Elective Reconstruction for Children With Congenital Adrenal Hyperplasia: Evaluating Association of Familial...

Improved patient/family education and shared decision-making (SDM) for congenital adrenal hyperplasia (CAH) is needed. We aimed to evaluate patient/family characteristics and management choices since implementation of an SDM tool for CAH.

CAH patients at a single institution from 2014 to 2024 were provided an SDM tool for management options. Families chose elective reconstruction of the clitoris and/or urogenital sinus (UGS) or only medically necessary procedures. Patients deferring surgery are followed for voiding difficulty, undergoing cystoscopy/vaginoscopy and imaging when indicated. UGS repair with introitoplasty is offered when obstruction is noted. Variables examined included race/ethnicity, procedures performed, clitoris and UGS anatomy, difficulty voiding, and imaging.

Sixteen patients fell into four categories: (1) full elective reconstruction (n = 4); (2) partial elective reconstruction (n = 2); (3) partial medically necessary reconstruction (n = 4) and; (4) deferral of reconstruction (n = 6). Common channel length of patients deferring surgery trended shorter without reaching statistical significance. When collapsed into non-Hispanic White versus persons of color, non-Hispanic White families were more likely to choose elective reconstruction versus delayed surgery (100% vs 0%, P = 0.001). Age in months at procedure was lower for elective reconstruction versus delayed reconstruction (8 versus 38, P = 0.05). Insurance type did not differ between groups.

Families of children with CAH who use an SDM process, combined with monitoring for voiding dysfunction, can choose to defer all or some reconstruction. Long-term follow-up and cultural and psychosocial factors influencing decision-making need to be investigated to ensure delivery of equitable care.