Braga Luís H P, Lorenzo Armando J, Tatsuo Edson S, Silva Ivani N, Pippi Salle João L
Division of Pediatric Urology, Hospital for Sick Children, Toronto, Ontario, Canada.
J Urol. 2006 Nov;176(5):2199-204. doi: 10.1016/j.juro.2006.07.063.
We present our experience with partial urogenital sinus mobilization in girls with congenital adrenal hyperplasia, with particular attention to vaginal caliber, introitus position, urinary continence and genital appearance.
We present data on a prospective evaluation of 24 girls with congenital adrenal hyperplasia who underwent feminizing genitoplasty using urogenital sinus mobilization with preservation of the pubourethral ligaments during a 4-year period. Urogenital sinus length determined by cystoscopy and degree of external genitalia virilization, defined according to Prader classification, were evaluated before reconstruction. At followup patients were examined while under sedation for evaluation of overall external genitalia cosmesis and calibration of the vagina. Urinary continence status and voiding efficiency were assessed clinically in toilet trained patients by voiding diary, and measurement of bladder capacity and post-void residual by ultrasound.
Patient age at operation ranged from 1 to 16 years (median 28.5 months), with a mean followup of 25 months (8 to 47). Degree of virilization was Prader type III in 3 children (12.5%), type IV in 16 (66.7%) and type V in 5 (20.8%). Urogenital sinus length was 2.5 cm or less in 17 children. Cosmetic results were good in 21 patients (87.5%) and satisfactory in 3 (12.5%). The vaginal and urethral openings were separate and identified at the surface of the vestibule in 21 girls. Adequate caliber of the mobilized vagina was achieved in 23 patients (95.8%). None of the 20 toilet trained children had urinary incontinence or recurrent urinary tract infections, with normal bladder emptying in 18 and small post-void residual in 2 (10%).
Urinary continence and excellent cosmetic appearance with adequate exteriorization of the vaginal and urethral openings can be achieved in most children with urogenital sinus anomaly treated with partial urogenital sinus mobilization.
我们介绍了在先天性肾上腺皮质增生症女孩中进行部分泌尿生殖窦游离术的经验，特别关注阴道口径、阴道口位置、尿失禁及外生殖器外观。
我们提供了对24例先天性肾上腺皮质增生症女孩进行前瞻性评估的数据，这些女孩在4年期间接受了保留耻骨尿道韧带的泌尿生殖窦游离术的女性化生殖器成形术。在重建前，通过膀胱镜检查确定泌尿生殖窦长度，并根据普拉德分类法评估外生殖器男性化程度。随访时，在镇静状态下对患者进行检查，以评估外生殖器整体美观情况及阴道校准。对已接受如厕训练的患者，通过排尿日记临床评估尿失禁状态和排尿效率，并通过超声测量膀胱容量和排尿后残余尿量。
手术时患者年龄为1至16岁（中位年龄28.5个月），平均随访25个月（8至47个月）。3例患儿（12.5%）为普拉德III型男性化，16例（66.7%）为IV型，5例（20.8%）为V型。17例患儿的泌尿生殖窦长度为2.5 cm或更短。21例患者（87.5%）的美容效果良好，3例（12.5%）令人满意。21例女孩的阴道和尿道开口分开且在前庭表面可识别。23例患者（95.8%）实现了游离阴道的足够口径。20例已接受如厕训练的患儿均无尿失禁或反复尿路感染，18例膀胱排空正常，2例（10%）排尿后残余尿量少。
大多数接受部分泌尿生殖窦游离术治疗泌尿生殖窦异常的儿童可实现尿失禁以及阴道和尿道开口充分外露的出色美容外观。
