{"id":7328,"date":"2019-02-25T14:37:22","date_gmt":"2019-02-25T03:37:22","guid":{"rendered":"https:\/\/blogs.rch.org.au\/news\/?p=7328"},"modified":"2019-02-25T14:53:28","modified_gmt":"2019-02-25T03:53:28","slug":"how-surgeons-used-a-3d-skull-to-save-little-jack-from-skull-condition","status":"publish","type":"post","link":"https:\/\/blogs.rch.org.au\/news\/how-surgeons-used-a-3d-skull-to-save-little-jack-from-skull-condition\/","title":{"rendered":"How surgeons used a 3D skull to save little Jack from skull condition"},"content":{"rendered":"
\n

When Jack Lambere Kalangis was born on June\u00a08, 2016, the large lump on his face immediately caused heartbreak and concern for his\u00a0mother, Boufa\u00a0Joses.<\/p>\n<\/div>\n

But living on the remote island of Akamp \u2014 a part of Vanuatu too small to register as a dot on most maps \u2014 nobody had any idea just how much danger he was in.<\/p>\n

\u201cI was worried, because I sometimes I was scared to carry him in public because of what people would think, and because he could get hurt,\u201d Ms Joses said.<\/p>\n

\"\"
Jack prior to life-saving surgery.<\/figcaption><\/figure>\n

Despite her attempts to shield him, a couple of holiday-makers from central Victoria saw Jack and felt compelled to act. Returning home, they asked a nurse friend how they could help the small boy, who in turn sought advice from a local doctor.<\/p>\n

The doctor then contacted\u00a0Children First Foundation (CFF).<\/p>\n

The charity sent photos of Jack\u2019s face to one of the world\u2019s leading craniofacial surgery teams at The Royal Children\u2019s Hospital and \u201cTeam Jack\u201d was born.<\/p>\n

\u201cHow fortunate for Jack and his family that those people were there at the time and had the fortitude to seek support for him,\u201d CFF chief executive Elizabeth Lodge said. But even as CFF volunteer Sally Peet sat on a plane in Port Vila on June 20 last year, waiting to fly Jack to Melbourne, things became unexpectedly complicated.<\/p>\n

Most people saw the lump on Jack\u2019s face as a cosmetic problem, but when veteran craniofacial surgeon Tony Holmes was handed a photo in early 2018, he knew exactly what it was: \u201ca death sentence\u201d.<\/p>\n

\u201cWe could easily diagnose what the problem was from the photograph, but we had no idea what was going on inside,\u201d he said.<\/p>\n

Dr Holmes has literally written the book on how to treat Jack\u2019s condition, known as a frontal ethmoidal encephalocele \u2014 or, as he describes it, \u201ca permeation of intracranial contents beyond the cranial cavity\u201d.<\/p>\n

In plain English, it means Jack\u2019s 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.<\/p>\n

The lumps on Jack\u2019s face were actually \u201cchunks of his brain\u201d covered only by a thin layer of skin \u2014 cosmetic concerns quickly became irrelevant.\u00a0Any bump could be fatal and there was no telling what damage had already occurred in his brain.<\/p>\n

Jack may have been in serious need of help, but Dr Holmes and his colleagues had handled many previous cases and were confident of saving him with little fuss. But when Jack finally made his dramatic arrival in Melbourne, everything changed.<\/p>\n

As unbelievable as it seemed, the brain extending out the front of Jack\u2019s head was only the tip of the iceberg. Scans revealed Jack had a second condition which caused the two-year-old\u2019s skull to form incorrectly at the back.<\/p>\n

Three-dimensional images confirmed his tiny skull was nowhere near big enough for his brain to fit inside.<\/p>\n

\"\"
Jack with Dr Burge, Dr Wray and Dr Holmes for his final check up.<\/figcaption><\/figure>\n

Jack was the first case of his type in the world \u2014 nobody had dealt with these issues at the same time.<\/p>\n

\u201cOn top of that the skull didn\u2019t grow properly. Not only did he have a hole, his head was small and tight.\u201d<\/p>\n

The craniofacial team had to call in neurosurgeon Alison Wray.<\/p>\n

\u201cThe brain has developed in a slightly different way, the formation of it is different,\u201d Dr Wray said. \u201cThat is something that we can\u2019t change. But, it was also being compromised because there wasn\u2019t enough room for it to be growing.\u201d<\/p>\n

Three-dimensional scans were used to make perfect replicas of Jack\u2019s imperfect skull, which RCH head of craniofacial surgery Dr Jonathan Burge took home so he could devote his spare time to cutting up and reassembling it, planning and perfecting the operation.<\/p>\n

The goal was to find a way of dividing the bones in Jack\u2019s head and reassembling them so they not only covered the hole above his nose, but also gave him the face his foetal development denied him.<\/p>\n

