One of the refreshing aspects of taking care of children is that, generally, they don’t have a mile long list of medications or an extensive known past medical history, unlike their adult counterparts. Unfortunately, this generality can lead us to become complacent and be caught off guard when a child presents with concerning symptoms or has unusual medical problems. We have previously discussed several “adult” illness that occur in children (ex, Pulmonary Embolism, Cholelithiasis, Nephrolithiasis) and one of those surprising entities is Stroke (CVA). While children may have an AVM that leads to hemorrhagic stroke, one important condition that may be on their PMHx list and can lead to CVA in moyamoya. Let’s take a moment to review moyamoya so we can remain vigilant!
Moyamoya: Basics
Moyamoya is a Japanese term that means “something hazy, like puff of smoke drifting in the air.” [Singhi, 2012]
Refers to the angiographic appearance of the abnormal collaterals that develop.
Cerebrovascular arteriopathy that leads to progressive stenosis and eventual occlusion.
It is not inflammatory and not due to atherosclerosis.
Stenosis is due to progressive intimal thickening and thrombosis formation.
Affects internal carotid, middle cerebral artery, and anterior cerebral artery [Singhi, 2012; Kim, 2010]
Can also affect posterior cerebral artery [Lee, 2015; Lee, 2014]
Rare cerebrovascular occlusive disease, but incidence varies between ethnic groups. [Smith, 2009]
Occurs in all ethnic groups.
Bimodal distribution: [Singhi, 2012]
One peak in childhood – usually before 10 years of age.
Second peak occurs during 4th decade of life.
Unknown cause
Likely combination of environmental and genetic factors
One large study found only 12% of cases to be familial. [Kim, 2010]
Moyamoya: What’s in a Name?
“Moyamoya,” “moyamoya syndrome,” “moyamoya disease” may be confused.
Moyamoya – generic term for the angiographic findings
Moyamoya Disease – bilateral, angiographic findings without associated clinical cause (i.e., idiopathic)
Moyamoya Syndrome – unilateral moyamoya or moyamoya found in association with systemic disorders that are known to be associated with moyamoya. [Singhi, 2012; Smith, 2012]
Genetic Disorders:
Down’s Syndrome
Neurofibromatosis type 1
Tuberous sclerosis
Marfan Syndrome
Alagille Syndrome
Hematologic Disorders:
Sickle cell disease and other hemoglobinopathies
Fanconi’s anemia
Hyperthryoidism
Congenital Heart Disease
Tubercular meningitis
Previous cranial therapeutic radiation
Moyamoya: Presentation
There are 4 main types of moyamoya based on clinical manifestations:
Infarct Type
Motor deficits (in one study, ~95% of cases) [Singhi, 2012]
Hemiparesis (44%) [Singhi, 2012]
Sensory symptoms
Some patients will present with ischemic symptoms precipitated by crying, coughing, blowing, or hyperventilation (which leads to hypocapnia and vasoconstriction) [Smith, 2009]
Transient Ischemic Attacks (TIAs) Type
Repeated TIAs is a presentation that needs to be respected.
Hemorrhagic Type
Hemorrhage is rare in children, but does occur. More common in adults. [Singhi, 2012]
Epileptic Type
May be initial presentation or recurrent in patient with known Moyamoya
Seizures on presentation rates vary: ~34% in [Singhi, 2012]; 19% in [Kim, 2010]
Surgery shown to help reduce recurrent seizure activity (97% seizure free) [Kim, 2010]
Other commonly encountered symptoms:
Headaches (33%) [Kim, 2010]
Surgery does not as greatly affect headache recurrence (26% still w/ headaches, and 16% developed new headaches) [Kim, 2010; Seol, 2005]
Fever
30% presented with fevers [Singhi, 2012]
Unclear association, but fever can lead to carotid artery constriction.
Moyamoya: Evaluation
Gold standard for evaluation is Intra-arterial cerebral angiography. [Singhi, 2012]
Better able to fully image the blood vessels.
Helps with correlation of Stage of Disease.
