There were problems with the growth of her face and therefore with the airways through the nasal spaces. We could expect breathing problems resulting in a tracheostomy, especially if her face doesn’t develop properly. Babies are already quite snuffly by nature. It doesn’t take much to restrict their already small airways.
It’s not always my decision which operations get bumped. All I can say for sure is that this baby became top priority as soon as she arrived.
We’ve done the ward rounds, which featured me, another consultant, two plastic surgeons, nurses, registrars and trainees. We’ve done the WHO briefing. I know everyone I’ll be working with and they understand what we expect. And as hard as it was to tell them, so do the parents.
We’re not about to embark on a quick fix. There’s no easy remedy for what Baby has. We can make a difference, that much I can be sure of, but how much? Plus, it won’t be easy and it won’t be quick. The operation we’re about to attempt will be the first of many. The various physical and functional problems caused by the syndrome are still going to be there when Baby wakes up. They will be for the rest of her life.
Today is just the first of multiple operations, all designed to allow the brain to grow and to give the skull and the face functionality. A secondary hoped-for outcome is to improve things from an aesthetic perspective. Basically, we want the child to pass what we call the ‘Tesco test’. I don’t know who coined it, but it means that when she’s old enough, I want her to be able to walk around a supermarket and hold her head up.
The first operation in a series carries more weight than the rest. We need to find a way to give space to the brain but without screwing up the operations further down the line. Everything needs to be meticulously planned, as it’s not enough to map out just what we’re going to do today. We’ve had to sketch out the whole of the next ten years of her life, which is why we work so closely with craniofacial plastic surgeons. In this case it’s my friend and colleague, David Johnson.
The Chiari malformation and the compression of the back of the brain needs urgent attention. The bone has worn away so much that it gives the appearance of fingers, mere slithers of carapace. In between is a more supple substance, but it’s not just dura. Some of it is actual brain.
Every heartbeat makes the brain pulsate a little, pushing against the inside of the skull. Despite the obvious difference in consistency between bone and brain, the bone is slowly worn away by the brain. The surface of the brain is rippled. The outer parts of each ripple – the mountains, if you like – called the gyri, wear away the adjacent bone first. In what we call the sulci – the dips or the valleys – there is less wear, so fingers of bone are left. Eventually, the gyri overlap and integrate these fingers of bone into the brain structure. The skull can look like a pepperpot – which is what we call the condition.
The stakes couldn’t be higher. We have to get those fingers of bone out without damaging the brain underneath and around. It’s complicated by the number of abnormal veins protruding from the back of the head. One misstep with the scalpel or dissectors and they become serious issues. At best, the veins might bleed. If they are major enough, they may cause a backup of blood in the brain and then a stroke. At worst, they’ll start to suck in air.
Anyone with a GCSE in biology knows that the heart is designed to hold blood, not air. Damaging one of these veins would be like sending air directly towards it on an expressway. One minute you’re dissecting the bits of bone, the next you have an air embolus on your hands – and within seconds, a potentially dead patient.
Think of deep-sea divers. If they surface too quickly they get an overload of nitrogen bubbles in their bloodstream, which can lead to lung problems and bring on a stroke and even be fatal. A baby on an operating table can experience exactly the same thing without getting its toes wet. Air in the bloodstream is like putting soap liquid into water. It creates bubbles that get pumped to the lungs, which then start to block off the blood flow in that direction, giving you a massive problem with oxygen delivery. I’d like to say it’s all just theory. Things you learn and hopefully avoid. But right from the get-go it’s all I can think about.
We make the incision into the scalp and the next step is to peel back the layer of skin to give me access to the skull. The scans are set to larger than life on my screen. I peep over my loupes to check once again for any veins in the cutting area. There are none flagged. Even so, I am constantly watching for any changes or bleeding as David makes the cut.
It goes to plan. We fold back the skin and begin phase two. I’m using very fine dissectors and an instrument similar to a microscopic spatula with which to lift the brain, then trying to nibble a bit of bone away with a small pair of pliers. It’s really slow, meticulous work. A drill would fly through in no time, but the risks of error are too great. This has to be done by hand, bit by bit by bit.
With every clean strike I inwardly breathe a sigh of relief. But if anything it only piles on the pressure. Each next movement seems to carry more danger, the greater likelihood of an accident.
We’ve been going nearly two hours when suddenly the anaesthetist says, ‘This isn’t good.’ Sumit Das is one of the best craniofacial anaesthetists around. Terrible taste in music, but he knows his stuff where it really counts. If he is worried, we all should be.
We pause. Look at him. Listen to his machines. The heart monitor sounds erratic. Something has gone wrong.
‘Change in oxygen levels,’ Sumit calls out. ‘Blood pressure dropping.’
‘What’s going on?’ I ask, already knowing the answer.
We start dripping then pouring water over the head, to avoid any more air being sucked in. ‘Air embolus.’ Even as he confirms my suspicions, he’s attaching a syringe to Baby’s drip.
‘Adrenaline,’ he announces, and starts to feed it into the patient, trying to provoke a response.
Nothing. In fact, worse than nothing. There’s a high-pitched continuous bleep. Exactly as you hear on every medical programme. The kind of bleep that means only one thing: flatlining.
