Sleiman Z, Zreik T, Bitar R, Sheaib R, Al Bederi A, Tanos V
Lebanese American University, Department of Obstetrics and Gynecology, Beirut, Lebanon.
Tiba IVF center, Hilla, Irak.
Facts Views Vis Obgyn. 2017 Sep;9(3):167-170.
Müllerian malformations result from defective fusion of the Müllerian ducts during development of the female reproductive system. The least common form of these malformations is Herlyn-Werner-Wunderlich syndrome characterized by obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). The most common presentation of this syndrome is a mass secondary to hematocolpos, pain, and dysmenorrhea. Clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan. We report two cases of this syndrome, featuring two very rare clinical presentations: hematosalpinx and pyocolpos. The clinical course of the pathology is not standard and each patient is treated accordingly.
苗勒管畸形是女性生殖系统发育过程中苗勒管融合缺陷所致。这些畸形中最不常见的形式是赫林-韦纳-温德利希综合征，其特征为半阴道梗阻和同侧肾异常（OHVIRA）。该综合征最常见的表现是血性阴道积血、疼痛和痛经继发的肿块。临床诊断极具挑战性，需要影像学检查，其中超声和磁共振成像在诊断、分类及治疗方案制定中起着至关重要的作用。我们报告两例该综合征病例，其具有两种非常罕见的临床表现：输卵管积血和脓性阴道积血。该病理的临床病程不典型，需对每位患者进行相应治疗。
