1972年Slotnick和Goldfarb首先报道5例单侧性索状卵巢综合征(unilateral streaked o- vary syndrome),后来文献又报道过2例,本文报告2例,对本征临床特点,内分泌检查、染色体核型分带等作了分析,并结合文献探讨其病因。病例1,27岁,因闭经和原发不孕4年就诊,儿童期无感染性疾病。14岁时阴毛和乳房发育,16岁月经初潮,但经量少,此后仅在使用雌、孕激素后才来潮。体检:身高169cm,体重54kg,乳房发育正常,皮肤和毛发生长和分布正常。妇科检查:阴道、子宫和附件正常。基础体温单相型,腹腔镜及染色输卵管镜检查(Chromosalpingoscopy)显示内生殖器正常,右侧卵巢发育不良,园而光滑,无排卵征象。左侧卵巢纤维索状,呈3～4× In 1972, Slotnick and Goldfarb first reported 5 cases of unilateral streaked o-lobe syndrome, and later reported 2 cases, this article reports 2 cases, the intrinsic clinical features, endocrine examination, chromosome karyotype Zonation and other analysis, combined with the literature to explore the etiology. Cases 1,27 years old, due to menopause and primary infertility 4 years of treatment, childhood non-infectious diseases. Pubic hair and breast development at 14 years of age, 16 years of menarche, but by the amount of less, only after the use of estrogen and progesterone before damp. Physical examination: height 169cm, weight 54kg, normal breast development, skin and hair growth and distribution of normal. Gynecological examination: the vagina, uterus and attachment are normal. Basic body temperature single-phase type, laparoscopy and staining Chromosalpingoscopy showed normal genitalia, right ovarian dysplasia, smooth park, anovulatory signs. Left ovarian fiber cord-like, was 3 ~ 4 ×