Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease

doi:10.1016/0887-8994(96)00045-8

Pediatric Neurology

Volume 14, Issue 3, April 1996, Pages 226-230

https://doi.org/10.1016/0887-8994(96)00045-8 Get rights and content

Abstract

We report a black girl with sickle cell anemia. On prophylactic exchange transfusion protocol, she experienced cerebrovascular accidents at 3 and 3.5 years of age, both associated with transient right hemiparesis. At 7.5 years of age, she presented with a partial motor seizure and a left hemiparesis. A cerebral angiogram demonstrated stenosis at the origins of both middle and anterior cerebral arteries bilaterally with extensive basal collateralization. She underwent uncomplicated bilateral encephaloduroarteriosynangiosis (EDAS) procedures using both superficial temporal arteries. At age 9 years, the patient presented with a severe headache and tunnel vision secondary to a stenosis of both posterior cerebral arteries. She underwent bilateral EDAS procedures using both occipital arteries. No complication was encountered. Postoperative cerebral angiogram demonstrated impressive neovascularity at the sites of all four EDAS procedures. Different treatment options of moyamoya disease are discussed.

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Cited by (35)

Cerebral Revascularization for Moyamoya Syndrome Associated with Sickle Cell Disease: A Systematic Review of the Literature on the Role of Extracranial-Intracranial Bypass in Treating Neurologic Manifestations of Pediatric Patients with Sickle Cell Disease
2020, World Neurosurgery
Citation Excerpt :
An extensive analysis was then performed to extract relevant clinical data with respect to surgical outcomes after revascularization and risk reduction of stroke after revascularization. Our final review and discussion are based on detailed analysis of data extracted from the final 13 articles.4-9,11-17 We conducted a retrospective review of patients with SCD with MMS at Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA to assess institutional outcomes.
Moyamoya syndrome (MMS) in patients with sickle cell disease (SCD) accentuates the risk of recurrent strokes. Chronic transfusion therapy (CTT) is an excellent option for preventing recurrent strokes in most patients with SCD. In SCD with MMS, CTT may fail as a long-term solution. Cerebral revascularization, in the form of extracranial-intracranial bypass, has been shown to prevent recurrent strokes in this cohort. We review the evolution of this paradigm shift in the management of SCD-associated MMS. A systematic review, adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol, was conducted. Our primary objectives were 1) to study the evolution of cerebral revascularization techniques in management of MMS in SCD and 2) to analyze the impact of neurosurgical intervention in this high-risk population. Four patients with SCD-associated MMS, who underwent indirect cerebral revascularization at our institute were retrospectively reviewed. A summary of 13 articles chronicling the advent and subsequent evolution of cerebral revascularization as a viable treatment strategy for stroke prevention in SCD-associated MMS is presented. The literature review suggests that early detection and surgical intervention (in addition to CTT) could significantly reduce stroke recurrence and improve neurocognitive outcome. Our short series of 4 patients also had a good outcome and no recurrence of strokes postoperatively. The literature emphasizes the use of a traditional standardized protocol for early identification (transcranial Dopplers, selective magnetic resonance angiography, and CTT). Early treatment and screening that involves early magnetic resonance angiography and referral to neurosurgery for revascularization may be considered for this high-risk population.
Surgical Revascularization for Pediatric Patients with Sickle Cell Disease and Moyamoya Disease in the Prevention of Ischemic Strokes: A Single-Center Case Series and a Systematic Review
2019, World Neurosurgery
Citation Excerpt :
However, this subset of patients is more prone to develop cerebral vasculopathy9 and although it is uncommon, they may develop moyamoya disease (MMD) over time, increasing the rate of recurrent ischemic insults compared with patients with SCD without MMD (57.9% vs. 28.0%; P < 0.05).10 For pediatric patients with SCD and MMD, the secondary prophylaxis for ischemic stroke remains uncertain but surgical revascularization has been shown to be a feasible alternative to decrease the rate of recurrent events in case reports and small case series.11-20 The present systematic review aimed to identify and analyze the available evidence on the safety and efficacy of surgical revascularization for pediatric patients with SCD in combination with MMD.
