First-ever complex Moyamoya brain surgery at our Children’s Hospital
A team of our expert neurosurgeons has successfully performed the first-ever Pial Synangiosis – a complex brain surgery to treat the rare condition, Moyamoya disease, in which the blood vessels (internal carotid arteries) that supply blood to the brain become narrowed.
Due to its progressive
nature, left untreated, Moyamoya, which affects about only one person in every million, can cause strokes, seizures, paralysis, brain damage and can be fatal. The name, which means “puff of smoke” in Japanese, refers to the wispy, smoke-like appearance of these new blood vessels on an x-ray.
The Pial Synangiosis, which usually takes three to four hours for each brain hemisphere, creates a new source of blood supply to the child’s brain suffering from severe restricted blood flow (ischemia).
During the surgery, the neurosurgeon first makes an incision in the scalp to expose a healthy blood vessel, the superficial temporal artery, which is normally connected to the scalp and not affected by Moyamoya. The artery is then separated from the surrounding tissue, while blood continues to flow through it. A window of bone is opened beneath the artery.
The surgeon then uses a microscope 
to carefully open each of the brain’s coverings, exposing the surface. They then place the artery onto the brain surface sewing the tissues around it to the surface of the brain. This keeps the artery in direct contact with the brain and stimulates the development of new blood vessels that grow from the donor scalp artery providing a vital source of blood to the brain.
The new blood vessels emerge, not only from the transplanted scalp artery but also from blood vessels sprouting from the covering of the brain around the opening in the skull.
Brave three-year-old, Jaimie-Lee, from Staffordshire, was first diagnosed with the condition last summer by the team at our Children’s Hospital after suffering a stroke. Soon after she had another meaning she was in urgent need of surgical treatment so that she could enjoy her childhood without the fear of her condition worsening.
She became the first child at our Children’s Hospital to undergo the Pial Synangiosis procedure last autumn. Mr Pasquale Gallo, Consultant Neurosurgeon and Craniofacial expert explained: “The procedure was vital for Jaimie’s recovery as currently there is no effective drug that can prevent the narrowing of the vessels that reduce blood flow to the brain.”
Jaimie’s carer, Lynda, said: “Jaimie is a happy little girl and we’re so thankful to those who helped us at the Children’s Hospital. She’s doing so much better after the surgery and is almost back to being herself. We couldn’t fault Mr Gallo, he’s been fantastic.”
Mr Gallo added: “In the past, other procedures have been performed for Moyamoya patients at our hospital but never a Pial Synangiosis bypass which is the gold standard for children suffering with the disease, like Jaimie-Lee.
“In the last ten years, all Moyamoya patients have been sent to Great Ormond Street Hospital for surgical treatment, which has resulted in additional costs and inconvenience for families from our region. Being able to perform Moyamoya Pial Synangiosis here will help to reduce this. We have already seen several surgical candidates in the past few months in the combined neurology-neurosurgery stroke clinic.”
The family from Staffordshire is relieved to see Jaimie-Lee back at home, happy and returning back to her usual self, enjoying her childhood without the many hardships brought on by Moyamoya disease. She will continue to have regular check-ups with our team.