How surgeons used a 3D skull to save little Jack

Last year our craniofacial surgery team received photos from the Children First Foundation (CFF) of a special little boy from the remote island of Akamp

Last year our craniofacial surgery team received photos from the Children First Foundation (CFF) of a special little boy from the remote island of Akamp — a part of Vanuatu too small to register as a dot on most maps.

Two year old Jack was born with a large lump on his face. Most people saw the lump as a cosmetic problem, but when veteran craniofacial surgeon Dr Tony Holmes was handed the photo in early 2018, he knew exactly what it was: “a death sentence”.

Jack had frontal ethmoidal encephalocele — or, as he described it, “a permeation of intracranial contents beyond the cranial cavity.”

This meant Jack’s brain was leaking out the front of his head through a hole where the bones of his face are supposed to fuse together at the top of his nose. Cosmetic concerns quickly became irrelevant – any bump could be fatal and there was no telling what damage had already occurred in his brain.

As unbelievable as it seemed, the brain extending out the front of Jack’s head was only the tip of the iceberg. Scans taken after he arrived in Melbourne revealed Jack had a second condition which caused his skull to form incorrectly at the back.

Three-dimensional images confirmed his tiny skull was nowhere near big enough for his brain to fit inside. Jack was the first case of his type in the world — nobody had dealt with these issues at the same time.

On August 7, 2018, neurosurgeon Dr Alison Wray and RCH head of craniofacial surgery Dr Jonathan Burge undertook the first major procedure to expand Jack’s skull, known as a posterior cranial vault expansion. Put simply, they cut away the entire back section of the toddler’s head and reattached it using expandable metal pins on each side underneath his skin.

Known as distracters, each of the pins had an arm sticking out through the skin like antenna on the sides of Jack’s head, which doctors would twist every morning and night.

Each time they twisted the arms the pins would stretch 0.6mm — effectively tearing the join in Jack’s skull so new bone would grow to cover the gap in the hours before it was again stretched.

Growing by 1.2mm a day, it took a tense 25 days to grow the 3cm needed for Jack’s head to be large enough to hold his brain.

On Tuesday November 27, it was Dr Holmes and Dr Burge’s turn to team up in the operating theatre, this time to remove his encephalocele and rebuild his face.

“This is the first time we have ever had to do this … it has worked a dream,” Dr Holmes said.

“It has all gone exactly to plan,” Dr Holmes said.

Jack returned to his island home in February and will be able to make whatever he wants out of life, though his impact will always be felt by the surgeons who helped rebuild him.

“Without sounding too cheesy, that is why you keep coming back to this job,” Dr Burge said.

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