Abstract W P421: Trend in Concurrent Hospitalization for Moyamoya and Sickle Cell Disease: An Analysis of Nationwide Inpatient Sample Data

BACKGROUND: The association between Moyamoya disease and sickle cell disease is well recognized in the literature. However, there is limited data on inpatient admission of concurrent sickle cell disease and Moyamoya disease. We sought to determine the trend in incidence of admissions of concurrent Moyamoya and sickle cell disease as well as the most common presentation of these admissions. METHODS: We reviewed the Healthcare Cost and Utilization Project's Nationwide Inpatient Sample (NIS) database from 2000-2011 for concurrent Moyamoya and sickle cell admissions using the ICD 9-CM codes We obtained data on gender, clinical presentation, procedures, co-morbidities and patient outcomes RESULTS: From 2000 to 2011, an estimated patients 756 (weighted (n)=3692) with co-existing Moyamoya disease and sickle cell disease were admitted. The incidence of admission for concurrent disease increased significantly from 0.04/100,000 admissions in 2000 to 0.21/100,000 admissions in 2011(figure 1). This was very significant using the Cochrane Armitage trend test(p<0.001). The most likely reasons for admissions were ischemic stroke(7.2)% followed by hemorrhagic stroke(2.8 and transient ischemic attack(1.2%) (p = 0.0116). The most commonly performed treatment procedures included packed cell transfusion(33.7%) followed by exchange transfusion (8.8%). However there was no significant change in mortality from 2000-2011. CONCLUSION: The number of hospitalizations due to concurrent Moyamoya and sickle cell disease has increased significantly over the last decade and are likely to present with a cerebrovascular accident. Thus, it is important to maintain a high degree of suspicion for Moyamoya disease in sickle cell disease patients presenting with neurological symptoms since this could potentially impact their management.

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Case Report: Acute Stroke in Young Adult Patient with Moyamoya Syndrome Secondary to Sickle Cell Disease

Blood ◽

10.1182/blood-2020-134202 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 13-13

Author(s):

Oladipo Cole ◽

Asia Filatov ◽

Javed Khanni ◽

Patricio Espinosa

Keyword(s):

Case Report ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Moyamoya Disease ◽

Conflicts Of Interest ◽

Acute Chest Syndrome ◽

African American Female ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Radiographic Findings

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.

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Five years of experience with hydroxyurea in children and young adults with sickle cell disease

Blood ◽

10.1182/blood.v97.11.3628 ◽

2001 ◽

Vol 97 (11) ◽

pp. 3628-3632 ◽

Cited By ~ 132

Author(s):

Alina Ferster ◽

Parvine Tahriri ◽

Christiane Vermylen ◽

Geneviève Sturbois ◽

Francis Corazza ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Laboratory Data ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Significant Difference ◽

History Of ◽

Ischemic Attack

The short-term beneficial effect of hydroxyurea (HU) in sickle cell disease (SCD) has been proven by randomized studies in children and adults. The Belgian registry of HU-treated SCD patients was created to evaluate its long-term efficacy and toxicity. The median follow-up of the 93 patients registered is 3.5 years; clinical and laboratory data have been obtained for 82 patients at 1 year, 61 at 2 years, 44 at 3 years, 33 at 4 years, and 22 after 5 years. On HU, the number of hospitalizations and days hospitalized dropped significantly. Analysis of the 22 patients with a minimum of 5 years of follow-up confirm a significant difference in the number of hospitalizations (P = .0002) and days in the hospital (P < .01), throughout the treatment when compared to prior to HU therapy. The probabilities of not experiencing any event or any vaso-occlusive crisis requiring hospitalization during the 5 years of treatment were, respectively, 47% and 55%. On HU, the rate per 100 patient-years of severe events was estimated to be 3.5% for acute chest syndrome, 1.2% for aplastic crisis, 0.4% for splenic sequestration; it was 0% for the 9 patients with a history of stroke or transient ischemic attack followed for an average of 4 years. No important adverse effect occurred. Long-term chronic treatment with HU for patients with SCD appears feasible, effective, and devoid of any major toxicity; in patients with a history of stroke, HU may be a valid alternative to chronic transfusion support.