\u201cA lot of craniofacial, the fun is in the planning,\u201d Dr Burge said. \u201cI think you make poor decisions when you feel rushed. It was nice not to feel pushed into a corner.\u201d<\/p>\n

A\u00a0plan was formed so Dr Wray could lead the first stage of their blueprint.<\/p>\n

\u201cHe is the architect and I am the carpenter,\u201d Dr Wray joked. \u201cThe problem was that it went from being one operation into two very big operations.\u201d<\/p>\n

On August 7, 2018, Dr Wray and Dr Burge undertook the first major procedure to expand Jack\u2019s skull, known as a posterior cranial vault expansion. Put simply, the pair cut away the entire back section of the toddler\u2019s head and reattached it using expandable metal pins on each side underneath his skin.<\/p>\n

Known as distracters, each of the pins had an arm sticking out through the skin like antenna on the sides of Jack\u2019s head, which doctors would twist every morning and night.<\/p>\n

Each time they twisted the arms the pins would stretch 0.6mm \u2014 effectively tearing the join in Jack\u2019s skull so new bone would grow to cover the gap in the hours before it was again stretched apart.<\/p>\n

Growing by 1.2mm a day, it took a tense 25 days to grow the 3cm needed for Jack\u2019s head to be large enough to hold his brain.<\/p>\n

\u201cIt looks good. He does still have a malformation of the brain but \u2026 he doesn\u2019t seem to be having any problems \u2026 so that is really encouraging,\u201d Dr Wray said.<\/p>\n

On Tuesday November 27, it was Dr Holmes and Dr Burge\u2019s turn to team up in the operating theatre, this time to remove his encephalocele and rebuild his face.<\/p>\n

\"\"
Jack is now ready to head back home to Vanuatu.<\/figcaption><\/figure>\n

\u201cThis is the first time we have ever had to do this \u2026 it has worked a dream,\u201d Dr Holmes said.<\/p>\n

From his cheeks up, the top section of Jack\u2019s troubled face was cut away and separated into different sections; two eye pieces, a forehead piece, and a nose.<\/p>\n

The pieces housing the holes for Jack\u2019s eyes were then tilted inward towards where his nose would go, so that they not only gave him a normal sha pe, but were closer together so they covered the hole where his brain had leaked out. While little of Jack\u2019s own nose could be used, bone grafts were shaped and attached to give him a new one.<\/p>\n

Finally, Jack\u2019s forehead was put back in place, though this time it was tilted forward to fit in with his newly enlarged skull and have the same as everyone else.<\/p>\n

\u201cIt has all gone exactly to plan,\u201d Dr Holmes said.<\/p>\n

If there was a problem inside Jack\u2019s head, it certainly did not show on the outside. At\u00a0Children First Foundation\u2019s Kilmore farm Jack is a whirlwind of energy, running rings around his carers, the other children undergoing their own surgery and builders undertaking renovations.<\/p>\n

\u201cIf he comes back we can put a bit more nose in.<\/p>\n

\u201cAlso, there is a worry that his nose has more bone in it than other people\u2019s, so he could break it \u2014 so no boxing or other high-contact sports.\u201d<\/p>\n

In her understated manner, Boufa takes in all the information, before asking a series of questions including her first priority: \u201cWill Jack be able to play soccer?\u201d<\/p>\n

Dr Holme\u2019s answer is a qualified \u201cyes\u201d, suggesting Jack try to become a goal-keeper because it would be too dangerous to head the ball.<\/p>\n

One by one Boufa\u2019s other concerns are handled, all with a positive response \u2014 Jack will be able to go on long walks; he will be able to play with friends; Jack will have a seemingly normal life.<\/p>\n

Jack will return to his island home next week able to make whatever he wants out of life, though his impact will always be felt by the surgeons who helped rebuild him.<\/p>\n

\u201cWithout sounding too cheesy, that is why you keep coming back to this job,\u201d Dr Burge said.<\/p>\n

Words Grant McArthur (Herald Sun), photo Alex Coppel<\/p>\n","protected":false},"excerpt":{"rendered":"

When Jack was born, the large lump on his face immediately caused heartbreak and concern for his mother, Boufa Joses. However with the help of life-saving surgery at the RCH, Jack can return home to Vanuatu as a healthy three year old.<\/p>\n","protected":false},"author":161,"featured_media":7335,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[8971,6],"tags":[],"class_list":["post-7328","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-featured","category-in-the-news"],"acf":[],"_links":{"self":[{"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/posts\/7328","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/users\/161"}],"replies":[{"embeddable":true,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/comments?post=7328"}],"version-history":[{"count":12,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/posts\/7328\/revisions"}],"predecessor-version":[{"id":7347,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/posts\/7328\/revisions\/7347"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/media\/7335"}],"wp:attachment":[{"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/media?parent=7328"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/categories?post=7328"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blogs.rch.org.au\/news\/wp-json\/wp\/v2\/tags?post=7328"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}