scholarly journals An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective

2019 ◽  
Vol 9 (1) ◽  
pp. 44
Author(s):  
Baba P. D. Inusa ◽  
Laura Sainati ◽  
Corrina MacMahon ◽  
Raffaella Colombatti ◽  
Maddalena Casale ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Doppler Ultrasound ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Risk ◽  
Transcranial Doppler Ultrasound ◽  
Ultrasound Screening ◽  
Cell Disease ◽  
Risk Distribution ◽  
The Impact

Background: Effective stroke prevention in sickle cell disease (SCD) is recommended for children with sickle cell anaemia. Effective implementation relies on the correct stratification of stroke risk using Transcranial Doppler Ultrasound (TCD), prior to committing children to long-term treatment with transfusion. Nevertheless, less than 50% of children with SCD in Europe receive annual TCD—one of the reasons being a lack of trained personnel. The present European multi-centre study was designed to promote the standardisation and delivery of effective screening. Methods: Fifty-five practitioners from differing professional backgrounds were recruited to the TCD training program. The impact of the training programme was evaluated in three European haematology clinics by comparing stroke risk classification and middle cerebral artery time-averaged maximum velocity (TAMMV) obtained from a cohort of 555 patients, before and after training. Results: 42% (23/55) of trainees successfully completed the program. The TAMMV, used to predict stroke risk at each Centre, demonstrated the highest values in Centre 3 (p < 0.0001) before training. The imaging-TCD TAMMV was also higher in Centre 3 (p < 0.001). Following training, the TAMMV showed closer agreement between centres for both imaging-TCD and non-imaging TCD. The stroke risk distribution of children at each centre varied significantly before training (p < 0.001), but improved after training (Fisher’s Exact: no treatment = 5.6, p = 0.41, treatment = 13.8, p < 0.01). The same consistency in stroke risk distribution following training was demonstrated with both non-imaging and imaging-TCD data. Conclusion: The attainment of competency in stroke screening using transcranial Doppler scanning (TCD) in sickle cell disease is more feasible for professionals with an ultrasound imaging background. A quality assurance (QA) system is required to ensure that standards are maintained. Further work is in progress to develop an achievable and reproducible QA program.

Transcranial Doppler Ultrasound in Peninsular Arab Patients With Sickle Cell Disease

2018 ◽  
Vol 38 (1) ◽  
pp. 165-172 ◽  
Author(s):  
Adekunle Adekile ◽  
Meaad Hassan ◽  
Akram Asbeutah ◽  
Mohamed Al‐Hinai ◽  
Omar Trad ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Doppler Ultrasound ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Transcranial Doppler Ultrasound ◽  
Cell Disease

Transcranial Doppler Ultrasound Velocity, Cerebral Vasculopathy, and Silent Infarcts In Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 269-269
Author(s):  
Peter M.K. de Blank ◽  
Daniel M Hayward ◽  
Robert Zimmerman, MD ◽  
Avrum Pollock ◽  
Janet L Kwiatkowski
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Doppler Ultrasound ◽  
Sickle Cell ◽  
Cerebral Artery ◽  
Transcranial Doppler ◽  
Transcranial Doppler Ultrasound ◽  
Cell Disease ◽  
Increased Risk ◽  
The Mean

