Vasospasm Following Hemispherectomy: A Case Report of a Novel Complication.

BACKGROUND: Hemispherectomy has been shown to be successful in treating medically intractable epilepsy, with favorable seizure-free outcome. However, the procedure remains to be 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 DESCRIPTION: Seventeen year-old-male 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 eight, he developed an acute decline in his neurological status requiring urgent intubation for airway protection. Magnetic resonance imaging revealed areas of restricted diffusion throughout bilateral hemispheres which 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 worsening clinical picture, he was transitioned to comfort care and passed away.

CONCLUSION: This is the first report in literature describing global vasospasm and delayed cerebral ischemia in a patient carrying COL4A1 mutation, following hemispherectomy. Despite minimal residual postoperative subarachnoid hemorrhage burden, we postulate that his COL4A1 gene mutation might have resulted in this exaggerated vasospasm. This needs to be studied in animal models of this genetic disorder.