Yan Jiayu,Chen Yajun,Peng Chunhui,et al.Clinical diagnosis and treatment of cloacal malformation in boys[J].Journal of Clinical Pediatric Surgery,2020,19(10):878-883.[doi:10.3969/j.issn.1671-6353.2020.10.004]
男童泄殖腔畸形诊治分析
- Title:
- Clinical diagnosis and treatment of cloacal malformation in boys
- Keywords:
- Digestive System Abnormalisties; Cloaca/AB; Male; Diagnosis; Therapy
- 分类号:
- R726.1;R726.2;R657.1
- 摘要:
- 目的 探讨男童泄殖腔畸形（cloacal malformation，CM）的临床特征、诊断方法、手术方式及预后情况。方法 回顾性分析2005年1月至2019年12月首都医科大学附属北京儿童医院普外科收治的4例男童CM的临床资料，并采用Rintala评分和排尿功能障碍评分系统（dysfunctional voiding scoring system，DVSS）进行随访，分别评估其排便功能和排尿功能。Rintala评分和DVSS评估需患儿年龄≥4岁。Rintala评分范围0~20分，<12分提示排便功能不良，12~16提示排便功能一般，≥17分提示排便功能良好；DVSS系统评分范围0~30分，评分越高提示功能越差。结果 4例患儿产后均发现无正常肛门，粪便、尿液从会阴部同一开口排出。3例全程在本院治疗，就诊年龄分别为5个月、5个月和1岁2个月；1例于外院行尿道成形术，术后出现直肠尿道瘘转至本院，就诊年龄为10岁9个月。4例患儿均为重度尿道下裂合并阴茎阴囊转位。会阴部查体结合辅助检查（泌尿系统超声、骶尾部X线平片和瘘道造影检查等）能明确诊断及合并畸形。全程在本院治疗的患儿依次行前矢状入路会阴肛门成形-尿道成形-其他畸形矫正的手术方案，术后1例出现直肠会阴瘘；外院术后患儿再次手术，采用后矢状入路骶会阴肛门成形+直肠尿道瘘修补术。全程在本院治疗的3例患儿获得随访，随访年龄分别至6岁、11岁（合并骶骨畸形）和15岁（合并骶骨畸形），Rintala评分分别为20分、12分和10分，DVSS评分分别为0分、10分和3分。结论 通过会阴部查体，结合相关辅助检查能明确诊断男童CM及合并畸形，该类患儿可依次行前矢状入路会阴肛门成形-尿道成形的手术治疗。术后排便功能与排尿功能评分与患儿有无骶骨畸形密切相关。
- Abstract:
- Objective To explore the clinical characteristics,diagnosis,surgery and prognosis of cloacal malformation (CM) in boys.Methods A retrospective analysis was conducted for 4 CM boys aged over 4 years from January 2005 and December 2019.Their defecation and urination functions were evaluated by Rintala score and dysfunctional voiding scoring system (DVSS) respectively.Based upon total Rintala score (0-20),three categories of defecation function were assigned:≥17 normal defecation function,12-16 mild defecation dysfunction and<12 severe defecation dysfunction;the higher total DVSS score (0-30),the worse urination function.Results All of them had one single perineal opening for passing stool and urine without normal anus after birth.The age of admission of three boys on full-course treatment was 5,5,12 and 2 months respectively.Another 129-month-old boy with rectourethral fistula after undergoing urethroplasty at another hospital was admitted.All were associated with severe hypospadias and penoscrotal transposition.Perineal examination plus auxiliary examinations could effectively diagnose the disease and associated malformations.Anorectal,urological and other associated anomalies were resolved successively and one boy had postoperative rectal perineal fistula.Posterior sagittal anorectoplasty and rectourethral fistula repair was performed for rectourethral fistula.Three boys were followed up until an age of 6,11 (with sacral abnormality) and 15 (with sacral abnormality).They had Rintala score of 20,12,10 and DVSS of 0,10,3 respectively.Conclusion Physical and auxiliary perineal examinations are crucial for diagnosing CM and associated malformations in boys.Perineal anoplasty and urethroplasty may be performed for CM boys.Defecation and urination functions are closely related with sacral abnormalities.
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备注/Memo
收稿日期:2020-02-03。
基金项目:中国工程院-外科扶持基金
通讯作者:陈亚军,Email:chenyajunmd@aliyun.com