J Korean Med Sci.  2011 Mar;26(3):399-403. 10.3346/jkms.2011.26.3.399.

Long-term Follow-up after Feminizing Genital Reconstruction in Patients with Ambiguous Genitalia and High Vaginal Confluence

• Park S
• Ha SH
• Kim KS

• KMID: 2157878
• DOI: http://doi.org/10.3346/jkms.2011.26.3.399

Abstract

We evaluated the long-term results of feminizing genital reconstruction in patients with genital ambiguity with high vaginal confluence. The medical records of 10 consecutive patients with ambiguous genitalia and high vaginal confluence who underwent feminizing genital reconstruction from 1996 to 2007 were reviewed. Seven patients had congenital adrenal hyperplasia, one had mixed gonadal dysgenesis, one had partial androgen insensitivity, and one had 5-alpha reductase deficiency syndrome. Median age at operation was 21 months (range, 2-47 months). Median follow up was 7.7 yr. Of the six patients who underwent feminizing genital reconstruction with the Gonzalez method, three had good results. Of the other three patients, one had a urethrovaginal fistula and underwent fistula repair 9 yr after, one had distal vaginal stenosis and underwent revision vaginoplasty 9 yr after, and one had a urethrovaginal fistula and urethral stricture. The patient with urogenital mobilization had persistent urogenital sinus. Feminizing genitoplasty using the Gonzalez method showed good long-term results in patients with ambiguous genitalia and Congenital adrenal hyperplasia. The procedure is less invasive than other approaches and results in excellent cosmetic outcomes; and complications can be corrected by relatively simple procedures.

Keyword

Feminization; Adrenal Hyperplasia, Congenital; Disorders of Sex Development

MeSH Terms

Child, Preschool
Disorders of Sex Development/*surgery
Female
Feminization/*surgery
Follow-Up Studies
Humans
Infant
Male
Penis/abnormalities
*Reconstructive Surgical Procedures
Urethral Stricture/surgery
Urogenital System/surgery
Vagina/*surgery

Figure

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  Fig. 1 Perioperative photography. (A) Inverted "U"-shape incision. (B) Posterior skin flap.

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  Fig. 2 Anterior preputial flap.

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  Fig. 3 Genital appearance immediately after surgery.

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  Fig. 4 Genital appearances. (A) Preoperative image. (B) Postoperative image at the 3 months after surgery.

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