Moyamoya Disease in an 18-Month-Old Female Caucasian Complicated by Cerebral Hyperperfusion Syndrome Following Indirect Revascularization

Abstract

Patient: Female, 18-month-oldFinal Diagnosis: Moyamoya diseaseSymptoms: SeizuresMedication: —Clinical Procedure: —Specialty: NeurosurgeryObjective:Rare co-existance of disease or pathologyBackground:Cerebral hyperperfusion syndrome is a rare complication of indirect revascularization due to moyamoya disease, but has not been reported previously in the pediatric population. We present a case of an 18-month-old girl with moyamoya disease that was treated with bilateral pial synangiosis and had complications consistent with cerebral hyperperfusion syndrome. This case report discusses the pathophysiological mechanisms involved in cerebral hyperperfusion in moyamoya syndrome.Case Report:An 18-month-old female Caucasian presented with seizures and weakness of the left side. Angiography confirmed bilateral cerebral moyamoya disease that was worse on the right side. Indirect revascularization with pial synangiosis was first performed on the right side to allow for healing. Five months later, pial synangiosis was then performed on the left side. Postoperatively, the patient experienced increased intracranial pressure (ICP), suggesting cerebral hyperperfusion syndrome. She was treated with a repeat lumbar puncture, a lumbar drain, and a lumbar shunt.Conclusions:This report demonstrates a case of cerebral hyperperfusion syndrome as a complication of moyamoya disease in a pediatric patient. Although the patient progressed well after placement of a lumbar shunt, this case demonstrates the occurrence of cerebral hyperperfusion syndrome as a complication of revascularization in pediatric patients and highlights the need for further research in this area.

Diabetes or calcium channel blocker contribute to cerebral hemodynamics after bypass surgery in adult patients with moyamoya disease

“…However, this study also found 4 cases of CHS after indirect revascularization, which is in line with a few other studies (15,16). Some possible theories for explaining this are as follows.…”

Encephaloduroarteriosynangiosis Procedure: A Treatment Option for Patients With Moyamoya Disease

“…23 The incidence of cerebral hyperperfusion syndrome has been found to be lower in the pediatric population, 12 likely because collateral vessels do not tolerate sudden reperfusion. 24 On the other hand, hypoperfusion or low blood pressure during or after revascularization can lead to perioperative ischemia; blood pressure management therefore should be determined on an individual basis to provide the best outcomes. Wound complications also can arise after revascularization during the harvesting of grafts and tissues from the scalp.…”

“…This defect leads to an area of the brain being hyperperfused, which can cause the patient to develop transient neurological deficits, hemorrhage, edema, or seizures 23 . The incidence of cerebral hyperperfusion syndrome has been found to be lower in the pediatric population, 12 likely because collateral vessels do not tolerate sudden reperfusion 24 . On the other hand, hypoperfusion or low blood pressure during or after revascularization can lead to perioperative ischemia; blood pressure management therefore should be determined on an individual basis to provide the best outcomes.…”