Long-term 10-year comparison of girls with congenital adrenal hyperplasia who underwent early and late feminizing genitoplasty

The appropriate time to perform feminizing genitoplasty in patients with congenital adrenal hyperplasia (CAH) with the 46, XX genotype is still debatable. The aim of our study was to evaluate the relationship between age at surgery and long-term surgical outcomes of patients who underwent feminizing genitoplasty.

We retrospectively analyzed 14 patients with CAH and 46, XX genotypes with feminizing genitoplasty (clitoroplasty + vaginoplasty) between 2005 and 2022. The patients were divided into two groups. Group 1 consisted of seven girls (n = 7/14) who have been operated before the age of 2 years. Group 2 consisted of seven girls (n = 7/14) who have been operated after the age of 2 years. The two groups are compared regarding anatomical assessments, overall cosmetic results, need for additional intervention using Creighton's criteria. Additionally, the cosmetical satisfaction of the patients/parents is questioned.

The mean age of the girls was 32.42 months (10–96 months) during operation time. The mean age of Group 1 patients (n = 7/14) who have been operated before the age of 2 years was 11.71 months (10–19 months). The mean age of Group 2 patients (n = 7/14) who have been operated after the age of 2 years was 53.14 months (36–96 months). The mean follow-up time was 10.57 years (3–18 years). There was no statistically significant difference between those operated on before and after two years in terms of anatomical assessments, overall cosmetic results and patient/parent satisfaction except the need for additional intervention (p = 0.049). In Group1 (operation age < 2 years old), five out of seven (71.42%) patients needed additional major surgery (four urogenital sinus re-mobilization, one redo-clitoroplasty). Those who received additional major surgery were the ones who were not satisfied. In Group 2 (operation age > 2 years old), two patients out of seven patients (28.57%) received major surgery (two redo-urethroplasties) and those patients were not satisfied. When patient/parent satisfaction was compared with additional surgical intervention, as expected, patients'/parents’ satisfaction increased as major surgical intervention decreased. This was statistically significant (p = 0.007). The main source of dissatisfaction was repeated surgery among the parents.

The possibility of this additional surgical intervention increases, and patient/parent satisfaction decreases in patients below the age of 2 years. The corrective surgeries can be differed until the gender identity of the patient matures and the patient autonomy in deciding whether this surgery should occur.