Folch M, Pigem I, Konje J C
Department of Obstetrics and Gynaecology, Leicester Royal Infirmary, England, UK.
Obstet Gynecol Surv. 2000 Oct;55(10):644-9. doi: 10.1097/00006254-200010000-00023.
Mullerian agenesis, a congenital malformation of the genital tract is the second most common cause of primary amenorrhea. Its etiology is poorly understood but it may be associated with renal, skeletal, and other abnormalities. The diagnosis is often made either radiologically or laparoscopically in patients in whom hormonal and karyotypic investigations for primary amenorrhea are normal. Two-dimensional ultrasound is not a reliable method of diagnosis, as exemplified by the two cases presented in this review; however, three-dimensional ultrasound may be a more sensitive diagnostic tool. The management is varied, but we conclude that the treatment of choice should be a nonsurgical approach aimed at creating a neovagina. Because of the implications for reproduction, these patients require psychological support, which should be offered as part of therapy.
Gynecologists and Family Physicians
After completion of this article, the reader will be able to describe the pathophysiology and clinical presentation of mülerian agenesis and to list the other abnormalities associated with this condition and to outline potential treatment options for a patient with müllerian agenesis.
苗勒管发育不全是一种生殖道先天性畸形，是原发性闭经的第二大常见原因。其病因尚不清楚，但可能与肾脏、骨骼及其他异常有关。对于原发性闭经患者，若激素和染色体检查正常，诊断通常通过放射学或腹腔镜检查做出。二维超声不是一种可靠的诊断方法，本综述中的两个病例就是例证；然而，三维超声可能是一种更敏感的诊断工具。治疗方法多样，但我们得出的结论是，首选治疗应是非手术方法，旨在创建一个新阴道。鉴于对生殖的影响，这些患者需要心理支持，应作为治疗的一部分提供。
妇科医生和家庭医生
读完本文后，读者将能够描述苗勒管发育不全的病理生理学和临床表现，列出与此病症相关的其他异常情况，并概述苗勒管发育不全患者的潜在治疗选择。