‘Bloody hell!’ he says. ‘She’s arresting.’
Several things happen at once. The first is that the music gets shut down. I don’t have to ask. I certainly don’t complain. I couldn’t hear it anyway. The most obvious reason for the heart stopping is that we’ve caught a vein. David folds back the scalp as I continue flooding the area to keep the puddles of water where we want them. It’s fast, frenetic, reactionary. But nothing compared to what the anaesthetist is doing.
His job, at its most basic level, is to monitor the patient’s heart rate and keep it comfortable. He doesn’t expect to be doing it manually. He’s got his hands underneath the little sheet covering Baby. His fingers are locked around the spine, leaving his thumbs free at the front for tiny but firm chest compressions. He starts to press and count. Press and count. Too hard and he’ll snap the rib cage and possibly damage the lungs or heart. Too soft and he won’t trigger a pulse.
Everyone knows what they’re doing. We discussed this likelihood during our pre-op gathering. Even so, dealing with it is something else. We’re all matter of fact. Calmness personified, at least on the outside. There’s only one goal: bring Baby back to life.
I’ve done as much as I can at my end. David and I watch as Sumit, his head barely a foot from mine, works away. Thirty seconds come and go. I’m beginning to feel the pressure.
‘Right,’ says Sumit, after a while longer, ‘let’s stop and see where we are.’ He leaves his hands in place, but stops compressions. There is an eerie silence as we all look at the screen. I hear something. It’s a beep. A small, faint beep. Followed by another. And another.
I look at the anaesthetist. The sweat is pouring off him. He checks his machines. He takes his hands off Baby. He looks at me. And he says, ‘Panic over. Let’s try not to do that again, shall we?’
I’ve said that a good scrub nurse runs the theatre. The surgeons oversee the actual operation, but only up to a point. T
he anaesthetist is the one looking out for the patient’s overall well-being. I’m the one who decides what we do, but the anaesthetist is the one who decides what we don’t do.
After a flatline situation there’s always a bit of analysis, a bit of reparation. He’ll try to carry out various treatments to get the heart rate and blood pressure into workable positions. If he has any doubts at all, he can call the operation off. In this instance, I think more inexperienced anaesthetists would have done. They’d have panicked.
Our guy doesn’t. He knows us. He’s aware that we’d discussed causing an air embolus as a possible outcome and he was ready. The worst had happened and we dealt with it as a team. Once satisfied that Baby was stable again, he saw no reason why the team couldn’t continue. Which we do – without further problems.
I say ‘we’ because in this case the other consultant in the room, David Johnson, is about to take over. My task is to assist with taking the bone off the back of the head, these islands of bone, all the way down to the junction between the skull and the spine. We call this ‘pepperpotting of the skull’ – because that is what the skull looks like – with multiple holes in it. It went right the way around the head, and by releasing the restrictions at the back, we can take the pressure off the brain.
If it’s my job to remove the errant bone and protect the brain, it falls to my expert consultant colleagues from the plastic surgery department, like David, to run most of the show. They make the skin incision and peel back the skin from the skull, plan the layout of the new skull, and put the bone back so as to create a bigger skull for the brain, and then close up. Except we don’t put it back in this case. I’ve already thrown away all the ‘fingers’ of bone. We are left with a space the size of my palm, which on a six-week-old baby is quite substantial, but David just stretches the scalp back over the affected area, essentially leaving no bone over the back of her brain. We needed to leave room for further growth. Our next operation, when it happened, would be as much as a year away thanks to this procedure.
It’s quite hard to imagine a large boneless part of your skull. I mean, how do you lie down? Surely you’d notice the soft bit at the back of your head? Fortunately, babies never seem to realize. I suppose everything else in the skull is so soft that they don’t recognize when the skull ends and just skin continues. You hold your breath when you see them lie back, but they don’t flinch. It’s amazing, really.
Operations like this involve such close work with the plastic surgeon. I’ve known many over the years. They’re all incredibly gifted and driven to help. They’re not just in the business of cosmetic improvement, although of course they do facelifts, tummy tucks and boob jobs. They’re here to give patients a shot at a normal life. To help them to pass the Tesco test.
We would go on to do seven operations jointly over five years on this child. Each one was designed to bring us closer to that goal. Each one essential.
It’s not only the skull under the hair we have to worry about. Children with this combination of conditions also have very small faces. Their facial bones are extremely restricted in growth. Without intervention, Baby’s eye sockets – or ‘orbits’ – would naturally remain small as she aged. That was the case until she reached two years old. Then we did an operation on the front of her head.
To the strains of Black Sabbath, I remove Baby’s forehead and the upper parts of the orbits around the eyes clean off, as though I were lifting a pair of spectacles. David then refashions them into larger ‘glasses’, and essentially we reshape and reposition this bone further forward on the skull. I find the whole process fascinating. I’m as much a student as anyone. It’s the plastic surgeon who maps out my cuts and is in charge of piecing the shapes back together into a new-look skull. I’m just a highly paid carpenter.
