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Case series and literature review of Moya moya disease in adults- evolution of new insights

Pal R, Chakrabarti R, Rajan S, Bhavani S
Cleveland Clinic, Clevealnd, OH, USA

Moyamoya disease (MMD) is a steno occlusive disease at the terminal portions of the intra cranial internal carotid arteries and compensatory development of a hazy network of small collateral vessels at the base of the brain. Although this disease is old, we bring forth some new insights. Due to changing technology and more advanced anesthesia medications, techniques and newer procedures and increasing complexity of the patient's diseases, we review five cases and anesthesia related to their management.Revascularization surgery procedures have shown to provide symptomatic benefit in a majority of these patients by augmenting collateral cerebral blood flow (CBF). The revascularization is achievable either by directly anastomosing an external carotid artery branch to a cortical artery ( EC-IC bypass)or indirectly by placing a vascularized tissue pedicle in direct contact with the brain( EDAM, EDAS), leading to an ingrowth of new blood vessels to the cortex. Goal of anesthetic management
The anesthetic management of these patients has evolved over the years with an increased understanding of the disease. These have specifically resulted from the identification of risk factors for perioperative complications and outcomes related to the use of anesthetic agents, importance of pain control, the increased use of regional anesthesia, and better monitoring techniques in providing high quality and safe patient care to patients with MMD. The goal for anesthesia is to maintain the balance between the oxygen supply and demand which is even more important in MMD to avoid neurologic morbidity because of the underlying pathophysiology. CBF should be maintained by avoiding hypotension and maintaining normocarbia while an increase in cerebral oxygen consumption (CMRO2) associated with laryngoscopy, tracheal intubation, and surgical events can be minimized by the use of appropriate depth of anesthesia.
We report 5 cases of adult patients with MMD who underwent different surgical procedures I,e Ec-IC bypass, EDAM and precut balloon angioplasty .
Peri operative management and complication
The effects of anesthetics, surgery, and perioperative stress response on severely altered cerebral hemodynamics are complex. Evidence suggests that sparing vital collateral vessels and minimum brain retraction during surgery are essential to avoid perioperative complications. Moreover, patients who underwent indirect revascularization had a greater risk of neurologic deterioration than those who had direct anastomotic procedures
1 Soriano SG, Sethna NF, Scott RM. Anesthetic management of
children with moyamoya syndrome. Anesth Analg. 1993; 77:1066–1070
2 Moyamoya Diseases: A Review of the Disease and Anesthetic Management
Tariq Parray, MD, Timothy W. Martin, MD, MBA, and Saif Siddiqui, MD
(J Neurosurg Anesthesiol 2011; 23:100–109)

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