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Year : 2015  |  Volume : 16  |  Issue : 2  |  Page : 23-29

Caring for a patient with moyamoya disease: A case report

Professor, College of Nursing, CMC, Vellore, India

Date of Web Publication23-May-2020

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Moyamoya disease is a rare cerebrovascular disorder that is characterized by progressive stenosis of distal portion of the internal carotid artery and its main branches. Moyamoya is a Japanese word for a “cloud of smoke” that depicts the appearance of collaterals as occlusion of the blood vessels occur due to stenosis. It mostly affects the younger age group and they develop signs and symptoms of transient ischemic attacks, seizures, infarction, and strokes. In children the disease is characterized by ischemic events compared to adults who develop haemorrhagic manifestations. Diagnosis is based on characteristic arteriographic appearance. Management is not very clear although revascularization techniques are being tried. The effects of multiple strokes in the lives of these individuals have a cumulative load in the quality of life. Therefore nurses have a great role to play in the management of patients during the acute as well as the rehabilitative phases. For a comprehensive understanding of the disease here is an article with the details of the disease such as epidemiology, etiology, pathophysiology, clinical manifestations, diagnostic measures, and the management presented along with a case report focusing on nursing management.

Keywords: Moyamoya disease, stroke, seizures, transient ischemic attack, nursing management

How to cite this article:
Sam PR. Caring for a patient with moyamoya disease: A case report. Indian J Cont Nsg Edn 2015;16:23-9

How to cite this URL:
Sam PR. Caring for a patient with moyamoya disease: A case report. Indian J Cont Nsg Edn [serial online] 2015 [cited 2023 Mar 26];16:23-9. Available from: https://www.ijcne.org/text.asp?2015/16/2/23/284857

  Introduction Top

Moyamoya is a progressive cerebrovascular condition due to stenosis of the intracranial vessels of the anterior, middle, and posterior cerebral arteries that results in ischemia and stroke in children. Also the decreased blood flow in the circulation of the brain causes formation of abnormal collaterals as a compensatory mechanism which gives an appearance of a puff of smoke due to bleeding from these vessels.

  Anatomy and Physiology Top

Blood is carried to the brain by two paired arteries namely the internal carotid and the vertebral arteries. The internal carotid arteries supply the anterior portion and the vertebral arteries supply the posterior portion of the brain. The right and left vertebral arteries join together to form a single basilar artery (Tortora & Derickson, 2006). The basilar artery and the internal carotid arteries communicate with each other in a ring at the base of the brain called the circle of Willis as shown in [Figure 1].
Figure 1: Blood circulation to the brain (Source: Zuccarello & Andaluz, 2013)

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  Case Report Top

A four year old girl child was admitted with complaints of transient episodes of left upper and lower limb weakness and one episode of left upper and lower limb focal motor seizure. The weakness lasted for few minutes to few hours and was associated with aphasia and transient blindness of both eyes. After this episode, there was a history of cognitive decline, hyperactivity, and reduced attention span. She was evaluated and diagnosed to have Moyamoya disease and was started on Tab. Ecospirin 75 mg once daily and a course of steroids for one and a half months. She underwent cerebral angiogram and magnetic resonance imaging (MRI) of brain which showed bilateral internal carotid artery stenosis and she underwent right encephaloduroarteriosynangiosis. After surgery her Glasco Coma Scale (GCS) Score was 6/15 (E1V1M4) and therefore a repeat computed tomography (CT) of the brain was done which showed an infarct that involved the left internal carotid artery territory. She was then taken for an emergency left frontotemperoparietal craniectomy. Her GCS Score after the second surgery was 5T/15 which was again followed by a CT brain that showed bilateral internal carotid artery territory infarcts with few viable areas in certain areas of the brain.