Stages progress from Stage 1 to Stage 6
Staging based on progressive stenosis and eventual occlusion of internal carotid artery (ICA) along with development of moyamoya collaterals at base of brain and development of external carotid artery (ECA) collateral vessels.
MRI/MRA has been found reliable and often used for screening for disease and monitoring, but surgery often based on intra-arterial angiography. [Singhi, 2012; Smith, 2012]
Moyamoya: Management
There is no effective pharmacotherapy to treat moyamoya. [Singhi, 2012]
Therapies aimed at underlying syndromes, if present, for Moyamoya Syndrome. [Smith, 2012]
Surgical revascularization to augment collateral circulation can be helpful.
Various techniques debated amongst neurosurgeons.
Typically reserved for patients with progressive ischemic symptoms or those with evidence of inadequate cerebral perfusion. [Singhi, 2012; Smith, 2012]
Prognosis is highly variable.
May have slow progression with intermittent events.
May have rapid neurologic deterioration.
Early diagnosis and active intervention before irreversible changes occur can lead to favorable outcomes. [Bao, 2014]
Sadly, even in the absence of CVA, there is still often a lower quality of life observed in the patient’s with Moyamoya. [Ball, 2016]
Moral of the Morsel:
Not every child has “no significant past medical history.” Some children have rather complex medical histories.
“Adult” medical problems can occur in children.
A child with a history of possible TIAs should not be dismissed… consider Moyamoya!
A child with Moyamoya may present with progression of disease… be aware of the increased risk for further ischemic disease.
References
Ball AJ1, Steinberg GK2, Elbers J3. Quality of Life in Pediatric Moyamoya Disease. Pediatr Neurol. 2016 Oct;63:60-5. PMID: 27473648. [PubMed] [Read by QxMD]
Moyamoya disease (MMD) is a progressive intracranial arteriopathy with high risk of stroke. Its impact on quality of life is unstudied. We surveyed children with moyamoya disease and compared their quality of life to chronically ill children and children with stroke to better understand the impact of this diagnosis. […]
Rashad S1, Fujimura M2, Niizuma K1, Endo H1, Tominaga T1. Long-term follow-up of pediatric moyamoya disease treated by combined direct-indirect revascularization surgery: single institute experience with surgical and perioperative management. Neurosurg Rev. 2016 Oct;39(4):615-23. PMID: 27180559. [PubMed] [Read by QxMD]
Moyamoya disease (MMD) is a rare occlusive cerebrovascular disease that mainly presents in children as cerebral ischemia. Prompt treatment with either a direct or indirect revascularization procedure is necessary for children with MMD in order to prevent repeated ischemic events. We herein present our experience with combined direct and indirect bypass surgery for the treatment of pediatric MMD as well as our uniquely designed perioperative proto […]
Bao XY1, Duan L, Yang WZ, Li DS, Sun WJ, Zhang ZS, Zong R, Han C. Clinical features, surgical treatment, and long-term outcome in pediatric patients with moyamoya disease in China. Cerebrovasc Dis. 2015;39(2):75-81. PMID: 25573764. [PubMed] [Read by QxMD]
There was few detailed demographic and clinical data about Chinese patients with moyamoya disease. Here we describe the clinical features, surgical treatment, and long-term outcome of pediatric patients with moyamoya disease at a single institution in China. […]
Lee JY1, Kim SK2, Phi JH2, Wang KC2. Posterior Cerebral Artery Insufficiency in Pediatric Moyamoya Disease. J Korean Neurosurg Soc. 2015 Jun;57(6):436-9. PMID: 26180612. [PubMed] [Read by QxMD]
The majority of clinical studies on moyamoya disease (MMD) have focused on anterior circulation. The disease involvement of posterior circulation in MMD, mainly in the posterior cerebral artery (PCA), has been mentioned since the early 1980s, and it has been repeatedly emphasized as one of the most important factors related to poor prognosis in MMD. However, its clinical features and outcome have only been elucidated during the last few years. In […]
