Quantitative Analysis of Extracranial Arterial Tortuosity in Patients with Sickle Cell Disease

Buch, Karen; Arya, Rahul; Shah, Bhavya; Nadgir, Rohini N.; Saito, Naoko; Qureshi, Muhammad M.; Sakai, Osamu

doi:10.1111/jon.12418

Cited by 16 publications

(12 citation statements)

References 32 publications

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“…However, the majority of SCD-related intra-cerebral and subarachnoid hemorrhages are associated not with collateral vessel rupture, but with aneurysm rupture (40, 41). Intracerebral aneurysms are also prevalent in SCD patients (25, 41), and tortuosity and ectasia are well-documented in humans and animal models (42–45). Whilst aneurysms are not significantly associated with collateral vessel formation (46) they do appear to form in the context of progressive vasculopathy, with a majority of patients with aneurysms having more than one (47).…”

Section: Stroke and Cerebrovascular Diseasementioning

confidence: 99%

Vascular Instability and Neurological Morbidity in Sickle Cell Disease: An Integrative Framework

et al. 2019

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It is well-established that patients with sickle cell disease (SCD) are at substantial risk of neurological complications, including overt and silent stroke, microstructural injury, and cognitive difficulties. Yet the underlying mechanisms remain poorly understood, partly because findings have largely been considered in isolation. Here, we review mechanistic pathways for which there is accumulating evidence and propose an integrative systems-biology framework for understanding neurological risk. Drawing upon work from other vascular beds in SCD, as well as the wider stroke literature, we propose that macro-circulatory hyper-perfusion, regions of relative micro-circulatory hypo-perfusion, and an exhaustion of cerebral reserve mechanisms, together lead to a state of cerebral vascular instability. We suggest that in this state, tissue oxygen supply is fragile and easily perturbed by changes in clinical condition, with the potential for stroke and/or microstructural injury if metabolic demand exceeds tissue oxygenation. This framework brings together recent developments in the field, highlights outstanding questions, and offers a first step toward a linking pathophysiological explanation of neurological risk that may help inform future screening and treatment strategies.

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Section: Stroke and Cerebrovascular Diseasementioning

confidence: 99%

Vascular Instability and Neurological Morbidity in Sickle Cell Disease: An Integrative Framework

et al. 2019

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“…The resulting constitutive equation can also be applied to other complex geometries without the need for lengthy experimentation and variable changes, enabling realistic simulations of blood flow in the vascular system and forming the basis for potential future patient-specific treatment design. Although behavior in capillaries is too complex to be described by a continuous model such as the Carreau-Yasuda model, the higher risk of SCD patients for vascular anomalies (31) points to the need for and potential benefit of the approach described here.…”

Section: Discussionmentioning

confidence: 99%

“…The role of blood rheology in cerebral aneurysm formation is an active research area, and there is a recognized association between SCD and some cerebrovascular anomalies (18,31). These observations suggest that an effective approach to treatment design for SCD patients could include both patient-specific blood rheology and patientspecific cerebrovascular geometry (via, e.g., magnetic resonance angiography).…”

Section: Discussionmentioning

confidence: 99%

An Experimental-Computational Approach to Quantify Blood Rheology in Sickle Cell Disease

Bazzi

Valdez

Barocas

et al. 2020

Biophysical Journal

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“…In addition, it has recently become clear that patients with SCD may have extra-cranial vasculopathy, including dissection as well as narrowing or occlusion ( Figure 5G,I ), detectable on imaging of the neck [ 50 , 51 ], and that this is associated with SCI [ 51 ]. Tortuosity is relatively common ( Figure 5I ) [ 52 ] and ectasia of the basilar and intracranial circulations has also been documented and is associated with low hematocrit [ 53 ]. It is worth considering separately the vasculopathy literature in the context of clinical stroke, SCI, and screening for stroke risk in asymptomatic patients.…”

Section: Qualitative Imaging In Steady-state Settingsmentioning

confidence: 99%

MRI detection of brain abnormality in sickle cell disease

Stotesbury

Kawadler

Saunders

et al. 2021

Expert Review of Hematology

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Introduction: Over the past decades, neuroimaging studies have clarified that a significant proportion of patients with sickle cell disease (SCD) have functionally significant brain abnormalities. Clinically, structural magnetic resonance imaging (MRI) sequences (T2, FLAIR, diffusion-weighted imaging) have been used by radiologists to diagnose chronic and acute cerebral infarction (both overt and clinically silent), while magnetic resonance angiography and venography have been used to diagnose arteriopathy and venous thrombosis. In research settings, imaging scientists are increasingly applying quantitative techniques to shine further light on underlying mechanisms. Areas covered: From a June 2020 PubMed search of 'magnetic' or 'MRI' and 'sickle' over the previous 5 years, we selected manuscripts on T1-based morphometric analysis, diffusion tensor imaging, arterial spin labeling, T2-oximetry, quantitative susceptibility, and connectivity. Expert Opinion: Quantitative MRI techniques are identifying structural and hemodynamic biomarkers associated with risk of neurological and neurocognitive complications. A growing body of evidence suggests that these biomarkers are sensitive to change with treatments, such as blood transfusion and hydroxyurea, indicating that they may hold promise as endpoints in future randomized clinical trials of novel approaches including hemoglobin F upregulation, reduction of polymerization, and gene therapy. With further validation, such techniques may eventually also improve neurological and neurocognitive risk stratification in this vulnerable population.

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Quantitative Analysis of Extracranial Arterial Tortuosity in Patients with Sickle Cell Disease

Cited by 16 publications

References 32 publications

Vascular Instability and Neurological Morbidity in Sickle Cell Disease: An Integrative Framework

Vascular Instability and Neurological Morbidity in Sickle Cell Disease: An Integrative Framework

An Experimental-Computational Approach to Quantify Blood Rheology in Sickle Cell Disease

MRI detection of brain abnormality in sickle cell disease

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