Uncommon Laparoscopic Findings in a Sexually Immature Agonadal XY Phenotypic Female

A defect of intrauterine development occurring early in male embryogenesis results in a broad range of XY agonadal phenotypes. A 16-year-old girl was referred for evaluation of primary amenorrhea and failure of sexual development. Gynecological examination revealed infantile but otherwise normal female external genitalia with clitoral hypotrophy, absence of hymen, and a tight vaginal introitus. The vagina was 4 cm long and ended in a blind pouch. No masses were detected in inguinal canals or in labia majora. Ultrasonography, computerized tomography, and magnetic resonance imaging failed to demonstrate internal genitalia or gonads. Endocrine evaluation showed elevated plasma concentrations of Follicle Stimulating Hormone and Luteinizing Hormone with undetectable estradiol levels. The kariotype from cultures of peripheral leucocytes was 46, XY. At laparoscopy no uterus or ovarian structures were found. Only two small fibrous oval masses with a thin peduncle were found along each lateral pelvic wall and were excised. Histological examination of these masses revealed that they resembled oviduct tissue. Therefore, the present agonadal XY phenotypic female is the third of such cases that have been reported to have only rudiments of fallopian tubes but is the first of these three cases that has been explored entirely by laparoscopy.