Acute stroke in sickle cell disease
- Alex George, MD, PhD
Alex George, MD, PhD
- Assistant Professor of Pediatrics
- Baylor College of Medicine
- Section Editors
- Donald H Mahoney, Jr, MD
Donald H Mahoney, Jr, MD
- Section Editor — Pediatric Hematology
- Professor of Pediatrics
- Baylor College of Medicine
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
Stroke is a common and potentially devastating manifestation of sickle cell disease (SCD) that can affect children and adults. Challenges in management include distinguishing acute stroke from other cerebrovascular manifestations of the disease such as meningitis, cerebral malaria, or seizure disorder, and distinguishing acute ischemic stroke from hemorrhagic stroke. Care of the patient with an acute stroke requires specialized expertise in exchange transfusion practices.
This topic discusses an approach to the acute assessment and treatment of stroke in children and adults with SCD.
Risk stratification and primary stroke prevention, as well as secondary prevention for individuals with SCD who have had an acute stroke or transient ischemic attack (TIA) are presented in detail separately. (See "Prevention of stroke (initial or recurrent) in sickle cell disease".)
DIAGNOSIS AND IMMEDIATE MANAGEMENT
Individuals with SCD are at risk of ischemic and hemorrhagic stroke, especially those with HbSS or HbS-beta0 thalassemia. It has been estimated that without intervention, 11 percent of patients with SCD will have a clinically apparent stroke by age 20 years, and one-fourth of patients with SCD will have a stroke by age 45. Stroke risk factors and epidemiology in patients with SCD are presented separately. (See "Prevention of stroke (initial or recurrent) in sickle cell disease".)
The diagnosis of suspected stroke involves a rapid initial clinical assessment accompanied by neuroimaging to differentiate ischemia from hemorrhage and to exclude stroke mimics.
- Kassim AA, Galadanci NA, Pruthi S, DeBaun MR. How I treat and manage strokes in sickle cell disease. Blood 2015; 125:3401.
- Hulbert ML, Scothorn DJ, Panepinto JA, et al. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. J Pediatr 2006; 149:710.
- http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines/index.htm (Accessed on September 30, 2014).
- Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014; 312:1033.
- Kernan WN, Ovbiagele B, Black HR, et al. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2014; 45:2160.
- Amlie-Lefond C, Rivkin MJ, Friedman NR, et al. The Way Forward: Challenges and Opportunities in Pediatric Stroke. Pediatr Neurol 2016; 56:3.
- Strouse JJ, Lanzkron S, Urrutia V. The epidemiology, evaluation and treatment of stroke in adults with sickle cell disease. Expert Rev Hematol 2011; 4:597.
- Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998; 91:288.
- Powars D, Wilson B, Imbus C, et al. The natural history of stroke in sickle cell disease. Am J Med 1978; 65:461.
- Adams RJ, Nichols FT. Sickle cell anemia, sickle cell trait and thalassemia. In: Handbook of Clinical Neurology, Vascular Disease Part III, Vinken PJ, Bruyn GW, Klawans HL (Eds), Elsevier, Amsterdam 1989. p.503.
- Powars D, Adams RJ, Nichols FT, et al. Delayed intracranial hemorrhage following cerebral infarction in sickle cell anemia. J Assoc Acad Minor Phys 1990; 1:79.
- Adams RJ. Neurologic complications. In: Sickle Cell Disease: Basic Principles and Clinical Practice, Embury SH, Robert P, Hebbel RP, et al (Eds), Raven Press, Ltd, New York 1994. p.599.
- Resar LM, Oliva MM, Casella JF. Skull infarction and epidural hematomas in a patient with sickle cell anemia. J Pediatr Hematol Oncol 1996; 18:413.
- Oyesiku NM, Barrow DL, Eckman JR, et al. Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg 1991; 75:356.
- Anson JA, Koshy M, Ferguson L, Crowell RM. Subarachnoid hemorrhage in sickle-cell disease. J Neurosurg 1991; 75:552.
- Overby MC, Rothman AS. Multiple intracranial aneurysms in sickle cell anemia. Report of two cases. J Neurosurg 1985; 62:430.
- DIAGNOSIS AND IMMEDIATE MANAGEMENT
- Clinical assessment
- Immediate management (all patients)
- Laboratory and other testing
- ISCHEMIC STROKE - ADDITIONAL MANAGEMENT
- Post-diagnosis evaluation
- INTRACRANIAL HEMORRHAGE - ADDITIONAL MANAGEMENT
- Treat bleeding
- Additional therapy according to type of lesion
- SUMMARY AND RECOMMENDATIONS