How Moyamoya disease affects children: a comprehensive guide

So you think your child may have Moyamoya disease? We asked Rabia Qaiser, MD, FAANS, a neurosurgeon at Riley Children’s Health, to offer her expertise in explaining the condition, what it means and how it may affect your child.

What is Moyamoya disease?

Moyamoya disease is a rare, neurological condition affecting the blood vessels in the brain. Derived from the Japanese term “Moyamoya,” meaning “puff of smoke,” the disease causes the brain’s blood vessels to appear like wisps of smoke when viewed on a diagnostic scan. It can occur in people of all ages, although it is most concerning and severe in children ages 5 – 10. While the cause of Moyamoya is unknown, genetic factors may play a role, particularly for those of Asian ancestry. It can also be seen in individuals with Down syndrome and sickle cell anemia.

What are the symptoms and effects of children with Moyamoya disease?

Moyamoya disease symptoms are caused by reduced blood flow to the brain and weakened blood vessels. Common symptoms include:

• Stroke or stroke-like symptoms
• Transient ischemic attacks or ministrokes
• Weakness or numbness on one side of the body
• Tingling on one side of the body
• Seizures
• Migraine-like headaches

Alongside the physical symptoms, children with Moyamoya disease may experience cognitive challenges such as difficulties with memory, attention and learning. Emotional and psychological factors may include anxiety or depression. Socially, your child my experience feelings of isolation.

“Moyamoya also greatly impacts childrens’ everyday routine,” Dr. Qaiser said. “Patients have to be mindful of contact sports, and they also need to make sure they stay well hydrated, given that dehydration can lead to strokes.”

How is Moyamoya syndrome diagnosed?

To diagnose Moyamoya disease, a specialist will do the following:

• Medical history. If you or a family member have a history of Moyamoya disease, your specialist will review your child’s medical history.
• Neurological exam. Here, a specialist will search for signs of weakness, numbness or tingling in the limbs.
• Magnetic resonance imaging (MRI). This test provides detailed images of brain structures, revealing the areas affected by reduced blood flow.
• Magnetic resonance angiogram (MRA) or computer tomography (CT) angiogram (CTA). These are imaging studies that provide precise mapping of the brain’s blood vessels.
• Cerebral angiogram. A procedure where dye is injected into the arteries to provide a detailed view of the blood supply in the brain. This test highlights blockages and any new blood vessels that may have formed, allowing to determine if there is a need for surgical treatment.

How are people with Moyamoya disease treated?

At Riley Children’s, a multidisciplinary team discusses cases monthly to determine the best options for each patient. Moyamoya disease treatment often involves a combination of medicine and surgery to improve blood flow to the brain and prevent strokes:

• Pial synangiosis surgery. This procedure allows new blood vessels to grow and restore flow to the brain. Statistics show post-surgical outcomes have decreased stroke risk to 0.08% (ischemic stroke) and 0.04% (hemorrhagic stroke), with an overall 99% 10-year-survival rate, according to a study published in Cureus: Journal of Medical Science in 2023.
• Aspirin. Patients with Moyamoya are prescribed a daily dose of aspirin, which is a widely accepted medical therapy for preventing strokes.

Once following these appropriate treatment options, patients can participate in usual activities of daily life, such as school and sports.

How can you support your child with Moyamoya disease?

While Moyamoya disease is an ongoing battle, there are several ways to support your child:

• Seek out resources to learn more about Moyamoya disease. Online medical publications such as Pubmed articles are always good places to start.
• Foster your child’s independence and confidence by teaching them about their condition and encouraging them to openly communicate their needs.
• Find support from family, therapists, peers and educators to ease the care process. Building resilience and enforcing positivity is vital to helping your child navigate their condition.

Ultimately, moyamoya is a complex condition significantly affecting children, and understanding its impacts is crucial to managing the disease.

About Dr. Rabia Qaiser

Dr. Qaiser is a Moyamoya specialist with dual training in pediatric neurosurgery as well as cerebrovascular and skull base neurosurgery. “This subject remains my passion to provide the best care for patients,” she says. Learn more about how she found her calling at a young age.

For more about Moyamoya disease, read Connor’s patient story and Marissa’s patient story.