Double uterus with a blind hemivagina and ipsilateral renal agenesis: Clinical variants in three adolescent women: Case reports and literature review

Abstract

Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis, Wunderlich-Herlyn-Werner syndrome (WHWS), is a complex congenital anomaly, with approximately 170 cases previously reported, affecting both miillerian and wolffian systems. Possibly due to an anomalous ovarian secretion of miillerian inhibitory factor, it consists of two müllerian hemiapparati, one of which is occluded, and unilateral renal agenesis (mostly on the obstructed side). Four main variants are known: complete hemivaginal obstruction, incomplete hemivaginal obstruction, complete hemivaginal obstruction with laterally communicating hemiuteri, and complete hemivaginal atresia. WHWS is mostly diagnosed in adolescents, with earlier and more severe symptoms when no communication exists between the hemiapparati. The authors report on three cases with different clinical presentations detected in adolescent girls. New etiologic insights, diagnostic procedures, and treatment are discussed. To preserve fertility, early diagnosis and conservative surgery are recommended.