Spence J E
Department of Obstetrics and Gynecology, Children's Hospital of Eastern Ontario, University of Ottawa, Canada.
J Pediatr Adolesc Gynecol. 1998 Feb;11(1):3-11. doi: 10.1016/s1083-3188(98)70100-7.
Congenital malformations of the vagina, cervix, and uterus, although rare, may have profound implications for the young gynecological patient. These anomalies are often detected in the adolescent period. For proper management, the physician requires a thorough understanding of normal embryology and sexual differentiation. Although clinical experience helps the gynecologist appreciate the disturbed anatomic configurations, each and every individual who presents with a defect must be thoroughly evaluated because genital tract aberrations do not necessarily follow any defined and consistent pattern. Other anomalies often coexist, particularly related to the renal tract, so a thorough assessment is warranted. Genital malformations can be particularly disturbing to the patient and her family because they not only have reproductive implications but also significant psychological and sexual overtones that need to be addressed and dealt with in a sensitive and reassuring manner. This report is meant to provide an overview of the various abnormalities encountered and guide the clinician by providing an approach to management. A more indepth discussion is best found in the classic textbooks (Rock JA: Surgery for anomalies of the müllerian ducts. In: Te Linde's Operative Gynecology (8th ed). Edited by J Rock, J. Thompson. Philadelphia, Lippincott-Raven, 1997; Edmonds DK: Sexual development anomalies and their reconstruction: upper and lower tracts. In: Pediatric and Adolescent Gynecology. Edited by J Sanfilippo, D Muram, P Lee, J Dewhurst. Philadelphia, W.B. Saunders, 1994; Jones HW Jr: Reconstruction of congenital uterovaginal anomalies. In: Female Reproductive Surgery. Edited by J Rock, A Murphy, HW Jones Jr. Baltimore, Williams & Wilkins, 1992).
阴道、宫颈和子宫的先天性畸形虽然罕见,但可能对年轻的妇科患者产生深远影响。这些异常通常在青春期被发现。为了进行恰当的处理,医生需要全面了解正常的胚胎学和性分化。尽管临床经验有助于妇科医生认识到解剖结构的紊乱,但每一位出现缺陷的个体都必须进行全面评估,因为生殖道畸形不一定遵循任何明确且一致的模式。其他异常常常并存,尤其是与泌尿道相关的异常,因此有必要进行全面评估。生殖器畸形可能会让患者及其家人特别困扰,因为它们不仅对生殖有影响,还带有重大的心理和性方面的弦外之音,需要以敏感且令人安心的方式加以处理。本报告旨在概述所遇到的各种异常情况,并通过提供一种处理方法来指导临床医生。更深入的讨论最好在经典教科书中查找(罗克JA:苗勒管异常的手术治疗。见:《泰林德手术妇科》(第8版)。J·罗克、J·汤普森编辑。费城,利平科特 - 拉文出版社,1997年;埃德蒙兹DK:性发育异常及其重建:上下生殖道。见:《儿科与青少年妇科》。J·桑菲利波、D·穆拉姆、P·李、J·德赫斯特编辑。费城,W.B. 桑德斯出版社,1994年;琼斯HW Jr:先天性子宫阴道异常的重建。见:《女性生殖外科》。J·罗克、A·墨菲、HW·琼斯Jr编辑。巴尔的摩,威廉姆斯与威尔金斯出版社,1992年)。