https://www.gosh.nhs.uk/news/experts-at-gosh-and-barts-team-up-for-world-first-operation/
Experts at GOSH and Barts team up for world-first operation
31 Aug 2023, 7 p.m.
More than 50 clinicians from Great Ormond Street Hospital and Barts Health NHS Trust teamed up to perform a lifesaving world-first operation.
Clinicians had always feared the risk of operating on Sami, 12, was too high due to complexities caused by Multisystemic Smooth Muscle Dysfunction Syndrome (MSMDS) – of which there are only 35 reported cases in the world. MSMDS is a genetic disease, with no cure, that can cause a wide range of problems, including dangerous enlargement of the arteries and a high risk of stroke.
As Sami’s condition worsened and his aorta became more and more dilated, his GOSH consultant Elena Cervi, called an emergency multi-disciplinary team meeting to see if they could undertake previously unthinkable surgery. They teamed up with Professor Aung Ye Oo, Lead for Aortic Surgery at Barts, who has a vast experience of adult thoracoabdominal aortic repair.
The operation
After many discussions with Sami’s family, an Ethics Committee meeting, and over 20 hours of planning, including a meeting of around 40 experts at GOSH and Barts on Christmas Eve 2021, the first operation went ahead on 30 December 2021.
The 12-hour operation involved bypass surgery where the circulation to his brain and lower body was stopped for a period. It allowed the team to control Sami’s circulation to ensure they got the perfect balance to allow them to begin to repair his aorta without it rupturing, while preventing a stroke. It has never been performed on any child in the world before.
The operation proved to be such a success that Sami has since had two further complex surgeries to minimise his risk of further acute deteriorations.
After three major operations and prolonged hospital stays for a total of nearly five months, Sami is now at home. He will come back regularly so the team can monitor his progress.
Sami and his family
As it’s a progressive disease we felt like we were on a one-way street. We thought nothing could be done and we were preparing for the worst. Then out of the blue his consultant Elena, who has worked with us for eight years, called on Christmas Eve. We knew that we only had weeks if not days before something catastrophic happened so we took the chance. From being on palliative care, it’s now three years down the line and we’re here, all because of Dr Cervi. She could have said there’s no chance and that would have been the end, but she went beyond, way beyond.
He’s always been very limited in what he can do and who he can play with, but having this surgery has opened the doors for us. He can get involved in sport and move around freely, he can fly now whereas we’ve never been able to go too far.
Sami is now back to school and is most looking forward to art and design technology lessons. He’s also hoping to visit America as he’ll be able to fly for the first time.
Sami (right) and his brother Salman
We never dreamed we’d be able to do something as brave as manipulating Sami’s circulation without causing harm. We never thought we’d be able to offer such a big operation, and now he’s had three of them and come out of the other side. To see him like this now is so good. Credit goes to Sami and his family and all the teams at GOSH and Barts who pulled this together. It’s such an amazing achievement.
The aortic team at Barts Health and I were delighted with the outcome of the complex multistage treatment for Sami and it is great to hear that he is recovering well at home.
Dr Cervi and Professor Oo
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