真性半陰陽の2例 - 本邦報告100例の統計的考察 -

Abstract

Two cases of true hermaphroditism were presented. Case 1; A 14-year-old junior-high school boy was admitted for bilateral development of the breast. At birth the perineal hypospadias and bilateral undescended testes were noticed. When he was 2 and 3 years of age he had been operated for the hypospadias and undescended testes at a hospital. But as the operation [or the hypospadias was imperfect, he was re-operated by Denis Browne's method at our hospital when he was 4 years of age. Breast development started at 12 years of age. Routine laboratory examinations were mostly normal. Sex chromatin studied on buccal smear was positive and the chromosome constitution of 46XX/46XY was established by peripheral leucocyte culture. Bilateral ovotestes were revealed by exploration for scrotal contents and by microscopic examination. Epididymis and vas deferens were found also bilaterally, but uterus, vagina and Fallopian tube were absent. Ovarian portions were removed. Case 2; A 6-year-old child, who was brought up as a boy, was admitted because of the perineal hypospadias and no scrotal contents on the left side. He was operated on for the hypospadias by Denis-Browne's method because simply diagnosed as hypospadias. However, postoperative voiding cystourethrography showed fairly well developed vagina opened to the urethra. Routine laboratory examinations were normal. Sex chromatin was positive and chromosomal investigation showed a karyotype of 46XX. Through surgical and histological examination, it was made clear that he had an ovotestis on the right side of the scrotum and an ovary on the left inguinal canal each with Fallopian tube and fimbria. Uterus was absent. Right ovarian portion and left ovary were removed. 100 cases of true hermaphrodites reported in Japan were reviewed and briefly discussed.