Duke is building the first program for Chiari malformation in the southeastern U.S. under the leadership of Gerald A. Grant, MD, chair of the Department of Neurosurgery, a national expert in treating the condition. It will be the third center of its kind in the country.
Grant says the focus is on building a program that provides comprehensive and holistic care for both children and adults across the lifespan.
“The diagnosis of Chiari malformation (type 1) is more common than we know because an MRI is required to make the diagnosis,” says Grant. “It is often a congenital problem, but it can be acquired, as well, and affects all ages.
“It is an especially unusual disorder because the diagnosis is not based on whether the patient has developed symptoms,” Grant adds. “We are studying the natural history of Chiari 1 over time with or without surgery.”
Classic Chiari symptoms typically present as a headache at the back of the head which worsens with a Valsalva maneuver such as a cough or straining. Patients might also present with symptoms due to cervical spine instability or weakness to developmental delays and breathing problems, which can cause sleep or eye-related problems.
Interdisciplinary expertise is essential to manage the comprehensive care of patients with Chiari malformation and syringomyelia. At Duke, the collaborative team includes neurosurgeons, neurologists who specialize in sleep issues, neuro-ophthalmologists, neuroradiologists, interventional radiologists who manage cerebrospinal fluid leaks, pain management specialists, and psychologists. Working as a team is essential to provide the individualized care and offer the best options to maximize quality of life, Grant says.
Before coming to Duke, Grant helped build the Chiari malformation program at Stanford University.
Patient Case
In his first surgical case since arriving at Duke, Grant performed surgery on a 5-year-old boy with Chiari malformation whose older brother has also been diagnosed with and previously treated surgically by Grant for the condition.
The patient had experienced speaking difficulties, including stridor, and recently reported headaches and neck pain. Because the patient’s symptoms were worsening rapidly and severely affecting his ability to function, Grant moved forward with a decompression surgery. “This surgery is important to do the right way the first time to avoid further complications and problems with these patients over time, in children especially,” he says.
The decompression surgery involved removing bone to create more space around the cerebellum and cervical spine, which helps restore the normal flow of spinal fluid and relieve pressure on the spinal cord, halting further damage.
Following the procedure, the patient no longer experiences headaches. Other symptoms are improving, although baseline issues persist that will require team-based follow-up and surveillance.
“Kids need to be followed closely after surgery over several years to ensure they are the best they can be since in rare cases, scarring, spinal leak, syrinx enlargement, and/or hydrocephalus might develop,” Grant explains. “Once they stop growing, the risk for those complications decreases, but we will still watch both brothers all through high school.”
