Herlyn-Werner-Wunderlich syndrome

Clinical History

17-year-old female virgin presented with recurrent lower abdominal pain related to menstruation.

Imaging Findings

Ultrasound revealed:
Haematometrocolpos with suspected uterine anomalies. Non-visualized left kidney at its anatomical position.

CT and MRI findings:
Didelphys uterus with haematometrocolpos, likely sequelae of obstructed/blind hemivagina with associated left renal agenesis.

Discussion

Background:
Herlyn-Werner-Wunderlich syndrome is a very rare cause of abdominal pain in young women. A triad of uterus didelphys, unilateral hemivagina obstruction due to a longitudinal septum and ipsilateral renal agenesis is diagnostic [1].
This syndrome is a combination of Müllerian duct anomalies and mesonephric duct anomalies. The incidence of didelphys uterus, related to HWW, is approximately 1/2000 to 1/28000. Mullerian duct anomalies have an incidence of 2–3% while HWW constitutes 0.16–10% of these Mullerian duct anomalies [1].
The complications of this syndrome can be classified as acute complications, such as pelvic pain due to haematocolpos / haematometra, pyohematocolpos and pyosalpinx as well as chronic or long-term complications such as endometriosis due to reflux form the fallopian tube, pelvic adhesions and increased risk of abortion or infertility [2, 3].
Clinical perspective:
Herlyn–Werner–Wunderlich syndrome (HWWS) is often a misdiagnosed entity. It usually presents at puberty with pelvic pain but rarely can present in adulthood in the form of pyometra, ischiorectal swelling, urinary obstruction, and primary infertility.
Imaging perspective:
Ultrasound is a helpful tool in the diagnosis of Müllerian duct anomalies by detection of haematocolpos, which appears as a pelvic fluid collection with low-level echoes which can make the diagnosis of genito-urinary tract anomaly easier, however, MRI is more sensitive in detecting the uterine contour, the shape of the intrauterine cavity, the character of the septum, as well as associated finding like endometriosis, pelvic inflammation and adhesions.
Outcome:
The patient underwent both laparoscopy and minilaparotomy with drainage of haematocolpos.
Teaching points:
-The diagnosis of HWW syndrome is always difficult due to the rarity of this syndrome, and a high index of suspicion is required as well as the knowledge of types of congenital anomalies associated . Early detection is important as immediate surgical intervention can provide pain relief and prevent further complications [4, 5].
- HWW syndrome should be suspected as cause of lower abdominal pain around the age of puberty with or without palpable lower abdominal mass or when imaging findings revealed unilateral haematocolpos and/or haematometrocolpos in didelphys uterus associated with ipsilateral renal agenesis.

Differential Diagnosis List

Final Diagnosis

Herlyn-Werner-Wunderlich syndrome

URL:	https://www.eurorad.org/case/11699
DOI:	10.1594/EURORAD/CASE.11699
ISSN:	1563-4086