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Treatment of a cerebral pial arteriovenous fistula in a patient with sickle cell disease-related moyamoya syndrome: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.12.peds14486 ◽

2015 ◽

Vol 16 (2) ◽

pp. 207-211 ◽

Cited By ~ 3

Author(s):

Anna Lo Presti ◽

Alexander G. Weil ◽

Aria Fallah ◽

Eric C. Peterson ◽

Toba N. Niazi ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Moyamoya Disease ◽

De Novo ◽

Transarterial Embolization ◽

Management Strategies ◽

Superficial Temporal Artery ◽

Temporal Artery ◽

Venous Aneurysm ◽

Cell Disease

Sickle cell disease (SCD) is an autosomal recessive hematological disorder, characterized by sickling of the red blood cells in response to a hypoxic stress and vaso-occlusive crises. It is associated with moyamoya-like changes on cerebral angiographic imaging in 43% of patients. Cerebral aneurysms, arteriovenous malformations, and dural arteriovenous fistulas (AVFs) have been described in association with SCD and moyamoya disease. However, the description of a pial AVF (pAVF) in a patient with SCD and/or moyamoya formation has not yet been reported. The authors present the case of a 15-year-old boy with SCD-associated moyamoya disease harboring a pAVF who developed a de novo venous aneurysm 8 months after undergoing indirect superficial temporal artery-middle cerebral artery (MCA) bypass that was complicated by bilateral ischemia of the MCA territory. The pAVF was successfully treated with transarterial embolization using Onyx. The authors describe the possible pathophysiological mechanisms and management strategies for this rare occurrence.

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Moyamoya Disease Predicts Progression of Cerebral Vasculopathy in Patients with Sickle Cell Disease Despite Chronic Transfusion Therapy

Blood ◽

10.1182/blood.v126.23.2071.2071 ◽

2015 ◽

Vol 126 (23) ◽

pp. 2071-2071 ◽

Cited By ~ 2

Author(s):

Alyssa M Schlenz ◽

Michael U Antonucci ◽

Rebecca Cafiero ◽

Nina-Serena Burkett ◽

Julie Kanter

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Moyamoya Disease ◽

Stroke Prevention ◽

Conflicts Of Interest ◽

The Body ◽

Cell Disease ◽

Transfusion Therapy ◽

Moya Moya Disease ◽

History Of

Abstract Introduction: Patients with sickle cell disease (SCD) develop multi-organ complications due to hemolysis, inflammation, and vascular occlusion that results in small vessel obstruction throughout the body. In the brain, however, large cerebral vessels are also damaged resulting in occlusion or stenosis and subsequent development of abnormal collateral vasculature and moyamoya disease. Chronic red cell transfusion (CRCT) therapy significantly reduces the risk of stroke in children with abnormal transcranial doppler (TCD) studies and is also effective in reducing stroke recurrence in those with a history of overt or silent stroke; however, it is unclear if CRCT halts or reverses the progression of vasculopathy. The present study evaluated cerebrovascular stenosis and moya moya disease as risk factors for progression of vasculopathy over time in a cohort of patients with SCD who were started on CRCT therapy as children for stroke prevention. Methods: A retrospective cohort study (with IRB approval) was used to evaluate cerebrovascular changes in patients on CRCT.Patients were included in the study if they had received CRCT for stroke prevention for at least 12 months and had at least two serial magnetic resonance imaging and angiography (MRI/MRA) studies for review. For the imaging analysis, the patient's MRI/MRA closest to the initiation of CRCT (i.e. baseline imaging) was compared to the most recent imaging available by a neuro radiologist who was blind to the patient's clinical history. Additional demographic information included the patient's current age, gender, indication for CRCT, years on CRCT, and laboratory results for pre-transfusion % hemoglobin S (HbS). Results: Forty patients with SCD (current age: M = 16.48, SD = 5.10; 23 male, 17 female) were included. Average duration of CRCT therapy was 9.96 years (SD = 5.67) and average pre-transfusion HbS levels were 42.52% (SD = 9.88). Patients were initiated on therapy due to: overt stroke (n = 19), silent stroke (n = 2), and abnormal TCD (n = 20). Of the 20 patients initiated on therapy due to abnormal TCD, 7 were found to have abnormal MRI at baseline consistent with silent stroke. One of these patients was also found to have co-occurring moyamoya disease, despite no evidence of prior overt stroke. At baseline, 45% (n = 18) of patients had abnormal MRA and 25% (n = 10) had moyamoya disease. Progression of vasculopathy occurred in 15% (n = 6) of patients, all of whom had a history of moya moya disease at baseline (5 patients with overt stroke and 1 with silent stroke). Of the remaining 3 patients with moya moya disease at baseline, 2 remained stable with no improvement and 1 demonstrated improvement on MRA. For patients with abnormal MRA, but no history of moya moya disease (n = 9), 5 demonstrated improvement (2 patients with silent stroke and 3 with overt stroke). Conclusions: Progression of vasculopathy was common among patients with baseline moyamoya disease despite CRCT. Also notable, however, was improvement in vasculopathy (as defined by reduction of stenosis) in 6 patients, the majority of whom had not developed moyamoya prior to the initiation of CRCT suggesting that more mild vasculopathy can be reversed with early intervention. Patients with moya moya disease warrant ongoing annual assessment as they may require vascular bypass to prevent further worsening. Future large, multi-site investigations are needed to identify improved biomarkers and further understand characteristics of patients who demonstrate improvement versus progression of vasculopathy on CRCT. Disclosures No relevant conflicts of interest to declare.

