CASE 18209 Published on 14.06.2023

Herlyn-Werner-Wunderlich Syndrome:  A case report with US, MRI and TC findings


Genital (female) imaging

Case Type

Clinical Cases


Antonia Aulicino, Marco Dugo, Caterina Galzerano, Roberto Galeotti

Department of Radiology, Sant’Anna Hospital, Ferrara, Italy


19 years, female

Area of Interest Anatomy, Genital / Reproductive system female ; Imaging Technique CT, MR, Ultrasound
Clinical History

A 19-year-old woman presenting a severe abdominopelvic pain, nausea without vomiting, which started the week before during her monthly period as a severe dysmenorrhea, not responding to NSAIDs. The woman also referred malodorous vaginal discharge for one month, and recurrent episodes of dysmenorrhea. The patient denied any recent abdominal trauma.

Imaging Findings

The abdominal Ultrasound (US), performed at our emergency ward, revealed an undefined hypoechogenic left pelvic mass (Fig.1) close to a  dismorfic uterus, associated to a plausible absence of the left kidney.

The Computer Tomography (CT) scan detected a likely bicornuate uterus (Fig.2) and a pelvic mass suggestive of hematometra/hematocolpos (Fig.3); in addition, it confirmed a left renal agenesia with a compensatory hypertrophy of the right kidney (Fig.4).

The Magnetic Resonance Imaging (MRI) findings demonstrated a uterine-vaginal anomaly consisting in a dydelphys uterus (Fig.5), a double vagina with blind left hemivagina (Fig.6) and ipsilateral renal agenesis; consequently, it showed, fluid accumulation in the obstructed hemivagina consisting in a quite large mass with hyperemic edges; the fluid presented typical methaemoglobina signal intensity resulting in a diagnosis of hematocolpo (Fig.7.)

Moreover, the exam revealed some mild fluid in the pelvic pouch.


Herlyn-Werner-Wunderlich (HWW) Syndrome is a rare congenital malformation also known as OHVIRA-Syndrome, characterized by a didelphys uterus, a double vagina with one obstructed hemivagina, associated to an ipsilateral renal anomaly (most likely agenesia or severe atrophy); the exact aetiology and pathogenesis of HWW syndrome is still unknown[1], [2].

Several factors influence the female uro-genital growth, first of all the development of the paramesonephric (Müllerian) and mesonephric (Wolffian) ducts. The mesonefric ducts are inductors for a correct Müllerian duct fusion, moreover, they play a key role in kidneys organogenesis. A developmental anomaly of the caudal portion of one of the mesonephric ducts may result in a unilateral renal agenesis associated with an imperforate hemivagina. Since the mesonephric duct is absent, the ipsilateral Müllerian duct is laterally displaced, preventing the fusion with the contralateral duct, ending in a didelphic uterus. The spared contralateral Müllerian duct evolves in a regular vagina, while the displaced once evolves in a blind sac because of the distance from the urogenital sinus, resulting in an obstructed hemivagina.[3]

Various uterine anomalies may arise from an impaired fusion of the paramesonephric ducts. They have been classified into seven categories by the American Society of Reproductive Medicine[4] and according to this classification the HWWS features the III class of uterine anomaly.

This condition is often discovered during puberty, right after menarche. [4] The increased hematcolpo or hematometra, due to the longstanding menstrual blood in the hemivagina, results in a palpable pelvic mass and dysmenorrea.  [4], [5]

Several complications may arise from this syndrome, the acute ones are usually pyohematocolpos, pyosalpinx, or pelviperitonitis, wheras long-term complications may be endometriosis, pelvic adhesions and increased risk of abortion or infertility. [6]

Sometimes the correct diagnosis may be late because, despite a dilated obstructed hemivagina,  the menstrual fluid flows regularly from the unobstructed system. [4]

Actually, techniques like US and MRI are largely used to study the genito-urinary system, in particular the MRI examination is crucial in detecting anatomical anomalies. However, since 1981 laparoscopic exploration has been regarded as the gold standard. [7]

The common treatment of HWWS consists in the resection of almost all the septum in the obstructed vagina, if possible. Fertility is usually preserved through this surgical procedure even if sterility is a rare occurrence in women with didelphic uterus. This syndrome has the best prognosis among the Mullerian duct anomalies. [7], [8]

Differential Diagnosis List
Herlyn-Werner-Wunderlich Syndrome
Ovarian cancer
Pelvic inflammatory disease
Peritoneal neoformation
Final Diagnosis
Herlyn-Werner-Wunderlich Syndrome
Case information
DOI: 10.35100/eurorad/case.18209
ISSN: 1563-4086