Herlyn-Werner-Wunderlich syndrome: A report of three cases in adolescents and adult woman

Abstract

We report three cases with Herlyn-Werner-Wunderlich syndrome in adolescents and young female. The objective of this report was to describe the clinical presentation, ultrasound (US) and magnetic resonance imaging (MRI) findings of Herlyn-Werner-Wunderlich syndrome. The three patients were 12, 13 and 34 years old, respectively. The reason for admission and clinical symptoms varied between the 3 patients, including menstrual cramps, vaginal bleeding, and dull pain in the hypogastric region. Nevertheless, the sonographic and MRI findings of all three cases were typical for HWWS, including didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis.

Keywords: Diagnostic imaging; Hematometrocolpos; Herlyn-Werner-Wunderlich syndrome; Renal agenesis; Uterus didelphys.

Fig. 1

Illustration of Herlyn-Werner-Wunderlich syndrome with complete vaginal septum, didelphys, hematometra and hematocolpos on the right (type 1 according to China's classification).

Fig. 2

Patient 1, a 12-year-old girl. Transabdominal ultrasound showing uterus didelphys and left hematocolpos.

Fig. 3

Patient 1, a 12-year-old girl. Axial and Coronal T2-Weighted, (A) uterus didelphys, hematometra, and hematocolpos on the left (arrows), (B) ipsilateral kidney agenesis.

Fig. 4

Patient 2, a 13-year-old girl. Transabdominal ultrasound showing uterus didelphys and right hematocolpos.

Fig. 5

Patient 2, a 13-year-old girl. Coronal (A, B) and sagittal (C) T2-weighted magnetic resonance image (A, C). Two separate uterine cavities (A, arrows) with dilatation of right hemivagina. Absent right kidney (B, arrow). High signal intensity fluid in T2W and hyperintense content in T1-weighted fat-suppressed sequence (not shown) in line with blood products.

Fig. 6

Patient 3, a 34-year-old female. Coronal T2-Weighted, (A) didelphys, (B) left hematocolpos.

Fig. 7

The process of formation of the genitourinary system.