Cerebrovascular Disease and Stroke
Conditions and Treatments

Conditions and Treatments

The Cerebrovascular Disease and Stroke Center at Cincinnati Children’s treats all vascular diseases of the brain and spine. In many cases, our patients have complex needs, with multiple conditions that affect their health. Our team works collaboratively with other specialists at Cincinnati Children’s to ensure that each child receives comprehensive, well-coordinated care.

An aneurysm is a bulging, weak area in the wall of an artery that supplies blood to the brain. A brain aneurysm typically does not cause symptoms and often goes unnoticed. In rare cases, a brain aneurysm ruptures, immediately releasing blood and causing a hemorrhagic stroke (bleeding in the brain). In these rare instances, there is a significant risk for brain damage or death.  

An arteriovenous malformation (AVM) is a complex tangle of abnormal connections between arteries and veins that is present at birth. It can raise a person’s blood pressure, and over time can reduce blood supply to surrounding skin and tissues. AVMs can increase a person’s risk of having a stroke or brain aneurysm. 

A cavernous malformation (CVM), also called a cavernoma, cavernous angioma or cavernous hemangioma, is a condition in which clusters of blood vessels in the brain or spinal cord form abnormally, creating “caverns” filled with slow-moving blood.  Some CVMs cause no symptoms, but larger ones may cause seizures, headaches and impaired speech or vision. 

CVMs can be found anywhere in the body, and can cause significant bleeding problems in the brain or spinal cord.  These malformations also can be attached to an important “normal” vein that the brain and spinal cord need to function normally. Some CVMs can be identified by a genetic abnormality. 

An ischemic stroke occurs when a blood clot blocks a vessel that supplies blood to the brain. When a person experiences multiple ischemic strokes over time, the strokes are considered chronic. Among the conditions that can cause chronic ischemic stroke are Moyamoya disease, certain heart conditions, sickle cell disease and trauma to an artery. 

A pseudoaneurysm occurs when the vessel wall is injured and blood flow through the vessel is disrupted or even cut off.  Blood collects outside the artery and a clot (or thrombosis) forms. This condition usually is caused by some kind of trauma to the artery. In contrast, a true aneurysm usually is caused by a defect that has been present since birth, or by some kind of disease process. 

A hemorrhagic stroke occurs when a blood vessel on or in the brain bursts. This causes bleeding in the brain, which leads to swelling and pressure. A hemorrhagic stroke can cause oxygen deprivation in the brain and permanent brain injury. Common causes of a hemorrhagic stroke include aneurysm, arteriovenous malformations, brain tumors, head trauma, high blood pressure and clotting disorders, such as sickle cell disease.

Moyamoya disease is a rare but very serious condition in which one or both of the internal carotid arteries (located in the front of the neck) become narrower over time. This causes blood flow to slow down, increasing a child’s risk of developing a blood clot. Tiny blood vessels form around the blocked artery as the body attempts to restore normal blood flow. The Japanese word “moyamoya” means “puff of smoke” − which is what the tiny blood vessels look like on an imaging test. 

If left untreated, Moyamoya disease can cause multiple strokes leaving brief or permanent weakness or mental decline. 

Moyamoya syndrome is a rare occurrence in which Moyamoya disease and another related condition are present.  Some of these related conditions include Down syndrome, Alagille, PHACE, neurofibromatosis, cancer and sickle cell disease. Radiation therapy also can induce Moyamoya disease. 

Some vascular conditions, such as arteriovenous malformation, Moyamoya disease and stroke, can cause movement disorders. Symptoms include involuntary movements such as tremors, dystonia (sustained muscle contractions) and others. 

Stroke sometimes occurs in children who are have a heart disorder, lymphoma (cancer of the immune system and white blood cells) or other serious medical conditions. Cerebrovascular specialists work in close collaboration with other specialists to ensure that patients receive well-coordinated care that addresses all of their medically complex needs.

Sinus pericranii is a rare disorder in which veins on the outside of the scalp are abnormally connected to veins in an area of the brain called the dural sinuses. Dural sinuses play a role in blood circulation in the brain.

The most visible symptom of sinus pericranii is a swollen mass on the scalp. The condition sometimes occurs along with other medical problems, such as developmental delays and macroencephaly (an abnormally large head in infants).

