CASE REPORT OF HERLYN-WERNER-WUNDERLICH (OHVIRA) SYNDROME: MRI FINDINGS AND RADIOLOGICAL GUIDE

Faryal Farooq

CASE REPORT OF HERLYN-WERNER-WUNDERLICH (OHVIRA) SYNDROME: MRI FINDINGS AND RADIOLOGICAL GUIDE

Faryal Farooq

Abstract

ABSTRACT

Herlyn-Werner-Wunderlich (HWW) syndrome, a rare Müllerian duct anomaly (MDA) consists of didelphic uterus (MDA III), hemivaginal septum and ipsilateral renal agenesis (Mesonephric anomaly). This is given the name of OHVIRA syndrome comprising of obstructed hemivagina and ipsilateral renal anomaly. Patients are usually diagnosed at puberty after menarche. Diagnosis is made solely on the basis of suspicion due to nonspecific symptoms and also because of seldom encounter of Mullerian Duct Anomalies (MDA) clinically. If the history, examination and imaging findings correlate, existence of this syndrome is raised. The role of imaging is to help detect, diagnose, and distinguish surgically correctable forms of Müllerian Duct Anomalies from inoperable forms.

We report a case of a 16-year-old girl with this condition who was diagnosed as uterus didelphys with unilateral hematocolpos and ipsilateral renal agenesis on the basis of sonography and confirmed by MRI. MRI proved to be of great help in correct diagnosis avoiding surgical interventions.

Patient was treated with drainage of hematocolpos and single stage vaginoplasty and followed up subsequently.

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References

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