Abstract Abstract 269 Background: Approximately one-fourth of children with sickle cell disease (SCD), type SS, show evidence of cerebral ischemia on magnetic resonance imaging (MRI) without overt neurologic symptoms. Children with these silent infarcts have an increased risk of neuropsychological abnormalities and overt stroke. The pathophysiology of silent infarcts is unclear. Elevated transcranial Doppler ultrasound (TCD) velocities in the internal cerebral artery (ICA) and middle cerebral artery (MCA) are associated with an increased risk of overt stroke, but have not been associated previously with silent infarct. However, prior studies of silent infarcts failed to examine the association with anterior cerebral artery (ACA) vessel abnormalities, despite a predominantly frontal distribution of these infarcts. In addition, the relationship of magnetic resonance angiography (MRA) abnormalities to silent infarcts has not been extensively studied, although children with abnormal TCD velocity who also have stenosis or occlusion of vessels by MRA have the highest risk of overt stroke. We hypothesized that elevated ACA velocity and/or significant vasculopathy of the cerebral vessels demonstrated by MRA would be associated with a higher risk of silent stroke. Methods: A retrospective analysis of children followed at our Sickle Cell Center with SCD, type SS or Sb0-thalassemia was performed. Children with TCD (with ACA velocity) and brain MRI/A performed within a year of each other were included. TCD studies performed while on chronic transfusions were excluded. The last eligible MRI/TCD combination was used for patients who had multiple studies. Laboratory values obtained within a year of the MRI also were analyzed. Results: Of the 254 eligible subjects, 54% were male and the mean age was 10.6 ± 5.2 years. Silent infarcts were present in 78/254 (30.7%); the location was frontoparietal in 68%. The mean time-averaged mean of the maximal velocity (TAMMvel) of qualifying STOP vessels (MCA, bifurcation, and ICA) was 139±35cm/s, while the mean TAMMvel of the ACA was 117±34cm/s, which is 84% of the velocity of the other anterior vessels. As previously reported, TAMMvel inversely correlated with age (r=-0.40, p<0.0001) and hemoglobin concentration (r=-0.30, p<0.0001). There was no significant difference in TAMMvel in STOP qualifying vessels (MCA, bifurcation, DICA; 137cm/s vs. 145cm/s, p=0.08) among those with and without silent infarct. However, silent infarcts were associated with abnormal TAMMvel (≥200cm/s, 69/239 with normal/conditional vs. 9/15 with abnormal TAMMvel p=0.01) in these vessels. TAMMvel in the ACA was significantly higher (125 cm/s vs. 113 cm/s, p=0.004) in children with silent infarcts, and elevated ACA TAMMvel (≥170cm/s) was associated with silent infarcts (70/242 with normal vs. 8/12 with elevated velocity, p=0.006). No other single vessel velocity was significantly associated with silent infarct. Abnormal ICA/MCA TAMMvel was associated with stenosis of these vessels by MRA (p<0.001), and abnormal ACA velocities were associated with ACA stenosis by MRA (p<0.001). Further, stenosis by MRA in the ICA/MCA was associated with silent infarct (p<0.006) as were abnormalities of the ACA vessels (p=0.001). Conclusions: Unlike prior studies, we demonstrate a significant association between abnormal ICA/MCA velocity and silent infarcts. We also show an association between ACA velocity and silent infarct, which may in part be due to the predominantly frontoparietal distribution of these lesions. This, together with the association of MRA abnormalities of all three anterior vessels (ICA, MCA, ACA) with silent infarcts suggests a possible role of larger cerebral vessel vasculopathy in the pathophysiology of silent infarcts. This could be due to decreased distal blood flow related to the larger vessel narrowing, embolization of thrombus in larger vessels, or small vessel vasculopathy associated with larger vessel disease. However, mechanisms other than larger vessel vasculopathy are also likely to be involved given that silent infarcts occur in a substantial number of children without elevated TCD velocity or vasculopathy by MRA. Nonetheless, assessment of TCD velocity and MRA abnormalities may help provide information on risk assessment for CNS disease in children with SCD. Disclosures: No relevant conflicts of interest to declare.

PO12-TU-02 Transcranial Doppler ultrasound in sickle cell disease: an experience from Oman

2009 ◽  
Vol 285 ◽  
pp. S222-S223
Author(s):  
G.R. Gujjar ◽  
Z. Lamki ◽  
Y. Wali ◽  
A. AlAsmi ◽  
A. Pathare ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Doppler Ultrasound ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Transcranial Doppler Ultrasound ◽  
Cell Disease

scholarly journals Transcranial Doppler screening for stroke risk in children with sickle cell disease: a systematic review

2017 ◽  
Vol 12 (6) ◽  
pp. 580-588 ◽  
Author(s):  
Sara Mazzucco ◽  
Marina Diomedi ◽  
Amrana Qureshi ◽  
Laura Sainati ◽  
Soundrie T Padayachee
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Risk ◽  
Screening Methods ◽  
Cell Disease ◽  
High Quality ◽  
Screening Practices

Background Sickle cell disease (SCD) is one of the most common causes of stroke in children worldwide. Based on the results of the Stroke Prevention Trial in Sickle Cell Anemia (STOP), annual transcranial Doppler ultrasound (TCD) screening for affected children is standard practice. However, the need for TCD surveillance programs could override the accuracy of the screening, affecting the correct stratification of stroke risk and subsequent clinical management of the target population. Aims To shed light on this issue, a systematic review of the literature on TCD screening for children and adolescents with SCD was carried out (CRD42016050549), according to a list of clinically relevant questions, with a particular focus on screening practices in European countries. Quality of the evidence was rated using the grading of recommendations assessment, development and evaluation. Summary of review Thirty-three studies published in English or French were included (5 randomized controlled trials, 8 experimental non-randomized, and 20 observational studies). The quality of the retrieved evidence ranged between low and high, but was rated as moderate or high most of the times. TCD is effective as a screening tool for the primary prevention of stroke in SCD children. There is no high-quality evidence on the effectiveness of alternative screening methods, such as imaging-TCD with or without angle correction or magnetic resonance angiography. No evidence was found on effectiveness of the screening on children on hydroxyurea and with genotypes other than HbSS and HbS/β0. No European data were found on screening rates or adherence of screening practices to the STOP protocol. Conclusions High-quality studies on alternative screening methods that are currently used in real-world practice, and on screening applicability to specific subgroups of patients are urgently needed. Considering the low awareness of the disease in European countries and the lack of data on screening practices and adherence, clinicians need up-to-date guidelines for more uniform and evidence-based surveillance of children with SCD.

