Evaluation of urogenital sinus surgery in disorder of sex development_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

MAO Yu , CHEN ShaoJi ,  TANG Yunman , WANG Xuejun , QIN Daorui , CHEN Yuejiao

Department of Pediatric Surgery at Children’s Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People’s Hospital, Chengdu Sichuan, 610072, P.R.China;

Corresponding author：

TANG Yunman, Email: tangyunman@126.com

Keywords：

Urogenital sinus surgery; disorder of sex development; genitoplasty; vaginoplasty

DOI：

10.7507/1002-1892.201705004

Video：

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ObjectiveTo explore the indication and effectiveness of urogenital sinus surgery in feminizing genitoplasty of disorder of sex development (DSD).MethodsA retrospective analysis was made on clinical data from 22 patients with DSD who underwent one stage feminizing genitoplasty between October 2010 and December 2015. The patients’ age ranged from 1 year and 2 months to 21 years, with the median age of 2 years and 1 month. According to the Prader classification criteria, the appearance of vulvas were rated as grade Ⅰ in 7 cases, grade Ⅱ in 6 cases, grade Ⅲ in 8 cases, and grade Ⅳ in 1 case. Cystoscopy was applied before feminizing genitoplasty in all patients. Low confluence of vagina and urethra was found in 19 patients, while high confluence was found in 3 patients. The mean length of urogenital sinus was 1.6 cm (range, 0.5-3.0 cm). The mean length of water-filled vagina was 4.4 cm (range, 3.5-5.5 cm). Cervix was detected at the end of vagina in 16 patients, meanwhile absence of cervix was detected in 6 patients. The same procedures of clitoroplasty and labioplasty were used in all patients. Three procedures of urogenital sinus surgery were applied, as the " cut-back” vaginoplasty in 6 patients, the " flap” vaginoplasty in 11 patients, and the partial urogenital sinus mobilization (PUM) in 5 patients.ResultsAll procedures were completed successfully and the incisions healed by stage Ⅰ. All patients were followed up 12-74 months, with the average of 30.5 months. The outcome of appearance evaluation was excellent in 13 patients (59.1%), good in 6 patients (27.3%), and poor in 3 patients (13.6%). Urinary incontinence, post-void residual, urinary infection, and urethrovaginal fistula were not found in 17 toilet trained patients.ConclusionUrogenital sinus surgery is the most critical step in feminizing genitoplasty of DSD. It can be finished in one stage procedure with clitoroplasty and labioplasty before puberty. If thoroughly evaluation before surgery is completed and the principle of different procedures is handled, the outcome will be satisfactory.

Citation： MAO Yu, CHEN ShaoJi, TANG Yunman, WANG Xuejun, QIN Daorui, CHEN Yuejiao. Evaluation of urogenital sinus surgery in disorder of sex development. Chinese Journal of Reparative and Reconstructive Surgery, 2017, 31(9): 1092-1097. doi: 10.7507/1002-1892.201705004 Copy

1.	Braga LH, Lorenzo AJ, Tatsuo ES, et al. Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for congenital adrenal hyperplasia. J Urol, 2006, 176(5): 2199-2204.
2.	Kanhere M, Fuqua J, Rink R, et al. Psychosexual development and quality of life outcomes in females with congenital adrenal hyperplasia. Int J Pediatr Endocrinol, 2015, 2015: 21.
3.	Ahmed SF, Rodie M. Investigation and initial management of ambiguous genitalia. Best Pract Res Clin Endocrinol Metab, 2010, 24(2): 197-218.
4.	David AD. Disorders of Sexual Development: Etiology, Evaluation, and Medical Management//CAMPBELL-WALSH Urology. 11th ed. Philadelphia: Elsevier, 2016: 4601-4635.
5.	Binet A, Lardy H, Geslin D, et al. Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype? J Pediatr Surg, 2016, 51(3): 465-468.
6.	Fortunoff S, Lattimer JK, Edson M. Vaginoplasty technique for female pseudohermaphrodites. Surg Gynecol Obstet, 1964, 118: 545-548.
7.	Jenak R, Ludwikowski B, González R. Total urogenital sinus mobilization: a modified perineal approach for feminizing genitoplasty and urogenital sinus repair. J Urol, 2001, 165(6 Pt 2): 2347-2349.
8.	Peña A. Total urogenital mobilization—an easier way to repair cloacas. J Pediatr Surg, 1997, 32(2): 263-268.
9.	Rink RC, Adams MC, Misseri R. A new classification for genital ambiguity and urogenital sinus anomalies. BJU Int, 2005, 95(4): 638-642.
10.	Tugtepe H, Thomas DT, Turan S, et al. Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients? J Pediatr Urol, 2014, 10(5): 948-954.
11.	Park S, Ha SH, Kim KS. Long-term follow-up after feminizing genital reconstruction in patients with ambiguous genitalia and high vaginal confluence. J Korean Med Sci, 2011, 26(3): 399-403.
12.	Yankovic F, Cherian A, Steven L, et al. Current practice in feminizing surgery for congenital adrenal hyperplasia; a specialist survey. J Pediatr Urol, 2013, 9(6 Pt B): 1103-1107.
13.	Sturm RM, Durbin-Johnson B, Kurzrock EA. Congenital adrenal hyperplasia: current surgical management at academic medical centers in the United States. J Urol, 2015, 193(5 Suppl): 1796-1801.
14.	Nordenskjöld A, Holmdahl G, Frisén L, et al. Type of mutation and surgical procedure affect long-term quality of life for women with congenital adrenal hyperplasia. J Clin Endocrinol Metab, 2008, 93(2): 380-386.
15.	M Selveindran N, Syed Zakaria SZ, Jalaludin MY, et al. Behavioural Problems in Children with 46XY Disorders of Sex Development. Int J Endocrinol, 2017, 2017: 5987490.
16.	Wang LC, Poppas DP. Surgical outcomes and complications of reconstructive surgery in the female congenital adrenal hyperplasia patient: What every endocrinologist should know. J Steroid Biochem Mol Biol, 2017, 165(Pt A): 137-144.

