全结肠型巨结肠误治的再手术诊疗经验及预后分析

Analysis of total colonic aganglionosis with mistreatment: reoperative experience and prognosis

目的 探讨既往误治的全结肠型巨结肠(total colonic aganglionosis,TCA)病例的误治原因、再手术流程及预后,以提高对TCA的临床认识。方法 回顾性分析2010年1月至2021年6月首都医科大学附属北京儿童医院收治的经病理证实为TCA的71例患儿临床资料,根据首诊是否接受合适的手术方案,将患儿分为误治组与无误治组。误治组17例,无误治组45例,确诊后放弃治疗9例。误治组首诊均在三级甲等医院(综合医院9例,儿科专科医院8例)。术前误诊10例,无误诊7例。分析误治组患儿首诊信息、再手术原因及诊疗流程。通过电话或门诊随访患儿预后,比较两组患儿术后并发症、生长发育和排便功能。排便功能评估采用Rintala评分,评估时患儿年龄需≥4岁。结果 术前误诊患儿均未接受巨结肠手术,无误诊患儿6例行巨结肠手术,1例行升结肠造瘘,手术年龄为26(4,240)d。首次术后出现腹胀16例(16/17,94.1%)、排便困难14例(14/17,82.4%)、呕吐13例(13/17,76.5%)、小肠结肠炎10例(10/17,58.8%)。无误诊患儿再手术原因主要为痉挛段残留(5/7,71.4%)。除2例首次术后明确痉挛段残留以及1例吻合口漏患儿外,其余均再次接受术中多点冰冻病理检查(14/17,82.4%)。误治组17例患儿均再次接受巨结肠根治手术。与无误治组(41例已完成根治手术)相比,误治组平均手术次数、造瘘比例和最终根治手术年龄均明显高于无误治组[手术次数,3(3,5)次比1(1,2)次,P<0.001;造瘘比例,12/17比16/41,P=0.028;根治手术年龄,525(197,1971)d比164(29,464)d,P=0.007]。随访到误治组15例,无误治组31例,随访时间为5.4(2.1,8.0)年。两组患儿术后并发症、生长发育情况和Rintala评分无显著差异。结论 TCA误治原因主要为术前误诊、手术经验不可靠和术中病理结果偏差。最终根治术前建议再次行多点冰冻病理检查明确诊断,必要时先行造瘘。首诊误治的TCA患儿的诊疗时间明显延长,但总体预后尚可。

Objective To explore the causes and reoperative procedures for mistreated cases of total colonic aganglionosis(TCA)and to evaluate their prognosis.Methods From January 2010 to June 2021,clinical data were retrospectively reviewed for 71 TCA children(17 with mistreatment,45 with non-mistreatment and 9 without radical operation)confirmed intraoperatively or by postoperative pathological examination at Beijing Children's Hospital.Clinical profiles and reoperative findings of mistreated cases were analyzed.And surgical approaches,postoperative complications,growth and bowel function recovery(Rintala score)were compared between children with mistreatment and non-mistreatment.Results All mistreated patients(100%)were initially treated at class 3A hospitals(9 general hospitals,8 pediatric hospitals).Among them,10(58.8%)were improperly operated due to an initial misdiagnosis and 5(29%)had residual aganglionosis during the first operation.Abdominal distension(94%),constipation(82%),vomiting(76%)and enterocolitis(59%)were the major reoperative symptoms.All cases underwent radical re-operation and 14(82.4%)had intraoperative pathological examination.Number of operations,proportion of ileostomy and age of the last radical operation in children with mistreatment were significantly higher than those with non-mistreatment[3(3,5)vs.1(1,2),P<0.001;12/17 vs.16/41,P=0.028;525(197,1971)vs.164(29,464)days,P=0.007].Fifteen children with mistreatment and 31 with non-mistreatment were followed up with a median time of 5.4(2.1,8.0)years.No significant differences existed in postoperative complications,growth or Rintala score.Conclusions The leading causes of mistreatment are initial misdiagnosis,unreliable surgical experience and incorrect intraoperative pathological examination.An intraoperative pathological examination should be performed during reoperation and ileostomy if necessary.The treatment duration of TCA is significantly prolonged.However,the overall prognosis remains decent.