Transcranial Doppler scanning and the assessment of stroke risk in children with haemoglobin sickle cell disease

2007 ◽  
Vol 93 (2) ◽  
pp. 138-141 ◽  
Author(s):  
Colin R Deane ◽  
David Goss ◽  
Sandra O’Driscoll ◽  
Sarah Mellor ◽  
Keith R E Pohl ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Risk ◽  
Cell Disease

Transcranial Doppler Screening Program Is Effective in Preventing Stroke in Children with Sickle Cell Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 714-714 ◽  
Author(s):  
Henrietta Enninful-Eghan ◽  
Renee H Moore ◽  
Rebecca Ichord ◽  
Janet L Kwiatkowski
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Screening Program ◽  
Treatment Protocol ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Neurological Event ◽  
The Impact

Abstract In the Cooperative Study of Sickle Cell Disease the incidence of stroke in SCD-SS was estimated to be 0.61 per 100 patient-years. Since that study, the use of transcranial Doppler ultrasonography (TCD) has become routine to detect children at high risk of stroke and regular transfusions have been shown to reduce the risk of stroke by over 95% in those with abnormal TCD studies. The impact of TCD screening on the overall incidence of stroke in children with SCD has not been studied extensively. We sought to determine the impact of our TCD screening and treatment protocol on the incidence of first stroke in a cohort of children followed at our Sickle Cell Center. Routine TCD screening was instituted at our Center in Oct, 1998. Our protocol includes annual TCD studies for children with normal TCD results (<170 cm/s), repeat study every 3 to 6 months in those with conditional results (170–199 cm/s), and within 1–4 weeks for children with abnormal results (≥200 cm/s). Chronic transfusion therapy is recommended for patients with confirmed abnormal TCD velocities. In the current study, the rate of stroke in the 8-y period prior to TCD screening (Sept 1, 1990-Aug 31, 1998 – Pre-TCD) was compared to the rate in the 8-y period after TCD screening began (Sept 1, 1998 – Aug 31, 2006 – Post TCD). Eligible subjects were patients less than 22 years old with a diagnosis of SCD-SS or SCD-Sβ0-thalassemia. Subjects with a history of stroke prior to Sept, 1990 or before enrollment in our Center were excluded. Cases of stroke or other neurological event were identified from our clinical database. The study neurologist reviewed all clinical data and radiological studies for each neurological event and classified events into one of the following categories: overt stroke - ischemic (neurological deficit conforming to a vascular territory with neuroimaging studies corresponding to the clinical deficit) or hemorrhagic not overt stroke (other neurological event), and indeterminate. Incidence rates for stroke were calculated and compared between the Pre and Post TCD groups using a test of binomial proportions. Subjects were followed until they had a stroke or neurological event, turned 22 years old, the end of the 8-y period or until the last clinic date. The pre-TCD group included 475 children with a total follow-up time of 3,137 person-years. Twenty-one patients had overt stroke, 3 had other neurologic events (1-seizure, 1-transient ischemic attack/syncope, 1-behavioral changes) and 2 were indeterminate. The post-TCD group included 530 children with 3,578 person-years follow-up. Two patients had overt stroke, 6 had other neurological events [1-diffuse encephalopathy with viral syndrome, 1-febrile seizure, 3-dizzy and/or syncope (one with hgb=2.7), 1-headache with <30 min arm/leg weakness – all with acute punctate infarcts whose location did not correspond to clinical presentation], and 1 was indeterminate. The incidence of overt stroke in the pre-TCD period was 0.67 per 100 person-years, compared with an incidence of 0.06 per 100 person-years in the post-TCD period (p < 0.001). The first stroke case in the post-TCD period was a 3.4 year-old with ACA velocities > 200 cm/s but no abnormal velocities in the ICA/MCA and the second occurred in a 1.2 year-old, prior to the age that screening is started. Thus, our TCD screening and treatment program has been successful in reducing the rate of first overt stroke, although small vessel ischemia, particularly in the setting of an additional insult such as severe anemia, may not be prevented. Further modifications such as the addition of ACA velocity to treatment criteria, earlier screening, or the addition of other neuroimaging studies might further reduce the risk of first stroke.

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