Baltsavias G1, Khan N, Valavanis A. The collateral circulation in pediatric moyamoya disease. Childs Nerv Syst. 2015 Mar;31(3):389-98. PMID: 25378261. [PubMed] [Read by QxMD]
The descriptions of collateral circulation in moyamoya have so far been a mixture of topography-based and vessels’ source-based analyses. We aimed to investigate the anatomy and systematize the vascular anastomotic networks in pediatric moyamoya disease. […]
Lee JY1, Choi YH, Cheon JE, Paeng JC, Ryu HW, Kim KJ, Phi JH, Wang KC, Cho BK, Chae JH, Kim SK. Delayed posterior circulation insufficiency in pediatric moyamoya disease. J Neurol. 2014 Dec;261(12):2305-13. PMID: 25212285. [PubMed] [Read by QxMD]
Approximately 30% of patients with moyamoya disease (MMD) have presented with involvement of the posterior circulation, mainly the posterior cerebral artery (PCA). Diagnosis of delayed progression of PCA stenosis in MMD may be difficult due to the diversity in clinical features. The goal of this study was to evaluate pediatric MMD patients with delayed PCA involvement after completion of revascularization of the anterior circulation. Forty-one pe […]
Singhi P1, Choudhary A, Khandelwal N. Pediatric moyamoya disease: clinical profile, literature review and sixteen year experience from a tertiary care teaching institute. Indian J Pediatr. 2013 Dec;80(12):1015-20. PMID: 23525975. [PubMed] [Read by QxMD]
To assess the clinical spectrum and outcome of pediatric moyamoya disease (MMD) in Indian sub-continent. […]
Smith ER1, Scott RM. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr. 2012 Apr;9(4):353-60. PMID: 22462697. [PubMed] [Read by QxMD]
Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain (“moyamoya disease”) or can be unilateral or found in association with systemic disorders (“moyamoya syndrome”). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke-the primary pr […]
Kim SK1, Cho BK, Phi JH, Lee JY, Chae JH, Kim KJ, Hwang YS, Kim IO, Lee DS, Lee J, Wang KC. Pediatric moyamoya disease: An analysis of 410 consecutive cases. Ann Neurol. 2010 Jul;68(1):92-101. PMID: 20582955. [PubMed] [Read by QxMD]
Moyamoya disease (MMD) is a cerebrovascular occlusive disease of the bilateral internal carotid arteries that causes a compensatory abnormal vascular network at the base of brain. The rare incidence and various surgical techniques applied have limited the clinical research on MMD. […]
Amlie-Lefond C1, Bernard TJ, Sébire G, Friedman NR, Heyer GL, Lerner NB, DeVeber G, Fullerton HJ; International Pediatric Stroke Study Group. Predictors of cerebral arteriopathy in children with arterial ischemic stroke: results of the International Pediatric Stroke Study. Circulation. 2009 Mar 17;119(10):1417-23. PMID: 19255344. [PubMed] [Read by QxMD]
Cerebral arteriopathies, including an idiopathic focal cerebral arteriopathy of childhood (FCA), are common in children with arterial ischemic stroke and strongly predictive of recurrence. To better understand these lesions, we measured predictors of arteriopathy within a large international series of children with arterial ischemic stroke. […]
Smith JL1. Understanding and treating moyamoya disease in children. Neurosurg Focus. 2009 Apr;26(4):E4. PMID: 19335128. [PubMed] [Read by QxMD]
Moyamoya disease, a known cause of pediatric stroke, is an unremitting cerebrovascular occlusive disorder of unknown etiology that can lead to devastating, permanent neurological disability if left untreated. It is characterized by progressive stenosis of the intracranial internal carotid arteries and their distal branches and the nearly simultaneous appearance of basal arterial collateral vessels that vascularize hypoperfused brain distal to the […]
Seol HJ1, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg. 2005 Nov;103(5 Suppl):439-42. PMID: 16302616. [PubMed] [Read by QxMD]
Patients with moyamoya disease complain of headache before surgery, after surgery, or in both periods. To clarify the pathophysiological features of these headaches which are one of the major symptoms in moyamoya disease after indirect bypass surgery, the authors reviewed data obtained in patients with moyamoya disease who underwent such surgery at their institute. […]
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