This systematic review aims to identify and analyze the available evidence on the safety and efficacy of surgical revascularization for pediatric patients with sickle cell disease (SCD) and moyamoya disease (MMD).
A systematic review was conducted following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The following databases were searched: PubMed, Ovid MEDLINE, and Scopus. Studies included in the review were original research articles in peer-reviewed journals in which individual participant data were available. The articles were thoroughly examined and compared on study design, outcomes, and results. The authors reviewed their institution's database to identify pediatric patients with SCD and MMD who underwent surgical revascularization and were included in the analysis.
A total of 53 patients were included and 82 hemispheres were intervened with direct or indirect surgical revascularization. Encephaloduroarteriosynangiosis (EDAS) was the most common procedure performed (42/82; 51.2%) followed by pial synangiosis (31/82; 37.8%). There was 1 intraprocedural complication. The median clinical follow-up was 37 months (interquartile range, 24.1–73.5 months) and during this period, 3 of 52 patients (5.8%) had ischemic strokes. All ischemic strokes occurred within the first 30 days after the surgery and the rate of ischemic stroke-free survival was 94.3% (95% confidence interval, 83.3–98.1). The estimated incidence rate of ischemic stroke was 1.42 events/100 patient-years (95% confidence interval, 0.46–4.4).
Our study suggests that surgical revascularization in pediatric patients with SCD and MMD is safe to perform and results in a low rate of future ischemic insults.
The Genetic Landscape of Cerebral Steno-Occlusive Arteriopathy and Stroke in Sickle Cell Anemia
2018, Journal of Stroke and Cerebrovascular Diseases
Citation Excerpt :
Chronic transfusion therapy prevents recurrent stroke in many individuals with SCA but is less effective in patients with existing SOA.13,14,24 Surgical revascularization may decrease stroke risk; however, its efficacy and optimal timing remain controversial.25-30 Transcranial Doppler ultrasonography is currently the only clinically validated screening tool for identifying individuals with SCA who are at higher risk of stroke.31-33
Sickle cell disease (SCD) is one of the most common autosomal recessive diseases in humans, occurring at a frequency of 1 in 365 African-American and 1 in 50 sub-Saharan African births. Despite progress in managing complications of SCD, these remain a major health burden worldwide. Stroke is a common and serious complication of SCD, most often associated with steno-occlusive cerebral arteriopathy, but little is known about its pathogenesis. Transcranial Doppler ultrasonography is currently the only predictive test for future development of stroke in patients with sickle cell anemia and is used to guide preventative treatment. However, transcranial Doppler ultrasonography does not identify all patients at increased risk for stroke, and progressive arteriopathy may occur despite preventative treatment. While sibling studies have shown a strong genetic contribution to the development of steno-occlusive arteriopathy (SOA) in SCD, the only genome-wide association study compared a relatively small cohort of 177 patients with stroke to 335 patients with no history of stroke. This single study detected variants in only 2 genes, ENPP1 and GOLGB1, and only one of these was confirmed in a subsequent independent study. Thus, the underlying genes and pathogenesis of SOA in SCD remain poorly understood, greatly limiting the ability to develop more effective preventive therapies. Dissecting the molecular causes of stroke in SCD will provide valuable information that can be used to better prevent stroke, stratify risk of SOA, and optimize personalized medicine approaches.
Antithrombotic therapy in neonates and children: Antithrombotic therapy and prevention of thrombosis, 9th ed: American college of chest physicians evidence-based clinical practice guidelines
2012, Chest
Neonates and children differ from adults in physiology, pharmacologic responses to drugs, epidemiology, and long-term consequences of thrombosis. This guideline addresses optimal strategies for the management of thrombosis in neonates and children.
The methods of this guideline follow those described in the Methodology for the Development of Antithrombotic Therapy and Prevention of Thrombosis Guidelines: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines.