The result looks a bit extreme until the rest of the face catches up. It also leaves gaps between bone pieces, which we pad with crumbs of sawn-off shards, then re-cover with the skin. The joy of the human body, though, is that the gaps will be filled by fully formed bone soon enough. It certainly makes our job easier.
With the children who are suffering from some genetic causes – so called syndromic craniosynostosis – it’s less about worrying over any gaps; we’re actually praying it doesn’t fill too fast. The whole reason for their particular condition is that it lays down bone too rapidly.
On Baby, we worked on the top part of the face first. In theory you could deal with everything at once, but it’s wasted time. Until children have their final ‘adult’ face, you’ll be redoing it again and again. It’s only around eight or nine, maybe ten, that you get a real sense of what they’re growing up to look like. Only then do you have something concrete to work with, and the final facial growth will sometimes only happen in the early twenties.
The whole process is hard for parents. I’m talking about from six to eight operations, but these aren’t back to back. They’re over a period of years. The parents and the patient have to live in the interim with a fairly different look for the child. And it’s not as if each operation is an obvious step forward. When we moved the whole ‘spectacle’ section of Baby’s head forwards, it didn’t look exactly right. But we weren’t doing it to improve her for that day or even that year. We were putting the infrastructure in place for her long-term maturity. She was literally going to grow into her face.
The advantage that parents have is knowledge. Being forewarned is forearmed, as they say. Some mums and dads, for example, are at work one day and they get a call to say their child has been hit by a car and is in intensive care. As regards the consequences, there’s sometimes no warning, no getting your head around it or preparing yourself. It’s a complete rug-pull.
On the other hand, when a child is born with a ‘medical’ condition affecting their skull structure, the issue is flagged early on and the fixing process is slow and methodical, so there’s plenty of time to adjust.
When we first sat down with Baby’s parents, it was a case of saying, ‘Your child’s skull isn’t developing normally. We’re going to take steps to adjust, but it’s merely the start of a long, long walk. Our priority today is to protect her brain function. Then we’ll work on her appearance – she might need or want further surgery to help her find her ideal place, if that’s what we all agree is needed. It shouldn’t be that a child should have to undergo surgery to conform to what the rest of the world thinks. But then again, it’s easy to moralize if you aren’t the kid getting bullied, the teenager left out of meet-ups, the adult feeling too shy to talk to someone special at the office party. It’s not going to happen overnight. But we will get there.’
At least that was the gist of it, but it took many hours to have that conversation. It starts with us saying hello in clinic, or on the ward. We go through everything so far, a full history and examination is done. Then we discuss the condition for a while. Later, after a break, our nurse specialist comes back. She goes through everything again – sometimes people like to ask her questions they feel might be too mundane to put to us. The ward nurses, all fantastic people with a wealth of experience, also help to discuss the recovery from operations. Play specialists may help with anxiety for the older kids, some of whom know exactly what’s going to happen to them and are distinctly unimpressed.
In some cases, where the patient has just one single growth line that’s fused early, we’re able to cut some of the skull out, reshape it, put it back together and hey presto – it’s done. We meet at clinic once a year, but usually that’s all that’s required. We might stay in touch for fifteen years and never do anything other than chat. You just never know.
More serious ‘plain neurosurgery’ cases will stay in my orbit for eighteen years. The follow-up is as important in some ways as the cutting and shaving. It doesn’t matter what level of treatment you get. You’re my patient until you stop being a child and beyond. From birth to voting age. And even after then, I guarantee I’ll be sticking my beak in and checking your files when my colleagu
es in the adult wing take on your case. However, craniofacial patients don’t get moved on – we keep them. Since we really are experts in their conditions, there is no benefit in transferring their care to someone who does less of this work.
One of the things I say to parents is, ‘We’re not looking for perfection. Not everyone out there can look like Angelina Jolie. God knows, I certainly don’t look like Brad! What we want is for them not to stand out.’ And that might sound like a low target, but think about all the people you see on a day-to-day basis. Look at your fellow travellers on your daily commute. How many stand out as beautiful or attractive or something special? Very few, I suspect. The rest of us just sort of jog along, nothing special, no one’s looking twice. We’re not going to win Sexiest Man Alive – and we’re not going to turn milk sour by looking at it either.
My goal is simple: I want my patient to be able to decide what he or she wants to do in their life. And to do it without feeling restricted because of the way they look and the way they function. I want them to be comfortable at school, at social functions as teenagers, at work. Maybe they’d like to go out to bars and clubs – chat people up, find a partner, get married. Maybe they don’t. The point is, I want the decision to be what they want, not what they feel they are able to do because of how they look. I want them to be happy people. It’s what we all wish for our children. And if I want it for my own offspring, then I want it for all my kids.
Obviously for the more severely affected patients, brain function can be an issue that becomes more apparent over time. Parents start to notice the difference between their child and others, not only physically but developmentally. Perhaps they are slower to move forward in school, they need one-to-one attention, sometimes even special schooling. That’s much more difficult for families to cope with. Baby’s parents were typical. They weren’t worried that she was slower to learn than her peers. They were looking ahead. ‘What’s going to happen to her when we’re gone?’ They never stop caring. Never stop putting Baby first.
Everything That Makes Us Human Page 11