Herlyn-Werner-Wunderlich Syndrome with Unilateral Hemivaginal Obstruction, Ipsilateral Renal Agenesis, and Contralateral Renal Thin GBM Disease: A Case Report with Radiological Follow Up

vagina is a rare disease entity that is often accompanied by ipsilateral renal agenesis. It was recognized as early as 1922 (1) and is sometimes referred to as HerlynWerner-Wunderlich (HWW) syndrome (2, 3). The association of renal agenesis with ipsilateral blind hemivagina was reported as Herlyn-Werner syndrome in 1971 (4), while the association of renal aplasia, bicornuate uterus with isolated hematocervix, and a simple vagina was reported by Wunderlich in 1976 (5). This complex of anomalies is observed in adolescents and young women with progressive dysmenorrhea, abnormal pain, menstrual irregularities and a pelvic mass. Early and accurate diagnosis of this entity is important, and a resection of the obstructing vaginal septum can provide pain relief and prevent further complications (6, 7). We report the initial and follow-up imaging findings of a case of HWW syndrome associated with thin glomerular basement membrane disease of the contralateral kidney, which to our knowledge is the first radiological report in the domestic literature.