Diane thought her spinal CSF leak had been thoroughly treated. But when her symptoms returned, it would take nearly eight years and surgery to fully address the underlying cause: a CSF-venous fistula.
It was summer 2008 when Diane, on vacation with her family, bent down to retrieve an outfit from her suitcase and felt her life change. “I literally saw stars,” she said. She felt confused, unsteady; her vision was blurry, and her head hurt. Perhaps this was a case of low blood sugar, she thought, and so she laid down, which seemed to help. But the pain was unrelenting. Within a week, the constant headache was so bad whenever she was upright that she suffered from nausea, vomiting, double vision, ringing in her ears, and hearing loss.
When she and her family returned from vacation, she immediately made an appointment with a neurologist, and after performing tests, her doctors diagnosed her with spontaneous intracranial hypotension due to a spinal CSF leak. They recommended bedrest and caffeine, theorizing that since this appeared to be a spontaneous leak—meaning it wasn’t caused by a needle or other trauma—it might resolve itself with time. But instead, for months she suffered with incredible pain. She was unable to drive, being upright for too long gave her double vision and made her vomit, and despite drinking so much caffeine she landed in the ER with her heart racing, nothing seemed to help. “I can’t keep doing this,” she told her doctors, and finally she was treated with the first of several epidural blood patches. Diane experienced only minimal relief from her first blood patch, but her second one provided her with two years of nearly pain-free living. When she felt her original symptoms slowly returning—the ringing in her ears, the jabbing head pain—she underwent a third blood patch. This one lasted for four years.
Diane was thrilled to be back in the midst of her busy life. As a small business owner running a wholesale bakery, and working as a food business consultant, she had no time for down-time, and she was grateful to be able to be upright and out of bed. But gradually she began to notice a creeping pain in her neck that eventually grew to be intolerable, to the point of almost causing her to pass out. She returned to her doctors for another brain MRI, and to her surprise, the imaging showed several of the textbook signs of severe spontaneous intracranial hypotension. Her leak was back. She was instructed to go home and not get out of bed, which she said was like “telling an ocean wave to stand still.” But on bedrest she went. Just like before, lying down helped with some of the pain, but it didn’t resolve the problem.
In the spring of 2016, almost 8 years since she’d bent over and seen stars, Diane received an email from her brother pointing her to an article about another woman suffering for years from a spinal CSF leak who’d ultimately found relief through being treated by a team of neuroradiologists. Soon thereafter, she traveled to meet with that team, who were able to evaluate her and identify the root of her problem: a little-known type of spinal CSF leak called a CSF venous fistula. This abnormal channel between the space where cerebrospinal fluid is and a vein outside the dura mater meant that her cerebrospinal fluid was being siphoned off through a rogue vein, so that no matter how much cerebrospinal fluid she produced, it was constantly being whisked away. It took several rounds of epidural patching with fibrin sealant and, ultimately, spinal surgery to resolve the CSF venous fistula. But today, after over 8 years of living with the effects of her spinal CSF leak, Diane is symptom-free. “I consider myself so lucky,” she says. “I just count my blessings that I was diagnosed early, and that I did have relief along the way.”
Overall, she says, this experience has emphasized for her the importance of resilience and perseverance. “What you always have to remember is, this is curable. You have to believe that this will be solved, you have to just keep going — and then you have to follow the path to the best people out there who can help you.”
If you have been suffering with daily headache that tends to be better when lying flat, or simply better in the morning and worse later in the day, with poor response to medications, ask your health care provider if your headache could be due to a spinal CSF leak.