Transcript Your Patient Has Moyamoya. Now What?

YOUR PATIENT HAS
MOYAMOYA
DISEASE.
NOW WHAT?
YEE L. KWAN, RN, CCRN
DUNAP, CLASS OF 2013
FEBRUARY 27, 2012
IT’S NOT A BRAND OF
SOY SAUCE…..
By the end of this presentation you should be
able to…….
1. Discuss/define Moyamoya Disease (MMD)
2. Discuss etiology, signs & symptoms of
MMD
3. Discuss anesthetic implications for your
patient with MMD
MY PATIENT
• 31 yo F
• Diagnostic lap, BSO
• PmHx:
•
•
•
•
•
•
•
Chronic pain
Migraines
Asthma
Hypotension
Cerebral bypass
TIAs
L sided weakness (transient)
“PUFF OF SMOKE”
Fig. 1 The haze of cigarette smoke and its resemblance of the vascular changes occurring in moyamoya disease. Adapted from
“Discovery of cerebrovascular moyamoya disease: Research during the late 1950s and early 1960s,” by H. Oshima, 2012, Child's
Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
ETIOLOGY & PATHO
• Genetically inherited?
• Most likely autosomal dominant
• No one really knows!
• Eccentric intimal thickening
• Fibroblast growth factor is increased
• Ongoing CNS inflammation w/slight BBB
impairment
Fig. 2. Moyamoya Vessel. [Online Image]. Obtained February 1, 2012, from
http://neurosurgery.stanford.edu/cerebrovascular/aneurysms.html
Figure. Angiography was performed twice, with an interval of 4 years and 8 months apart.
Choi H et al. Neurology 2007;68:954-954
©2007 by Lippincott Williams & Wilkins
CLINICAL
PRESENTATION
• Ischemic stroke
• Frontal lobe
• Dysarthria
• Aphasia
• Hemiparesis
• Headache
• Syncope
• Visual symptoms
• Intellect impairment/cognitive dysfunction
TREATMENTS
• Direct bypass
• STA-MCA anastomosis
• STA-ACA anastomosis
• Indirect bypass
• EDAS
• EMS
• EDAMS (EDAS + EMS)
• Combined bypass
• Craniotomy with dural inversion, cervical
sympathectomy, omental transposition, burr holes
• Antiplatelet Agents
• ASA
• Ticlopidine (Ticlid)
• Calcium Channel Blockers
• Nimodipine (Nimotop)
• Nicardipine (Cardene)
• Anticonvulsants
PREOP
• Preop eval
• Baseline neuro status
• Document, document, DOCUMENT!
• Home meds
• Continue until DOS
• Premeds
• IVF
• Anxiolytics
INDUCTION
• Induction agents
• Inhalation vs. IV
• Careful titration
• Monitoring
• Standard monitors
• +/- as needed for patient acuity
MAINTENANCE
• Drugs
• Ventilation
• BP, Fluids & UOP
• Hematocrit
• Temp
EMERGENCE & POSTOP
• Neuro check
• Pain management
THE GOAL…..
Medical Disorders Associated with Moyamoya Disease
Congenital syndromes
Apert syndrome
Down syndrome
Hirshprung disease
Marfan syndrome
Neurofibromatosis Type 1
Turner syndrome
Immunologic disorders
Graves disease
Hematologic disorders
Aplastic anemia
Fanconi anemia
Sickle cell anemia
Thalassemia
Neoplasia
Wilms tumor
Craniopharyngioma
Parasellar tumors
Infectious
Tuberculous meningitis
Leptospirosis
Vascular disorders
Atherosclerotic disease
Cardiomyopathy
Coarctation of aorta
Hypertention
Others
Radiation injury
Pulmonary sarcoidosis
Nephrotic syndrome
IN SUMMARY…..
• Not just at Duke
• What’s the baseline?
• Status quo
• Not the time to “run the patient dry”
• Supply = demand
QUESTIONS FOR
THOUGHT…..
• 100% FiO2?
• What’s your threshold for wakeup time?
• TIVA vs. regular?
References:
De Buysscher, P., Moerman, A., Bove, T., De Pauw, M., Wouters, P., & De Hert, S. (2011). Value of cerebral oxygen saturation monitoring
during cardiopulmonary bypass in an adult patient with moyamoya disease. Journal of Cardiothoracic and Vascular Anesthesia. doi:
10.1053/j.jvca.2011.11.002
Hedenstierna, G., & Rothen, H. U. (2000). Atelectasis formation during anesthesia: Causes and measures to prevent it. [Review]. Journal of
Clinical Monitoring and Computing, 16(5-6), 329-335.
Hedenstierna, G., & Edmark, L. (2010). Mechanisms of atelectasis in the perioperative period. [Research Support, Non-U.S. Gov’t Review].
Best Practice & Research. Clinical Anaesthesiology, 24(2), 157-169.
Kraemer, M., Horn, P. A., Roder, C., Khan, N., Diehl, R. R., Berlit, P., & Heinemann, F. M. (2012). Analysis of human leucocyte antigen genes
in Caucasian patients with idiopathic Moyamoya angiopathy. Acta Neurochirurgica, 154(3), 445-454. doi: 10.1007/s00701-011-1261-5
Kuroda, S., & Houkin, K. (2008). Moyamoya disease: current concepts and future perspectives. [Research Support, Non-U.S. Gov’t Review].
Lancet Neurology, 7(11), 1056-1066. doi: 10.1016/S1474-4422(08)70240-0
Morgan, G. E., Mikhail, M. S., & Murray, M. J. (2006). Clinical Anesthesiology (4th ed.). New York: Lange Medical Books/McGraw Hill,
Medical Pub. Division.
Oshima, H., & Katayama, Y. (2012). Discovery of cerebrovascular moyamoya disease: research during the late 1950s and early 1960s.
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. doi: 10.1007/s00381-012-1708-x
Parray, T., Martin, T. W., & Siddiqui, S. (2011). Moyamoya disease: A review of the disease and anesthetic management. [Review]. Journal of
Neurosurgical Aanesthesiology, 23(2), 100-109. doi: 10.1097/ANA.0b013e3181f84fac
Rodriguez-Hernandez, A., Josephson, A. S., Langer, D., & Lawton, M. T. (2011). Bypass for the prevention of ischemic stroke. [Review].
World Neurosurgery, 76(6 Suppl), S72-79. doi: 10.1016/j.wneu.2011.06.032
Sato, K., Shirane, R., Kato, M., & Yoshimoto, T. (1999). Effect of inhalational anesthesia on cerebral circulation in Moyamoya disease.
Journal of Neurosurgical Anesthesiology, 11(1), 25-30.
Zipfel, G. J., Fox, D. J., Jr., & Rivet, D. J. (2005). Moyamoya disease in adults: the role of cerebral revascularization. Skull base : Official
Journal of North American Skull Base Society ... [et al.], 15(1), 27-41. doi: 10.1055/s-2005-868161