1. Braga LH, Lorenzo AJ, Tatsuo ES, et al. Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for congenital adrenal hyperplasia. J Urol, 2006, 176(5): 2199-2204.
2. Kanhere M, Fuqua J, Rink R, et al. Psychosexual development and quality of life outcomes in females with congenital adrenal hyperplasia. Int J Pediatr Endocrinol, 2015, 2015: 21.
3. Ahmed SF, Rodie M. Investigation and initial management of ambiguous genitalia. Best Pract Res Clin Endocrinol Metab, 2010, 24(2): 197-218.
4. David AD. Disorders of Sexual Development: Etiology, Evaluation, and Medical Management//CAMPBELL-WALSH Urology. 11th ed. Philadelphia: Elsevier, 2016: 4601-4635.
5. Binet A, Lardy H, Geslin D, et al. Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype? J Pediatr Surg, 2016, 51(3): 465-468.
6. Fortunoff S, Lattimer JK, Edson M. Vaginoplasty technique for female pseudohermaphrodites. Surg Gynecol Obstet, 1964, 118: 545-548.
7. Jenak R, Ludwikowski B, González R. Total urogenital sinus mobilization: a modified perineal approach for feminizing genitoplasty and urogenital sinus repair. J Urol, 2001, 165(6 Pt 2): 2347-2349.
8. Peña A. Total urogenital mobilization—an easier way to repair cloacas. J Pediatr Surg, 1997, 32(2): 263-268.
9. Rink RC, Adams MC, Misseri R. A new classification for genital ambiguity and urogenital sinus anomalies. BJU Int, 2005, 95(4): 638-642.
10. Tugtepe H, Thomas DT, Turan S, et al. Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients? J Pediatr Urol, 2014, 10(5): 948-954.
11. Park S, Ha SH, Kim KS. Long-term follow-up after feminizing genital reconstruction in patients with ambiguous genitalia and high vaginal confluence. J Korean Med Sci, 2011, 26(3): 399-403.
12. Yankovic F, Cherian A, Steven L, et al. Current practice in feminizing surgery for congenital adrenal hyperplasia; a specialist survey. J Pediatr Urol, 2013, 9(6 Pt B): 1103-1107.
13. Sturm RM, Durbin-Johnson B, Kurzrock EA. Congenital adrenal hyperplasia: current surgical management at academic medical centers in the United States. J Urol, 2015, 193(5 Suppl): 1796-1801.
14. Nordenskjöld A, Holmdahl G, Frisén L, et al. Type of mutation and surgical procedure affect long-term quality of life for women with congenital adrenal hyperplasia. J Clin Endocrinol Metab, 2008, 93(2): 380-386.
15. M Selveindran N, Syed Zakaria SZ, Jalaludin MY, et al. Behavioural Problems in Children with 46XY Disorders of Sex Development. Int J Endocrinol, 2017, 2017: 5987490.
16. Wang LC, Poppas DP. Surgical outcomes and complications of reconstructive surgery in the female congenital adrenal hyperplasia patient: What every endocrinologist should know. J Steroid Biochem Mol Biol, 2017, 165(Pt A): 137-144.

Chinese Journal of Reparative and Reconstructive Surgery

Evaluation of urogenital sinus surgery in disorder of sex development

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