，手术是其有效治疗手段。

Objective To explore the diagnosis and treatment of adult Hirschsprung＆#39;s disease. Methods Retro-spective analysis was performed on the clinical data of 21 cases of adult Hirschsprung＆#39;s disease from January 2004 to January 2014. Results There were 17 males and 4 females aging from 15 to 52 years old in the selected 21 cases, among which there were 4 cases with meconium delay discharge history, 11 cases with intermittent constipation and abdominal distention since infant. Bowel stricture, transition and dilation could be examined by barium enema in 14 cases. Typical manifestations at cramping section, migrating section, expanding section were observed in 14 cases through barium enema. Anal manometry showed that the recto-anal inhibition reflex was absent in 6 patients. All cases were diagnosed by pathological examination. Soave procedure was performed in 4 patients, with Swenson procedure in 4 patients, Rehbein procedure in 8 cases, modified Duhamel procedure in 5 cases. In all cases, patient symptoms were completely resolved and the function of defecation was improved after operation. Conclusion The final diagno-sis depends on the history of constipation, barium enema, anal manometry and pathological examination. Surgery can achieve satisfactory outcomes in patients with adult Hirschsprung＆#39;s disease.