Background Vascular anomalies are a heterogeneous group of disorders seen in children and adults. A standard nomenclature for classification has been offered by the International Society for the Study of Vascular Anomalies (ISSVA). Its application is important for communication among the multiple specialties involved in the care of patients and for planning treatment, as well as for research and billing. We hypothesized that terminology still is not uniformly applied, and that this could have an impact on treatment. Methods We retrospectively reviewed the medical records of patients with non-brain lesions from our institutional vascular anomalies database seen 2010–2016 for whom at least one clinic visit, radiologic imaging report, and pathology report were available, to compare diagnoses among and within disciplines, and treatment recommendations. Diagnoses and referral patterns by community healthcare providers also were reviewed. Results Of 400 patients seen during the targeted time interval, 35 had clinical, imaging, and pathology reports. Agreement in terminology from initial clinic notes with imaging and pathology reports was noted in only 3 cases (9%). “Hemangioma” was often misused; “lymphangioma” and “cystic hygroma” persist as diagnostic labels. Community healthcare providers referred vascular malformations with a diagnosis of “mass” or “hemangioma” in 17/18 cases where that information was available. Incomplete or mislabeling of vascular anomalies sometimes delayed referrals to appropriate clinics, though it did not have a major impact on treatment. Conclusions An understanding of vascular anomalies as tumors or malformations is not uniform. Ongoing education will be needed to promote consensus terminology and facilitate referrals.

A 4-year-old boy with a history of epilepsy presented with multiple seizures and subsequent hemiparesis. He was found by his parents in the early morning having a tonic-clonic convulsion. He was febrile at that time, and the episode resolved within 10 minutes, with return to normal baseline. The next day, he had multiple episodes of tonic-clonic seizures over 3-4 hours. He was brought for evaluation and found to have right-sided hemiparesis, which persisted for >48 hours. Magnetic resonance imaging of the brain demonstrated changes consistent with diffuse edema throughout the left cerebral hemisphere, as shown on T2-weighted, fatsaturated axial, and T2-weighted coronal images (Figures 1 and 2).The patient's history, examination, and radiologic findings were consistent with hemiconvulsion-hemiplegiaepilepsy syndrome, a consequence of prolonged focal status epilepticus usually affecting young children and characterized by hemiplegia and radiologic evidence of hemispheric edema on the contralateral side. The pathophysiology is not well understood but appears to involve cytotoxic edema, with younger patients being more vulnerable because of the increased susceptibility of immature brains to the development of unilateral ictal discharges. 1 The patient improved with supportive care, although he remained with residual hemiparesis at 2-month follow-up. ■