Total vaginal replacement with sigmoid colon for partial testicular feminilizing syndrome in pediatric age

A 46XY patient, raised as girl, affected by androgen insensitivity syndrome with an incomplete receptor block (partial testicular feminilizing syndrom e), underwent a total vaginal reconstruction, bilateral orchiectomies and v ulvoplasty at the age of 11 years. At the time of the operation, the clitor is was mildly enlarged and a short urogenital sinus was present with a norm al urethral opening and a very small vaginal introitus. The vagina was tiny , blind-ending and useless and, obviously, no female internal genitalia wer e found. Two large hemiscrotums with full size testes were present. The pat ient has a normal female attitude. The vaginal replacement was performed us ing a segment of sigmoid colon. Eight days after the operation the patient and her mother were taught how to dilate and irrigate the neovagina once a day. At one month follow-up, the neovagina showed a good looking introitus, the mucorrea was significantly decreased, a size 22 Heger dilator was easi ly introduced inside the vagina and a vaginogram was satisfactory. Moreover , the two labia majora, made with hemiscrotum flaps, gave a normal appearan ce to the external genitalia. In conclusion, the preliminary result of this case confirms the feasibility of vaginal reconstruction in pediatric age, with advantage of early restoration of a normal genital anatomy in a one st age operation before the usual age of puberty. The sigmoid neovagina seems to be the procedure of choice for total vaginal replacement in children, fo r good cosmetic and hygienic results and for simple and temporary postopera tive management.