0.08%
world's population affected by Atlanto-occipital assimilation
Illinois parents find care for son’s rare spinal condition at Stead Family Children’s Hospital
After a 13-hour surgery last Halloween, Luke Johnston connected with his Iowa care team including the hospital facility dog, Corrin
Luke Johnston’s parents searched the country for a surgeon who could perform the high-risk operation their son needed for his rare medical condition. They found that expertise close to home at University of Iowa Health Care Stead Family Children’s Hospital.
“When he was born, they did lots of different tests because of multiple congenital markings,” his mother, Amy Johnston, says of Luke’s birth at their local hospital near Peoria, Illinois. “It was very traumatizing. We felt he was doing great, but they were telling us all of these things they were worried about.”
Luke was born with fused ribs, scoliosis, a tethered spinal cord, congenital ptosis, clubfoot, and webbed fingers. He also failed his newborn hearing screening.
Blood tests revealed Luke had Saethre-Chotzen syndrome, a genetic disorder characterized by fused bones. He underwent tethered spinal cord surgery and webbed finger repair at their local hospital before he turned 1.
“Stuff that’s not supposed to be connected - is fused together,” his dad, Joey Johnston, says of the disorder. “For Luke, that means his C1 vertebra was fused to his skull.”
Luke’s deafness is related to the syndrome, Joey Johnston says, “because the bones inside his ear were fused together and not formed correctly, so they don’t work the way they’re supposed to.”
Saethre-Chotzen sydnrome is rare - it affects about one in 50,000 babies. On top of that, Luke in 2024 was diagnosed with an even rarer condition.
‘Luke’s World Tour’
Because Luke suffers from sleep apnea, he underwent a sleep study, and his pulmonologist ordered an MRI to ensure a common treatment would be beneficial.
A continuous positive airway pressure machine, commonly called a CPAP machine, keeps the airways open during sleep. The MRI determined a CPAP machine would work for Luke, but it also unveiled another condition.
Luke was diagnosed with atlanto-occipital assimilation, in which portions of his spine were pushed into where his brain stem and spinal cord meet, putting him at risk of paralysis, even from a short fall.
“The condition is extremely rare, with only 0.08% of the world’s population affected. It’s even rarer for a child to have this,” Amy Johnston says. “Typically, adults get this in bad car accidents. We think this was something he was born with.”
His parents were sent to their local neurosurgeon, who referred the couple to the few hospitals in the United States that perform the needed surgery.
“We did what we call ‘Luke’s World Tour’ and visited all three hospitals,” Amy Johnston says. “Everybody talked so highly of Stead Family Children’s Hospital we ended up going there. They were so confident that they could let Luke live his life again.”
“Something that really drew us there was that Dr. Arnold Menezes, the neurosurgeon, had written articles on the syndrome,” she adds.
Signs point to Stead Family Children’s Hospital
Patients from as far as Indonesia, Japan, and South Africa have made the journey to Iowa City specifically to see Menezes, MD, and Joey Johnston says the doctors they visited in Boston were impressed the world-renowned surgeon conferred on Luke’s case.
Other signs solidified their choice.
“I was at a rest stop in Missouri and a guy saw Luke in his neck brace and came over to recommend going to Iowa,” Joey Johnston says. “His daughter had surgery there with Dr. Menezes. It was like everybody was pointing us in that direction.”
A Halloween surgery
The date for Luke’s cervical spinal fusion surgery at Stead Family Children’s Hospital happened to fall on Halloween.
“He was upset over this,” Amy Johnston says, “but the staff was so amazing, they made us cry. All of them dressed up in costume before surgery to make it feel like our Halloween-loving 7-year-old wasn’t missing out.”
During the 13-hour surgery, performed by neurosurgeon Brian Dlouhy, MD, his parents were regularly updated and say they trusted his care team.
Luke was on a ventilator in the intensive care unit after the surgery.
“That staff held our hand during some of the most difficult days of our lives,” Amy Johnston says, and Luke found a friend to inspire him, as well.
Upon seeing Corrin, one of the hospital’s facility dogs, Luke smiled - this was the first time his parents had seen their son smile in a week.
“Corrin was such a vital piece to Luke’s healing,” Amy Johnston says. “Luke was willing to get out of bed for Corrin and do therapies with her, as well. She played an important role in Luke’s recovery, for sure.”
"For the worst possible thing to happen to your kid, it was the best possible scenario."
Amy Johnston
Luke’s Mom
Unforgettable experience
Within that time, Luke and his parents were able to experience “The Hawkeye Wave” from the hospital during a Hawkeye football game.
“It was a night game, so everybody put their phones on to wave and Luke just started crying,” Amy Johnston says. “It made him feel like he wasn’t alone, and all of those people were there to cheer him on and help him get better. He won’t ever forget that."
Luke was an inpatient for 10 days and his parents are happy they chose Stead Family Children’s Hospital for their son’s surgery.
“I felt like it was the first time I could breathe in months,” Amy Johnston says. “I felt like we were in the best hands.”
Now 8 and in second grade, the Dunlap, Illinois, boy enjoys art, puzzles, coding, and writing books, including a book about surgery he shared with his class. His parents say Luke is both sensitive and silly and likes to make other people laugh.
Since the surgery, restrictions on his movement have gradually been lifted, and he continues to be seen at Stead Family Children’s Hospital for checkups and in specialty clinics.
“We’re definitely part of the team,” Amy Johnston says, noting Luke’s care team let him pick out the color of his brace and try to involve him as much as possible. “Just little things like that were a big deal to Luke. For the worst possible thing to happen to your kid, it was the best possible scenario.”
Photos from Kids Day 2025
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