Menezes Maria, Corbally Martin, Puri Prem
Childrens Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin-12, Ireland.
Pediatr Surg Int. 2006 Dec;22(12):987-90. doi: 10.1007/s00383-006-1783-8.
Although various surgical procedures have been described to treat Hirschsprung's disease (HD), few studies have evaluated the long-term results of these children. The purpose of this study was to assess the long-term clinical outcome and bowel function of patients with HD. The hospital records of 259 consecutive patients with a confirmed histological diagnosis of HD during 1975-2003 were examined. Data was assessed for age at presentation, sex, clinical presentation, associated anomalies, level of aganglionosis, surgical procedures, complications and bowel function. Follow up was carried out by personal/telephone interviews with patients or their parents. Of the 259 patients with HD, 200 were males (77.2%) and 59 females (22.8%). Intestinal obstruction was the presenting feature in 147 patients (56.8%), intestinal perforation in 5 (1.9%), enterocolitis in 30 (11.6%) and constipation in 77 (29.7%). Thirty-nine patients (15.1%) had associated Down's syndrome. Two hundred and nine patients (80.7%) had rectosigmoid disease, 31 (12%) had long segment disease and 19 (7.3%) had total colonic aganglionosis. Forty-three patients (16.6%) had preoperative enterocolitis. Primary colostomy was performed in 160 patients and a primary pull through in 90. Seven patients had a sphincteromyectomy for ultrashort HD. Two patients died prior to treatment. Various pull through procedures were performed in these patients. Postoperative complications included: pelvic abcess in 2, rectal stricture in 10, perianal excoriation in 7, anastomotic leak in 8, intestinal obstruction in 3, wound dehiscence in 1, stomal prolapse/stenosis in 5, rectovesical fistula in 2 and enterocolitis in 56. Five patients underwent a redo pull through and 46 required a post pull through sphincterectomy. At the time of follow-up, 27 were lost to follow-up, 9 died, 18 had permanent stomas and 4 were too young to assess bowel function. Of the remaining 194 patients, bowel function was normal in 132 (68%). Twenty patients (10.3%) had soiling and 42 (21.7%) had constipation requiring laxatives or enemas. There was no difference in bowel function in relation to type of pull through operation. Only 34% of patients with Down's syndrome had normal continence. The majority of patients with HD continue to have disturbances of bowel function for many years before attaining normal continence.
尽管已经描述了多种用于治疗先天性巨结肠症（HD）的外科手术方法，但很少有研究评估这些患儿的长期治疗结果。本研究的目的是评估HD患者的长期临床结局和肠道功能。我们查阅了1975年至2003年间259例经组织学确诊为HD的连续患者的医院记录。评估的数据包括就诊时的年龄、性别、临床表现、相关畸形、无神经节段水平、手术方法、并发症和肠道功能。通过对患者或其父母进行个人访谈/电话访谈进行随访。在259例HD患者中，男性200例（77.2%），女性59例（22.8%）。147例患者（56.8%）以肠梗阻为首发症状，5例（1.9%）为肠穿孔，30例（11.6%）为小肠结肠炎，77例（29.7%）为便秘。39例患者（15.1%）伴有唐氏综合征。209例患者（80.7%）为直肠乙状结肠型病变，31例（12%）为长段型病变，19例（7.3%）为全结肠无神经节症。43例患者（16.6%）术前患有小肠结肠炎。160例行一期结肠造口术，90例行一期拖出术。7例超短段HD患者行括约肌切除术。2例患者在治疗前死亡。这些患者实施了各种拖出手术。术后并发症包括：盆腔脓肿2例，直肠狭窄10例，肛周皮肤擦伤7例，吻合口漏8例，肠梗阻3例，伤口裂开1例，造口脱垂/狭窄5例，直肠膀胱瘘2例，小肠结肠炎56例。5例患者再次行拖出术，46例需要在拖出术后行括约肌切除术。在随访时，27例失访，9例死亡，18例有永久性造口，4例因年龄太小无法评估肠道功能。在其余194例患者中，132例（68%）肠道功能正常。20例患者（10.3%）有污粪，42例（21.7%）有便秘需要使用泻药或灌肠。肠道功能与拖出手术类型无关。唐氏综合征患者中只有34%的患者控便功能正常。大多数HD患者在实现正常控便之前多年仍存在肠道功能紊乱。
