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赫林-韦尔纳-温德利希综合征的诊断与治疗:一例报告

Diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome: a case report.

作者信息

Wdowiarz Kinga, Skrajna Adrianna, Reinholz-Jaskólska Małgorzata

机构信息

Department of Obstetrics and Gynaecology, Praski Hospital, Warsaw, Poland.

出版信息

Prz Menopauzalny. 2021 Apr;20(1):52-56. doi: 10.5114/pm.2021.104034. Epub 2021 Mar 1.

Abstract

INTRODUCTION

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It is one of the rare anomalies of the genitourinary system and is usually identified in the first few years after menarche.

CASE REPORT

An 18-year-old patient presented with painful menses for 1 year and was suspected for vaginal haematoma. Symptoms appeared 5 years after menarche.

DISCUSSION

Magnetic resonance imaging of the pelvis revealed a congenital anomaly of the genital tract. Two steps of surgical management were performed. First, a hysteroscopic incision of the vaginal septa and evacuation of vaginal haematoma were performed. Subsequently, the vaginal septum was partially excised during laparohysteroscopy.

CONCLUSIONS

Ipsilateral renal agenesis was confirmed by ultrasound during hospitalization, and the diagnoses of obstructed hemivagina and ipsilateral renal anomaly syndrome were established.The aim of this study was to present the diagnostic and therapeutic approaches in HWW syndrome, and to draw attention to the challenges in its diagnosis, which can coexist with multiple complications.

摘要

引言

赫林-韦纳-温德利希(HWW)综合征是一种罕见的先天性异常,其特征为双子宫伴盲端半阴道和同侧肾缺如。它是泌尿生殖系统罕见的异常之一,通常在初潮后的头几年被发现。

病例报告

一名18岁患者因痛经1年就诊,怀疑为阴道血肿。症状在初潮后5年出现。

讨论

骨盆磁共振成像显示生殖道先天性异常。进行了两步手术治疗。首先,进行宫腔镜下阴道纵隔切开术及阴道血肿清除术。随后,在腹腔镜宫腔镜联合手术中部分切除阴道纵隔。

结论

住院期间超声证实同侧肾缺如,诊断为梗阻性半阴道和同侧肾异常综合征。本研究的目的是介绍HWW综合征的诊断和治疗方法,并提请注意其诊断中可能与多种并发症并存的挑战。

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