De Castro neophaloplasty for children with congenital aphalia: a new approach for a complex dilemma

Abstract

Purpose Congenital aphalia is a rare but complex male genital abnormality. The preferred approach in the past was female gender reassignment. However new concepts in ambiguous genitalia have questioned this strategy and opened the possibility for a cosmetic repair in childhood and future prosthesis implantation at puberty. We present a video describing a 12 year old boy with congenital aphalia whose posterior urethra emerged in distal rectum closed to the linea pectinea. Material and methods With the patient in the prone position an ASTRA approach enabled careful urethral isolation from the distal rectum. The patient was repositioned in the supine position and the urethral stump was left in the perineal area close to the scrotum. A skin flap was produced from the inferior abdominal wall keeping its vascularization in the suprapubic area. The flap was rotated caudally, partially defatted and prepared for tubularization in order to produce a phallic appearance. A 16cm buccal mucosa graft was obtained from the lower lip and cheek and tubularized over a urethral catheter. The neourethra was anastomosed to the posterior urethra and the distal part of the neourethra was anchored in the flap. The neophallus was completed by creating a “coronal-like” line. Results The patient had nice cosmetic healing of the penis but developed a perineal urethral fistula and stricture and awaits clinical revision. The psychological impact of the surgery for the patient was extremely favourable and successful. Conclusions The De Castro neophaloplasty is a feasible alternative for congenital aphalia and should be considered in these complex cases. Longer follow-up and larger experience with the technique is desired and therefore multi-institutional cooperative work should be encouraged.