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Successful Regional Anesthetic for a Parturient with Moyamoya Syndrome

Case Reports in Anesthesiology ◽

10.1155/2020/1785041 ◽

2020 ◽

Vol 2020 ◽

pp. 1-3

Author(s):

Anjalena Pasam ◽

Akshatha Kamath ◽

Hattiangadi Sangeetha Kamath ◽

Joel Yarmush ◽

Khaja Ahmed

Keyword(s):

Sickle Cell Disease ◽

Cesarean Section ◽

Sickle Cell ◽

Moyamoya Disease ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Careful Monitoring ◽

The Usa ◽

Spinal Epidural ◽

Regional Anesthetic

Anesthesia for Cesarean section could be challenging due to the physiological changes during pregnancy, but it can be more complicated if associated with sickle cell disease and moyamoya disease. The moyamoya syndrome is nothing but sickle cell disease complicated by cerebral vasculopathy. Incidence of moyamoya disease in the USA is 0.086/100,000 people. We report a case of a pregnant woman with sickle cell disease and moyamoya syndrome, who underwent a successful spinal epidural for primary cesarean section, with careful monitoring of blood pressure.

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Response to Letter to the Editor: Impact of Bariatric Surgery on Outcomes of Patients with Sickle Cell Disease: a Nationwide Inpatient Sample Analysis, 2004–2014

Obesity Surgery ◽

10.1007/s11695-019-03956-8 ◽

2019 ◽

Vol 29 (8) ◽

pp. 2624-2624 ◽

Cited By ~ 2

Author(s):

Thomas R. McCarty ◽

Prabin Sharma ◽

Siddhartha Yadav ◽

Julius N. Ngu ◽

Basile Njei

Keyword(s):

Bariatric Surgery ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Nationwide Inpatient Sample ◽

Cell Disease ◽

Sample Analysis ◽

Letter To The Editor

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Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events

Blood ◽

10.1182/blood.v99.9.3144 ◽

2002 ◽

Vol 99 (9) ◽

pp. 3144-3150 ◽

Cited By ~ 171

Author(s):

Scott R. Dobson ◽

Kenton R. Holden ◽

Paul J. Nietert ◽

Joel K. Cure ◽

Joseph H. Laver ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Proportional Hazards ◽

Recurrent Stroke ◽

Neuropsychological Testing ◽

Cell Disease ◽

Conventional Angiography ◽

Risk Of Recurrence ◽

Cerebrovascular Events ◽

Ischemic Attack

Abstract We conducted a retrospective study to determine whether the presence of moyamoya collaterals influenced the risk of recurrence of cerebrovascular events (CVEs: stroke or transient ischemic attack) in patients with sickle cell disease placed on chronic transfusions after a stroke. Forty-three patients with homozygous sickle cell anemia (HbSS) and 1 with HbSOArab (16 females, 28 males) who had suffered strokes while under the age of 18 were studied. All patients had been on transfusions aimed at maintaining the sickle hemoglobin (HbS) level below 30%. They were followed for a mean of 6.6 years (2.2 to 20.4 years). The presence of collaterals was diagnosed based on either magnetic resonance angiography or conventional angiography. Eighteen (41%) of the 44 patients suffered recurrent CVEs. Nineteen (43%) (6 females, 13 males) patients had moyamoya collaterals. Eleven (58%) of these 19 experienced 21 total recurrent CVEs, including 4 strokes in 4 patients (21%). In comparison, 7 (28%) of 25 patients without moyamoya collaterals experienced 9 recurrent CVEs (P < .05) with only 1 recurrent stroke (4%). Moyamoya patients were also more likely to have 2 recurrent CVEs (42% vs 8%,P < .05) as well as poorer neuropsychological testing results. A proportional hazards regression analysis indicated that patients with moyamoya were more than twice as likely to incur a subsequent CVE (hazard ratio, 2.40; 95% confidence interval, 0.85, 6.75). We conclude that up to 41% of patients with sickle cell disease experience recurrent CVEs after an initial stroke despite chronic transfusions and that the risk of recurrence is significantly higher for those who have moyamoya collaterals.

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