  Epidemiology Top

Moyamoya disease is originally thought to be confined to the Asian population but was found in other races too. Incidence is highest in Japan. Disease is seen to largely peak during the first decade and a smaller peak is observed in the fourth decade of life. Bimodal age distributions are seen. A female predominance occurs (Baba, Houkin, & Kuroda, 2008). Based on ethnicity as ratio to whites was 4.6 for Asian Americans, 2.2 for African Americans, and 0.5 for Hispanics (Uchino, Johnston, Becker, & Tirschwell, 2005).

  Etiology Top

The disease is said to be idiopathic. It is however believed by many to be hereditary. A family history of10% of patients is found with this disorder. Familial Moyamoya disease is autosomal dominant with incomplete penetrance that depends on age of genomic imprinting factors (Mineharu et al., 2008).

  Pathophysiology Top

The exact pathogenesis of Moyamoya disease is unknown. The gene ring finger protein 213 (RNF213) has been identified in the pathogenesis of this disease (Ma et al., 2013). The disease causes constrictions primarily in the internal carotid artery and extends to the middle and anterior cerebral arteries and also the branches of the internal carotid artery. The constrictions of the arteries occur due to proliferation within the arterial lumen causing external fibrocellular intimal thickening. When the artery becomes completely blocked, there is a fine collateral circulation that is formed. The collaterals are either thin and dilated or thick and stenosed (Lim, Cheshier, & Steinberg, 2006). Although patients often survive on the collateral circulation there is a danger of development of blood clot or mural thrombi, within these arteries that causes stroke (Takagi, Kikuta, Nozaki, & Hashimoto, 2007). There is often repeated strokes that results in severe functional impairment or even death.

  Clinical Manifestations Top

These patients are identified to develop various manifestations leading to functional impairment (Starke et al., 2009).

The child had developed a transient ischemic attack followed by a seizure due to which she had weakness of the left upper and lower limbs, aphasia and difficulty in vision in both the eyes for a brief period of time.

  Diagnostic Measures Top

Along with history and clinical examination the following diagnostic tests are performed to identify and confirm on the disease and its extent

Magnetic Resonance Angiogram (MRA): It is performed during an MRI by injecting a contrast agent into the blood stream so that arteries of the brain can be seen. In those with Moyamoya the anterior cerebral artery is seen as profuse collateral network of vessels.

Angiogram: A catheter is inserted into the major arteries of the external and internal carotid and vertebral arteries and contrast dye is injected after which X-ray images are taken as shown in [Figure 3].
Figure 3: The right carotid artery shows occlusion of the carotid bifurcation with collaterals creating the appearance of “puff of smoke” (Source: Steinberg et al., 2015)

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Computed Tomography Angiography (CTA): It involves injecting a contrast agent into the blood stream so that arteries of the brain can be seen. This test provides the best pictures of both blood vessels through angiography and soft tissues through CT.

Computed Tomography Perfusion: It involves injecting a contrast agent into the blood stream to study the amount of blood flow reaching the brain and determine areas at risk for stroke.

  Management Top

Management is difficult because of the desire to prevent both infarction and haemorrage. Surgery is generally recommended for those with recurrent or progressive TIAs or strokes with the goal of revascularization (Guzman et al., 2009; Kuroda et al., 2008; Ozgur, Aryan, & Levy, 2006; Roach et al., 2008; Scott et al., 2004).

The surgical procedures that are done for Moyamoya can be of two types - direct and indirect surgical methods for revascularization.

1. Direct: The superficial temporal artery is anastamosed to the middle cerebral artery for revascularization of the area affected to achieve instant improvement in blood flow. This surgery is attempted for preventing cerebral haemorrhage however its effectiveness is still under study (Kanaguchi, Okano, & Sakaki, 2000)

2. Indirect: It is preferred for children under 10 years of age. The various indirect methods are explained below:

  • Encephalo-duro-arterio-synangiosis (EDAS): The superficial temporal artery is sutured to the surface of the brain. Over time angiogenesis occurs that results in small arterial vessels that supply the brain.
  • Encephalo-myo-synangiosis (EMS): The temporalis muscle is dissected and is placed onto the surface of the brain. Over time new vessels form between the blood-rich muscle and the brain.
  • Encephalo-duro-arterio-myo-synangiosis (EDAMS): This is a combination of the techniques of both EDAS and EMS.
  • Omental transposition: The omentum is placed on the surface of the brain which results in new vessels that will grow into the brain and improve blood supply.
  • Multiple burr holes: Small holes are made in the skull that allows growth of new vessels into the brain from the scalp.

