CLITOROPLASTY FOR GENITAL VIRILIZATION IN GIRLS WITH CONGENITAL ADRENAL HYPERPLASIA_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

ZENGLi , ZHANGJie , HUANGGuizhen , HUANGYidong , MAXue ,  HUANGLugang

Department of Pediatric Surgery, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P. R. China;

Corresponding?author：

HUANGLugang, Email: lugang992001@aliyun.com

Keywords：

Clitoroplasty; Genital virilization; Disorders of sexual development; Congenital adrenal hyperplasia

DOI：

10.7507/1002-1892.20160231

Video：

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Objective To investigate the effectiveness of clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate for genital virilization in girls with congenital adrenal hyperplasia (CAH). Methods Between June 2000 and October 2015, 27 girls with genital virilization were treated with clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate. The chromosomal karyotype was 46, XX in all patients, whose age ranged from 3 to 11 years (mean, 3.9 years). According to Parder's virilization scale, 5 cases were classified as stage Ⅱ, 18 cases as stage Ⅲ, and 4 cases as stage Ⅳ. The hormone tests showed decreased cortisol level and increased testosterone and 17-hydroxyprogesterone levels. They were diagnosed with non-salt-wasting CAH. Results All of the patients underwent clitoroplasty successfully. The mean operation time was 74 minutes (range, 58-95 minutes). Incision healed primarily. The patients were followed up 6 months to 10 years (median, 19 months). The external genitalia had good appearance, without necrosis of flap and the glans clitoris, abnormal sensation of the glans clitoris, or clitorism recurrence. Five girls had breast development and normal menstrual cycle during adolescence, and 1 patient who was followed up for 10 years gave birth to a son. Conclusion Clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate is a relatively ideal method for treating genital virilization in girls. It has less complications, good aesthetic and functional results. There are satisfactory outcomes when combining with the endocrine treatment.

Citation： ZENGLi, ZHANGJie, HUANGGuizhen, HUANGYidong, MAXue, HUANGLugang. CLITOROPLASTY FOR GENITAL VIRILIZATION IN GIRLS WITH CONGENITAL ADRENAL HYPERPLASIA. Chinese Journal of Reparative and Reconstructive Surgery, 2016, 30(9): 1134-1138. doi: 10.7507/1002-1892.20160231 Copy

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2.	Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet, 2005, 365(9477):2125-2136.
3.	Gozar H, Pascanu I, Ardelean M, et al. Surgical reconstruction of the genitalia in a 3-year-old infant with a 46XX karyotype:case report. Aesthetic Plast Surg, 2014, 38(3):549-553.
4.	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.
5.	Latrech H, Skikar I, Gharbi Mel H, et al. Disorder of sexual development and congenital heart defect in 47XYY:clinical disorder or coincidence? Case Rep Endocrinol, 2015, 2015:802162.
6.	Nakashima S, Ohishi A, Takada F, et al. Clinical and molecular studies in four patients with SRY-positive 46, XX testicular disorders of sex development:implications for variable sex development and genomic rearrangements. J Hum Genet, 2014, 59(10):549-553.
7.	Juniarto AZ, van der Zwan YG, Santosa A, et al. Application of the new classification on patients with a disorder of sex development in Indonesia. Int J Endocrinol, 2012, 2012:237084.
8.	Guercio G, Rey RA. Fertility issues in the management of patients with disorders of sex development. Endocr Dev, 2014, 27:87-98.
9.	楊洋, 周曉玉, 周曉光. 21-羥化酶缺乏先天性腎上腺皮質增生癥52例臨床分析.中國當代兒科雜志, 2015, 17(6):613-617.
10.	梁玲, 辛穎. 47例先天性腎上腺皮質增生癥的臨床特點及診治分析.中國優生與遺傳雜志, 2014, 22(1):119-121.
11.	Speiser PW, Azziz R, Baskin LS, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency:an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab, 2010, 95(9):4133-4160.
12.	Speiser PW, Azziz R, Baskin LS, et al. A summary of the endocrine society clinical practice guidelines on congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency. Int J Pediatr Endocrinol, 2010, 2010:494173.
13.	Reifsnyder JE, Stites J, Stites J, et al. Nerve sparing clitoroplasty is an option for adolescent and adult female patients with congenital adrenal hyperplasia and clitoral pain following prior clitoral recession or incomplete reduction. J Urol, 2016, 195(4 Pt 2):1270-1274.
14.	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.
15.	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.
16.	Leslie JA, Cain MP, Rink RC. Feminizing genital reconstruction in congenital adrenal hyperplasia. Indian J Urol, 2009, 25(1):17-26.
17.	Savanelli A, Alicchio F, Esposito C, et al. A modified approach for feminizing genitoplasty. World J Urol, 2008, 26(5):517-520.
18.	Poppas DP, Hochsztein AA, Baergen RN, et al. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol, 2007, 178(4 Pt 2):1802-1806.
19.	Pippi Salle JL, Braga LP, Macedo N, et al. Corporeal sparing dismembered clitoroplasty:an alternative technique for feminizing genitoplasty. J Urol, 2007, 178(4 Pt 2):1796-1800.
20.	林小梅, 吳本清, 黃進潔, 等.先天性腎上腺皮質增生癥1例CYP21A2基因突變分析.中國當代兒科雜志, 2013, 15(11):942-947.
21.	Chen W, Xu Z, Nishitani M, et al. Complement component 4 copy number variation and CYP21A2 genotype associations in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hum Genet, 2012, 131(12):1889-1894.

