Posterior hypospadias: long-term followup after reconstructive surgery in the male direction.

Posterior hypospadias with a scrotal or perineal meatus results from a defect in step 3 of male sexual differentiation. The different etiological factors underlying this condition result in a broad spectrum of presentations ranging from the isolated form to complex ambiguity of the external genitalia, such as male pseudohermaphroditism. Between 1952 and 1988 a total of 92 patients with posterior hypospadias underwent a 2-stage reconstruction at our department. A retrospective study was performed with the aim of evaluating the long-term results in these patients. Our special interest focused on the functional and cosmetic results, exocrine and endocrine functions, as well as the sexual lives of the patients. While satisfactory results were obtained in two-thirds of the 42 male patients available for long-term followup, there were 13 patients who at followup still presented with complex sexual ambiguity. In 6 of these patients androgen receptor defects were detected by means of biochemical as well as molecular-biological investigations. Our data emphasize the importance of androgen metabolism for male sexual development and underline the necessity of careful evaluation in these children.