Neuroradiologic appearance of intracranial vascular lesions associated with multiple lymphangioendotheliomatosis with thrombocytopenia

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare congenital vascular disorder characterized by a multitude of lesions involving the skin and gastrointestinal tract with associated thrombocytopenia. Due to the rarity of this disorder, the incidence of intracranial involvement is unknown and only 6 cases have been described in the literature to date. Here we present a case of MLT with a series of neuroimaging studies across several years of the patient's life showcasing the natural history of the intracranial vascular lesions associated with MLT. The features presented in this case report highlight the importance of CNS imaging in patients with MLT in both emergent and nonemergent settings.