Herlyn-Werner-Wunderlich Syndrome in a young female mimicking acute appendicitis

Abstract Objective Herlyn-Werner-Wunderlich (HWW) Syndrome, also known as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, is a very rare congenital urogenital tract anomaly. The incidence of the syndrome less than 1/1,000,000 females. OHVIRA radiologically presents Wunderlich triad: uterus didelphys, obstructed hemivagina ipsilateral renal agenesis. Clinical presentation progressive dysmenorrhea beginning with menarche. If unilateral agenesis diagnosed in female newborn, gynecological exams should be performed to exclude corresponding genital anomalies. We present here case mimicking acute appendicitis. Methods A 12-year-old recently menarched presented our emergency department lower abdominal pain nausea without vomiting. started two days earlier it was first such episode. abdomen tender right quadrant no alarming signs. WBC count CRP were normal. Abdominal ultrasound suggested appendicitis (without clear visualization appendix) revealed left Performed MRI confirmed uterine malformation 2 bodies, cervical uteri closed hemi-vagina hematocolpos. Results With diagnosis healthy appendix cause identified. patient hospitalized for one night control treatment oral contraception introduced suppress hormonal cycle. Since not well controlled returned later. Indication surgical made blood filled incised, drained marsupialised. Oral (Dienogest) prescribed. Further follow-up corrective surgery are planned. Conclusion may mislead physicians presuming other pathologies In young women presenting pain, taken into account. Ultrasound imagery choice, especially fertile women. Rapid complete necessary treat correctly prevent complications. Moreover, detected genitourinary malformations always lead further investigations concomitants pathologies.