Perioperative Management of Pediatric Patients with Moyamoya Arteriopathy

Abstract

Pediatric patients with moyamoya arteriopathy are at high risk for developing new onset transient or permanent neurologic deficits secondary to cerebral hypoperfusion, particularly in the perioperative period. It is therefore essential to carefully manage these patients in a multidisciplinary, coordinated effort to reduce the risk of new permanent neurologic deficits. However, little has been published on perioperative management of pediatric patients with moyamoya, particularly in the early postoperative period during intensive care unit admission. Our pediatric neurocritical care team sought to create a multidisciplinary periprocedural evidence- and consensus-based care pathway for high-risk pediatric patients with moyamoya arteriopathy undergoing anesthesia for any reason to decrease the incidence of periprocedural stroke or transient ischemic attack (TIA). We reviewed the literature to identify risk factors associated with perioperative stroke or TIA among patients with moyamoya and to gather data supporting specific perioperative management strategies. A multidisciplinary team from pediatric anesthesia, neurocritical care, nursing, child life, neurosurgery, interventional neuroradiology, neurology, and hematology created a care pathway for children with moyamoya undergoing anesthesia, classifying them as either high or standard risk, and applying an individualized perioperative management plan to high-risk patients. The incidence of neurologic sequelae before and after pathway implementation will be compared in future studies.

Risk Factors and Imaging Biomarkers Associated With Perioperative Stroke in Pediatric Moyamoya Arteriopathy

“…Therefore, it is essential to identify this high-risk group preoperatively and take precautions to prevent perioperative stroke, such as with intraoperative and postoperative protocols that stress protective strategies such as minimizing fluctuation in carbon dioxide in order to avoid cerebral vasoconstriction or diversion of flow away from cerebral territories affected by moyamoya; hyperhydrating; and avoiding hypotension relative to a patient's baseline blood pressure. 28,29 Although resource-intensive, the consistent use of strategies to uphold these management principles may help decrease the incidence of perioperative ischemia in high-risk children. 30,31 Our study is limited by its small sample size, in addition to its retrospective nature.…”

Advances in the Diagnosis and Treatment of Pediatric Arterial Ischemic Stroke

“…Moyamoya accounts for about 8% of childhood AIS and is associated with high rates of stroke recurrence [37]. The perioperative period is a particularly high-risk epoch for moyamoya-related stroke, which remains a challenge in moyamoya care due to the need for angiography and surgical management [191,192]. While long-term stroke prophylaxis may include antiplatelet therapies and/or surgical revascularization, acute stroke is typically managed with augmentation of cerebral perfusion pressure with fluids and flat head of bed.…”

“…Consideration can be given to vasopressors in the right clinical context, such as if the patient is hypotensive or there are other indicators of cerebral hypoperfusion [193]. Pain and agitation control is of paramount importance, as hyperventilation can lead to cerebral vasoconstriction, thereby exacerbating cerebral hypoperfusion [191]. Maintenance of normocarbia and normoglycemia and minimization of metabolic demand through normothermia and prompt seizure control are also important neuroprotective measures.…”

“…Once appropriate patients are selected for surgery, identification of high-risk patients and meticulous perioperative management, which includes rigorous blood pressure management and maintenance of normocarbia, is critical to minimizing risk of perioperative infarction [191,192]. When revascularization is performed at high-volume moyamoya centers, length of stay and costs are decreased without more complications [245].…”