Combined Robotic and Perineal Vaginal Pull-Through for a Fully Virilized Congenital Adrenal Hyperplasia Female
Duncan R. Morhardt, MD, PhD, Briony Varda, MD, MPH, Richard N. Yu, MD, PhD, David A. Diamond, MD.
Boston Childrens Hospital, Boston, MA, USA.
BACKGROUND: Herein we present our initial reconstructive approach for a severely virilized (Prader V) 16-month-old 46XX classic congenital adrenal hyperplasia patient. Following an inconclusive genitogram, endoscopy revealed a high confluence of the vagina inserting into the verumontanum in the posterior urethra. After discussion with the family, who wished to begin reconstruction while preserving future options, an isolated robotic vaginal mobilization and pull-through were performed.
METHODS: Cystoscopy confirmed a high confluence. A 4 French (F) Fogarty catheter was placed into the vagina via the confluence. A 12mm camera port and two 8mm working ports were used. Dissection of the anterior and posterior vagina was performed with careful avoidance of the cervical and vaginal blood supply, ureters, and rectum. The distal vagina was transected and the posterior urethra closed with 6-0 PDS suture. A perineal dissection was then performed through an inverted U-shaped incision. The perineal flap was mobilized. A plane along the posterior urethra was developed. The previously mobilized vagina was delivered through the perineum with a laparoscopic grasper where it was approximated to the perineal flap. A 10F Foley catheter was left for 2 weeks for urethral healing. The patient was discharged on postoperative day two.
RESULTS: The robotic approach made for facile identification and dissection of the vagina with minimal blood loss. The confluence was readily apparent. Immediate postoperative results were encouraging.
CONCLUSIONS: This combined approach permits excellent visualization of and access to the vagina during dissection. It also facilitates posterior urethral closure with minimal blood loss and provides an alternative to the ASTRA procedure, which would required direct rectal access to the vagina.
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