Ovarian Torsion in an Adolescent with Beckwith-Wiedemann Syndrome and Unilateral Tubo-ovarian Hyperplasia.

BACKGROUND

Beckwith-Wiedemann syndrome (BWS) is the most common pediatric overgrowth syndrome. BWS has a broad phenotypic presentation along with an increased propensity to develop various embryonal tumors. There are very few reported cases of gonadal hyperplasia in BWS patients in the existing literature.

CASE: We describe a 13-year-old girl with BWS who presented with an episode of abdominal pain and was found to have torsion and necrosis of a markedly hyperplastic right ovary and fallopian tube. We present a brief literature review on ovarian hyperplasia in BWS patients for which we used an online search of the databases PubMed, Embase, Ovid Medline, and Cochrane.

RESULTS AND

CONCLUSION

Through an extensive literature search, we only found 3 previous reports of ovarian hyperplasia in BWS patients, all in postmortem specimens. Our case highlights a potentially important aspect of visceral organ hyperplasia in patients with BWS that could remain indolent until adolescence and might present as an abrupt-onset abdominopelvic catastrophe.