[46, XX true hermaphroditism: a case report with a review of 37 cases reported in Japan].

: The patient was a 17-year-old male who had undergone chordectomy at the age of 5 years. The patient visited us with the chief complaint of ectopic urethral opening and breast growth at the end of May, 1980. Urethrography revealed a well developed vagina and a unicordial uterine. The upper portion of bilateral gonads were cystic and nodular by palpation. Sex chromatin examination using buccal smear revealed X chromatin positive and Y chromatin negative. Chromosomal constitution was 46, XX karyotype and H-Y antigen was positive. Since the patient hoped to remain as male, he received gonadectomy, hysterectomy and implantation of thesis prosthesis into his scrotum. Histopathologically, bilateral gonads were ovotestes and neither vas nor fallopian tube was present. Postoperatively, he received supplement therapy with testosterone with successful results concerning his secondary sexual characters.