Herlyn-Werner-Wunderlich syndrome

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 (2^nd part) paramesonephric ducts and distal divergent (3^rd 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.