尿生殖窦游离术在治疗女性先天性肾上腺皮质增生症的疗效评估

Outcomes of urogenital sinus mobilization for congenital adrenal hyperplasia

目的探讨尿生殖窦游离（UGSM）术在治疗女性先天性肾上腺皮质增生症（CAH）的疗效。方法对2008年9月至2014年7月收治的13例一期完成阴蒂整形＋UGSM的女性cAH患儿临床资料进行回顾性分析，分为尿生殖窦部分游离（PUM）和尿生殖窦整体游离（TUM）两组，评估围手术期情况，通过DAN．PSS调查表了解术后下尿路症状及尿控情况，并采用改良的CAH女性外阴重建后外观评分标准对术后会阴部外观进行评分。结果PUM组8例，TUM组5例，中位手术年龄分别为3．2岁和5．9岁，共同通道中位长度分别为1．1cm和3．0cm，中位手术时间分别为180rain和200min。两组术后中位随访时间分别为42个月和50个月。PUM组中术后出现明显下尿路症状（LUTS）的共2例，1例为经常性的遗尿，1例为经常性的尿频尿急伴急迫性尿失禁。TUM组中术后出现明显LUTS的共3例，1例为中度的排空障碍伴轻微的排尿费力，1例为经常性的遗尿，1例为经常性的尿频尿急伴急迫性尿失禁。两组术后LUTS比较无明显差异，且术后均可获得良好的尿控能力。13例术后会阴部外观中4例美观，6例一般，3例不理想，两组比较无明显差异，共6例术后阴道口暴露不满意，专科医师建议二次手术。结论UGSM是目前治疗女性CAH尿生殖窦畸形非常有效的手术方式，可在青春期前一期完成，术前需全面评估尿生殖窦情况，术后可获得良好的尿控能力及满意的女性会阴外观。

Objective To evaluate the outcomes of urogenital sinus mobilization （UGSM） for congenital adrenal hyperplasia （CAH）. Methods From September 2008 to July 2014,13 cases of CAH underwent cliteroplasty and UGSM in one stage. They were divided into two groups of partial urogenital mobilization （PUM） and total urogenital mobilization （TUM）. Their perioperative conditions were assessed. And postoperative postoperative lower urinary tract symptoms （LUTS） were evaluated by DAN-PSS scale and postoperative perineal appearance was graded by improved rating scale. Results The procedures of PUM （n = 8） and TUM （n ： 5） were performed. The median operative age was 3. 2 years for PUM versus 5.9 years for TUM, median length of common channel 1.1 cm for PUM versus 3. 0 cm for TUM,median operative duration 180 min for PUM versus 200 rain for TUM, median follow-up time 42 months for PUM versus 50 months for TUM. In PUM group, 2 cases presented obvious LUTS. One had frequent enuresis and another urinary incontinence. In TUM group, 3 cases presented obvious LUTS, there were moderate incomplete emptying （n = 1 ）, enuresis （n = 1） and urinary incontinence （n = 1）. LUTS and perineal appearance had no inter-group difference. All cases had excellent postoperative urinary continence. The cosmetic outcomes were satisfactory （n = 4） ,average （n = 6） and unsatisfactory （n = 3）. Six cases of poor vaginal exposure were recommended for re-operation. Conclusions As an ideal treatment for female CAH,UGSM may be completed in one stage with cliteroplasty. Overall preoperative assessment of urogenital sinus status yields excellent cosmetics and functional outcomes.