The most common types of head, neck and spine tumors in children are gliomas and medulloblastomas.

Head, neck and spine tumors may be benign or malignant. The medical team “grades” these tumors to indicate how serious they are. Low-grade tumors (I and II) tend to grow slowly, and high-grade tumors (III and IV) are fast-growing, malignant and spread into healthy brain tissue. 

A pseudotumor, also called idiopathic intracranial hypertension, occurs when pressure inside the skull increases for an unknown reason. Symptoms mimic a brain tumor, but no tumor is present. Symptoms can include headaches behind the eyes, ringing in the ears, nausea, vomiting or dizziness, and others.

Vein of Galen malformation is a rare type of arteriovenous malformation (AVM) deep in the brain. It occurs when blood from cerebral arteries rushes too quickly into a large vein called the vein of Galen. The blood flows away from the brain instead of toward it. This puts pressure on the heart and lungs and can lead to heart failure or pulmonary hypertension, a type of high blood pressure that affects arteries in the lungs and in the heart. 

Vein of Galen malformation occurs during early prenatal development and often is visible on a prenatal ultrasound. It must be treated soon after birth. 

Bow hunter’s syndrome is a stroke caused by turning the head to the side in a forceful way. The motion causes pressure on the arteries in the neck, which interrupts the flow of blood. A stroke occurs when blood flow becomes blocked or the blood vessel bursts. Symptoms are the same as those of a regular stroke (dizziness, vision disturbances, trouble speaking, etc.) and usually occur on only one side of the body. 

Treatment Options

Anti-clotting drugs, such as aspirin, can help prevent the formation of blood clots in children who are at risk for mini-strokes and strokes due to cerebrovascular disease.

Cincinnati Children’s offers a number of other endovascular procedures to treat cerebrovascular conditions. These include:

  • Endovascular embolization, which uses glue or coils to plug a lesion in the artery caused by aneurysm, an arteriovenous malformation or other abnormal blood vessels in the brain. The goal is to cut off the blood supply in the problem area and reduce the risk of rupture.
  • Stent placement, which involves inserting a tiny mesh device within the artery to maintain blood flow. Stenting is sometimes used to prevent a stroke in patients with venous hypertension or pseudotumor.
  • Selective intra-arterial chemotherapy infusion for tumors of the head, neck and spine. This therapy involves injecting chemotherapy drugs into a selected artery, which means the drugs reach the tumor in a more concentrated and potentially more effective form. Cincinnati Children’s is the only pediatric hospital in the United States where this therapy is available.

Direct arterial bypass is a complex surgical procedure used to treat an artery that has been damaged, blocked or narrowed due to Moyamoya disease. This revascularization procedure, also known as “superficial temporal artery to middle cerebral artery (STA-MCA) bypass,” involves connecting a blood vessel from the scalp directly to a blood vessel on the outer surface of the brain. The goal is to create a new pathway for the blood to flow.

Indirect revascularization is a type of surgery used to encourage new blood vessel growth to the brain for patients with Moyamoya disease. It involves laying and suturing an artery, muscle or other tissue on the surface of the brain. Over time, new blood vessels form, leading to improved blood flow to the brain. Indirect revascularization techniques include:

  • Pial synangiosis
  • Dural inversion
  • Encephaloduroarteriosynagiosis (EDAS)
  • Encephalomyosynangiosis (EMS)
  • Omental transposition/transfer

Deep brain stimulation (DBS) is used to treat tremors and dystonia (movement disorders) that sometimes occur when a child has a cerebrovascular disease such as chronic stroke or arteriovenous malformation.

Performed by a neurosurgeon, this minimally invasive procedure involves placing a stimulation electrode on abnormal brain tissue in order to alter its function, control tremors and improve movement. DBS is reversible and adjustable, and does not destroy the area of brain tissue but stimulates it instead. Our neurosurgeons perform this procedure using intra-operative magnetic resonance imaging, which provides real-time guidance during surgery.

Only a small number of medical centers specialize in providing a comprehensive DBS program for children and adolescents. Our team collaborates with specialists at the Cincinnati Children’s Movement Disorders Clinic to ensure that children receive comprehensive care before and after the implant surgery.