A Rare Cause of Abdominal Pain: Herlyn-Werner-Wunderlich Syndrome

Abstract

A Rare Cause of Abdominal Pain: Herlyn-Werner-Wunderlich Syndrome Abstract Herlyn-Werner-Wunderlich (HWW) syndrome is a congenital urogenital syndrome. Typical features are unilateral blind-ending hemivagina with uterine didelphy and ipsilateral renal agenesis. Patients usually progress asymptomatically until menarche, and they are diagnosed with radiological imaging by applying to the hospital with these conditions such as abdominal pain, abdominal bloating, primary amenorrhea along with menarche. In this case report, it is aimed to present a patient who applied to our emergency department with abdominal pain and is diagnosed with HWW syndrome. In this case, it is emphasized that emergency physicians should regard to HWW syndrome in the differential diagnosis of abdominal pain. Keywords: Herlyn-Werner-Wunderlich, Müllerian Anomaly, Abdominal Pain Introduction Abdominal pain constitutes an important part of emergency admissions. It has been observed that 40% of these not complying with the known symptoms and concluded any clear and understandable outcome and come up with a spontaneous sight (1). It is very difficult to determine the prevalence and incidence of uterine anomalies due to the use of different classifications, diagnosis with different methods and the absence of symptoms of many anomalies. On the other hand, in all studies conducted between 1950 and 2007, it was determined as 6.7% in the general population and 7.3% in the infertile population and women with recurrent pregnancy loss, this rate varies between 3% and 25% (2). No matter how the exact factors that caused these anomalies are not clear, it has been determined in studies that ionizing radiation, infective processes and some medicine (Diethylstilbestrol (DES), Thalidomide, etc.) exposed during the genital development process causing Müllerian anomalies (3). Herlyn-Werner-Wunderlich (HWW) syndrome is among the uterine anomalies and important for early diagnosis of complications and permanency of fertility. Most of the patients consult with these complaints such as abdominal pain, abdominal mass, bloating, inability to menstruate and acute abdominal pain(4). Dysmenorrhea is the main symptom of HWW syndrome and it is usually established after puberty. The increase in pain is related to a rise in the volume of hematocolpus caused by an obstructed hemivagina. Endometriosis, inflammation, twisted cysts, and appendicitis are other differential causes of pelvic pain and these must be excluded. Although it is possible to interfere diagnoses with similar symptoms and there is not a typical examination finding, differential diagnosis can be easily made with scanning methods. Observing unilateral blind-ending hemivagina and uterus didelphy and ipsilateral renal agenesis on computed tomography (CT) or ultrasonography (USG) imaging performed on the patient is important for diagnosis(5). Making diagnosis in time makes it possible to prevent possible complications with further examination, treatment and surgical procedures depending on the condition of the anomaly (6). In this case, it is aimed to present the HWW Syndrome that emergency physicians detect in a 19-year-old female patient who applied to the emergency department with the abdominal pain. Case Report A 19-year-old female patient has consulted to the emergency department with the abdominal pain. On admission to the emergency service, it has been observed that the general health of the patient is good and conscious. First vital signs; blood pressure: 115/75 mmHg, saturation: 96%, heart rate: 88 beats/min, fever: 36.6oC.In physical examination; there is tenderness and defense in the right inguinal and hypogastric area of the abdomen. Advanced vaginal examination could not made to the patient in the emergency room, but it is learned that she has the virginity in this statement. No significant finding is detected in the patient's other systemic examination. In her background, it is learned that the patient has occasional abdominal pain, but not having consistent dysuria or dysmenorrhea. The patient has not applied to any health institution with this complaint beforehand. By closing the oral intake of the patient in the emergency room, IV hydration isstarted, and necessary laboratory tests are requested.In the test results; b-Hcg