Masculinizing genitoplasty in intersex patients

We reviewed cosmetic and functional outcome of masculinizing genitoplasty (MPG) in intersex patients assigned as a male performed in our service for more than 12 years.

A total of 57 patients underwent MPG in our department during a 20-year period. Of these cases MPG was performed using modern 1-stage surgical techniques from 1991 to 2003 in 39 (68%). Of the 39 children 8 (21%) had 17beta-hydroxysteroid dehydrogenase deficiency, 2 (5%) had 3beta-hydroxysteroid dehydrogenase deficiency, 8 (21%) had 5alpha-reductase deficiency, 2 (5%) had mixed gonadal dysgenesis, 1 (2.6%) was a true hermaphrodite, 1 (2.6%) had Klinefelter's syndrome, 1 (2.6%) had partially androgen insensitivity syndrome and 16 (41%) had idiopathic male pseudohermaphroditism. The presenting disease was severe proximal penoscrotal hypospadias in 30 cases (77%), perineal hypospadias in 9 (23%) and müllerian duct opening in the perineum along with the urethral meatus in 16 (28%). Median patient age at surgery was 1.8 years. MPG was performed with a transverse pedicled preputial island flap as an onlay in 29 cases (74%). The remaining 10 patients (26%) underwent tubularization of the mucosa in the perineal area and end-to-end anastomosis to a tube made from the pedicled prepuce. Scrotal transposition as well as orchiopexy was performed in some patients as an independent operation so as not to jeopardize the perineal and preputial flaps.

In 23 (59%) of the 39 children 1 operation achieved satisfactory cosmetic and functional results in terms of good urinary stream and straight phallus during erection. Three (7%) children presented with various degrees of breakdown of the urethroplasty and required a repeat operation. In 5 patients (12.8%) a small urethral fistula developed and closure was performed.

A 1-stage male genitoplasty for male pseudohermaphroditism is accompanied by a reasonable incidence of major complications. It should be performed in early childhood to avoid psychological and social anxiety by the child and parents.