关键词: 先天性肾上腺皮质增生症, 性别发育异常, 尿生殖窦部分游离术

Abstract: Objective To evaluate the effect of partial mobilization of urogenital sinus in the treatment of sexual dysplasia(DSD) caused by female congenital adrenal hyperplasia(CAH). Methods The clinical data of 46,XX DSD children caused by CAH who were diagnosed and treated in the First Affiliated Hospital of Xinjiang Medical University from April 2016 to November 2019 were retrospectively analyzed.Preoperative cystoscopy was performed to measure the common channel length of the urogenital sinus,and 15 children suitable for partial mobilization of the urogenital sinus(PUM) combined with clitoroplasty and labioplasty to complete the feminization of the external genitalia in one stage were selected.Postoperative perineal appearance,urodynamic studies,and lower urinary tract symptoms were followed up to assess efficacy. Results All children were followed up for 6 months,1 year and 2 years after operation.The common channel length of the urogenital sinus was 0.9-1.9cm,with an average of 1.45cm.The incisions of the children were healed by first intention;13 cases had realistic vaginal shape,1 case had large and small labia minora,poor symmetry,1 case had vaginal stenosis,but none had lower urinary tract symptoms(LUTs);15 cases who underwent PUM had postoperative urodynamic examination:maximum urinary flow rate:12-27ml/s,no abnormality of bladder during filling period,bladder capacity in the normal range,no urine leakage;no abnormality of maximum voiding pressure.Conclusion PUM has an ideal therapeutic effect on 46,XX DSD children with short common channel of urogenital sinus(less than 1.9cm),with realistic.

Key words: congenital adrenal hyperplasia, disorders of sex development, partial urogenital sinus mobilization