Baby Michaela home after Monash Children’s Hospital repairs hole in her head

Herald Sun, 15 April 2016, Grant McArthur

Baby Michaela home after Monash Children’s Hospital repairs hole in her head exposing her brain.

ON Christmas Day Christy Puyk was delighted to hear her baby Michaela cry after a trouble-free labour, then feel her placed on her chest for their first cuddle knowing everything had worked out perfectly.

But next to them Michaela’s father Keith Miller and the stunned Angliss Hospital staff suddenly realised something wasn’t right.

“I noticed straight way — it literally looked like somebody had cut a porthole straight out of the top of her head,” Mr Miller told the Herald Sun.

“You could see her brain.

“The brain was not outside of her head, but there was no … it looked like a normal head, it wasn’t raised, there was no lump.

“It was our first baby and I was thinking — is this normal, I don’t think this is normal.”

Despite newborn Michaela appearing to be in otherwise perfect health, it was obvious she had a 10x4cm section of the top of her head missing, exposing her precious brain to the world.

Within a minute the doctors had removed the newborn from her mum and were huddled in the corner with her, trying to work out what they were seeing.

“We saw what it was, but she just looked so healthy,” Ms Puyk said.

“She was crying and doing everything normal, so it was really hard to grasp just how serious it was.

“Ten to 15 minutes later she was taken down to the special care section and we didn’t know much about what was going because they (doctors) didn’t really know.”

Three hours after her birth Michaela was transferred to the specialist care at Monash Children’s Hospital and diagnosed with a cutis aplasia — an extremely rare condition where the skin at the top of her head fails to form — but in a case far more severe than anyone had previously seen.

Not only was Michaela missing skin on top of her head, but the bones which would normally meet near the centre of her skull were sitting much lower than normal to the side, meaning her brain had no protection.

The situation then became even more dire when it emerged she also had a hole in protective membrane covering her brain and Michaela’s cerebrospinal fluid was leaking out.

Facing risks of infection, an accidental knock to her brain or damage to a blood vessel that would result in bleeding to death, Michaela was facing a 30-50 per cent chance of not surviving long enough to close her head.

On Boxing Day, Monash neurosurgeons cleaned up area around Michaela’s exposed brain before plastic surgeon Rory Maher grafted skin from the newborn’s back and covered part of the hole.

“The main concern is of infection and it is quite alarming for the parents — but they coped amazingly,” Mr Maher said.

“We skin grafted it, but that was just to try and reduce the wound. But she still required dressings over the front third of it because the graft could not take over the area with fluid leaking meant the skin could not survive.

“The actual intervention was a relative simple things to do … at the end of the day she has healed it herself because the wound, just by natural healing, has got smaller and smaller.”

Under constant watch in neonatal intensive care, Michaela had to undergo twice weekly lumbar punctures to drain off the leaking brain fluid, as well as a weekly general anaesthetic so the dressings on her head could be changed.

Another two skin grafts saw the wound gradually begin to be closed over and Michaela began wearing a helmet in place of her missing skull.

“Christie and myself couldn’t do anything except the same thing all the doctors were doing — wait, wait and keep hoping,” Mr Miller said.

“The graft got smaller, smaller and smaller and, eventually, it was the size of a pinhead with a tiny leak and we could see the light.”

For three and a half months there was little the doctors or parents could do other than try to avoid infection and hope Michaela’s head would seal.

“Those first couple of weeks were just terrible. Seeing her with all those breathing tubes, her not being able to move and me not being able to hold her, it was the worst feeling in the world,” Ms Puyk said.

“She is perfectly healthy except for that one bit.

“From the start they were pretty straightforward, saying that with infections if anything gets in she won’t survive it because it will go straight into her brain.

“It was horrible waiting, but she was in the right place and we knew she was getting the best care, so that made it easier.”

Finally on Tuesday, after almost four months and infection free, Michaela was discharged to her Narre Warren home for the first time.

While the bones are still to form at the top of her skull, the gap has halved already and it is hoped Michaela will have a fully protected brain by her first birthday.

Until then she will have to wear a helmet, but Monash Children’s paediatrician Dr Cathy McAdam said the main danger has passed.

“I am expecting that by the time she goes to school she will just be like every other kid,” she said.

For Ms Puyk there is no way to describe the joy having her daughter home.

“I have not had a smile off my face since she got here.”

“Before she was born we decided we did not want to baby her. If she falls over we are just going to pick her up and keep her going.

“Now we want to do the same thing, but we’ll probably be more cautious.”