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Vascular Diseases of the Brain and Spinal Cord

What are vascular diseases of the brain and spinal cord?

Many problems can affect the blood vessels of a child’s brain, but they are rare.

Several malformations of arteries and veins in the brain and spinal cord are present at birth and sometimes genetic.

The vascular (blood vessel) diseases seen most often are:

Who gets vascular diseases of the brain or spinal cord?

Children rarely have vascular diseases of the brain or spinal cord. When they do, the defect is usually present at birth (congenital).

We don’t know what causes many of these abnormalities. Moyamoya disease and cavernous malformations may be genetic.

What is your experience with vascular diseases of the brain and spinal cord?

Diagnosis and treatment of these rare problems need the expertise of a team of specialists such as we have at Children’s. Our neurosurgeons have the specialized equipment needed as well as experience in diagnosing and operating on children and babies with all of these abnormalities.

Moyamoya is one of the vascular problems of the brain and spinal cord and is a particular area of emphasis for our neurosurgeons. We specialize in pial synangiosis, the surgery we use to treat Moyamoya.

Doctors from across the United States refer patients to us for this surgery. We do about six of them every year.

Our neurosurgeons also operate on adults with similar abnormalities at Harborview Medical Center, a part of UW Medicine.

What do you use to treat vascular disease of the brain or spinal cord?

Tests

We use a number of screening tools that help us understand your child’s problems and to plan treatment.

The diagnosis of a vascular disease of the brain or spinal cord can require many tests:

Embolization

This is a procedure an interventional radiologist may do. He does an angiogram and then puts very small coils or particles into the child’s vascular (blood vessel) lesions to block their blood supply.

This causes the vessels to clot, which may treat the problem or make it easier to operate on.

Surgery considerations

We treat vascular abnormalities with a variety of surgeries. The choice of treatment depends on how ill your child is when diagnosed, her medical history and age, and the findings from her studies and tests.

We may embolize the lesion and then be able to surgically remove the lesion.

We sometimes treat lesions with the Gamma knife process. This is not a knife, but a high-intensity radiation treatment aimed right at the lesions.

Vascular diseases can be life threatening. Your child may arrive at Children’s very ill from bleeding from a lesion; we may operate right away to drain the blood. Your child’s surgeon decides if he needs surgery and discusses the operation with you. Some children with vascular lesions also develop hydrocephalus.

Specific vascular diseases

What is an aneurysm?

An aneurysm (pronounced ANN-yer-iz-m) is a bubble-like widening of an artery. It is usually due to a weakness in the wall of an artery and present at birth. As with some other vascular diseases of the brain and spinal cord, we usually diagnose an aneurysm when it bleeds.

Generally, people’s aneurysms do not bleed until they are in their 40s or 50s, though they can bleed in children. Of the children who bleed, 80 percent are teenagers.

Signs of an aneurysm vary greatly. If an aneurism ruptures and bleeds, it causes a subarachnoid hemorrhage and possibly symptoms such as:

  • Severe headaches
  • Throwing up
  • Change in mental status
  • Coma
  • Cranial nerve problems
  • Seizures
  • Sensitivity to light (photophobia)

What is an arteriovenous malformation?

Normally arteries and veins are connected by tiny vessels called capillaries. When the arteries and veins reach an area without capillaries, an arteriovenous (pronounced are-TEAR-io-venus) malformation (AVM) can develop. The result is a tangle of small abnormal vessels that bleed easily. An AVM can cause many problems.

Ninety percent of AVMs are in the upper portions of the brain. About 10 percent show up in the spinal cord.

We usually diagnose the AVM when it first bleeds. The AVM can bleed without warning and without any obvious cause.

Though most AVMs bleed in adults 20 - 40 years old, they can bleed in children. A child’s risk of bleeding in the area is about 3 percent each year. These lesions can bleed over and over again. Learn more about AVMs.

What is a cavernous malformation?

A cavernous malformation is a collection of well-defined blood vessels. They are also called cavernous hemangioma, cavernous angioma and cavernoma.

Doctors can see these lesions on an MRI scan or a CT scan, but not on a cerebral angiogram. Some of these malformations are passed through families, a process involving genetics.

Cavernous malformations usually occur in the upper portion of the brain. A child can have more than one. They can bleed and we often diagnose them after a child bleeds from one of them for the first time. Some children with cavernous malformations have seizures.

How do you treat aneurysms, arteriovenous malformations and cavernous malformations?

Our concern is always whether the lesion will bleed and cause damage. A thorough work-up of your child and her abnormality helps our neurosurgeons decide what treatment is best — embolization, Gamma knife, or a craniotomy and removal (resection) of the lesion.

Your child’s neurosurgeon bases his treatment recommendation on the location, size and specific details of your child’s lesion.

What is Moyamoya disease?

Moyamoya (pronounced MOY-a-MOY-a) disease causes thickening of the walls of the major blood vessels to the brain where they enter the skull.

This thickening causes the vessels to narrow. In response, the body tries to create a new path for blood flow by forming a fine network of new blood vessels at the base of the brain.

Watch the special about Susana's battle with this rare brain disease.

The fine network of blood vessels appears like a puff of smoke on an angiogram. This disease was first described in Japan where Moyamoya means ‘puff of smoke.’

Moyamoya can be present at birth (congenital), but it worsens over time. Decreased blood flow to the affected area may cause intermittent symptoms of strokes and/or seizures.

Moyamoya may occur after radiation treatment to the brain for a brain tumor. It also occurs with:

How do you treat Moyamoya disease?

Our neurosurgeons treat this illness with an operation called a pial synangiosis. It involves cutting out the temporal artery from the child’s scalp.

Next we open her skull and attach the artery onto the surface of her brain. The artery then grows new vessels down into the brain. This gives the area a new blood supply.

Your child’s doctor may operate on the effected side or on both sides if needed. Read more about our experience treating Moyamoya disease.

What is a vein of Galen malformation?

This is a rare malformation. We see two to three infants a year with vein of Galen malformation.

The vein of Galen is a vein deep in the brain. A vein of Galen malformation is an aneurysm (a ballooning) of this vein and involves other vessels that drain into it.

The malformation can become large, causing several other problems like hydrocephalus, heart failure and poor blood supply to the brain tissue.

We usually see this problem in newborns. The malformation’s increased blood flow may stress the infant’s heart causing congestive heart failure. The baby experiences less blood supply to the rest of her brain and there is often progressive loss of brain function.

Between 30 to 40 percent of infants with vein of Galen malformation die. Sixty to 80 percent of babies who develop heart failure die.

How do you treat a vein of Galen malformation?

Our first treatment of a newborn with a vein of Galen malformation is to embolize the lesion to clot off and lessen its blood flow. Babies often need repeated embolizations.

Often the baby develops hydrocephalus or may develop it later in childhood.

As with all conditions, treatment for a vein of Galen malformation is highly individual and based on your child’s needs.