A rare case of obstructed hemivagina with uterus didelphys and ipsilateral renal anomaly syndrome

Abstract

Obstructed hemivagina and ipsilateral renal anomaly syndrome, also known as Herlyn-Werner-Wunderlich syndrome, represents a rare congenital anomaly characterized by the presence of an obstructed hemivagina with uterus didelphys and concomitant ipsilateral renal abnormalities. Typically, the clinical presentation includes cyclical abdominal pain, vaginal discharge, and/or a vaginal mass occurring post-menarche. Accurate diagnosis requires a high index of suspicion among clinicians, coupled with a comprehensive understanding of the distinctive features associated with this anomaly. Herein, we present the case of a 13-year-old female patient who complained of persistent lower abdominal pain. Magnetic resonance imaging confirmed the diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome, and the patient was successfully treated with a laparotomy involving blood aspiration and hysterectomy. This case report highlights the significance of clinical awareness, prompt diagnosis, and timely therapeutic interventions to mitigate the adverse effects and optimize outcomes in individuals affected by obstructed hemivagina and ipsilateral renal anomaly syndrome.

Keywords: Congenital abnormalities; Müllerian anomalies; renal agenesis; uterine anomaly.

Figure 1.

Computerized tomography scan of the abdomen and pelvis displaying the absence of the right kidney (red circle).

Figure 2.

(a) Coronal IV contrast-enhanced image of the abdomen demonstrating the absence of the right kidney. (b) Selected T2 coronal images of the pelvis illustrating the presence of both ovaries: the right ovary (blue arrow) and the left ovary (green arrow). (c) Selected axial T1 fat-saturated magnetic resonance imaging with gadolinium highlighting two separate uterine bodies. The right uterus (green arrow) is associated with a bloody collection (hematometrocolpos—orange arrow), while the left uterine body is indicated by the yellow arrow.