The child underwent encephaloduroarterio- synangiosis surgery.

  Nursing Management Top

Nursing management of a patient with Moyamoya is presented using nursing process approach (Hickey, 2009; Smeltzer & Bare, 2010).

1. Nursing Diagnosis: Ineffective cerebral perfusion related to decreased cerebral blood flow secondary to occlusion in the cerebral artery

Expected Outcome: Adequate cerebral perfusion is restored as evidenced by normal GCS Score and Intra Cranial Pressure (ICP)


  • Assessed neurological status every 2 hours to identify changes indicative of worsening or improving condition
  • Monitored neurological response to activities as it can alter with changes in positioning or movement
  • Planned nursing care activities to minimize increase in ICP
  • Prevented flexion at the neck, shoulder, hip or knee to avoid obstruction of blood flow
  • Monitored respiratory status to assess changes in neurological status

Evaluation: Her neurological status was only 6T/15 after the first surgery and later reduced to 5T/15 after the second surgery. During the rehabilitative process it had improved to 7T/15.

2. Nursing Diagnosis: Ineffective airway clearance related to inability to cough out secretions as manifested by adventitious sounds, and diminished breath sounds

Expected Outcome: Patent airway is maintained as evidenced by normal breath sounds


  • Assisted her in a semi- Fowlers position for maximum intrathoracic pressure and promote maximum chest expansion
  • Provided nebulisations of Salbutamol, Ipravent and N-Acetyl Cysteine for mobilising the secretions
  • Monitored the oxygen saturation levels
  • Provided tracheostomy care every 4 hourly
  • Changed the position every 2 hourly to ensure postural drainage and prevent stasis of secretions
  • Performed tracheobronchial suctioning every 2 hourly and as needed to remove secretions and improve oxygenation
  • Performed chest physiotherapy to enhance removal of secretions
  • Ensured adequate fluid intake to liquefy secretions

Evaluation: Her oxygen saturation levels ranged between 98 100%.

3. Nursing Diagnosis: Impaired physical mobility related to paralysed extremities secondary to coma

Expected Outcome: Optimal physical mobility is enhanced as evidenced by improved range of motion of extremities and absence of contractures


  • Assessed and documented the range of motion possible and muscle strength
  • Assessed the positioning ability to determine the extent of the problem and plan appropriate interventions
  • Maintained proper position or alignment with pillows to prevent contractures
  • Performed passive exercises to maintain joint and muscle functioning
  • Provided adequate periods of rest with exercise to facilitate rehabilitation

Evaluation: She had no muscle atrophy but had developed dystonia due to the disease process and it was controlled with Inj. Midazolam 5ml/hour during acute phases.

4. Nursing Diagnosis: Impaired verbal communication related to global edema and craniectomy

Expected Outcome: Verbal communication is restored within accepted limits


  • Assessed communication deficits present, to determine type and plan appropriate interventions
  • Provided positive reinforcement and praise to build confidence
  • Spoke slowly to allow adequate time to understand to avoid over-whelming with verbal stimuli
  • Provided verbal prompts and reminders to stimulate speaking

Evaluation: There was no verbal communication that was established till discharge.

5. Nursing Diagnosis: Self care deficit related to altered loss of consciousness

Expected Outcome: Self care needs are met involving the mother as evidenced by groomed appearance


  • Assessed the level of motor and sensory abilities every 4 hourly to determine the level of care needed
  • Bathed her daily to meet the hygienic needs
  • Performed range of motion exercises as tolerated to maintain joint range of motion and muscle strength
  • Provided catheter care to reduce risk of infection
  • Provided her with comfort devices of alpha bed and pillows
  • Provided her perineal care to reduce infection

Evaluation: Her mother had learnt all aspects of care demonstrating confidence before discharge.