We suggest that where possible, pediatric hematologists with experience in thromboembolism manage pediatric patients with thromboembolism (Grade 2C). When this is not possible, we suggest a combination of a neonatologist/pediatrician and adult hematologist supported by consultation with an experienced pediatric hematologist (Grade 2C). We suggest that therapeutic unfractionated heparin in children is titrated to achieve a target anti-Xa range of 0.35 to 0.7 units/mL or an activated partial thromboplastin time range that correlates to this anti-Xa range or to a protamine titration range of 0.2 to 0.4 units/mL (Grade 2C). For neonates and children receiving either daily or bid therapeutic low-molecular-weight heparin, we suggest that the drug be monitored to a target range of 0.5 to 1.0 units/mL in a sample taken 4 to 6 h after subcutaneous injection or, alternatively, 0.5 to 0.8 units/mL in a sample taken 2 to 6 h after subcutaneous injection (Grade 2C).
The evidence supporting most recommendations for antithrombotic therapy in neonates and children remains weak. Studies addressing appropriate drug target ranges and monitoring requirements are urgently required in addition to site- and clinical situation-specific thrombosis management strategies.
Antithrombotic therapy in neonates and children: American College of Chest Physicians evidence-based clinical practice guidelines (8th edition)
2008, Chest
This chapter about antithrombotic therapy in neonates and children is part of the Antithrombotic and Thrombolytic Therapy: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Grade 1 recommendations are strong and indicate that the benefits do, or do not, outweigh risks, burden, and costs, and Grade 2 suggests that individual patient values may lead to different choices (for a full understanding of the grading, see Guyatt et al in this supplement, pages 123S-131S). In this chapter, many recommendations are based on extrapolation of adult data, and the reader is referred to the appropriate chapters relating to guidelines for adult populations. Within this chapter, the majority of recommendations are separate for neonates and children, reflecting the significant differences in epidemiology of thrombosis and safety and efficacy of therapy in these two populations. Among the key recommendations in this chapter are the following: In children with first episode of venous thromboembolism (VTE), we recommend anticoagulant therapy with either unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH) [Grade 1B]. Dosing of IV UFH should prolong the activated partial thromboplastin time (aPTT) to a range that corresponds to an anti-factor Xa assay (anti-FXa) level of 0.35 to 0.7 U/mL, whereas LMWH should achieve an anti-FXa level of 0.5 to 1.0 U/mL 4 h after an injection for twice-daily dosing. In neonates with first VTE, we suggest either anticoagulation or supportive care with radiologic monitoring and subsequent anticoagulation if extension of the thrombosis occurs during supportive care (Grade 2C). We recommend against the use of routine systemic thromboprophylaxis for children with central venous lines (Grade 1B). For children with cerebral sinovenous thrombosis (CSVT) without significant intracranial hemorrhage (ICH), we recommend anticoagulation initially with UFH, or LMWH and subsequently with LMWH or vitamin K antagonists (VKAs) for a minimum of 3 months (Grade 1B). For children with non-sickle-cell disease-related acute arterial ischemic stroke (AIS), we recommend UFH or LMWH or aspirin (1 to 5 mg/kg/d) as initial therapy until dissection and embolic causes have been excluded (Grade 1B). For neonates with a first AIS, in the absence of a documented ongoing cardioembolic source, we recommend against anticoagulation or aspirin therapy (Grade 1B).
Hemoglobinopathy
2006, Pediatric Critical Care

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Case report: Watters FestschriftEncephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease

Abstract

Pediatr Neurol

J Pediatr

Surg Neurol

J Pediatr

Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain

Arch Neurol

Moyamoya disease—A review

Stroke

Moyamoya disease

Dev Med Child Neurol

Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults

Am J Roentgenol

Pial synangiosis for moyamoya syndrome in children

Pediatr Neurosurg

A familiar occurrence of moyamoya disease: Report of three Japanese families

J Neurol Neurosurg Psychiatry

Neurofibromatosis and intracranial arterial occlusive disease

Neuroradiology

Moyamoya and Fanconi's anemia

Pediatrics

Occlusion of large cerebral vessels in sickle cell anemia

N Engl J Med

Cerebral angiographic features in tuberculous meningitis

Neurology

Moyamoya disease caused by leptospiral cerebral arteritis

Chinese Med J

Familial moyamoya associated with biochemical abnormalities of connective tissue

Neurology

Case report: Watters Festschrift
Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease