Usually a visit to the doctor means a diagnosis and a prescribed treatment that improves or cures the patient’s problem. However, people born with a Chiari malformation are often misdiagnosed many times because their symptoms mimic other medical conditions or their doctors aren’t familiar with this rare neurological disorder. People with the mildest form of Chiari malformation may never experience symptoms and first learn about their condition from a diagnostic imaging test for a different medical issue. Others may develop symptoms during childhood or even in their late 20s and 30s. From that point, they often begin a long process to find an accurate diagnosis and a correct course of treatment.

Chiari malformation is a structural defect in the brain that occurs during fetal development. A portion of the cerebellum, the bottom part of the brain, drops out of the skull and crowds the spinal cord, which pressures both the brainstem and the spinal cord. This pressure blocks the flow of cerebrospinal fluid, known as CSF. Cerebrospinal fluid surrounds and protects the brain and spinal cord, so a Chiari malformation disrupts the natural flow of CSF, compresses brain and spinal tissue, and increases pressure within the brain. Difficulties with balance and coordination, numbness or tingling in the face, arms and legs, vision or hearing problems, muscle weakness, trouble swallowing, fatigue, dizziness and headaches are signs of Chiari malformations.

Anjolina Ross, age 22, was diagnosed with a Chiari malformation at age 18. Her migraine headaches, tingling in her hands, feet and face made it hard for her to work. Ross comments, “I started having headaches around age 12, but it took years to get diagnosed.” She tried countless medications and treatments, but eventually none of these worked. “There was nothing else I could do but surgery, and I was more scared of having migraines than of having surgery.” As a working mother with a one year old daughter, she said, “I was always worried that in the middle of playing with my daughter, I’d get a migraine, and I couldn’t do anything about it till my husband came home. This scared me.”

Bonnie Bassford, age 37, works full-time as an office manager, is a wife and a mother of two young children. Bassford remarks, “Sometimes people have multiple symptoms and often each symptom is treated on its own. That’s one reason a Chiari malformation can take so long to diagnose.” Like Anjolina Ross, Bassford suffered with chronic headaches as well as numbness in her arms and legs. “I learned to live with these conditions for over 15 years, until the dizziness came along.” An MRI showed a significant Chiari malformation and a syrinx, a fluid-filled cyst or bubble in her spinal cord. Because of the blockage of cerebrospinal fluid (CSF) by her cerebellum, when her heart beat, her CSF was forced into her spinal cord, rather than flowing naturally around it. This causes pressure on the spinal cord from within, resulting in neurological symptoms.

Bassford had Chiari decompression surgery at a medical center out of the area and this surgery led to several others. Over the course of these surgeries and her growing frustration with the increasing amount of untreatable headaches, she learned about Dr. Khalid Kurtom, MD, FAANS, neurosurgeon at Chesapeake
Neurological Surgery, part of University of Maryland Shore Medical Group, an affiliate of University of Maryland Shore Regional Health, and Clinical Assistant Professor in the Neurosurgery Department at the University of Maryland School of Medicine. “After being a patient of Dr. Kurtom, I can attest that he is the most compassionate person, the most understanding of my symptoms and the easiest to understand doctor I’ve met throughout this whole ordeal,” she said.

“Chiari malformation is typically treated with a Chiari decompression,” Dr. Kurtom explained. “This involves removing a small portion of bone at the site of compression and expanding that area. There are different nuances of how this is done based on the surgeon’s preferences. Surgery is usually successful in alleviating symptoms, but this may take time since the brain and CSF need to adjust to the extra space provided by the procedure. The two most notable complications are CSF leak, possibly needing CSF shunting, and infection. The nuances of the surgery performed may play a role in minimizing these complications. Overall, this is a successful operation, and the benefits definitely outweigh the risks of surgery.”

Dr. Kurtom placed a shunt behind Bassford’s right ear and then ran a tube internally from behind the ear down through her chest and into her abdomen. Bassford said he described this shunting procedure as “basic plumbing—if you have too much CSF in the brain, you have to redirect this fluid from the top down into the abdomen, which naturally reabsorbs the fluid.” 

“The amount of fluid drained is regulated by a valve that can be programmed with a magnet from outside the scalp,” Dr. Kurtom said. “Drainage can be increased or decreased by adjusting this valve in the office.”

Dr. Kurtom advised Bassford that adjustments might be needed and they were, but every time, Dr. Kurtom quickly responded. He told her they were aiming for perfection, “and he kept adjusting my shunt 'til the setting was just right,” she said. Soon Bassford had no daily headaches, something that had not happened before. “He gave me my life back, an even better one than before my Chiari malformation was diagnosed over two years ago,” she said.

Michelle Newnam Lowrey, age 44, had her symptoms, including headaches, vertigo and hearing problems, misdiagnosed for over ten years before her Chiari malformation was identified during a MRI, when the technician noticed it and urged her to see a doctor. Because of the Chiari malformation, she had two syringes in her spine. She met with the chairman of neurosurgery at the University of Maryland and he recommended Dr. Kurtom, who had completed a NIH clinical fellowship with one of the leading Chiari malformation experts in the world. Lowrey, a former elementary teacher, a wife and mother of four young children whom she is homeschooling, also suffered debilitating fatigue until her decompression surgery. ”Now I feel normal fatigue at the end of the day. Dr. Kurtom’s decompression procedure is a minimally invasive surgery—a hallmark of his,” she said. The Chiari malformation and syringes also affected her senses, but now she said, “My body is acclimating to the CSF flowing correctly, and all my senses are beginning to return.”

Ross, Bassford and Lowrey all emphasized that patients must be their own advocates because Chiari malformations are so difficult to diagnose. Lowrey adds, “A Chiari malformation can look like so many other problems and so many doctors don’t know that it exists, although more are learning about it, and MRIs have helped.”

Dr. Kurtom is the only neurosurgeon on the Eastern Shore that does this surgical procedure. “There are not many options or resources for patients regarding this pathology,” he said. “Most neurosurgeons do not treat this pathology, and the few that do the operation have no uniform way of performing the procedure. This results in significant confusion regarding the surgery, how it is performed, expected outcomes and patients’ experiences.”

Bonnie Bassford has set up a Facebook page, Chesapeake Chiarians (www.facebook.com/chesapeakechiarians), and hopes to start a support group on the Eastern Shore. “You can’t look at someone and know he or she has a Chiari malformation,” she said. “It can be a debilitating disorder that affects not only the patient, but also the spouse, children, family and friends.” She posts encouraging quotes, facts and tips on her page. “I want to give people with Chiari malformations and CSF disorders a place to come together and relate, words to encourage them that there is hope and treatments that work, as well as to educate the public on what we Chiarians struggle with on a daily basis.” Dr. Kurtom remarks, “I encouraged Mrs. Bassford to start this group because I think this is a great idea and will add much needed support and resource for patients in the local community. I am sure many will benefit from her efforts.”

“People shouldn’t get discouraged and give up when they have a condition that impacts the quality of their lives,” Anjolina Ross said and Bassford agrees. “It’s not impossible to overcome this battle,” Bassford said. “You need to advocate for yourself and find hope again. Dr. Kurtom can make an accurate diagnosis and treatment plan because he is well educated and trained in Chiari malformations.” Chesapeake Neurological Surgery is an affiliate of University of Maryland Shore Medical Group, the multi-specialty practice of University of Maryland Shore Regional Health. For additional information about the services provided by Khalid Kurtom, MD, FAANS, neurosurgeon at Chesapeake Neurological
Surgery, which is located at 403 Purdy Street, Suite 204, in Easton, call 410-820-9117.