Purpose or Case Report: To demonstrate an unusual association of VECTERL and uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndromes in fetal MRI with post mortem MRI correlation.
Methods & Materials: 35-year-old female, 31weeks pregnant, with severe oligohydramnios. The prenatal US was limited to evaluate both kidneys. Fetal MRI was requested before termination of pregnancy.
Results: Fetal MRI showed multiple cystic lesions in the right kidney (MCDK) in the right renal fossa. The left kidney was not identified. There was a large T1hypo/T2isointense globular-shaped lesion in the pelvic cavity, anterior to the rectum, likely turbid-fluid filled in the vagina (hydro/ hydrometrocalpos). Severe fetal lungs hypoplasia was noted. Findings were suggestive of possible OHVIRA syndrome.

Termination of pregnancy was obtained due to multiple anomalies with severe fetal lung hypoplasia. Post mortem MRI was obtained.

Post mortem MRI showed baby girl with OHVIRA syndrome. MCDK of the right kidney is also noted, associated with large ASD, double SVCS, thoracic dextroscoliosis with T9 hemivertebra, suggestive of VACTERL syndrome.

Conclusions: OHVIRA Syndrome is a rare congenital anomaly of the female genital tract, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It occurs due to arrest of the midline fusion of the Mullerian and Wolffian ducts, either completely or incompletely. Incidence of Mullerian duct anomalies ranges from 0.8% to 4%. The true incidence of the OHVIRA syndrome is unknown, estimated 0.1-3.5 % of all Mullerian anomalies. Most of the patients are usually diagnosed at puberty after menarche due to recurrent severe dysmenorrhea and/or chronic pelvic pain.

VACTERL is an association of congenital malformation, characterized by the presences of at least three of the following: vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal and limb anomalities. The etiology is unknown. Diagnosis is based on the clinical presentation at birth. Antenatal diagnosis can be challenging.

The association between OHVIRA and VACTERL syndromes has never been published. This is the first case report of OHVIRA and VACTERL syndromes association.

We will demonstrate embryological development of the Mullerian and Wolffian ducts to help understanding spectrum of the anomalies in OHVIRA syndrome as well as imaging checklists in fetal MRI in diagnosis of OHVIRA and VACTERAL anomalies.