P038: ANESTHETIC MANAGEMENT OF A PATIENT PRESENTING WITH MOYAMOYA DISEASE UNDERGOING ENCEPHALO-DURO-ARTERIO-PIAL SYNANGIOSIS: A CASE REPORT
Stephen Alcos, DO1, Romulo Cuy, MD2; 1Larkin Community Hospital, 2Nicklaus Children's Hospital
Introduction: Moyamoya disease (MMD) is a rare (incidence 0.086/100,000), occlusive cerebrovascular disorder that presents as transient ischemic attacks progressing to strokes, fixed neurologic deficits, and seizures in children if left untreated. MMD is characterized by an abnormal vascular network of collateral vessels developing at the base of the brain typically visualized on cerebral angiogram. Various surgical revascularization procedures have been shown to provide long-term symptomatic benefit in the majority of patients. However, MMD poses clinical challenges to the anesthesiologist due to the brain’s compromised circulation. Specifically, MMD patients are at greater risk for ischemic neurologic events during the perioperative period and may develop stroke, transient ischemic attack, intracranial hemorrhage, and infection. The following report illustrates the anesthetic management of a child undergoing surgery for MMD.
Case Presentation: A previously healthy 11-year-old Hispanic female presented with a history of numbness and tingling of the right arm and leg intermittently. A cerebral angiogram revealed nearly complete occlusion of the left internal carotid artery with collateralization. A diagnosis of MMD was made followed by surgical correction with an encephalo-duro-arterio-pial synangiosis. Anesthetic management for this patient focused on avoiding cerebral ischemia, which could be accomplished by maintaining normocapnia, normothermia, normovolemia, adequate depth of anesthesia, and maintenance of oxygen supply-demand ratio in the brain. Despite careful anesthetic management, the patient did suffer from postoperative transient neurologic deficits intermittently for two weeks. During her hospital stay, medical management consisted of increased hydration, adjustments of aspirin levels, and ruling out seizures. After stabilization, the patient was discharged to home and advised to follow up in clinic. Even though this patient presented with postoperative transient neurologic deficits, the long-term outcome appears to be positive.
Conclusion: Neurosurgery for MMD is a high-risk surgery as it involves the brain and its compromised circulation. Despite adherence to current anesthetic recommendations, patients may still have transient neurological deficits postoperatively. However, patients can still become symptom-free and resume full activity in the long term. It is crucial for surgery that the anesthesiologist have experience in managing children being treated for MMD, as the type of anesthesia they require is very different from the standard anesthetic children receive for almost any other type of neurosurgical procedure. There has yet to be a general consensus as to which anesthetic technique is superior to another in the management of MMD. However, it is agreed upon that perioperative complications can be forfeited by ensuring optimal control of blood pressure, central venous pressure, and urine output. This may be accomplished more successfully by invasive monitoring prior to the procedure and continuing these monitors postoperatively.