Long‐term stroke risk in children with sickle cell disease screened with transcranial doppler

Adams, Robert J.; McKie, Virgil; Carl, Elizabeth; Nichols, Fenwick T.; Perry, R.E.; Brock, Katherine; McKie, Kathleen T.; Figueroa, Ramón; Litaker, Mark S.; Weiner, Steven J.; Brambilla, Donald

doi:10.1002/ana.410420505

Cited by 362 publications

(320 citation statements)

References 17 publications

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“…The cost-effectiveness, portability and repeatability of TCD make it an ideal neuroimaging technique, especially for patients with SCD who need regular monitoring for primary stroke prevention 2,5,6 . Patients with SCD are at risk for a spectrum of brain injuries such as subclinical infarction, acute ischemic stroke and hemorrhage due to the high level of circulating irreversibly-sickled red blood cells and their adherence to the vascular endothelium.…”

Section: Discussionmentioning

confidence: 99%

Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence

Rodrigues

Adegoke

Campos

et al. 2017

Arq. Neuro-Psiquiatr.

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Transcranial doppler (TCD) is a strategic component of primary stroke prevention in children with sickle cell disease (SCD). This study was conducted to examine the TCD characteristics of children with SCD in nine different medical centers in Brazil. Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121±23.83cm/s and 124±27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9.19±5.92 years) were evaluated with TCD for the first time. Conclusions: The SCD patients were evaluated with TCD at an older age, representing an important missed opportunity for stroke prevention. Since TCD screening in patients with SCD is important to detect those at high risk for stroke, it is recommended that this screening should be made more readily available.

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Section: Discussionmentioning

confidence: 99%

Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence

Rodrigues

Adegoke

Campos

et al. 2017

Arq. Neuro-Psiquiatr.

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“…One hundred ninety children were screened with TCD and followed for an average of 29 months; 23 had a TAMX >170 cm/s; strokes occurred in seven patients, including six among the 23 patients with abnormal TCD. This result was confirmed by a subsequent study [23] including 125 more children, which showed that a TAMX ≥200 cm/s in the terminal ICA, or in the MCA, indicates a 10% risk for stroke per year, compared to a 2% risk in patients with normal TCD.…”

Section: Indicationsmentioning

confidence: 57%

“…In STOP-I [18], the diagnostic distribution at initial TCD examinations was: 67% normal, 17.6% conditional, 9.3% abnormal, and 6% inadequate. In a French cohort [14], TCD was abnormal in 21% of SCA-patients at a median age of 3.2 years (range, 1.3-8.3).…”

Section: Indicationsmentioning

confidence: 94%

“…Most strokes are due to a macro-angiopathy affecting the terminal internal carotid arteries and proximal middle and anterior cerebral arteries, with smooth muscle hyperplasia and intimal fibrosis that lead to progressive stenosis and occlusion with moyamoya-like collateral development [17]. Chronic transfusions are effective in reducing the risk of (1) recurrent ischaemic stroke, and (2) a first stroke in children with HbSS who have abnormally high velocities on TCD [18].…”

Section: Indicationsmentioning

confidence: 99%

“…In the randomised multi-centre North American STOP Istudy (stroke prevention trial in sickle cell anaemia) [18], 1,934 HbSS or HbSB0 children from 2 to 16 years old were screened with TCD. Children with TAMX ≥200 cm/s in the MCA or ICA were randomised into two groups.…”

Section: Indicationsmentioning

confidence: 99%

See 2 more Smart Citations

Transcranial Doppler in children

Verlhac

2011

Pediatr Radiol

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Transcranial Doppler US, a non-invasive tool for evaluating the cerebral arteries, has evolved significantly during the last two decades. This review describes the practical procedure, and summarises and illustrates its established and "work-in-progress" indications in children. Indications for a transcranial Doppler US examination include, but are not limited to: (1) evaluation of cerebral blood flow velocities in the circle of Willis in patients with sickle cell anaemia to guide transfusion therapy; (2) diagnosis and follow-up of vasculopathy, such as moyamoya disease; (3) diagnosis and monitoring of acute cerebrovascular disorders in intensive care patients, in particular following traumatic brain injury, and during cardiovascular surgery; and (4) confirmation of a clinical diagnosis of brain death by documentation of cerebral circulatory arrest.

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Big Strokes in Small Persons

Adams¹

2007

Arch Neurol

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S ickle cell disease (SCD) is understood on a genetic and a molecular level better than most diseases. Young children with SCD are at a very high risk of stroke. The molecular pathologic abnormalities of SCD lead to microvascular occlusion and intravascular hemolytic anemia. Microvascular occlusion is related to painful episodes and probably causes microcirculatory problems in the brain. The most commonly recognized stroke syndrome in children with SCD is large-artery infarction. These "big strokes" are the result of a vascular process involving the large arteries of the circle of Willis leading to territorial infarctions from perfusion failure or possibly artery-to-artery embolism. We can detect children who are developing cerebral vasculopathy using transcranial Doppler ultrasonography (TCD) and can provide effective intervention. Transcranial Doppler ultrasonography measures blood flow velocity in the large arteries of the circle of Willis. Velocity is generally increased by the severe anemia in these patients, and it becomes elevated in a focal manner when stenosis reduces the arterial diameter. Children with SCD who are developing high stroke risk can be detected months to years before the stroke using TCD. Healthy adults have a middle cerebral artery velocity of approximately 60 cm/s, whereas children without anemia have velocities of approximately 90 cm/s. In SCD, the mean is approximately 130 cm/s. Two independent studies have demonstrated that the risk of stroke in children with SCD increases with TCD velocity. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) (1995-2000) was halted prematurely when it became evident that regular blood transfusions produced a marked (90%) reduction in first stroke. Children were selected for STOP if they had 2 TCD studies with velocities of 200 cm/s or greater. Children not undergoing transfusion had a stroke risk of 10% per year, which was reduced to less than 1% per year by regular blood transfusions. Stroke risk in all children with SCD is approximately 0.5% to 1.0% per year. On the basis of STOP, if the patient meets the high-risk TCD criteria, regular blood transfusions are recommended. A second study was performed (2000-2005) to attempt withdrawal of transfusion in selected children in a randomized controlled study. Children with initially abnormal TCD velocities (Ն200 cm/s) treated with regular blood transfusion for 30 months or more, which resulted in reduction of the TCD to less than 170 cm/s, were eligible for randomization into STOP II. Half continued transfusion and half had cessation of transfusion. This trial was halted early for safety reasons. There was an unacceptably high rate of TCD reversion back to high risk (Ն200 cm/s), as well as 2 strokes in children who discontinued transfusion. There are no evidence-based guidelines for the discontinuation of transfusion in children once they have been identified as having high risk based on TCD. The current situation is undesirable because of the longterm effects of transfusion, including iron overload. ...

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Long‐term stroke risk in children with sickle cell disease screened with transcranial doppler

Cited by 362 publications

References 17 publications

Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence

Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence

Transcranial Doppler in children

Big Strokes in Small Persons

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