Hip pain as a teen leads to life-threatening diagnosis

Lauren Casey was 15 and a tenor sax player in her Minnesota high school marching band when the pain in her hip and lower back became severe. It hurt when she walked and when she stood for hours at her fast-food job. Soon, she began feeling like her ribs were popping out of place.

She thought maybe carrying around her 10-pound instrument, much of its weight on her neck, was the problem.

“It was heavy for someone tiny,” she said.

Casey’s parents took her to a string of doctors during her teenage years — internists, an osteopath, a physical therapist — but none could solve what ailed her. One said her problem was a “twisted pelvis.” Another, she said, suggested “it was just in my head.”

“It was doctor after doctor shutting me down,” said Casey, now 24.

In college, Casey sought alternative treatments, such as one focused on manipulating her musculoskeletal system. But the pain always returned, she said.

After more than eight years of seeking help from myriad medical professionals who failed to identify what was wrong with her, she essentially gave up. Casey concentrated on her studies and decided to tough out the near-constant pain.

Then, her family moved to the Chicago area, and her father urged her to try a new physician. She made an appointment with a chiropractor who ordered X-rays and an MRI of her entire spine and neck — something no previous doctor had requested. What appeared on those images was so unusual, Casey was quickly referred to a neurosurgeon. That, she said, began the fraught path to solving her years-long pain.

“I lived for 22 years not knowing what this was,” she said. “But I’m glad someone found out what was wrong. … I just didn’t realize it would be such a big deal.”

Born this way

Casey’s condition began in the womb. Unlike typically developing babies, her upper spine failed to fully unfurl, which left her neck tucked slightly downward at birth. Her skewed anatomy only worsened during adolescence, doctors said.

Over the years, she adjusted, torquing her body in various ways to compensate for her misaligned spine, doctors theorized. This caused her hip and lower back pain to flare as a teenager. Casey said sleeping propped up on pillows bothered her neck, and sometimes led to migraines. But none of her doctors during those earlier years considered her neck or cervical spine to be the culprit, she said.

At college in Wisconsin, Casey hunkered down and focused on earning her computer science and math degree. She stopped playing tenor sax. Over-the-counter pain killers and a heating pad helped her endure, she said.

“The pain definitely slowed me down and made things hard to enjoy,” she said. “But I kept pushing through it.”

In 2023, after landing her first job out of college as a software engineer, she decided to try a chiropractor at Aligned Modern Health in Kildeer, Illinois.

That chiropractor, no longer with the practice, ordered the images of Casey’s entire spine. Suddenly, the abnormalities in her upper cervical spine and the base of her skull were clear.

“I’d never seen this in a patient before,” said chiropractor Hailey Spencley, also part of the practice, who reviewed the radiology findings and then called Casey back to the clinic.

“This was not something I wanted to do over the phone,” Spencley said. “I had her come in so we could go over the results and get her the care she needed.”

A rare disorder

Two referrals later, in October 2023, Casey met with Russ Nockels, a neurosurgeon and chief of spine surgery at Endeavor Health Northwest Community Hospital in Arlington Heights. Nockels explained that Casey’s pain was the result of a rare, congenital condition called basilar invagination. During fetal development, a bony knob called the dens, or odontoid peg, is supposed to form squarely atop the spine to provide stability for the neck and head. A little like “a lollipop on a stick,” according to another neurosurgeon who worked on the case.

For Casey, that typical alignment never evolved; instead, the peg ended up pressing backward into the base of her skull and, over years, had begun to impale Casey’s brain stem. By the time neurosurgeons got involved, Casey’s spinal cord had compressed by about 50%.

“The whole stability system failed,” said Nockels, adding that Casey’s condition only impacts around one to two people per million worldwide, according to an estimate. “It was really bad.”

So bad, he told Casey, that a simple fall or short stop in a car could have been fatal.

Sammy Khalili, the head and neck surgeon on Casey’s care team at Endeavor Health, added: “She was one hit, one cough away from being a paraplegic.”

When Casey first learned of her condition, she said her “heart dropped” with fear. “I went from ‘There’s nothing wrong with you’ to ‘Actually, you have to have surgery or else you’re risking your life every day,’” Casey said.

The surgery was complex and risky, largely because of its location in the cranio-cervical region, where the spinal cord and brain stem connect and a critically important hub of the nervous system controlling functions that maintain life.

“She could have wound up unable to breath, unable to move anything, literally a head on a pillow for the rest of her life, with no feeling, no movement in any part of body,” Nockels said. “Or she could have died.”

Through the nose

Decades ago, the most common method to treat this condition was to go in through the patient’s mouth, said Amin Kassam, another neurosurgeon working on Casey’s case. Doctors would retract the top opening of the mouth, cut the back of the throat, remove the peg completely, then place rods and screws in the area for stabilization.

In the early 2000s, Kassam said, he and colleagues pioneered a technique in which the peg was removed through the nose, which was less invasive — with no manipulation of the mouth or throat — and posed a lower risk of infection. For Casey, the team strategized about a unique variation: “We thought, ‘Why are we taking the peg out? There’s nothing wrong with it. It’s just in the wrong spot,’” Kassam said.

They developed a plan: create a slot at the bottom of Casey’s skull, and instead of taking out the peg, move it forward into the slot. This allowed them to dis-impale her spinal cord and brain stem; then, with X-ray guidance, the doctors could slowly let her head extend and tilt backward to its natural position.

But this region of the head and neck is rich with critical arteries, blood vessels and nerves. So, Melanie Fukui, a neuroradiologist on the care team, built a 3D model of Casey’s brain and skull, mapping out the important anatomy and structures before surgery.

“When you have a deformity like this, the relationships (between bodily structures) are not typical,” said Fukui, who likens herself to an “air traffic controller” in the operating room. “You go in there with drills, and these surgeons, they have a sixth sense of where things are, but in this case, that doesn’t always work because things aren’t where they’re supposed to be.”

In January 2024, Casey arrived for the first of her multistage operations.

“I was terrified because so much could go wrong, but at the same time, I didn’t have a choice but to go through with it,” she said.

Kassam’s surgical team went in through her nose and created a channel and slot for the peg, while Nockels placed a carbon fiber ring — a halo — around her head for stabilization.

Nockels then began to move the odontoid peg toward the correct position, but not all the way. After Casey spent three days in the ICU in traction to allow the peg to move slowly out of the skull base, Nockels turned Casey face down on the operating table. Then, using cobalt chrome rods and MRI-compatible screws, he manipulated the peg fully into its proper alignment.

Kassam said that to his knowledge, Casey was “the first person to undergo this procedure,” in which the head was returned to its correct position without removing the odontoid peg.

Casey, now living in Ponder, Texas, endured an arduous recovery: The halo remained on for about a month, then she wore a series of hard and soft collars around her neck. At first, she had to sleep sitting up in a chair. Swallowing was difficult, and she required help from her parents. “There was lots and lots of soup,” she said.

Casey lost significant rotation capability in her neck and still lives with mild scoliosis, spinal stenosis and bone spurs. Overall, though, the surgery was a success. Her hip pain is gone, and she has started walking regularly. She took a vacation to Las Vegas this summer and said she was amazed by her “seven straight days of no pain.”

Now, she can do what she wants “as long as it’s not jet-skiing or something crazy.”

Mostly, she’s just grateful.

“I’m lucky it turned out the way it did,” she said. “... I feel like I can live my life.”