• Amr Morsi, Andres Maldonado, Dennis Lal, Moosa Ahsan N V ANV Department of Neurology, Cleveland Clinic Foundation, Cleveland, Ohio, USA., Elia Pestana-Knight, and William Bingaman.
• Department of Neurosurgery, Cleveland Clinic Foundation, Cleveland, Ohio, USA. Electronic address: morsia@ccf.org.
• World Neurosurg. 2020 May 1; 137: 357-361.

BackgroundHemispherectomy has been shown to be successful in treating medically intractable epilepsy, with favorable seizure-free outcomes. However, the procedure is technically challenging with high rates of in-hospital complications. We present a unique case of functional hemispherectomy complicated by diffuse cerebral vasospasm and subsequent death in a patient with COL4A1 gene mutation.Case DescriptionA 17-year-old boy presented with right hemispheric epilepsy and a previously diagnosed autosomal dominant heterozygous COL4A1 gene mutation (c.4380T>G;p.Cys1460Trp). Functional hemispherectomy was performed without complications. On postoperative day 8, he developed an acute decline in neurologic status requiring urgent intubation for airway protection. Magnetic resonance imaging revealed areas of restricted diffusion throughout bilateral hemispheres that was explained by severe vasospasm and minimal cerebral blood flow seen on cerebral angiography. Intra-arterial calcium channel blocker infusion and balloon angioplasty were attempted without improvement in perfusion. With a worsening clinical picture, he was transitioned to comfort care and died.ConclusionsThis is the first report in the literature describing global vasospasm and delayed cerebral ischemia following hemispherectomy in a patient carrying COL4A1 gene mutation. We postulate that his COL4A1 gene mutation might have resulted in this exaggerated vasospasm despite minimal residual postoperative subarachnoid hemorrhage burden. This hypothesis needs to be studied in animal models of this genetic disorder.Copyright © 2020 Elsevier Inc. All rights reserved.

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