True hermaphroditism with bilateral ovotestis: a case report

Summary A true hermaphrodite with a 46 XX/47 XXY karyotype, gynaecomastia, hypospadia and scrotal gonads was investigated. Gonadectomy performed at 14 years of age revealed bilateral ovotestes. The ovarian portion contained follicles of all developmental stages. The testicular portion was immature consisting of seminiferous cords with Sertoli cells at various steps of differentiation and few germ cells within massive aggregates of collagenous connective tissue. Leydig cells as well as germ cells remained in an embryonic stage of development. Sections of a differentiated Wolffian duct (ductuli efferentes, epididymis, vas deferens) as well as of a Mullerian duct (hypoplastic fallopian tube) were found adjacent to both gonads. Postoperative treatment consisted of androgen substitution therapy leading to progression of puberty.