Department of Paediatric Surgery and Urology, Cambridge University Addenbrooke's Hospital, Cambridge, UK.
Department of Pediatric Surgery, Aghia Sophia Children's Hospital, Athens, Greece.
Int J Gynaecol Obstet. 2023 Mar;160(3):762-770. doi: 10.1002/ijgo.14362. Epub 2022 Aug 12.
Obstructive vaginal and uterine anomalies including imperforate hymen, transverse vaginal septum, and vaginal and/or cervical atresia or aplasia, might rarely present in infancy or childhood with hydrocolpos and/or hydrometra but they usually go unrecognized until presentation with amenorrhea and hematocolpos and/or hematometra in puberty. They should always be included in the differential diagnosis of a suprapubic and/or introital mass; in the latter case, vaginal vascular malformations and vaginal tumors should also be considered. Uterovaginal aplasia typically manifests with amenorrhea in puberty and needs to be differentiated from complete androgen insensitivity syndrome and gonadal dysgenesis of genetic males. Uterine fusion anomalies usually present with fertility and/or obstetrical complications in adulthood. However, a unicornuate uterus with a blind rudimentary contralateral horn containing functioning endometrium, and didelphys or septate uterus with a deviating obstructive septum might present in childhood or puberty with sequelae related with secretions or menstrual retention. This review provides a collective account of the most clinically important information about vaginal and uterine anomalies in childhood and adolescence for clinicians involved in the care of young females with the aim to provide guidance in appropriate evaluation and management.
阴道和子宫梗阻性异常包括处女膜闭锁、阴道横隔、阴道和/或宫颈闭锁或发育不全,在婴儿期或儿童期可能很少表现为积水性阴道和/或积水性子宫,但通常在青春期出现闭经、积血性阴道和/或积血性子宫时才被发现。它们在鉴别耻骨上和/或阴道入口肿块的病因时应始终被纳入考虑;在后一种情况下,还应考虑阴道血管畸形和阴道肿瘤。子宫阴道发育不全通常在青春期出现闭经,需要与完全雄激素不敏感综合征和遗传男性的性腺发育不良相鉴别。子宫融合异常通常在成年后出现生育和/或产科并发症。然而,单侧子宫伴盲的、发育不全的对侧残角含有功能性子宫内膜,以及双子宫或纵隔子宫伴偏离的梗阻性纵隔,可能在儿童期或青春期因分泌物或月经潴留而出现相关后遗症。本文综述了与儿童和青少年阴道和子宫异常相关的最具临床意义的信息,为涉及年轻女性护理的临床医生提供了指导,旨在帮助他们进行适当的评估和管理。