Congenital adrenal hyperplasia: Does repair after two years of age have a worse outcome?

Abstract

Introduction: Congenital Adrenal Hyperplasia (CAH) is the commonest cause of disorders of sex development (DSD) in children. The timing of surgery, early versus late, is a subject of current debate. We hypothesize that surgery for congenital adrenal hyperplasia after age two results in a worse outcome than procedures performed earlier in the neonatal period."

Patients and methods: Retrospectively evaluated children underwent feminizing genitoplasty the period from 2003 to 2015. Sixty-one children included in the study. They were divided into two groups; Group I: those repaired before 2 years of age (early repair), Group II: those repaired after 2 years of age (late repair). We compare both groups as regards the timing, stages of the genitoplasty, genital anatomical assessment, overall cosmetic results and further treatment recommendations.

Results: Group I: included 35 children with mean age at presentation 1.73 ± 2.27months (3 days-10.0 months) group II: included 26 children with mean age at presentation 18.78 ± 32.25 months (3 days-150.0 months). 88.5% of children were operated in single stage. Overall cosmetic outcome is good in 94.3% in group I versus 19.2% in group II (p < .001), satisfactory in 5.7% in group I versus 53.8% in group II (p < .001), poor in 0% of group I versus 26.9% in group II (p = .002). 94.3% of children in group I needed no further surgeries versus 26.9% of group II (p < .001).

Discussion: a current unsolved debate is when to perform the feminizing genitoplasty in children with congenital adrenal hyperplasia (CAH), some are pushing to wait till puberty and others are advocating early reconstruction. To take this debate a further forward, we studied retrospectively our operated children and stratified them according to age into below 2 years and after 2 years of age and we found that earlier (before 2 years of age) is better then late (above 2 years of age) repair.

Conclusions: Better anatomical findings were significantly observed in patients with early surgical intervention (before the age of 2 years).

Keywords: Adrenogenital syndrome; Congenital adrenal hyperplasia; Long term follow up.