6. Nursing Diagnosis: Interrupted family processes related to comatose family member as manifested by inability to adapt to health crisis of family member and lack of communication

Expected Outcome: Family process is enhanced as evidenced by adequate coping and cooperation by the family members


  • Assessed effect of child on the family as a whole to determine extent of problems
  • Taught and assisted family members to provide care to enable them to be an integral part of the care
  • Facilitated family communication and realistic planning to meet the needs with minimal disruption
  • Provided accurate information to family regarding the condition of patient for better understanding and coping

Evaluation: Family members cooperated with the health team members in providing care to the child.

7. Nursing Diagnosis: Disturbed sensory perception related to decreased loss of consciousness as manifested by inaccurate interpretation of environment, signs of fear, anxiety, and restlessness

Expected Outcome: Sensory perception is restored to maximum


  • Assessed the level of consciousness to determine the extent of the problem
  • Administered sedative (Inj. Midazolam 5 ml/ hr) as continuous infusion to reduce restlessness
  • Kept the room quiet and lights dim, and provided a calm environment to avoid frightening her
  • Assisted and supported her during uncomfortable procedures like Intravenous cut down that was necessary and also during dystonic episodes

Evaluation: She remained calm most of the time.

8. Nursing Diagnosis: Acute pain related to dystonia as manifested by grimaces and moaning

Expected Outcome: Pain is minimized as evidenced by absence of facial grimaces and moaning


  • Administered Tab. Diazepam 7.5mg TID and Tab. Baclofen 10mg BD for dystonia
  • Encouraged gentle range of motion to reduce joint stiffness and promote circulation
  • Massaged the muscles to promote comfort
  • Controlled the environment to encourage rest as pain was exhausting

Evaluation: She had intermittent episodes of pain which could be noticed through her facial grimaces.

9. Nursing Diagnosis: Potential for injury related to seizure activity secondary to cerebral irritation

Expected Outcome: Injury is prevented


  • Monitored for seizure activity to identify and start interventions immediately
  • Kept the side rails up and provided pillows all around for protection against falls and injury
  • Administered antiepileptics ( Inj. Sodium Valproate and Inj. Levetiracetum) regularly
  • Ensured that temperature was brought down to decrease brain’s oxygen demand

Evaluation: She did not develop seizures.

The child at the time of discharge continued to have dystonia but was made pain free with Inj. Midazolam which had to be continued. Follow up revealed that she developed no further complications. She was nursed at home by her mother with the help of rigorous training that was given during her hospital stay by the nurses.

  Conclusion Top

Moyamoya progression can be slow with rare intermittent events or have a rapid neurologic decline. Though it is not possible to reverse the primary disease process yet it is possible to prevent complications. Surgical interventions are necessary to improve blood flow and provide protection against strokes. Therefore prompt therapy and nursing care goes hand in hand to achieve the best outcome. Nursing care rendered needs to be comprehensive and complete. The nurse needs to ensure that adequate education is provided to caregivers for ongoing care even at home.

Conflicts of Interest: The author has declared no conflicts of interest.

