E2220. Herlyn-Werner-Wunderlich Syndrome: A Rare Case Report
  1. Deepa Dayanandan Susha; St. John's Medical College
  2. Soumya Cicilet; St. John's Medical College
We present a rare case of Mullerian duct anomaly- Herlyn Werner Wunderlich syndrome , also known as obstructed hemivagina and ipsilateral renal anomaly(OHVIRA) syndrome in a 15-year old girl who presented with severe pelvic pain in the month following her menarche. Early diagnosis was done by transabdominal sonography (TAS) and magnetic resonance imaging (MRI) wherein the typical findings of OHVIRA were confirmed and a complication of hematocolpus was identified and managed on an emergency basis.

Educational Goals / Teaching Points
Early diagnosis of OHVIRA is essential to avoid possible complications of the same. The most common complication encountered in a typical case of OHVIRA is hematocolpus as a result of obstructed hemivagina which often results in cyclical dysmenorrhea and persistent pelvic pain . The less common complications encountered are presence of dysplastic kidneys , ectopic ureters and recurrent miscarriages . The least and rare complication encountered is reported to be clear cell carcinoma of the cervix. MRI imaging aids in early diagnosis and management of the same.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
The most proposed theory of etiopathogenesis is considered to be due to the improper or variant fusion of the paired paramesonephric ducts resulting in complex uterine anomalies. TAS of the pelvis revealed imaging features of a uterus with didelphys configuration and endometrial thickening involving the bilateral uterine horns. There was evidence of a large cystic mass with internal echoes and absent internal vascularity within the right endocervical canal communicating with the right uterine horn. There was no evidence of any solid components/papillary projections within the cyst.The visualised bilateral ovaries were normal. Right kidney was not visualised in renal fossa with evidence of compensatory hypertrophy of the left kidney. Plain and contrast MRI performed on the following day confirmed the TAS findings with evidence of duplication of the uterus, cervix and vagina. The right endocervical canal was distended with contents of non-enhancing acute on chronic blood product signal intensity and was noted to communicate with the right uterine horn . The distal portion of the right endocervical canal revealed an obstructing septum .The distal right hemivagina, left endocervical canal and left hemivagina appeared collapsed.Bilateral ovaries were normal. Screening of abdomen showed absence of right kidney in right renal fossa with compensatory hypertrophy of the left kidney. A diagnosis of Herlyn Werner Wunderlich syndrome was made.

OHVIRA , being a rare anomaly was earlier diagnosed on the basis of invasive surgical interventions. The advent of MRI has aided in the early diagnosis and management , thus avoiding unnecessary surgical interventions and possible complications of the same.MRI further aids in early diagnosis of a rarely reported complication of clear cell cervical carcinoma .