Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since 1922. Only a handful of these cases have been associated with pyocolpos. Mullerian duct anomalies have an incidence of 2-3%. While OHVIRA constitutes 0.16-10% of these Mullerian duct anomalies. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and a pelvic mass. The pelvic mass is the collection of blood products within the obstructed hemivagina. The first study in the diagnostic work-up is usually ultrasonography, which typically demonstrates a pelvic fluid collection which can simulate other disease processes thus confounding the diagnosis. MRI findings of the pelvis reveal a didelphic uterus. Imaging of the abdomen reveals agenesis of the ipsilateral kidney. MRI is beneficial in characterizing the didelphic uterus and vaginal septum for pre-operative planning. Understanding the imaging findings, in conjunction with the clinical presentation, is critical for early diagnosis in attempting to prevent complications such as endometriosis or adhesions from chronic infections with subsequent infertility.

Keywords: Herlyn-Werner-Wunderlich syndrome; Mullerian duct anomalies; OHVIRA; didelphic uterus.

Figure 1

Diagram depicting OHVIRA. Didelphic uterus with left hemivaginal obstruction (LV), hydrometrocolpos of the left uterine horn (LU) and ipsilateral renal agensis. Occasional ectopic ureter (Ur) insertion into the obstructed hemivagina can occur.

Figure 2

17 year old female with OHVIRA and pyocolpos. Endovaginal longitudinal view of the uterus (8 MHz EV transducer, Phillips iU22) demonstrating an obstructed left hemivagina (arrow) with retained echogenic fluid. Uterus is also visualized in this image (arrowhead). Color Doppler demonstrates no flow in the collection.

Figure 3

17 year old female with OHVIRA and pyocolpos. Initial noncontrast coronal T2 FSE weighted sequence (3T Siemens, 5mm slice thickness, TR 3,800, TE 120) demonstrates a didelphic uterus with obstructed left uterine horn (large arrow) and left hemivagina (arrowhead). Note the right uterine horn (small arrow).

Figure 4

17 year old female with OHVIRA and pyocolpos. Coronal follow-up non-contrast enhanced T2 fat saturated MRI (3T Siemens, 5mm slice thickness, TR 3,822.8, TE 154) through the abdomen demonstrating absence of the left kidney (arrow) with reacumulation of fluid in the obstructed hemivagina status post IR drainage (arrowhead). Note the right uterine horn (small arrow).

Figure 5

17 year old female with OHVIRA and pyocolpos. AP fluoroscopic image (Siemens Artis Zee, kvp 70, mA 47) of post transvaginal drainage catheter placement into the infected hemivaginal fluid collection.

Figure 6

The American Fertility Society Classification Of Mullerian Anomalies: The spectrum of fusion anomalies. Reprinted with permission.