Herlyn-Werner-Wunderlich syndrome: A case report in a young woman, with literature review

Abstract

Herlyn-Werner-Wunderlich syndrome is a rare complex congenital disorder, with combined Müllerian and mesonephric duct anomalies, presenting with uterus didelphys, unilateral blind hemivagina and ipsilateral renal agenesis. Hemivaginal obstruction usually leads to impairment of normal menstrual flow, resulting in symptoms after menarche, namely dysmenorrhea, pelvic pain or infertility. Age of presentation depends on the anatomical features of this anomaly. We report a case of a 21-year-old female presenting with few symptoms and incidental findings on transvaginal ultrasound, with typical findings of this disorder on magnetic resonance imaging, which remains the gold standard imaging technique for thorough assessment of Herlyn-Werner-Wunderlich syndrome, allowing for a correct diagnosis and adequate surgical management. Our case also highlights some unusual features, such as the presence of a blind ectopic ureter, with hematic content, and an incomplete septum within the obstructed hemivagina.

Keywords: Hematocolpos; Herlyn-Werner-Wunderlich syndrome; Magnetic resonance; OHVIRA; Renal agenesis; Uterus didelphys.

Fig. 1

T2-weighted images showing two separate uterine bodies, widely splayed on axial view (A) and two cervices on coronal view (B) - uterus didelphys – as well as agenesis of right kidney with compensatory hypertrophy of left kidney (C).

Fig. 2

T2-weighted images revealing two cystic lesions on the anterior aspect of the vagina (stars) on sagittal view, corresponding to the obstructed anterior hemivagina, with an incomplete transverse septum inside (A), communicating with the right uterine cervix on coronal view (arrow) (B); T1-weighted axial image with fat-suppresion showing high sinal of the anterior hemivagina, concurring with hematocolpos (C).

Fig. 3

T2-weighted sagittal (A) and axial (B) images after administration of vaginal gel, delineating the abnormal anatomy: the obstructed anterior hemivagina, with T2 “shading,” and the unobstructed posterior hemivagina, distended with gel.

Fig. 4

T2-weighted coronal image showing a small kinked tubular structure (arrows) adjacent to the obstructed hemivagina, corresponding to a blind right ectopic ureter (A); T1-weighted axial image with fat suppression shows the high T1 signal of the ureter (arrow), due to hematic reflux from hematocolpos.

Fig. 5

Illustration of our case of HWWS: uterus didelphys, unilateral blind right hemivagina and ipsilateral renal agenesis. The right hemivagina presents an inner incomplete transverse septum. An ectopic right ureter with reflux of hematic content is also seen.