Coexistence of Ebstein Anomaly and Cerebral Cavernous Malformation: A Case Report

In most patients who have Ebstein anomaly (EA), neurological symptoms are either associated with thromboembolic episodes or due to arrhythmic complications. Cerebral cavernous malformation (CCM) is a vascular anomaly made up of sinusoidally dilated blood vessels, often presenting with seizure activity or intracranial bleeding. There has never been any documented report regarding coexisting EA and CCM. Herein, we have reported a 32-year-old female with an established history of Ebstein anomaly diagnosed in 2013 who had a history of generalized tonic-clonic fits since 2020. Neuroimaging studies showed the presence of a right frontal lobe mass with typical "popcorn-like" appearance and hemosiderin ring on MRI, indicative of cerebral cavernous malformation. The patient declined surgery and was managed conservatively with levetiracetam. With the occurrence of further fits in 2025, neuroimaging showed similar findings but without the presence of intratumoral bleeding. Subsequently, she underwent a left frontoparietal craniotomy for GTE of the lesion with cavernoma on histopathology. The neurological status of the patient was stable during her discharge on the fourth postoperative day. This case documents a previously unreported coexistence of Ebstein anomaly and cerebral cavernous malformation in a single patient. Given the rarity of both conditions, coexistence is coincidental. This indicates the need for genetic and molecular studies to determine whether a real biological association exists.