回肠拖出Soave术治疗全结肠型巨结肠的中远期疗效评估

目的评估回肠拖出Soave术治疗全结肠型巨结肠患儿的肛门直肠功能、营养状况及中远期疗效。方法对1999年1月至2010年12月经手术治疗的8例接受直接回肠拖出Soave术的全结肠型巨结肠患儿进行随访，随访时间18～147个月，平均随访时间（62.5±44.0）个月；均行体格测量及实验室检查的营养评估；采用问卷进行直肠肛门功能Kelly评分及生活质量评分。 结果8例患儿中，4例（50%）肛门外形正常，4例（50%）肛周轻度糜烂；4例（50%）排便次数3～5次/d； 8例直肠肛门功能Kelly评分3～6（5.1±1.0）分，7例（87%）获得较满意评分。生活质量评分7～11（9.6±1.2）分。疗效优6例（75%）。对7例10岁以下患儿采用WHO评估标准，其中年龄别身高正常4例（571%），轻度营养不良2例（28.6%），中度营养不良1例（14.3%）。结论 回肠拖出Soave术治疗全结肠型巨结肠患儿术后中远期疗效满意，虽轻、中度营养不良的发生率较高，但大部分患儿发育正常。

Long-term follow-up of total colonic aganglionosis post straight ileo-anal pull-through surgical treatment

Objective To investigate the long-term results of total colonic aganglionosis and to evaluate their nutritional status. Methods eight patients treated for TCA between January 1999 and December 2010 attended the study. Eight patients received straight ileo-anal pull-through. During the follow-up（ range 18 - 147 months, average 62.5 ±44 months）, anorectal functions with Kelly score, quality of life （ QOL）, height, weight and lab tests were assessed by clinical visit. Results The average Kelly score was 5.1 ±1.0 （ range 3 - 6）, seven patients （87%）had satisfactory anorectal functions. QOL was good in six patients （75 % ）, average score was 9.6 ±1.2. According to the WHO standard for children less than ten years old, in terms of height-for-age （HFA） ,57.1% and 28.6% were normal and mild malnutrition whereas 14.3% was medium malnutrition in seven patients. Conclusions Patients with TCA can have satisfactory outcomes on long-term follow up. However, mild and medium malnutrition were common in these patient.