Surgical Complications Following Early Genitourinary Reconstructive Surgery for Congenital Adrenal Hyperplasia-Interim Analysis at 6 Years

OBJECTIVE: To review the intermediate- to long-term surgical complications following feminizing reconstructive surgery for patients with congenital adrenal hyperplasia (CAH) at a single tertiary center. Genitourinary reconstructive surgery is pivotal to favorable cosmetic and functional outcomes for patients with CAH.

MATERIALS AND METHODS: We conducted a retrospective review identifying 26 patients from April 2003 to April 2015 who underwent genitourinary reconstructive surgeries. Demographic data and surgical postoperative complications (per Unplanned Postoperative Morbidity in Children [UPMC] and Clavien-Dindo classification) were evaluated.

RESULTS: The average age at the time primary surgery was done at our institute was 17 ± 20 (5-87) months; the average length of follow-up was 72.56 ± 36.95 (4.5-142) months. The average length of the common urogenital sinus was 4.5 ± 1.9 (2.5-6.4) cm, and 15 out of 22 (68%) patients had high confluence. A total of 7 complications were observed in 7 (27%) patients, 3 patients had Clavien grade I and UPMC 0 (dysuria, stitch dehiscence, wound separation), and 2 had Clavien grade II and UPMC 1 (urinary tract infection). Two (7.6%) patients required revision surgery, and both of them had Clavien grade IIIB. One patient had UPMC score 5 (suprapubic tube insertion for urinary retention secondary to cicatrization of the surgical site).

CONCLUSION: Genitourinary reconstructive surgery for CAH patients is well tolerated and is successful in toddlers, with low (7.6%) complication rate. Revision surgery has successful outcome both functionally and cosmetically. In toilet-trained girls, urinary continence can be achieved in all patients following feminizing genitoplasty.