Assessment of Urogenital Sinus Mobilization and Feminizing Genitoplasty in Children with Congenital Adrenal hyperplasia

Fathy, Mohamed; Ali, Ibrahim; Moustafa, Alaa A. E.; Mammdouh, Mohamed M.; Akoula, Ahmed; Ahmed, Ahmed H.; Elbadry, Mohamed; Tagreda, Ibrahim; Negm, Mohamed A.

doi:10.21608/ejsur.2024.314610.1175

Assessment of Urogenital Sinus Mobilization and Feminizing Genitoplasty in Children with Congenital Adrenal hyperplasia

Document Type : Original Article

Authors

¹ Department of Pediatric Surgery Unit, Faculty of Medicine, Minia University Hospitals, Egypt.

² Department Pediatric Surgery, Assiut University Pediatric Hospital, Assiut, Egypt.

³ Department of Urology, Faculty of Medicine, Minia University Hospitals, Egypt.

⁴ Department of Urology, Faculty of Medicine, Al-Azhar University, Al-Azhar, Egypt.

⁵ Department of Pediatric Surgery Unit, Qena Faculty of Medicine, South Valley University, Qena, Egypt.

10.21608/ejsur.2024.314610.1175

Abstract

Background: The objective of surgical intervention in females with urogenital sinus (UGS) and congenital adrenal hyperplasia (CAH) is to maintain bladder function, restore normal appearance and function of the external genitalia, and enable normal sexual and reproductive development throughout adulthood. We aimed to assess female patients who underwent UGS mobilization as part of feminizing genitoplasty treatment.
Methods: This study utilized a retrospective descriptive database of 20 patients who underwent feminizing genitoplasty and UGS mobilization in a single session for patients with CAH.
Results: The mean age for the 20 subjects included in the study was 27.3 ±10.55 months. Of these patients, 6 (30%) were classified as Prader III, whereas 14 (70%) were Prader IV. The length of the UGS was determined to be < 2.0 cm in 2 (10%), 2.0 cm in 5 (25%), 2.5 cm in 8 (40%), and ≤ 3.0 cm in 5 (25%) cases. The vaginal orifice was retracted with abnormal introitus in 2 (10%) cases and was seated in a normal site in the posterior portion of the vestibule in 18 (90%) patients. The labia minora had a satisfactory cosmetic appearance and looked relatively normal in all the studied patients. The labia majora had a satisfactory cosmetic appearance in 18 (90%) and retracted in two cases (10%). Residual urine occurred in 3 (15%) patients. There was a complete absence of urinary dribbling in all patients. In our study, a urogenital fistula was observed in one patient, accounting for 5% of the cases. However, no cases of recto-vaginal fistula were identified.
Conclusions: Feminizing genitoplasty combined with urogenital sinus mobilization is an effective method for patients with CAH in early childhood. Furthermore, Good cosmetic genitalia appearance and urinary continence can be achieved in the majority of patients.

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