The life of a four-year-old UK national girl suffering from a rare, progressive cerebrovascular disorder called Moyamoya disease (MMD), has been saved with indirect revascularisation (restoring blood flow) surgery. 

 

The first-of-its-kind surgical procedure in the UAE, performed by Dr Gopalakrishnan CV, Consultant and Head- Neurosurgery and Spine Surgery at Medcare Orthopaedics and Spine Hospital along with his team, saved the child’s life without needing treatment abroad. 

 

Moyamoya is a rare disease characterised by a progressive narrowing of the internal carotid artery, the major blood vessel that provides the brain’s blood supply. Research shows that the annual incidence of moyamoya disease is 0·5–1·5 per 100 000 individuals in east Asian countries but as low as 0·1 per 100 000 in other regions, including North America. Although MMD can be initially treated with medication, surgical intervention is required in cases of progressive disease or recurrent symptoms. 

 

Moyamoya

Dr Gopalakrishnan said: “The narrowing of blood vessels leads to the formation of collateral blood vessels that resemble a ‘puff of smoke’, translating to Moyamoya in the Japanese language. Although Moyamoya disease can be present in children and adults, it is most commonly found among children between the ages of five and 10 years old. The disease is also the most prevalent in East Asian countries. Thus, this patient’s case was quite rare.”

 

The child had a history of suspected transient ischemic attack, which are strokes that only last a few minutes. During an otherwise regular day of play, she suddenly experienced slurred speech, facial asymmetry, and mild right upper arm weakness. 

 

Following an MRI brain and MR angiogram, the doctors discovered significant narrowing of a portion of both internal carotid arteries, with the right artery completely obliterated, consistent with a diagnosis of Moyamoya disease. The diagnosis was confirmed by a 4-vessel digital subtraction angiogram which provides an image of the blood vessels in the brain to detect a problem with blood flow.

 

Dr Gopalakrishnan said: “After the diagnosis, we decided to opt for surgery, which involved combined bypass using the EDAMS (encephaloduroarteriomyosangiosis) technique that would provide us with multiple sources for indirect revascularisation.” 

 

Accompanying risks

“There were risks since EDAMS was not commonly performed in the UAE and up until now, patients had to seek this lifesaving surgery abroad. In fact, this was going to be one of the first attempts to perform the surgery using the EDAMS technique in the UAE,” he further added.

 

According to Dr Gopalakrishnan, revascularisation surgery, such as indirect or direct bypass surgery, is the primary surgical treatment for MMD. With direct bypass surgery, the surgeon connects the superficial temporal artery (STA), which are the terminal branches of the external carotid artery to the cortical branches of the middle cerebral artery providing oxygenated blood to the brain and improving the blood supply.

 

On the other hand, indirect bypass or EDAMS is a procedure where the surgeon uses the branches of the superficial temporal artery and a temporalis muscle flap, and places them directly on the surface of the brain. This helps the brain create new blood vessels, triggered by a lack of oxygenated blood. 

 

This procedure was augmented by multiple cranial holes to improve the chances for collateral blood flow by providing a conduit for external blood supply on to the brain surface. 

 

EDAMS procedure

The patient underwent EDAMS procedure for the arteries on both sides, as she could not undergo a direct bypass since the calibre of her vessels was very small. More than often in children the vessels are not large enough to sustain a direct bypass.  

 

Speaking about the experience, the mother of the patient said: “We are grateful to Dr Gopalakrishnan and his team for the outstanding care they provided to our daughter. Their expertise, compassion, and dedication gave me hope and strength throughout the entire process. Thanks to their skilled interventions, my daughter's life has been saved, and she can now look forward to a brighter future. We are eternally grateful for their unwavering support and care."-- TradeArabia News Service