Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease

Griessenauer, Christoph J.; Lebensburger, Jeffrey D.; Chua, Michelle; Fisher, Winfield S.; Hilliard, Lee; Bemrich-Stolz, Christina J.; Howard, Thomas H.; Johnston, James M.

doi:10.3171/2014.12.peds14522

Cited by 40 publications

(33 citation statements)

References 34 publications

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“…A range of revascularization procedures has become an option for patients with internal carotid artery occlusion and moyamoya collaterals. 85 Regrettably, no RCT has been introduced to rigorously assess standard care of regular blood transfusion therapy vs neurosurgery and regular blood transfusion therapy. Given the unknown risk-to-benefit ratio of alternative donor (haploidentical) or nonmyeloablative HSCT or revascularization procedures for secondary stroke prevention, we strongly recommend that single or preferably multi-institutional studies be registered with clinicaltrials.gov so we can collectively learn from these variations in practices with no clear superior strategy for secondary stroke prevention.…”

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confidence: 99%

Central nervous system complications and management in sickle cell disease

DeBaun

Kirkham²

2016

Blood

198

231

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With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.

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confidence: 99%

Central nervous system complications and management in sickle cell disease

DeBaun

Kirkham²

2016

Blood

198

231

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“…An alternative concern for the transfusion patients would be the hypothesis that patients on chronic transfusion have suffered a more severe clinical course that includes kidney damage. Similar to patients on chronic transfusion for stroke who, despite strict HbS control, continue to develop CNS vasculopathy and new strokes, these patients may represent a subset of patients who may require novel therapies or SCA transplant to prevent continued organ damage [43, 44]. …”

Section: Discussionmentioning

confidence: 99%

Evaluating risk factors for chronic kidney disease in pediatric patients with sickle cell anemia

et al. 2017

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Background Patients with Sickle Cell Anemia (SCA) have an increased prevalence of nephropathy and mortality from chronic kidney disease (CKD). Methods We evaluated the association of hyperuricemia and nocturnal hypertension with lower estimated glomerular filtration rate (eGFR) by cystatin-C among patients 10-21 years old with HbSS or SB0 thalassemia during a non-acute clinic visit. eGFR was obtained in 83 participants, uric acid in 81, and 24 hour ambulatory blood pressure monitoring (ABPM) was performed in 44 participants. Vital signs, CBC, CMP, LDH, medications, and urine microalbumin/creatinine were measured. Hyperuricemia was defined as a uric acid level ≥5.5 mg/dL. Nocturnal hypertension was defined as >25 % of nocturnal readings >95th percentile according to norms established by the American Heart Association Statement on ABPM in children and adolescents. Results The mean eGFR was statistically significantly lower among patients with hyperuricemia than with normal uric acid levels (143 vs 161 mL/min/1.73m2, respectively). Fourteen of 44 (32 %) participants had systolic nocturnal hypertension and 12 of 44 (27 %) had diastolic nocturnal hypertension. The mean eGFR was statistically significantly lower among participants with nocturnal systolic and diastolic hypertension than normal nocturnal blood pressure. In a regression model, nocturnal hypertension and hyperuricemia were associated with a lower eGFR. Conclusion Two risk factors for CKD, nocturnal hypertension and hyperuricemia, were associated with lower eGFR in older children and adolescent patients with SCA, and warrant long-term studies of their association with progression to CKD in this population.

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“…In this cohort study, both overt and silent stroke recurrence declined after cerebral revascularization surgery, providing further evidence that indirect cerebral revascularization is beneficial in children with SCD and moyamoya. Our report adds 12 cases of cerebral revascularization surgery in children with SCD, increasing the published total to 74 children, encompassing 118 cerebral hemispheres . In all of these series, overt stroke recurrence is decreased following revascularization surgery.…”

Section: Discussionmentioning

confidence: 91%

“…Our report adds 12 cases of cerebral revascularization surgery in children with SCD, increasing the published total to 74 children, encompassing 118 cerebral hemispheres. [9,13,[17][18][19][20] In all of these series, overt stroke recurrence is decreased following revascularization surgery. In addition, we provide the first indication that cerebral revascularization may reduce SCI recurrences in children receiving transfusion therapy.…”

Section: Discussionmentioning

confidence: 93%

Reduction in Overt and Silent Stroke Recurrence Rate Following Cerebral Revascularization Surgery in Children with Sickle Cell Disease and Severe Cerebral Vasculopathy

Hall

Leonard

Smith

et al. 2016

Pediatr Blood Cancer

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Background Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. Methods This was a retrospective cohort study of all children with SCD and moyamoya treated at two children’s hospitals. Clinical events and imaging studies were reviewed. Results Twenty-seven children with SCD and confirmed moyamoya receiving chronic transfusion therapy were identified, of whom 12 underwent indirect cerebral revascularization. Two subjects had post-operative transient ischemic attacks and another had a subarachnoid blood collection, none of which caused permanent consequences. Two subjects had surgical wound infections. Among these 12 children, the rate of overt and silent infarct recurrence decreased from 13.4 infarcts/100 patient-years before revascularization to 0 infarcts/100 patient-years after revascularization (p=0.0057); the post-revascularization infarct recurrence rate was also significantly lower than the overall infarct recurrence of 8.87 infarcts/100 patient-years in 15 children without cerebral revascularization (p=0.025). Conclusion The rate of overt and silent infarct recurrence was significantly lower following indirect cerebral revascularization. A prospective study of cerebral revascularization in children with SCD is needed.

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Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease

Cited by 40 publications

References 34 publications

Central nervous system complications and management in sickle cell disease

Central nervous system complications and management in sickle cell disease

Evaluating risk factors for chronic kidney disease in pediatric patients with sickle cell anemia

Reduction in Overt and Silent Stroke Recurrence Rate Following Cerebral Revascularization Surgery in Children with Sickle Cell Disease and Severe Cerebral Vasculopathy

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