Related Experiment Video

Updated: May 11, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
05:23

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

Cerebrovascular complications in children with sickle cell disease

M De Montalembert1, W Wang

  • 1Department of Pediatrics, Hôpital Necker Enfants Malades and Sickle Cell Reference Center, Paris, France.

Handbook of Clinical Neurology
|April 30, 2013
PubMed

Insights

Children with sickle cell disease (HbSS) face high stroke risk. Annual Transcranial Doppler (TCD) screening and monthly transfusions can prevent strokes in high-risk children.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Vascular Medicine

Background:

  • Sickle cell disease (HbSS) historically led to significant mortality and morbidity from cerebrovascular accidents in children.
  • Children with HbSS have an 11% risk of stroke before age 20, with peak incidence of ischemic stroke between 2-5 years and hemorrhagic stroke between 20-29 years.
  • Vessel occlusion in sickle cell disease involves intimal proliferation, inflammation, cell adhesion, hypercoagulability, and dysregulated vascular tone, potentially causing silent infarcts and cognitive decline.

Purpose of the Study:

  • To highlight the effectiveness of Transcranial Doppler (TCD) ultrasonography in identifying children at high risk for stroke.
  • To present evidence supporting stroke prevention strategies in pediatric sickle cell disease.
  • To outline current recommendations for screening and treatment of cerebral vasculopathy in children with sickle cell disease.

Main Methods:

  • Utilized Transcranial Doppler (TCD) ultrasonography for early detection of high-risk children.
  • Reviewed findings from a randomized study on stroke prevention through monthly transfusions in children with abnormal TCD results.
  • Examined the efficacy of chronic transfusion in reducing stroke recurrence and considered hematopoietic stem cell transplant as a treatment option.

Main Results:

  • Transcranial Doppler (TCD) ultrasonography effectively identifies children at high risk for clinical strokes.
  • Monthly transfusions significantly reduced the incidence of first-time strokes in children with abnormal TCD findings.
  • Chronic transfusion therapy greatly reduces, but does not eliminate, the risk of stroke recurrence after a first event.

Conclusions:

  • Annual TCD screening for children aged 2-16 years is recommended, with monthly transfusions for those identified as high-risk.
  • Hematopoietic stem cell transplant is a viable option for children with cerebral vasculopathy and an HLA-identical sibling.
  • Early detection and intervention are crucial for managing cerebrovascular complications in pediatric sickle cell disease.

More Related Videos

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry08:23

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry

Published on: November 5, 2019

Related Concept Videos

Hemorrhagic Stroke ll: Pathophysiology01:29

Hemorrhagic Stroke ll: Pathophysiology

A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
Hemorrhagic Stroke l: Introduction01:17

Hemorrhagic Stroke l: Introduction

A hemorrhagic stroke is an acute neurological event that occurs when a weakened cerebral blood vessel ruptures, allowing blood to accumulate within or around the brain. The sudden release of blood forms a focal hematoma that increases intracranial pressure, displaces neural tissue, and can obstruct cerebrospinal fluid pathways. These effects may be compounded by intraventricular extension of the hemorrhage, cerebral edema, or compression of adjacent structures, all of which contribute to...
Cytotoxic Edema: Pathophysiology01:21

Cytotoxic Edema: Pathophysiology

Cytotoxic edema is a form of cerebral edema characterized by intracellular swelling of neurons, astrocytes, and other glial cells. It develops when the mechanisms responsible for maintaining ionic gradients across the cell membrane become impaired. Under normal physiological conditions, the sodium–potassium ATPase actively transports sodium ions out of the cell and potassium ions into the cell, preserving osmotic balance and enabling electrical signaling. This pump requires a continuous supply...
Ischemic Stroke ll: Pathophysiology01:15

Ischemic Stroke ll: Pathophysiology

An ischemic stroke occurs when a cerebral blood vessel becomes obstructed, most often by a thrombus or embolus, interrupting the delivery of oxygen and glucose to brain tissue. Because neurons rely on continuous aerobic metabolism, energy failure begins within minutes of reduced perfusion. The region receiving the least blood flow becomes the infarct core, an area of irreversible cellular death. Surrounding this core lies the penumbra, a zone of hypoperfused but still viable tissue that is...
Complications of Diabetes Mellitus01:22

Complications of Diabetes Mellitus

Diabetes mellitus is a chronic metabolic disorder characterized by persistent hyperglycemia due to insulin deficiency, resistance, or both. Prolonged hyperglycemia disrupts metabolic homeostasis and leads to acute and chronic complications.Acute ComplicationsAcute complications result from sudden metabolic imbalance.Diabetic ketoacidosis (DKA) mainly appears in type 1 diabetes but may also develop in type 2 diabetes, particularly under extreme stress. It arises from severe insulin deficiency,...