Zakariya Elmoustafa with parents Ibrahim and Ahlam and siblings Ahmad and Rukaya. Picture: Tony Gough Source: Herald Sun Jun 24, 2012
FOR the first year of his life, Zakariya Elmoustafa’s tiny brain fought to keep developing inside a skull that had stopped growing.
It was the most severe case of a fused skull ever seen by Victorian doctors, robbing the toddler of even the basic abilities of being able to smile or look his father Ibrahim Elmoustafa and mother Ahlam Elakkoumi in the eyes. But a series of radical surgeries at the Royal Children’s Hospital to prise open his skull have not only saved Zakariya’s life, they have allowed him to interact with his world for the first time.
“The hardest thing was seeing people take a second or third glance at him,” said Ms Elakkoumi. “As a parent you want to protect your children from anything, so I’ve just had to hold him much closer. But now he’s free to live life.” Zakariya, now aged two, was born with an unusually shaped head – a rare syndrome called pancraniosynostosis – seen no more than twice a decade in Victoria.
A newborn’s skull is made of separate bones, allowing it to fit through the birth canal and accommodate the rapidly growing brain in the first two years of life.But the plates in Zakariya’s skull were fused together at birth, leaving the back of his skull so flat that it was constricting his growing brain.
Neurosurgeon Alison Wray and plastic surgeon David Chong took on the case, a unique combination of craniofacial skills that has also seen them involved in the separation of conjoined twins Trishna and Krishna.
“It was the most severe case I’d ever seen,” Ms Wray said “We weren’t able to engage with him. He wouldn’t look at you and it didn’t appear like he was seeing and interacting with his world.” But before his constricted brain could be relieved, Zakariya needed open-heart surgery at two months to ensure his heart could pump blood effectively.Once he was medically stable and could tolerate anaesthetic, surgeons scoured the world for techniques they could borrow to buy them time.
They needed to increase the size of his skull quickly while waiting for his heart to strengthen so he could survive more complex surgery.
At age six months, four tension-loaded springs were inserted into bone across the crown of his head. They opened gradually on their own over two months to increase his head circumference. And the results were instant. Two days after surgery Zakariya – for the first time in his life – smiled at his parents and was able to focus and follow objects. But the bigger operation was to follow, when the 12-month-old had four “distractors” screwed across bones in his skull.
When his skull cap was removed during surgery, the brain instantly relaxed 1 1/2cm, shocking proof of how squeezed it was. His parents were taught how to wind the cranks each day to slowly open his skull, taking advantage of the unique ability of children to regrow bone and increase the size of his skull. And the series of radical operations not only saved his life, but appear to have unlocked great intellectual and emotional potential. Zakariya is now performing above average in several areas just a year after the final operation. “It’s just like his brain has kicked into gear,” Ms Elmoustafa said. “He’s catching up in every possible way and that’s a miracle.”