Spotting the Culprit: A Rare Case of Hematometra in a Menstruating Female with Fraser Syndrome

Background: Fraser syndrome is a rare autosomal disorder that is associated with multiple genetic abnormalities. Discussed here is one abnormality associated with the genital tract.

Case Description: A 19-year-old female with Fraser syndrome presented to the pediatric emergency department for abdominal distention and pain for two days, noted to have hematometra on CT guided imaging. Decision was made to proceed with total abdominal hysterectomy bilateral salpingoophorectomy.

Conclusion: This case highlights the rarity of Fraser syndrome with associated hematometra secondary to both internal and external genital abnormalities post-menarche. Hematometra should be a differential diagnosis in patients with syndromes that are associated with genital abnormalities as failure to drain the retained blood and tissue products, can act as a foci for infection.