Presentation
Acute urinary tract infection.
Patient Data
Diffuse thickened wall and mucosal trabeculations of the urinary bladder, suggestive of cystitis.
Uterus and vagina:
non-visualization of the uterus, suggestive of uterine agenesis
non-visualization of the upper vagina, suggestive of upper vaginal agenesis
Right ovary: Small size (1.3 x 1.6 cm) with normal sonographic features, present at right side of the pelvis.
Left ovary: Small size (2 x 1cm) with normal sonographic features, present at left iliac fossa.
Left kidney is not present in left renal fossa or elsewhere in the abdomen, suggestive of left renal agenesis.
Solitary right kidney with normal size and location at right renal fossa.
Absent uterus and upper 2/3 of vagina, suggestive of Müllerian agenesis.
Both ovaries are present with normal size and MRI features.
Solitary right kidney.
Case Discussion
Features are suggestive of uterine and upper vaginal agenesis with both ovaries present, suggestive of class I Müllerian duct anomaly (Müllerian agenesis) with left renal agenesis, in keeping with Atypical form (type B) of Mayer-Rokitansky-Küster-Hauser syndrome. This anomaly is confirmed to have normal female Karyotyping (46xx).
The patient presented with acute cystitis. Urine analysis revealed pus>100 cells/HPF. The Müllerian anomaly was incidentally suggested on pelvic ultrasound. She has also a history of primary amenorrhea which was not previously investigated.
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