男性半陰陽の3例と本邦文献の検討

概要

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Three male hermaphrodites, equivalent to group A-2a by Jones' classification, have been reported. Case 1: Presence of left testis in her left atrophic scrotum of a 4-year-old girl resulted in change of her legal sex. Phallus was small and external urethral orifice located in perineum; perineal hypospadias. Uterus, normal in size for his (her) age, and right testis were disclosed in intraabdominal cavity. Case 2: A 23-year-old male, complaining of bilateral vacant scrotum. Penis fully developed without hypospadias. Laparotomy revealed well developed uterus and bilateral testes intra-abdominally. Case 3: At the time of herniotomy a 19-year-old male was found to have uterus in right inguinal canal, right testis in right scrotum and left one in abdominal space, close to the entrance of right inguinal canal, so-called hernia uteri inguinalis. In these 3 cases sex chromatin was negative and karyotype was 46XY. The following hypotheses have been presented for the etiology of male hermaphroditism. 1) Reduced or ineffective mullerian suppressing hormone secretion. 2) Diminished response of mullerian duct to mullerian suppressing hormone. 3) Malfunction of fetal testes during the period of the critical stage, 50th to 60th day in human embryo. Statistical analysis has been attempted on 47 cases, 44 cases being reported in 3 major Japanese journals related to urology over the past 16 years (1959 to 1974).