ESPE2024 Poster Category 3 Adrenals and HPA Axis (22 abstracts)
1Lady Ridgeway Hospital for Children, Colombo, Sri Lanka. 2University of Colombo, Colombo, Sri Lanka
Background: Virilizing congenital adrenal hyperplasia (CAH) is a complex disorder which requires a multidisciplinary approach. Exact timing of genitoplasty in children with virilization varies on the available resources, patients’ perspectives, and cultural influences. This study is aim ed to assess surgical interventions based on the degree of virilization in girls with congenital adrenal hyperplasia from a single center.
Methods: Nineteen patients attending routine endocrine and surgical clinics were analyzed retrospectively using medical records. The indications, timing, anatomy, surgical interventions, early and late complications, and parental satisfactions were studied.
Results: There were 16 patients with classic CAH. 12 had salt wasting and 04 were simple virilizing CAH. One had non-classic CAH. The degree of external virilization was assessed using Prader scale. There were 11 (57%) patients with Prader stage III while 5 (26%) had stage IV and 3 were stage II (16%). The preoperative anatomical assessment was performed in all patients using ultrasonography, examination under anesthesia and lower genito-urinary endoscopy. One patient had high vaginal- urethral confluence. 18 patients underwent feminizing genitoplasty while surgery was deferred in one patient due to male gender identity at 3-years of age. Age at surgery ranged from 14 months to 13 years. 17 patients had surgery at the mean age of 40 months. All Prader scale IV patients needed clitoroplasty, vaginoplasty and labioplasty. Out of the Prader stage III patients one did not need clitoroplasty, others underwent all 3 components. Stage II patients underwent selective components of the procedure depending on the anatomy. No postoperative complications like urinary tract infections, vaginal strictures, or bleeding were noted. All patients underwent subsequent EUA and vaginal calibration. No vaginal scaring was noted. None needed additional major revision of anatomy or redo surgery. The parental satisfaction on the cosmetic and the functional outcome of the surgery assessed verbally revealed satisfactory outcome.
Conclusion: Early surgical intervention for girls with severe degree of virilization due to congenital adrenal hyperplasia has resulted in good anatomical outcomes without significant increase in the complication rates. Assessment of patients’ perspective on the outcome will be studied comparing the cohort who did not undergo surgical intervention.