An unusual presentation of a patient with OHVIRA syndrome: A case report

SMSG Gunarathna1, SB Uduwerella2, JA Gunsingha2, S Wijesingha3 , BAS Fernando4

                                                       1.Specialty Doctor, Wrexham Maelor Hospital, UK

                                                       2.Consultant Obstetrician and Gynaecologist, General Hospital, Matara.Sri Lanka

                                                       3.Consultant Radiologist, General Hospital, Matara.Sri Lanka

                                                       4.Consultant Obstetrician and Gynaecologist, Wrexham Maelor Hospital, UK

Introduction

OHVIRA syndrome is referred to as uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis.
The reported incidence in the general population is 0.1%–3.8%. However, often this is incorrectly identified, incorrectly reported and, inappropriately treated.
The paired mesonephric and paramesonephric ducts represent the indifferent stage of the fetal internal genital system.
The paramesonephricductsofeithersidefusetoformthefemalegenitaltractexceptthe lower portion of the vagina. This process is referred to as Müllerian organogenesis.
Defects in this Mullerian organogenesis lead to complex malformations of the female genital tract such as OHVIRA syndrome.   

Case history