  References Top

Baba, T., Houkin, K., & Kuroda, S. (2008). Novel epidemiological features of Moyamoya disease. Journal of Neurology, Neurosurgery and Psychiatry, 79(8), 900 - 904.  Back to cited text no. 1
Guzman, R., Lee, M., Achrol, A., Bell-Stephens, T., Kelly, M., Do, H. M., … Steinberg, G. K. (2009). Clinical outcome after 450 revascularization procedures for Moyamoya disease. Journal of Neurosurgery, 111(5), 927 - 35.  Back to cited text no. 2
Hickey, J. V. (2009). The clinical practice of neurological and neurosurgical nursing (6th ed.). Philadelphia: Lippincott publications.  Back to cited text no. 3
Kanaguchi, S., Okano, S., & Sakaki, T. (2000). Effect of direct arterial bypass on the prevention of future stroke in patients with hemorrhagic variety of moya moya disease. Journal of Neurosurgery, 93, 397 - 401.  Back to cited text no. 4
Kuroda, S., Nakayama, N., Ishikawa, T., Houkin, K., Kamiyama, H., & Iwasaki, Y. (2008). Treatment Strategies and long-term prognosis in Moyamoya disease. Surgery for Cerebral Stroke, 36(5), 361- 366.  Back to cited text no. 5
Lim, M., Cheshier, S., & Steinberg, G. K. (2006). New vessel formation in the central nervous system during tumour growth, vascular malformations, and Moyamoya. Current Neurovascular Research, 3(3), 237-245.  Back to cited text no. 6
Ma, J., Liu, Y., Ma, L., Huang, S., Li, H., & You, C. (2013). RNF213 polymorphism and Moyamoya disease: A systematic review and meta-analysis. Neurology India, 61(1), 35 - 9.  Back to cited text no. 7
Mineharu, Y., Takenaka, K., Yamakawa, H., Inoue, K., Ikeda, H., Kikuta, K. I., … Koizumi, A. (2006). Inheritance pattern of familial Moyamoya disease: Autosomal dominant mode and genomic imprinting. Journal of Neurology Neurosurgery and Psychiatry, 77(9), 1025 - 1029.  Back to cited text no. 8
Ozgur, B. M., Aryan, H. E., & Levy, M. L. (2006). Indirect revascularisation for paediatric Moyamoya disease: The EDAMS technique. Journal of Clinical Neuroscience, 13(1), 105 - 108.  Back to cited text no. 9
Roach, E. S., Golomb, M. R., Adams, R., Biller, J., Daniels, S., de Veber, G., … Smith, E. R. (2008). Management of stroke in infants and children: A scientific statement from a special writing group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke, 39(9), 2644-91.  Back to cited text no. 10
Scott, R. M., Smith, J. L., Robertson, R. L., Madsen, J. R., Soriano, S. G., & Rockoff, M. A. (2004). Long-term outcome in children with Moyamoya syndrome after cranial revascularization by pial synangiosis. Journal of Neurosurgery, 100(2 Supplementary Pediatrics), 142 - 149.  Back to cited text no. 11
Smeltzer, S. C., & Bare, B. (2010). Brunner and Suddarth’s textbook of medical surgical nursing (12th ed.). Philadelphia: Lippincott publication.  Back to cited text no. 12
Starke, R. M., Komotar, R. J., Hickman, Z. L., Paz, Y. E., Pugliese, A. G., Otten, M. L., … Connolly Jr, E. S. (2009). Clinical Features, surgical treatment, and longterm outcome in adult patients with Moyamoya disease. Journal ofNeurosurgery, 111(5), 936 - 942.  Back to cited text no. 13
Steinberg, G. K. (2015). Aneurysms. Retrieved from http ://neuro surgery. stanford. edu/cerebrovascular/aneur ysms.htm.  Back to cited text no. 14
Takagi, Y., Kikuta, K., Nozaki, K., & Hashimoto, N. (2007). Histological features of middle cerebral arteries from patients treated for Moyamoya disease. Neurologia Medico-Chirurgica, 47(1), 1-4.  Back to cited text no. 15
Tortora, G. J., & Derickson, B. (2006). Principles of anatomy and physiology (11th ed.). New Jersey: John Wiley and Sons.  Back to cited text no. 16
Uchino, K., Johnston, S. C., Becker, K. J., & Tirschwell, D. L. (2005). Moyamoya disease in Washington State and California. Neurology, 65(6), 956 - 958.  Back to cited text no. 17
Zuccarello, M., & Andaluz, N. (2013). Moyamoya Disease. Retrieved from http://www.mayfieldclinic.com/PE- Moyamoya.htm#.VhIWuG5EVOc.  Back to cited text no. 18


  [Figure 1], [Figure 2]


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