19例一穴肛畸形患儿术后排便排尿功能预后及生存质量报告

Prognoses of defecation, urination and quality-of-life in children with one-point anal malformation after operation: a report of 19 cases

目的评估一穴肛畸形患儿术后的排便排尿功能预后及生存质量(quality of life,QOL)。方法回顾性分析2009年1月至2021年12月首都医科大学附属北京儿童医院新生儿外科收治且获得规律随访的19例"一穴肛畸形"患儿的临床资料。对于年龄<4岁的患儿,通过特定排便及排尿症状进行排便及排尿功能的评价;对于年龄≥4岁的患儿,采用Rintala评分量表进行排便功能评估,评分范围0~20分,总分≥17分提示排便功能良好,总分12~16分提示排便功能一般,总分<12分提示排便功能不良;采用排尿功能障碍和尿失禁症状评分系统(dysfunctional voiding and incontinence scoring system,DVAISS)进行排尿功能评估,评分>8.5分提示排尿功能异常。选用儿童QOL普适性核心量表PedsQLTM4.0中文版对本研究随访时年龄>2岁患儿的QOL进行评估。通过Spearman相关分析探索Rintala评分量表、DVAISS评分量表得分与QOL量表得分的相关性。结果19例患儿于关瘘后随访7~136个月,平均随访61.5个月。1例患儿因直肠黏膜脱垂再次手术,1例患儿因肠梗阻再次手术。在本研究年龄<4岁的6例患儿中,4例患儿出现需开塞露辅助排便或污裤的症状;2例患儿出现排尿淋漓不尽及污裤等症状。在年龄≥4岁的13例患儿中,7例患儿排便功能良好;10例患儿排尿功能正常。随访时年龄>2岁的16例患儿儿童生存质量普适性核心量表PedsQLTM4.0中文版家长报告表得分为(83.5±11.6)分。Rintala评分量表得分与QOL量表得分存在正相关关系(r=0.64,P=0.018);DVAISS评分量表得分与QOL量表得分存在负相关关系(r=0.66,P=0.014)。结论多数一穴肛畸形患儿术后排便功能与排尿功能预后较好,但共同管长度≥3 cm的患儿预后较差。排便或排尿功能较差会对患儿的QOL评分产生负面影响。针对一穴肛患儿应建立完善的多学科随访模式。

Objective To explore the long-term follow-up outcomes of defecation,urination and quality-of-life of cloacal malformation.Methods A retrospective analysis was conducted for 19 cases from January 2009 to December 2021.For children aged under 4 years,urination and defecation functions were evaluated by specific micturition and defecation symptoms.For children aged over 4 years,defecation and urination functions were evaluated by the scores of Rintala and Dysfunctional Voiding and Incontinence Scoring System(DVAISS).Based upon total Rintala score(0-20),three categories of defecation function were assigned:≥17 normal defecation,12~16 mild defecation dysfunction and<12 severe defecation dysfunction.Total DVAISS score>8.5 was judged as abnormal urination.The Chinese version of Pediatric Quality-of-Life Inventory Version 4.0(PedsQLTM4.0)Generic Core Scales was applied for assessing the quality-of-life(QOL)of children aged over 2 years during follow-ups.Spearman’s rank correlation test was utilized for assessing the associations between the scores of Rintala,DVAISS and QOL.Results During a follow-up period of 61.5(7-136)months after colostomy closure,one child was re-operated each for rectal mucosal prolapse and intestinal obstruction.Among 6 children aged under 4 years,4 children(66.7%)developed constipation or fecal soiling.Two children(33.3%)had urinary incontinence and wet pants.Among 13 children aged over 4 years,7 children(53.8%)had normal defecation and 10 children(76.9%)had normal urination.The mean Chinese version of PedsQLTM4.0 scales cores of 16 children were(83.5±11.6)points.There were a positive correlation between Rintala and QOL scores(r=0.64,P=0.018)and a negative correlation between DVAISS and QOL scores(r=0.66,P=0.014).Conclusions Most prognoses of postoperative defecation and urination are satisfactory for cloacal malformations.However,the prognosis is poor for children with a common channel longer than 3 cm.Poor defecation or urination has a negative impact on QOL score.Multidisciplinary long-term follow-ups are recommended due to a great complexity of cloacal malformations and associated anomalies.