男孩肛门直肠畸形临床特征分析及学龄前期排便功能评估

Clinical characteristics and evaluations of bowel function at preschool age in boys with anorectal malformations

目的分析男孩肛门直肠畸形(anorectal malformations,ARM)临床特征并评估其学龄前期的排便功能障碍的特点,为ARM术后早期肠道管理提供依据。方法收集2017年1月至2019年11月在南京医科大学附属儿童医院手术治疗的ARM男患儿的临床资料。采用Rintala排便功能评分表于2023年1月进行横断面调查,最终89例患儿完成调查问卷,根据Krickenbeck分型分组,其中泌尿系瘘(rectourological fistula,RUF)组36例,皮肤瘘(rectoperineal fistula,RPF)组53例。调查评分包括7个方面:控便能力、便意、排便频率、污便、失禁、便秘、社会问题。计量资料先进行正态分布检验,符合正态分布的采用t检验;不符合正态分布的采用Mann-Whitney U检验。结果RUF组肛门成形手术年龄及手术体重明显大于RPF组(z=-6.931,P<0.001;z=-6.967,P<0.001);RUF组脊髓发育异常发生率明显高于RPF组(χ^(2)=7.924,P=0.005);RUF组骶骨指数低于RPF组,同时低骶骨指数率明显高于RPF组(z=-4.907,P<0.001;χ^(2)=26.991,P<0.001)。89例患儿总体排便能力良好,功能正常(Rintala评分≥17)和良好(Rintala评分11~17)比例分别为50.6%(45/89)和41.6%(37/89);RUF组排便功能差于RPF组,其中排便正常比例RUF组低于RPF组,排便良好比例RUF组高于RPF组(χ^(2)=40.21,P<0.001)。排便功能障碍特点分析提示RUF组在Rintala排便能力总得分、控便能力、便意、排便频次、污便及失禁方面得分均明显低于RPF组(P<0.001)。比较各年龄亚组(4.0~4.5岁,4.5~5.0岁,5.0~5.5岁,5.5~6.0岁)总体排便功能得分,RUF组总体排便功能得分在各年龄段间差异无统计学意义(χ^(2)=0.640,P=0.887);而RPF组各年龄阶段间差异有统计学意义(χ^(2)=8.638,P=0.035),其中5.0~5.5岁及5.5~6.0岁年龄段排便功能得分高于4.0~4.5岁(P=0.014;P=0.031)。结论RPF患儿比RUF患儿有相对正常的骶骨发育和脊髓发育,学龄前期排便能力相对正常,并有随年龄增长逐步提升的趋势;RUF患儿排便能力相对较弱,且随年龄改善趋势不明显。控便能力障碍、便意障碍、污便及失禁是RUF男患儿术后早期肠道管理的重要方向。

Objective To explore the clinical characteristics of bowel dysfunction at preschool age in boys with anorectal malformations(ARM)to provide rationales for optimizing early bowel management of ARM.Methods In January 2023,a cross-sectional Rintala questionnaire survey was conducted among boys with different types of ARM who underwent surgical anal reconstruction between June 2017 and November 2019.A total of 89 consenting boys were divided into two groups of rectourological fistula(RUF,n=36)and rectoperineal fistula(RPF,n=53)according to Krickenbeck classification.Seven items of capability of holding back defecation,feeling/reporting an urge of defecating,fecal frequency,soiling,incontinence,constipation and social problems were included.The measurement data should be subject to normal distribution test first,and those conforming to normal distribution should be subject to t test,Mann Whitney U test was used for those non-normal distribution.Results Age at anal reconstruction and weight at surgery were significantly higher in RUF group than those in RPF group(z=-6.931,P<0.001;z=-6.967,P<0.001);sacral ratio was lower in RUF group than that in RPF group(z=-4.907,P<0.001).The rates of low sacral ratio and spinal abnormality were higher in RUF group than those in RPF group(χ^(2)=26.991,P<0.001;χ^(2)=7.924,P=0.005).Normal(Rintala score≥17)and good(Rintala score 11-17)function were found in 50.6%and 41.6%of patients with ARM respectively.Bowel function in RUF was significantly worse than that in RPF,including a lower rate of normal function and a higher rate of good function(χ^(2)=40.21,P<0.001).After analysis of the features of bowel dysfunction in RPF/RUF,the scores of total bowel function,capability of holding back defecation,feeling/reporting of an urge of defecating,soiling and accident were significantly lower in RUF than in RPF(P<0.001).No differences existed in score of total bowel function among four sub-age-groups,including 4.0~4.5,4.5~5.0,5.0~5.5 and 5.5~6.0 years old(χ^(2)=0.640,P=0.887).The trends of bowel function towards to improvement was obvious in RPF group(χ^(2)=8.638,P=0.035),including higher total bowel function score in 5.0~5.5 years and 5.5~6.0 years than that in 4.0-4.5 years old.Conclusions The development of sacrum and spine was relatively normal in RPF as compared to RUF.The bowel function at preschool age in RPF patients is satisfactory and improves with age.However,bowel dysfunction in RUF remains prevalent and does not improve with age in preschool age,including inadequate capability of defecation control and feeling/reporting of an urge of defecating,soiling and incontinence.These concerns are vital for early bowel management.