Herlyn-Werner-Wunderlich syndrome

Case contributed by Dr Suresh Babu Boddapati

Presentation

Complaints of cyclical lower abdominal pain associated with menstruation.

Patient Data

Age: 20 years
Gender: Female
MRI

Pelvis

MRI pelvis shows duplication of uterus, cervix & vagina (uterus didelphys, bicollis) with right-sided hematocolpos and ipsilateral renal agenesis in abdominal screening sections. 

Bilateral ovaries are normal. 

Ultrasound

Abdomen and pelvis

Duplication of uterus, cervix & vagina with collection in right hemivagina and ipsilateral renal agenesis & compensatory enlargement of left kidney. 

Case Discussion

A classic case of OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly), also known as Herlyn-Werner-Wunderlich (HWW) syndrome

OHVIRA syndrome is a rare congenital anomaly characterized by uterine didelphys, obstructed hemivagina and ipsilateral renal anomaly. The given case shows duplication of uterus with two distinct uteri and cervices- classified as Class 3 according to Americal Fertility Society (AFS) classification 1 of uterine anomalies. It is classified as U3BC2V2 following the comprehensive new classification of ESHRE/ESGE 2.

  • U3b: Abnormal fundal outline, characterized by external indentation (at the fundal midline exceeding >50% of uterine wall thickness) completely dividing the uterine corpus up to the level of the cervix
  • C2: Two distinct cervices, could be either fully divided or partially fused
  • V2: Longitudinal obstructing vaginal septum

To fully understand the associations in OHVIRA syndrome, Acein proposed a theory 3 according to which fused paired (2nd part) paramesonephric ducts and distal divergent (3rd part)  paramesonephric ducts contribute to the formation of uterus and cervix. Here the development and positioning of the paired paramesonephric ducts are influenced by the mesonephric ducts.

Unlike the conventional theory, vagina develops from mesonephric duct, lined by cells from Mullerian tubercle.

  1. failure of development of ureteric bud from mesonephros leads to agenesis of ipsilateral ureter and kidney
  2. failed positioning of paramesonephric ducts results in two hemiuteri and two hemicervices
  3. arrest of development of ipsilateral mesonephric duct leads to failure of distal hemivagina to develop resulting in obstructed hemivagina

Hence the spectrum of anomalies in OHVIRA syndrome can be explained by failure of mesonephric duct induced Mullerian development 3.

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Case information

rID: 80160
Published: 15th Jul 2020
Last edited: 21st Jul 2020
Inclusion in quiz mode: Excluded
Institution: NRIIMS Visakhapatnam

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