[Nerve-sparing, feminizing genital reconstruction to correct genital virilism: Experience of 25 children with adrenogenital syndrome].
The reconstruction of the virilized genitalia in females with adrenogenital syndrome (AGS) is carried out sparing the dorsal neurovascular bundle either through clitoral recession or reduction with simultaneous vaginoplasty and clitoroplasty. Within seven years, 25 girls aged 6 months to 16 years (mean 2 1/2 years) with AGS had a nerve-sparing genital reconstruction with resection of the clitoral shaft in one session as described by Praetorius. 23 children had a C21-defect and 1 a C11 beta-hydroxylase defect; one child had an external virilisation. All children were followed over an average period of 50 months: 21 had a good cosmetic result, the glans had a normal blood supply and was sensitive to a prick test; in 2 girls nerve integrity was demonstrated by somato-sensory evoked potentials. 2 children had small disturbing skin folds of the newly created labia and 2 girls had a somewhat scrotal appearance of the labia. The introitus caliber measured 14 F in girls operated before 6 years of age; however, a 14- and 16-year-old required intermittent bougienage.
Altwein
J E
JE
Urologische Abteilung, Universitätskinderklinik und Poliklinik Ulm.
Homoki
J
J
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D002987:Clitoris / Q000601:surgery*; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006801:Humans; D007223:Infant; D013518:Surgery, Plastic; D014844:Vulva / Q000098:blood supply / Q000294:innervation / Q000601:surgery
Nervenerhaltende, schaftresezierende Feminisierungsoperation zur Korrektur des virilisierten Genitales: Erfahrungsbericht über 25 Kinder mit adrenogenitalem Syndrom.