Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) with a tailgut cyst
Diagnosis almost certain
Presentation
Primary amenorrhea
Patient Data
Age: 25 years
Gender: Female
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The uterus is not visualized as well as the fallopian tubes with no distinct vagina. Both ovaries are well-visualized, containing several follicles.
The left lumbar fossa is occupied by the spleen and the left kidney is in an ectopic position in the left pelvic region.
A multiloculated cystic mass is seen in the retrorectal space, suggestive of a tailgut cyst.
No sacrococcygeal abnormalities are seen.
Case Discussion
Mayer-Rokitansky-Küster-Hauser syndrome belongs to class I Mullerian duct anomalies
Associated renal anomalies may be seen up to 30-40% of cases, but tailgut cyst is rarely associated with uterine anomalies.
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