Herlyn-Werner-Wunderlich syndrome complicated with pyocolpos: An unusual cause of postabortal sepsis

Abstract

Obstructive mullerian anomalies give rise to a spectrum of clinical presentations and are uncommon in routine gynecologic practice. The patient usually becomes symptomatic in early reproductive years. Recurrent pelvic pain, dysmenorrhea, enlarging abdominopelvic mass, and abnormal vaginal discharge are the common presenting symptoms. We describe a rare case of a mullerian anomaly getting diagnosed 13 years after attaining menarche during the evaluation of postabortal sepsis. Patient presented 2 weeks following evacuation carried out for missed abortion, with acute abdominal pain, fever and foul smelling discharge per vaginum. The anomaly was identified as uterus didelphys with obstructed left hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) complicated by pyocolpos. She was successfully managed by single-stage transvaginal septum resection under laparoscopic control.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with septic shock: A case report

“…OHVIRA syndrome presenting with acute abdomen has been reported in the literature. 3 Sharma et al reported OHVIRA syndrome with sepsis, 8 but the septic complication in this case was a result of surgical treatment of miscarriage, not the natural course of disease. OHVIRA syndrome is associated with a favorable prognosis if proper diagnosis is made at a young age and appropriate treatment is provided, but if the diagnosis is incorrect, serious complications can occur.…”

OHVIRA syndrome with Orofacial clefts: a rare association

“…9,15 Sharma et al, reported OHVIRA syndrome with sepsis the index case is associated with orofacial defect which has not been reported in literature so far. 16 The case was diagnosed early and manged promptly leading to reestablishment of regular painless menstrual cycles.…”

Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome

“…Herlyn-Werner-Wunderlich syndrome is extremely rare. To our knowledge, there are five cases of pregnancies associated to HWWS, [12][13][14][15] seven cases of HWWS with pyocolpos, [16][17][18][19][20][21][22] one case of pregnancy in HWWS with pyocolpos, 23 and two cases of pregnancy with pyocolpos in a uterus didelphys. 24,25 According to the European Society of Human Reproduction and Embryology (ESHRE) and the European Society for Gynecological Endoscopy (ESGE) 26 consensus on the Classification of Female Genital tract congenital anomalies, HWWS appears to be included in Class U3B uterine anomaly, class C2 cervix anomaly, and class V3 vaginal anomaly.…”