Weston’s story: Reshaping what’s possible

When 1-year-old Weston and his parents go for walks, people are captivated by his big grin and expressive eyes. And they’re delighted by the blue, green and yellow trains on the helmet on his head.

“Weston has to wear his helmet for 23 hours a day. But it’s normal for him. And when we say, ‘It’s time to put your helmet back on,’ he starts to giggle,” says Catherine, Weston’s mom.

Weston’s helmet looks playful. But its purpose is serious. It actively supports the growth and development of Weston’s skull and brain.

Two conditions most people have never heard of

Weston’s dad was teaching in his classroom when he received a call from Catherine and had to step out. She was talking calmly, but the news wasn’t good.

Catherine was relaying to Hayden that she’d just discussed the results of an advanced imaging test called a fetal MRI with her maternal fetal medicine (MFM) specialist, a test to investigate something abnormal looking with their baby's kidneys.

The test confirmed that instead of having two separate kidneys, Weston had a single, fused kidney.

The test also revealed another, more serious problem that Hayden had never heard of: pediatric craniosynostosis.

A calm wait-and-see approach

Catherine’s MFM recommended Catherine and Hayden contact the FETAL Center at Children’s Health to schedule a virtual appointment with Alex Kane, M.D., Plastic and Craniofacial Surgeon at Children's Health℠ and Professor at UT Southwestern.

They immediately liked Dr. Kane, who calmly and clearly spoke to them about the varieties of craniosynostosis and the need to take a wait-and-see approach.

He explained that the human skull is designed to grow as the brain grows. There are several joints in the skull (called sutures) that allow that to happen. When kids have craniosynostosis, some of their sutures are fused together in a way that doesn’t give the brain the room it needs to grow.

Dr. Kane said they’d have to wait until Weston was born to know for sure how many of his sutures were fused and then they could develop a care plan.

“I told the family that we have a few different ways to help make sure there’s enough room for a child’s brain to grow -- and that our team has a lot of experience performing those procedures,” said Dr. Kane.

Not fitting in the boxes

Weston was born with a unique head shape, even for kids with craniosynostosis.

“Most kids born with craniosynostosis have a single fused suture, making their head long and narrow or bulging in one place. But Weston had multiple fused sutures – his head was shaped kind of like a four-leaf clover,” says Brad Edward Weprin, M.D., Pediatric Neurosurgeon at Children’s Health and Professor at UT Southwestern.

His head shape was not the only unique thing about Weston’s case.

Craniosynostosis is often linked to other genetic syndromes, but genetic testing revealed Weston had none of them.

Developing a care plan

At just four weeks old, Weston’s parents took him to meet Dr. Kane.

“Dr. Kane went over the big picture and the options moving forward without making it sound super scary,” says Catherine.

Weston needed surgery because of increased pressure on his brain, which put him at risk of severe developmental challenges.

Often, craniosynostosis is treated with a major surgery called open cranial vault remodeling, where surgeons take a child’s skull off, reshape it and put it back on. Kids need to be a year old to have this surgery because their skull bones need to be firm enough to hold the fixation devices they use to remodel the skull.

Weston’s team determined that a less invasive type of surgery called a strip craniectomy would be a good option for Weston.

“Weston’s family was very fortunate to have an early, correct diagnosis before he was even born, which doesn't happen very often. His early diagnosis allowed us to be able to perform a strip craniectomy which can only be done at 3 months of age, when a child’s skull is still very flexible,” says Dr. Weprin.

During a strip craniectomy, small strips of skull bone are removed to create more space for the brain to grow. This surgery involves less blood loss and a much quicker recovery than cranial vault remodeling surgery.

Weston had strip craniectomy surgery on a Friday and was discharged from the hospital on Sunday.

A different child after surgery

On Sunday, after the expected swelling went away, his parents immediately noticed Weston seemed so much happier and healthier.

In the weeks before surgery, Weston was quiet and wasn't smiling much. His parents wondered if he was uncomfortable, even though a retinal exam showed his intracranial pressure was OK.

“It was a huge blessing that they scheduled him for surgery so soon,” says Catherine. “Because he was a whole different baby after. He was happier, he was smiling, his color was better – and he just looked and seemed better than he ever had.”

Strip craniectomy surgery is typically followed by helmet therapy – and five days after surgery, Catherine took Weston to see Tiffany Graham, M.S., Associate Professor at UT Southwestern to be custom fitted for his first helmet that the family painted to be a Texas Ranger’s themed one.

“Helmet therapy basically restricts skull growth in the places you don't want it to grow and leaves space where you do want it to grow, using a tiny bit of pressure, but nothing uncomfortable,” says Dr. Kane.

Developing completely normally

So far, helmet therapy has worked very well, and Dr. Kane, Dr. Weprin and Graham will continue to monitor his helmet therapy treatment plan until he’s around 18 months old.

When Weston takes off his helmet to show off his strawberry blond locks, you’d never know he’s been through what he has.

In terms of his development, Weston is completely on track for growth, feeding and motor skill markers. Weston started his life out with gastric reflux and needing feeding therapy – and in the first percentile for weight.

“Now, he’s in the 70th percentile,” says Catherine. “He can’t get enough green beans, avocados and bananas. And he’s crawling, pulling up and ready to start walking.”

Looking back and focusing on the future

Catherine now describes Weston as “an outdoor baby.” He loves going for walks where he leans forward in his stroller to look around at the trees, people and dogs that they pass. He especially loves going outside each evening to greet his dad as he walks up the driveway by squealing “Da Da Da.”

“Weston just loves being around people,” says Catherine. “I think it’s because, from a very young age, he's had to go to so many doctor's appointments. He’s spent his whole life around a lot of adults and kids in many different settings – and it’s made him very social.”

Weston still attends several types of follow-up appointments, but his parents and care team are very optimistic about his future.

He’s had no issues related to his cranial surgery – or his fused kidney, which is being monitored by Israel Nosnik, M.D., Pediatric Urologist at Children’s Health.

“It's a totally random thing to have your child be born with two rare conditions. And it was a terrifying ordeal, especially with it being our first kid. But a lot of growth happened for us as people and parents – and a lot of learning,” says Hayden. “And the end result has been amazing.”

To which Catherine adds,

Learn more

The Pediatric Plastic and Craniofacial Surgery Program treats kids of all ages with craniofacial differences, including all types of pediatric craniosynostosis, with a combination of compassionate care, innovative treatment and advanced surgical techniques.