Complete corporeal preservation clitoroplasty: new insights into feminizing genitoplasty

Abstract

Introduction: 46,XX Congenital adrenal hyperplasia (CAH) remains the first cause of genital virilization and current surgical techniques aim to restore female aspect of genitalia while preserving dorsal neurovascular bundle but not at the expense of not preserving erectile tissue. We aim to report our experience with a new surgical technique for clitoroplasty, completely preserving corporeal bodies, neurovascular bundles without dismembering the clitoris, in four patients with over a year follow up.

Materials and methods: After IRB approval four patients with 46,XX CAH and Prader 5 and 3 external genitalia, underwent feminizing genitoplasty. Complete preservation of erectile tissue was accomplished without a need to dissect dorsal neurovascular bundle. Glans size allowed no need for glanular reduction and there was no need to dismember the corporeal bodies.

Results: Four patients 12 to 24-months-old underwent complete corporeal preservation clitoroplasty (CCPC), mean age was 18.5 months, mean follow up was 10.25 months. Vaginoplasty was performed in all patients with partial urogenital mobilization (PUM) and Urogenital Sinus flap (UF), only one severely virilized patient required a parasagittal pre-rectal approach to mobilize the vagina. We had no complications until last follow up.

Conclusion: To our knowledge, we are introducing the concept of CCPC without the need of disassembling the corporeal bodies, neurovascular bundle and glans. It stands as a new alternative for feminizing genitoplasty with complete preservation of erectile tissue and no dissection of neurovascular bundle. Although there is still lacking long-term follow-up, it represents a new step in conservative reconfiguration of the external virilized female genitalia.

Keywords: Adrenal Hyperplasia, Congenital; Clitoris; Surgical Procedures, Operative.

Figure 1. Patient 1, 24 months old. A) Virilized External Genitalia, Prader V. B) Well-defined (male-like) external sphincter with a verumontanum appearance distal to the vaginal confluence (Arrow head) C) Vagina and Cervix (Arrow) D) A 3 fr Fogarty catheter in the bladder and a 6 fr Foley catheter in the vagina both going through the urogenital sinus (UGS).

Figure 2. Patient 1, 24 months old A) and B) Clitoral dissection preserving the dorsal neurovascular bundle (NVB) intact C. The clitoris and the NVB (Arrow head) dissected from the UGS (Arrow). D) Non-dismembered clitoris (Arrow head) with its NVB preserved.

Figure 3. Patient 2, 12 months old. A) and B) Detachment of the two hemicorpora in the midline (Arrow), up to the middle third of the phallic shaft. C) and D) Completed, corporeal sparing clitoroplasty showing a 3.5cm reduction in clitoral length. The proximal two thirds of each hemicorpora were laterally mobilized and anchored to the pubic bone (Red Dotted arrows).

Figure 4. A) Patient 3, 14 months old 46,XX with virilized external genitalia showing a Prader III configuration. B) External genitalia appearance at ten months follow-up after complete corporeal preservation clitoroplasty. C) and D) Patient 1, 24 months old, External genitalia appearance at 18 months follow-up after clitoroplasty.