Gold M A, Schmidt R R, Parks N, Traum R E
Department of Obstetrics and Gynecology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
J Reprod Med. 1997 Jun;42(6):375-7.
Bilateral tuboovarian absence is extremely rare and is associated with infantile sexual development, primary amenorrhea and primary infertility.
A 23-year-old woman presented for evaluation of primary amenorrhea. Her examination revealed hypoplastic breasts, genitalia and uterus; ovaries could not be identified. Marked estrogen deficiency was confirmed by endocrinologic testing. The karyotype was normal female. The patient was started on combined hormone replacement therapy and subsequently developed normal menses; physical maturation progressed normally. At the age of 29 she underwent diagnostic laparoscopy for evaluation of her fertility potential, at which time the absence of both ovaries and distal fallopian tubes was confirmed.
Bilateral tuboovarian absence is an extremely rare cause of primary amenorrhea and is associated with infantile sexual development and primary infertility. Its etiology includes tuboovarian torsion and congenital malformation. In this case, congenital malformation appears to have been the more likely cause.
双侧输卵管卵巢缺如极为罕见，与幼稚型性发育、原发性闭经和原发性不孕相关。
一名23岁女性因原发性闭经前来评估。检查发现其乳房、生殖器和子宫发育不全；未发现卵巢。内分泌检查证实存在明显的雌激素缺乏。核型为正常女性。患者开始接受联合激素替代治疗，随后月经恢复正常；身体发育正常进展。29岁时，她接受了诊断性腹腔镜检查以评估其生育潜力，此时证实双侧卵巢和远端输卵管缺如。
双侧输卵管卵巢缺如是原发性闭经的极其罕见原因，与幼稚型性发育和原发性不孕相关。其病因包括输卵管卵巢扭转和先天性畸形。在本病例中，先天性畸形似乎是更可能的原因。
