Timely treatment gives new lease of life to an 11-year-old diagnosed with Moyamoya disease, a rare cerebrovascular disorder

Arjun (name changed), an 11-year-old boy from Devanahalli taluk near Bengaluru, experienced an episode of seizure a year ago. This was progressively followed by a decline in his ability to see and weakness on the left side of his body.

A CT angiogram of the brain at Aster Whitefield Hospital in June last year showed a “puff of smoke” appearance - a hallmark sign of Moyamoya disease, a rare cerebrovascular disorder often leading to continuous strokes. This disease mostly affects children around the age of 10 years and adults around the age of 40 years while progressing without warning.

Known to have a prevalence rate of 0.35 to 0.94 per 1,00,000 people, mainly in the areas of Japan and Korea, Moyamoya disease causes the narrowing of key brain arteries, prompting the brain to form smaller compensatory blood vessels to meet its oxygen needs. These new vessels, however, are often insufficient, appearing as a “puff of smoke” in imaging scans. When the brain’s demand for blood outpaces the supply, patients experience strokes or other neurological events, as in Arjun’s case. Left untreated, Moyamoya can lead to recurrent strokes, disability, and even death.

Timely surgical intervention

The medical team — comprising Swaroop Gopal, Group Director, Aster International Institute of Neurosciences and Spine Care (specialising in vascular and endovascular surgery) and Suraj Gopal, Associate Consultant, Neurosurgery at the hospital — decided to go ahead with a revascularisation surgery in August last year.

This is an intricate procedure but offers a reliable method of restoring blood flow to the brain. The surgeons isolated a scalp artery, the superficial temporal artery (STA), and connected it to an artery in the brain, creating an anastomosis. This connection enabled blood from the STA to eventually supply the brain, circumventing the narrowed arteries and improving oxygen delivery.

However, revascularisation demands careful handling of delicate arteries. Isolating and suturing these tiny vessels required flawless execution. The post-operative prognosis for this disease includes stabilising the symptoms with continuous evaluation. Arjun was discharged two days after his surgery and has been scheduled for regular follow-up to monitor his condition. Moyamoya disease is known to be bilateral—it affects both sides of the brain. Hence, revascularisation procedures may also be needed for the opposite side of the brain.

“Arjun is doing well now. After the diagnosis, a Digital Subtraction Angiography (DSA) was performed to evaluate issues with the blood flow. This involved inserting a catheter into the leg artery and guiding it to the brain’s blood vessels, utilising high-quality X-rays to image the blood vessels,” said Dr Swaroop Gopal, adding that the entire revascularisation procedure was done under a microscope.

Dr. Suraj Gopal said, “Moyamoya is a surgically treatable disease. Early recognition of the symptoms is crucial for the diagnosis and subsequent treatment.”