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[梗阻性半阴道并同侧肾异常:阴道积脓的罕见病因。病例报告及文献复习]

[Obstructed hemivagina and ipsilateral renal anomaly: unusual cause of piocolpos. Report a case and review of literature ].

作者信息

Cortés-Contreras Diana Karen, Juárez-Cruz Patricio Manuel, Vázquez-Flores José, Vázquez-Flores Al David

出版信息

Ginecol Obstet Mex. 2014 Oct;82(10):711-5.

Abstract

OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) by acronym and abbreviations in English or Herlyn Werner Wunderlich syndrome is a rare congenital malformation caused by an alteration in the Mullerian ducts and Wolffian Ducts. Which is characterized by a triad: uterus didelphys, obstructed and ipsilateral renal agenesis hemivagina still uncertain etiology. Patients are usually asymptomatic until menarche where the most common clinical presentation is pelvic pain, followed by a vaginal or abdominal mass, normal menstrual periods, infertility, and vaginal discharge rarely appears. The case of a female patient of 15 years, nubile with chronic fetid vaginal discharge, initially diagnosed and treated as pelvic inflammatory disease occurs, however because it is an exceptional condition with the background of the patient, by complementary studies were conducted where pelvic ultrasound revealed pyocolpos and absence of left kidney, uterus didelphys, blind hemivagina by other imaging studies, where we could integrate Herlyn-Werner-Wunderlich syndrome. In conclusion, abnormalities in the development of the Miillerian ducts are difficult to diagnose early, so you must have the embryological knowledge, conduct thorough clinical assessment and detailed picture in whom the coridition is suspected to identify malformations coexisting urinary tract and vaginal defects with the importance of preserving reproductive success through appropriate planning of surgical approach, given that the fertility rate in these patients is comparable to the average.

摘要

OHVIRA(梗阻性半阴道和同侧肾异常)是英文首字母缩写词,或称为赫林·沃纳·温德利希综合征,是一种由苗勒管和中肾管改变引起的罕见先天性畸形。其特征为三联征:双子宫、梗阻性半阴道和同侧肾发育不全,病因尚不确定。患者通常在初潮前无症状,最常见的临床表现是盆腔疼痛,其次是阴道或腹部肿块、月经正常、不孕,很少出现阴道分泌物。一名15岁的青春期女性患者,有慢性恶臭阴道分泌物,最初被诊断并当作盆腔炎进行治疗,然而由于这是一种基于该患者背景的特殊情况,通过进行补充检查,盆腔超声显示积脓阴道以及左肾缺如,其他影像学检查显示双子宫、盲半阴道,据此我们可以诊断为赫林 - 沃纳 - 温德利希综合征。总之,苗勒管发育异常早期难以诊断,因此必须具备胚胎学知识,对疑似病例进行全面的临床评估和详细检查,以识别并存的泌尿系统畸形和阴道缺陷,鉴于这些患者的生育率与平均水平相当,通过适当规划手术方法来保留生殖成功率非常重要。

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