Importance of clinical examination in the recognition of Herlyn-Werner-Wunderlich syndrome: case report

Abstract

Introduction: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital disease of the Mullerian ducts, in which there was a didelphys uterus, hemivagina obstructed by the septum, and ipsilateral renal agenesis. The most common clinical presentation is progressive pelvic pain, dysmenorrhea, and a palpable mass. In some cases, the patient may have normal menstruation due to a hemivagina obstruction, which ends up delaying the diagnosis. Case Report: Female patient, 13 years old, admitted with complaints of pelvic pain, intermittent and progressive, a year ago, associated with menstrual irregularity. Performed a radiological propaedeutics and the initial gynecological physical examination was suppressed, the patient is misdiagnosed with ovarian cyst torsion and submitted to exploratory laparotomy. In the postoperative period, after reassessing the clinical history and undergoing a gynecological exam, an anatomical abnormality was observed in the region of the vaginal vestibule, compatible with the vaginal septum and hematocolpo. Colpotomy was performed with drainage of approximately 900 ml of chocolate-like blood and marsupialization of the vaginal septum. Conclusions: The importance of clinical examination for early diagnostic completion and to avoid unnecessary surgical procedures is noted, especially in the management of rare and intriguing cases such as HWWS.