TREATING THE RARE MOYAMOYA DISEASE

Doctors at MUH have a special interest in the management of moyamoya disease and other complex cerebrovascular diseases. A multidisciplinary team meets regularly to plan the best treatment for these patients.

 

Moyamoya is an idiopathic disease with patients developing spontaneous, gradual narrowing of the carotid arteries - in turn, restricting blood flow to the brain. In time, the artery can become occluded. When patients finally present to a specialist with moyamoya disease, severe narrowing of the artery has usually already occurred. They often present with a stroke or transient ischemic attack.

"When diagnosed, the disease is often well advanced," said Professor Marcus Stoodley, who trained in advanced neurosurgery at both Stanford University and the University of Chicago in the US and now directs the neurosurgery laboratory at the Faculty of Medicine and Health Sciences.

"Indeed, even diagnosis remains a challenge. Given that most neurological specialists aren't looking for something as rare as moyamoya, it is frequently missed until it is quite late. This means that, because of the prolonged reduction in blood flow to the brain, we see paralysis, loss of speech and loss of vision in these patients coming to Macquarie University Hospital - along with the risk of increased attacks."

Currently, there is no effective non-surgical treatment for the occluded arteries in moyamoya. If caught early enough, the current best option is to perform bypass surgery to enable blood to flow around the blockage.

This is done by directly connecting arteries to the brain, by an anastomosis connecting the superficial temporal artery from the scalp to the middle cerebral artery on the surface of the brain.

Macquarie University Hospital's advanced medical imaging plays a key role in diagnosis of patients and in decisions for the hospital's surgeons around how best to manage individual patients.

Patients coming to Macquarie University Hospital for surgical treatment are cared for by a multidisciplinary team that involves careful intra-operative and post-operative management. Anaesthesia, for example, needs to be administered quite differently to normal neurosurgery anaesthesia.

Post-operative care is carried out by medical and nursing staff experienced in moyamoya disease management - with ICU providing specialised care in blood pressure management, for example.

"Generally with moyamoya, we see most patients either presenting as children between the ages of 5 and 10, or as adults in their 40s and 50s" said Professor Stoodley. 

"Studies show that if children remain untreated, they generally don't fare well, often later having multiple strokes that result in intellectual and physical disability. We know that children treated do better than those not treated.