World-first surgery saves boy with rare condition | Our news

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World-first surgery saves boy with rare condition

The lead for aortic surgery at Barts Health NHS Trust was part of an extraordinary team of more than 50 clinicians who performed an operation on a 12-year-old that had never been carried out on any child in the world before.  

As Sami has Multisystemic Smooth Muscle Dysfunction Syndrome (MSMDS), it was considered a high risk for surgery to be performed. 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. There are only 35 reported cases across the world.  

The team at Great Ormond Street Hospital (GOSH), who were treating Sami decided to bring in Professor Aung Ye Oo, Lead for Aortic Surgery at Barts and his team. Professor Oo has vast experience in adult thoracoabdominal aortic repair and is one of only a very few surgeons in the world with the knowledge and skills to help.  

Following 20 hours of planning, discussions with Sami’s family and an Ethics Committee meeting, the first operation took place 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 the team got the perfect balance, allowing them to begin to repair his aorta without it rupturing, while preventing a stroke. As the first operation was a success, Sami has since had two further surgeries to minimise his risk of further acute deteriorations. This consisted in improving his cerebral circulation and subsequently addressing the remaining exceedingly dilated part of the aorta. 

Sami is now at home after three major operations in just over a year and prolonged hospital stays for a total of nearly five months. He will come back regularly so the team can monitor his progress. 

The 12-year-old is now starting back to school and is most looking forward to art and design technology lessons.  

Professor Oo said: “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.” 


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