Abstract
ABSTRACTIntroduction: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.
Highlights
One of the most complex problems of pediatric urology is the surgical management of disorders of sex development (DSD)
To our knowledge there are no previous descriptions of a complete corporeal preservation clitoroplasty (CCPC) technique and hereby we present our surgical approach
Vaginoplasty was performed in all patients with partial urogenital mobilization (PUM) and Urogenital Sinus flap (UF) technique
Summary
One of the most complex problems of pediatric urology is the surgical management of disorders of sex development (DSD). The most frequent cause is enzymatic deficiency of 21-hydroxilase, followed by 11β-hydroxylase. The lack of these enzymes manifests with varying degrees of virilization of the external genitalia as the result of excess androgenic metabolites [1,2,3]. Management of DSDs should be conducted by a transdisciplinary team that allows proper genetic counseling, accurate diagnosis, classification of the DSD, individualized surgical planning and a comprehensive decision-making process involving the family [1, 4]. Most families opt for early surgery, considering the positive implications for children’s psychosocial development, relieving parents distress and restoring “normal” external genital configuration. Szymanski et al evaluated parental decisional regret after FG, they reported that 20.5% of parents, reported some regret, in contrast no parent would have chosen again delayed surgery [1, 4,5,6]
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