Vertical fusion defects of the mullerian duct - septate uterus and transverse vaginal septum

Case contributed by Amr Farouk
Diagnosis certain

Presentation

Abdominal distension and vulval mass.

Patient Data

Age: 25 days
Gender: Female

The pelvis & lower abdomen center are seen occupied by bi-compartmental longitudinally positioned thick-walled cystic structure with thick mid-line wall. It measured 3 X 5.4 X 5.6 cm along its maximal axial & craniocaudal dimensions. The cavities are seen distended with fluid density reading with a mean attenuation 3-5HU. The urinary bladder is seen compressed opposite anterior abdominal wall.

A cystic lesion is seen sagging from the introitus with area of collapsed wall seen at the level of the presumed vagina being continuous superiorly with the thick midline wall of the fore-mentioned lesion. It measured 3.3 X 4.5 cm along its maximal axial & craniocaudal dimensions.

The left kidney shows an oval-shaped thick wall cyst at its mid-zone posteriorly showing high-density fluid reading a mean attenuation 15 HU & measuring 1.8 X 1.4 cm in diameters.

Case Discussion

Combined manifestation of vertical fusion defects of the Mullerian duct in the form of septate uterus and transverse vaginal septum causing massive retained secretions & bulging vaginal septum. 

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