Daughter’s Chiari malformation managed with decompression procedures
As a toddler, Sonja Young’s daughter, Louise, struggled to reach developmental milestones.
“We suspected something amiss from the time she was born when her oral-motor control issues were evident in her struggle to feed,” explains Sonja. “She gagged easily and drooled.”
Along with feeding issues, Louise, had poor balance, weak hands, weak core muscle strength and an awkward gait. Her language development was also delayed. When it came time for school, she tested into special education preschool.
| Louise attends a four-year college and in the summer
is a lifeguard at a northern Michigan camp.
Both Sonja and her husband were frustrated with the direction of their daughter’s medical care. They wanted her doctor to seek the underlying cause of her struggles. Recalls Sonja, “I believed there was an explanation for her symptoms.”
In their search for a diagnosis, the Youngs sought the help of pediatric developmental specialists; otolaryngologists or ENTs; allergists; gastroenterologists; cardiologists, neurologists; neurosurgeons; PT/OT/speech therapists; and a neuro-psychologist.
In 1999, after moving to Michigan, her family sought a new medical team for Louise. A brain MRI was ordered for cerebral palsy. While it came back negative, a neurologic condition known as Chiari malformation was noted. A subsequent MRI not only confirmed Chiari malformation, but also showed syringomyelia. At last, the Youngs had an answer.
But what did the diagnosis mean? Louise was 8-years-old. What was her prognosis?
According to the National Institutes of Health, Chiari malformations are structural defects in the part of the brain that controls balance. The resulting pressure on the brain and brainstem may block the flow of cerebrospinal fluid. A related disorder, syringomyelia, is a condition in which excess fluid builds up within the central canal of the spinal cord.
Louise has the most common form of Chiari malformation known as type 1 CM. While there is no cure for this condition, it can be managed. For those experiencing symptoms, like Louise, surgery is one of the few treatment options.
Three months after diagnosis, Louise had her first decompression procedure with the hope of stabilizing her symptoms. This was followed by a second surgery performed in 2003 by Holly Gilmer, M.D., director, Pediatric Neurosurgery, Beaumont Children’s Hospital.
During the decompression procedure, Dr. Gilmer says, “I create space by removing the back wall of the skull and back segment of the vertebrae, also called a C1 laminectomy. This relieves pressure on the spinal cord. Patients usually return to school in three weeks and full activity in six weeks.”
Says a grateful Sonja, “These surgeries kept Louise’s symptoms from progressing, stabilizing her. Over time she has learned to compensate for her deficits. Some symptoms will likely never be resolved, but she’s gained strength and endurance.”
Dr. Gilmer says that with the advancement in imaging technologies, type 1 CM is not as rare as once believed. In fact, she estimates about 20 percent of the population have this condition.
Fast forward nine years and Louise, 20, is attending a four-year college more than 100 miles from home. The sophomore is majoring in Actuarial Science. Her goal - to work for an insurance company or the government upon graduation. During the summer, she’s a lifeguard at a camp in northern Michigan.
Louise’s advice to others with CM, “Never give up. You can do whatever you set you mind to.”
Editor’s note: Beaumont’s Neuroscience Center of Excellence will open a Chiari malformation clinic in Royal Oak this year.
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