Herlyn-Werner-Wunderlich Syndrome: Sonographic and Magnetic Resonance (MR) Imaging Findings of This Rare Urogenital Anomaly

Abstract

Background: Herlyn-Werner-Wunderlich syndrome is a rare congenital urogenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Children usually have progressive pelvic pain after menarche, palpable mass due to hemihaemato(metro)colpos or pelvic inflammatory disease. The diagnosis usually requires a suspicion of this rare genitourinary syndrome.

Case reports: We present ultrasonography and MR imaging findings of this rare anomaly in two cases.

Conclusions: Early recognition of this rare syndrome can lead to an immediate, proper surgical intervention and is necessary to prevent complications and preserve future fertility. Ultrasound and MR imaging findings can collectively delineate uterine morphology, indicate the absence of ipsilateral kidney and show obstructed hemivagina.

Keywords: Magnetic Resonance Imaging; Ultrasonography, Doppler, Color; Urogenital Abnormalities.

Figure 1

A 13-year-old girl with pelvic pain and bad-smelling, green-coloured vaginal discharge, case I. On axial sonography at the level of the vaginas, (A) more to the left side (star), both hemivaginas are dilated and have fluid echoes. Axial T2W images at the level of uterus (B) and vaginas (C) show uterus didelphis and dilated right (arrow) and left (star) hemivaginas with slightly hypointense fluid contents consistent with haematocolpos. Note the tubular cystic structure in the wall of the left hemivagina (arrowhead).

Figure 2

A 15-year-old girl with dysmenorrhea, case II. On a sagittal view, the right kidney (A) was absent and the left kidney (B) was hypertrophic. Axial sonography image (C) demonstrates dilated left hemivagina with echogenic content (arrowhead) and normal right hemivagina (arrow). Axial T2W images at the level of uterus (D) and vaginas (E) show uterus didelphis and dilated left hemivagina with slightly hypointense fluid consistent with haematocolpos (star) and normal right hemivagina (arrow).