Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Mullerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. PRESENTATION OF CASE: In this report, the authors present the case of a 12-year-old patient presenting with progressive and disabling abdominal pain after the onset of menarche diagnosed with HWWS, describe the various diagnostic modalities and treatment options available, along with a current review of the literature. DISCUSSION: With normal external genitalia, HWWS is usually asymptomatic until menarche when patients present with worsening abdominal pain during menses and a palpable pelvic or abdominal mass. Untreated, HWWS may lead to a number of complications including endometriosis, infertility, and spontaneous abortion. CONCLUSION: Greater awareness of HWWS will lead to earlier detection and is the key to alleviating patient suffering and avoiding potentially severe complications. (C) 2019 The Authors. Published by Elsevier Ltd on behalf of US Publishing Group Ltd.