Extensive surgery helping little Leni West get new outlook on life

Leni West was born with a rare condition which doesn’t leave any room for her brain to grow. The toddler has already undergone three facial reconstructions and is about endure more surgery as her parents ready her for school - and the real world.

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Before she goes to school for the first time Leni West will get a new look.

The extensive plastic surgery will help her breathe better but hopefully it will also make her fit in a bit better.

At three, she is blissfully unaware of the stares she gets but her parents Tim and Lisa know all too well that the world can be cruel to those who look different.

“We want to raise awareness about facial differences, because people can be so cruel and they look at her funny. I just wish we could spark up a conversation. Yes, she does look different but she is still an amazing little girl,” Mrs West told The Sunday Telegraph.

Lisa West with daughter Leni, three, who was born with her skull plates fused. Picture: Richard Dobson

Leni has already undergone extensive reconstructive surgery for the condition she was born with.

Crouzon Syndrome is a genetic disorder that leads to the premature fusion of seams (sutures) in the skull. When she was born, it was clear from her head shape she would need urgent surgery because her fused skull was hampering her brain growth.

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“The developing brain usually drives skull growth, so if the sutures are fused, typically you get raised pressure in the head and that can lead to all sort of developmental problems,” Dr Adam Fowler, neurosurgeon with the craniofacial unit at the Children’s Hospital Westmead, said.

To help kids like Leni go to www.radiothon.org.au

“They had cut her eye to eye and took a strip of bone that was fused together out. She needed full blood transfusion, it was a pretty scary time,” the Baulkham Hills mum, who is pregnant with her second child, said.

Plastic and reconstructive surgeon Dr Damian Marucci has operated on Leni three times so far.

Leni has undergone several operations since her birth.

The operations including opening her skull.

“What we did was try to release some of the tension on the brain by removing strips of skull bone that had fused early. That got us out of jail for a while, gave us time while her brain kept growing, but everything fused up again and we needed to move the back of her head, the skull backwards to give the brain more room,” he said.

Called a posterior vault expansion, plates and screws were inserted in the bone.

“You can turn the screw half a millimetre twice a day and what it does is slowly push the bone back and stretches the bone,” Dr Marucci said.

Plastic surgeon Damian Marucci.

At age two, last July, the toddler underwent more surgery to remodel the front sector of her skull and forehead to free up more space for her brain but also to bring her forehead forward to protect her eyes.

But the big one is to come.

Before she goes to school in 2021, she will have what is called a mid-face advancement, which will expand her face forward to make space for her airways, relieving the need for a tracheotomy to aid her breathing. The condition also affects the airways due to the restrictive bone growth.

“We are going to bring her cheekbones forward in order to improve her upper airway and to give her lower globe of the eye more support. It’s called a Le Fort 3 advancement. She will be wearing a frame that screws into the side of her head and will slowly pull her face forward over a couple of months,” Dr Marucci said.

“It will improve her breathing and it will also improve the aesthetic and normalise her face a bit more.”

Leni with her parents Lisa and Tim.

What is a constant challenge for her parents however, is the inability of so many to accept the face of difference with grace.

“Other kids will look at her funny, then she starts dancing and they start dancing with her and they realise she is just like them, she has an infectious personality,” Mrs West, 35, said.

“But I can’t get used to the comments. My instinct is to take her away. She doesn’t realise she is different yet, but that stuff will get harder.”

Mr West said most people were okay but “you do get the odd person who will say something”.

“Or they clear out quickly, the parents feel uncomfortable in a playground because Leni looks a bit different and rather than have their child in the situation and say something they just remove the child. She can clear a playground in 10 seconds, which is also hard,” he said.

They hope society can become a bit more accepting of difference, especially as Leni faces the challenge of school when she turns five.

“Yes she looks different but apart from that she is so friendly and she will go out of her way to say hello to the other kids and give them a high five,” Mr West said.

“Once she goes to school, I just hope she finds a really good circle of friends that take her under their wings and protect her.”

Originally published as Extensive surgery helping little Leni West get new outlook on life