The unilateral streak gonad syndrome (Slotnick-Goldfarb syndrome)

Two new cases of unilateral streak gonad syndrome are presented. The review of the literature makes it possible to clearly delineate the anatomical, endocrinological and genetic aspects of this syndrome, characterized by secondary amenorrhea, sterility, a streak gonad in the left adnexa and a hypoplastic ovary in the other, while uterus and tubes are normal. Particular attention is devoted to the etiopathogenesis of the syndrome, as well as to the problem of spontaneous sexual development and ovarian function in the streak gonad syndrome.