1. ?Stephen YN, Thomas HS. Campbell's urology. 10th ed. Philadelphia:W.B. Saunders, 2012:3599-3628.
2. Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet, 2005, 365(9477):2125-2136.
3. Gozar H, Pascanu I, Ardelean M, et al. Surgical reconstruction of the genitalia in a 3-year-old infant with a 46XX karyotype:case report. Aesthetic Plast Surg, 2014, 38(3):549-553.
4. 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.
5. Latrech H, Skikar I, Gharbi Mel H, et al. Disorder of sexual development and congenital heart defect in 47XYY:clinical disorder or coincidence? Case Rep Endocrinol, 2015, 2015:802162.
6. Nakashima S, Ohishi A, Takada F, et al. Clinical and molecular studies in four patients with SRY-positive 46, XX testicular disorders of sex development:implications for variable sex development and genomic rearrangements. J Hum Genet, 2014, 59(10):549-553.
7. Juniarto AZ, van der Zwan YG, Santosa A, et al. Application of the new classification on patients with a disorder of sex development in Indonesia. Int J Endocrinol, 2012, 2012:237084.
8. Guercio G, Rey RA. Fertility issues in the management of patients with disorders of sex development. Endocr Dev, 2014, 27:87-98.
9. 楊洋, 周曉玉, 周曉光. 21-羥化酶缺乏先天性腎上腺皮質增生癥52例臨床分析.中國當代兒科雜志, 2015, 17(6):613-617.
10. 梁玲, 辛穎. 47例先天性腎上腺皮質增生癥的臨床特點及診治分析.中國優生與遺傳雜志, 2014, 22(1):119-121.
11. Speiser PW, Azziz R, Baskin LS, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency:an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab, 2010, 95(9):4133-4160.
12. Speiser PW, Azziz R, Baskin LS, et al. A summary of the endocrine society clinical practice guidelines on congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency. Int J Pediatr Endocrinol, 2010, 2010:494173.
13. Reifsnyder JE, Stites J, Stites J, et al. Nerve sparing clitoroplasty is an option for adolescent and adult female patients with congenital adrenal hyperplasia and clitoral pain following prior clitoral recession or incomplete reduction. J Urol, 2016, 195(4 Pt 2):1270-1274.
14. 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.
15. 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.
16. Leslie JA, Cain MP, Rink RC. Feminizing genital reconstruction in congenital adrenal hyperplasia. Indian J Urol, 2009, 25(1):17-26.
17. Savanelli A, Alicchio F, Esposito C, et al. A modified approach for feminizing genitoplasty. World J Urol, 2008, 26(5):517-520.
18. Poppas DP, Hochsztein AA, Baergen RN, et al. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol, 2007, 178(4 Pt 2):1802-1806.
19. Pippi Salle JL, Braga LP, Macedo N, et al. Corporeal sparing dismembered clitoroplasty:an alternative technique for feminizing genitoplasty. J Urol, 2007, 178(4 Pt 2):1796-1800.
20. 林小梅, 吳本清, 黃進潔, 等.先天性腎上腺皮質增生癥1例CYP21A2基因突變分析.中國當代兒科雜志, 2013, 15(11):942-947.
21. Chen W, Xu Z, Nishitani M, et al. Complement component 4 copy number variation and CYP21A2 genotype associations in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hum Genet, 2012, 131(12):1889-1894.

Chinese Journal of Reparative and Reconstructive Surgery

CLITOROPLASTY FOR GENITAL VIRILIZATION IN GIRLS WITH CONGENITAL ADRENAL HYPERPLASIA

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