When Rob arrived at Mayfield, more than 800 miles from his New England home, he was using leg braces and a cane or walker. He could walk only with significant assistance. During the previous 16 months he had seen four neurosurgeons, had undergone five surgeries, had suffered serious surgical complications, and had seen his mobility and hope all but vanish.
Dr. Robert Bohinski, a Mayfield neurosurgeon with special expertise in Chiari malformation, accomplished what the other doctors could not. In a rare procedure that he has performed only a few times in the last decade, he placed a shunt inside Rob’s spinal cord. When Rob returned a year later for a follow-up visit, he was walking without braces or even a cane.
"It is a great story," Dr. Bohinski reflects. "I am so happy for him."
A Chiari (pronounced kee-AR-ee) malformation occurs when the indented bony space at the back of the skull is smaller than normal, causing the cerebellum – the part of the brain responsible for balance -- to migrate into the spinal canal. When this occurs, pressure is exerted on the lower stem of the brain, and the flow of cerebrospinal fluid, which bathes and cushions the brain and spinal cord, can be blocked. A cyst, also known as a syrinx, can develop inside the spinal cord.
Looking back, Rob says he has been affected by Chiari since childhood, when he suffered from headaches but never connected them to any kind of medical condition.
"I first began to sense that something was wrong in July of 2009 when my left arm became hypersensitive to cold in a very unusual, painful type of way," Rob says. "It progressed to tingling and numbness over the following weeks and months. This was followed by strange electric-like shocks to my collar bones about six months later."
After a particularly bad attack in February 2010, Rob went to his local hospital emergency room. The attending physician ordered an MRI, which revealed Chiari malformation of the brain. "She explained the problem to me and indicated that my case was severe," Rob says. "She recommended that I be admitted and that a neurosurgeon be called to perform correction surgery. I was totally unprepared for such news and just wanted to go home to think about it and learn more."
Rob returned the next day, and additional scans revealed a small syrinx in his cervical spine. The surgeon referred Rob to a regional New England medical center. A month later, Rob underwent decompression surgery, which was intended to collapse the syrinx. Unfortunately, complications ensued.
"A couple of days after returning home, I noticed clear fluid flowing from the incision and returned to the office to be checked out," Rob says. "They dealt with the problem by adding a couple of sutures in the incision, and I was sent home. However, the leaking continued, and two weeks later, after a cough, the incision split open with a massive amount of drainage."
Rob was treated with an external lumbar drain for 11 days and then underwent a revision decompression surgery. He was hospitalized for a couple of weeks before being discharged. Still, Rob did not feel well. And over the next several months, weakness spread to his extremities. His left leg began to drag when he walked. In the spring of 2011, Rob was referred to yet another neurosurgeon. New scans revealed that the syrinx had significantly worsened. Rob was then referred to a major metropolitan hospital whose experts, he says, "thought a shunt was needed but were uncertain where to place it."
Rob was ready for one last expert opinion.
"Along the way, I had become aware of Mayfield," he says. "The Center was a considerable distance from our home in New England, but it was clear that the time had come to get an opinion from a major Chiari center.
"By this time, I was unable to walk without leg braces and a cane. I was unable to bend my left ankle or even wiggle my toes. I was having pain in my arms and was also experiencing dizziness, sweating, brain fog, sleep apnea, shortness of breath and depression. My bed had been moved to the living room. I could not go up the steps or bathe myself without assistance. More than anything else, my spirit was being crushed worrying over the welfare of my family and the concern that we could lose our home and everything we owned. I had gone to so many doctors and had only progressively gotten worse. Hanging on for hope was becoming difficult, and I doubt I would have been able to make it without the support of family and friends in the community."
Rob contacted Mayfield and forwarded all of his medical information and scans. Dr. Bohinski took his case, advising that a thoracic spinal shunt was needed.
A scan taken one year after placement of the shunt shows improvement of the syrinx.
"Shunt placement is not known to have a high success rate," Dr. Bohinski says. "But after all of us reviewed his case, we agreed that his Chiari malformation was appropriately decompressed and that placing a shunt was the only thing left to do."
The diagnosis and treatment, Dr. Bohinski stresses, are difficult and are not commonly performed. Furthermore, he did not make major promises. He advised Rob that their best hope was to halt further progression of disability. Resigned to the reality that the remainder of his life would continue to be severely limited, Rob underwent surgery on August 14, 2012.
"I don't recall much about waking up from my surgery," Rob says. "I was very groggy, but the next day I was able to wiggle my toes a little. While this may seem like next to nothing, it was huge for me and a sign that encouraged me to renew my fight. Later that day a therapist got me out of bed, and with the aid of a walker and security belt walked with me down the hall."
Dr. Bohinski prescribed occupational and physical therapy every day for the remaining two weeks of Rob’s stay in Cincinnati. By the end of that time period, Rob was able to put on his shoes and socks independently and walk unassisted with the aid of a walker and leg braces. He continued his therapy in New England, and after three months replaced the walker and leg braces with a cane. By six months he was walking without any aids at all and "making progress in regaining my life."
Dr. Bohinski could not have been more pleased when Rob walked in the door for his one-year follow-up appointment. "He proclaimed that I was his miracle patient, as he expected that I would require a walker and braces for the rest of my life," Rob says. "He then reviewed my MRI results, which showed that my syrinx had collapsed and my spinal cord appeared almost normal."
Today, Rob says, he continues to regain function. "I still have some problems, namely severe muscle spasms if I exert myself," he says. "I also experience instability and dizziness when I first wake up in the morning. But overall, I have much of my life back. And I have renewed hope of making further progress and ultimately returning to my pre-Chiari a level of activity."
Hope Story Disclaimer -"Robert''s Story" is about one patient's health-care experience. Please bear in mind that because every patient is unique, individual patients may respond to treatment in different ways. Results are influenced by many factors and may vary from patient to patient.
"... overall, I have much of my life back. And I have renewed hope of making further progress and ultimately returning to my pre-Chiari a level of activity."