Granström Anna Löf, Danielson Johan, Husberg Britt, Nordenskjöld Agneta, Wester Tomas
Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
Institution of Women's and Children's Health, Uppsala University, Uppsala, Sweden.
J Pediatr Surg. 2015 Nov;50(11):1865-9. doi: 10.1016/j.jpedsurg.2015.06.014. Epub 2015 Jun 24.
Hirschsprung's disease (HSCR) is treated surgically. There is a risk for faecal incontinence and constipation postoperatively. The long-term bowel functional outcome in adults and quality of life are sparsely studied. The aim of this study was to assess bowel function and quality of life in patients who had undergone surgery for HSCR during childhood.
All patients treated between 1969 and 1994 at St. Göran's Children's Hospital in Stockholm were invited to participate in the study. After consent, the patients received questionnaires containing general questions, validated questions on bowel function, questions about urinary function, SF-36 health survey (SF-36) and the Gastrointestinal Quality of Life Index (GIQLI). Clinical data were extracted from the case records. Controls matched for sex and age were randomly selected from the National Swedish Population Register.
48 of 60 (80%) invited patients responded to the questionnaires. Nine patients were excluded since the HSCR diagnosis could not be confirmed. The median age of the included patients was 28 (20-43) years. Most patients had undergone Soave's operation (73.4%) and two patients had a stoma at the time of follow-up. The bowel function was impaired in the HSCR group compared to controls, especially problems with flatulence, need to strain at defecation and several defecations for emptying. Patients in the HSCR group also had significantly more problems with faecal incontinence than controls. Quality of life according to SF-36 did not differ significantly between patients and controls, but the GIQLI score showed a significantly worse outcome in the HSCR group compared to the controls.
General quality of life in adults treated for HSCR during childhood is comparable to controls. However, they have impaired bowel function and gastrointestinal quality of life.
先天性巨结肠症（HSCR）通过手术治疗。术后存在大便失禁和便秘的风险。针对成人的长期肠道功能结局及生活质量的研究较少。本研究旨在评估儿童期接受HSCR手术患者的肠道功能及生活质量。
邀请了1969年至1994年期间在斯德哥尔摩圣戈兰儿童医院接受治疗的所有患者参与研究。获得同意后，患者收到包含一般问题、经验证的肠道功能问题、泌尿功能问题、SF-36健康调查（SF-36）以及胃肠道生活质量指数（GIQLI）的问卷。临床数据从病例记录中提取。从瑞典国家人口登记册中随机选取年龄和性别匹配的对照组。
60名受邀患者中有48名（80%）回复了问卷。9名患者因无法确诊HSCR而被排除。纳入患者的中位年龄为28（20 - 43）岁。大多数患者接受了Soave手术（73.4%），2名患者在随访时有造口。与对照组相比，HSCR组的肠道功能受损，尤其是腹胀、排便时需用力以及多次排便才能排空等问题。HSCR组患者的大便失禁问题也明显多于对照组。患者和对照组在SF-36评估的生活质量方面无显著差异，但与对照组相比，HSCR组的GIQLI评分结果明显更差。
儿童期接受HSCR治疗的成人总体生活质量与对照组相当。然而，他们的肠道功能及胃肠道生活质量受损。
