Department of Paediatric Surgery | Cairo University Specialized Paediatric Hospital (CUSPH) & Cairo University Children's Hospital (CUCH) (email)
Background and Rationale: Congenital adrenal hyperplasia in females leads to virilization of the external genitalia and an anomalous lower genitourinary tract. Hormonal therapy controls the endocrinopathy. Surgical repair of the anomaly is indicated to allow raising a female child with external genitalia matching her gender and to avoid psychological complications. Surgery at an earlier stage is believed to be easier and of better results than when deferred to adolescence.
Objectives: To study the anatomical abnormalities of genotypic females with virilized atypical genitalia due to congenital adrenal hyperplasia and their impact on surgery and to review the surgical techniques of feminizing genitoplasty and their early short-term outcomes. We aim to highlight the multidisciplinary management for these complex cases and to discuss the difficulties and challenges met during this.
Patients & Methods: Genotypic prepubertal females with virilized atypical genitalia due to congenital adrenal hyperplasia were included. Radiological studies in the form of sonography to visualize the uterus and gonads and genitography for delineation of the urogenital tract then followed. Endoscopic assessment (cystoscopy) was done at the time of surgery with catheter placement in both the vagina and urethra to guide the surgical procedure. Surgical genitoplasty aimed at a single-stage full correction for all cases preferring partial urogenital mobilization.
Outcome parameters: Achievement of an acceptable female appearance of the external genitalia, achievement of adequately sized and positioned vaginal and urethral orifices, and occurrence of short term complications.
Conclusions: Feminizing genitoplasty can be done safely between the age of 6 months and one year. Flap vaginoplasty and cut-back exteriorization are only suitable for low confluence. Partial urogenital mobilization is a safe and effective technique that is suitable to most cases.
KASR EL AINI MEDICAL JOURNAL. 01/2014; 20(1):17-27.