K. Heller1, K. Van Leeuwen2, G. Madura3, D. Salevitz4, J. Coronado2, R. Chawla5, E. Hansen2, G. Grimsby6, E.M. Weidler2 1Phoenix Children’s Hospital, Department Of Surgery, Phoenix, AZ, USA 2Phoenix Children’s Hospital, Department Of Pediatric Surgery, Phoenix, AZ, USA 3Mayo Clinic In Arizona, Alix School Of Medicine, Phoenix, AZ, USA 4Mayo Clinic In Arizona, Department Of Urology, Phoenix, AZ, USA 5Phoenix Children’s Hospital, Department Of Pediatric Endocrinology, Phoenix, AZ, USA 6Phoenix Children’s Hospital, Department Of Pediatric Urology, Phoenix, AZ, USA
Introduction: Controversy surrounding genital reconstruction for patients with congenital adrenal hyperplasia (CAH) has resulted in strategies aimed at improving the education and consent process for families of infants. This study reports on the choices caregivers have made since implementation of a shared decision-making tool for CAH.
Methods: All patients with CAH at a single institution were enrolled in a prospective study since 2008. A shared decision-making tool was implemented in 2015 including sections on knowledge of condition, surgical controversies, what families should be asking their providers, and surgical expectations. Families either choose elective reconstruction of the clitoris and/or urogenital sinus (UGS) or only medically necessary procedures aimed at improving voiding function. Patients who defer reconstruction are followed closely for voiding difficulty and, when present, undergo cystoscopy/vaginoscopy and voiding cystourethrogram. If there is evidence of obstruction, an introitoplasty with repair of UGS is performed. Variables examined included race/ethnicity, procedures performed, anatomy of the clitoris and UGS, and difficulty voiding.
Results: Sixteen patients were included. Patients fell into four categories: 1) Full elective reconstruction including vaginoplasty and clitoroplasty (n=4); 2) Partial elective UGS repair only (n=2); 3) Partial medically necessary UGS repair only (n=4) and; 4) Deferral of any reconstruction (n=6). Length of the common channel was not available in all patients but, in general, patients with a shorter common channel opted for deferral or UGS repair alone compared to patients with longer common channel. Families who chose elective reconstruction more often identified as non-Hispanic white compared to families who chose observation or medically necessary procedures (Table 1). When collapsing the racial/ethnic groups into non-Hispanic white versus persons of color (POC), there is a statistically significant difference when choosing elective reconstruction versus choosing to delay surgery (p = 0.001). Age at procedure was statistically significant when comparing elective vs delayed reconstruction (p = 0.05). Insurance type (private vs public) and common channel length were not statistically significant between the two groups.
Conclusion: Families of infants with CAH who use a shared decision-making process, combined with objective measurement of voiding dysfunction, can choose to defer all or some perineal reconstruction. Continued long-term follow up of this cohort can further inform families in terms of timing and need for procedures. Cultural and psychosocial factors influencing decision-making need to be investigated further to ensure delivery of equitable care.