An MD’s story of her IC diagnosis reads just like so many others. In 1998, she suddenly had terrible burning urethral pain. She’d had urinary tract infections before, so she thought this was another one and took antibiotics...

... But when her symptoms didn’t go away, and cultures didn’t show a urinary tract infection, her gynecologist suspected IC. Then, he referred her to a urologist who performed cystoscopy with hydrodistention under anesthesia and found glomerulations. In addition, Karen* had other conditions that so often go hand-in-hand with IC—migraine, allergy, eczema, irritable bowel syndrome. It was a common story. Her urologist diagnosed IC.

But that wasn’t the whole story. Five weeks before her IC symptoms started, she had fallen on her tailbone after she was knocked over at an ice-skating rink.

Her pelvic pain continued to grow, and nothing seemed to help, not pentosan polysulfate (Elmiron), not DMSO instillations. She began to need more and more pain medications. Her physicians tried one medication after another.

Frustrated, in 1999 her urologist sent her to David Foster, MD, a pelvic pain specialist in Rochester, to look for any other source of her pain. Because she had had a fall, he sent her to get a magnetic resonance image (MRI) of her lumbar, sacral (tailbone), and pelvic areas, just to be sure that there was no fracture. The radiologists saw nothing that they thought could be causing the problem, although they did note a small Tarlov (nerve root) cyst at the third sacral level (called S3, for short).

Dr. Foster suggested that she consult a neurosurgeon about whether the Tarlov cyst could be the cause of her pain. She talked to two different neurosurgeons who both said it was not the problem.

Karen’s* pain level kept increasing. When she first looked for help, her pain was at a 3 or 4 on a 10-point scale. Quickly, it escalated to 4 to 5, then 6. “When I stopped working a year and a half later, it was level 6 to 7,” she recalled. Stopping work meant giving up her busy surgical ophthalmology practice.

Then, she joined the ICA; got “phenomenal” advice, support, and encouragement; and vowed to pursue every treatment avenue available to her. In 2001 she had an Advanced Neuromodulation Systems (ANS) sacral neurostimulation device implanted. It barely helped.

By then, she had developed complex regional pain syndrome and allodynia (pain from things that aren’t normally painful). Vulvodynia was beginning to develop as well. By 2004, Susan needed large doses of opioid medications. “I got absolutely desperate,” she said, “my pain was an 8 to 9, and I was almost suicidal.”

She went to a second gynecologic pain specialist in Rochester, Fred Howard, MD, who suspected that her symptoms might be caused by damaged pudendal nerves because she had tenderness along the paths of these nerves, which serve the urethral/clitoral, perineal, and anal areas. He referred her to a neuroradiologist to get injections of anesthetics around both pudendal nerves under computed tomography (CT) guidance. “While I was on the table,” she remembered, “I was perfect. I had no pain. It was great. I thought, ‘Fantastic! I’m going to have eight hours without pain.’ I stood up, and my pain returned within 10 minutes.”

Just to make sure, she tried the procedure a second time a few weeks later. “It was the same story. While I was on the table, I was perfect. As soon as I stood up, it was a flop.” Even more discouraged and depressed, the news from Dr. Howard that she just didn’t have pudendal neuralgia was hard to take. But, he said, “It’s just got to be something closer to your spinal cord. What could it be?”

Karen’s* husband piped up, “What about that Tarlov cyst?” That was an “Aha!” moment for Dr. Howard, who knew that the thinking about Tarlov cysts and their effects had changed since her MRI in 1999. “We have to find someone who knows more about these,” he said.

Karen* found the Tarlov Cyst Support Group’s comprehensive website (www.tarlovcyst.net), with information for patients and articles from knowledgeable specialists. She also started reading the recent medical literature about Tarlov cysts, and more puzzle pieces of her story fell into place. By 2004 it was known that even a very small (less than 1 cm) Tarlov cyst can cause pain. In Karen’s* case, her fall caused a sudden, dramatic increase in her spinal fluid pressure and broke a membrane between the cyst and the spinal fluid column, allowing fluid to flow in and out of the cyst. Before the accident the Tarlov cyst was silent. Afterward, it caused severe, progressive pain.

Tarlov cysts produce pain when spinal fluid pressure increases inside them. That can happen when someone stands up, or does anything that increase pressure in the abdomen, such as coughing, bending, squatting, or lifting.

Karen* realized that was happening to her. It was why she felt great when she was lying on the table during the anesthetic injections and worse when she got up. It was why she was pain free when she first woke up in the morning but worse after she got up and as the day went on.

The usual symptoms of Tarlov Cyst disease are back pain, pain that shoots down the leg (sciatica), or pain in the buttocks or tailbone. (See “Is Nerve Damage Causing Your Pain?” box.) But when the cysts cause trouble, they can also cause urinary and gynecologic symptoms. One recent survey of 800 patients detailed on the Tarlov disease website showed that 46 percent had problems with frequent urination. And because Karen’s* problem cyst was at the nerve root of S3 where some of the nerve fibers that serve the bladder and the pudendal nerve originate, the cyst produced bladder, urethral, and vulvar pain.

As she read more, she also discovered things about Tarlov Cyst disease that were bound to confuse the picture for anyone familiar with IC symptoms. Five to nine percent of adults are thought to have these cysts, and nearly 90 percent of  Tarlov Cyst disease patients are women. In addition, most of the same foods and beverages that bother many IC patients also bother Tarlov Cyst disease patients with urinary symptoms—acidic fruits, juices, and vegetables; spicy foods; alcoholic and carbonated beverages; and more.

What she read in the medical literature also gave her hope. Surgical treatment helped 12 out of the 14 patients with bladder symptoms whose cases were in the articles—a success rate of 86 percent.

In 2004 there were three medical centers in the United States where experienced neurosurgeons were doing research on and treating Tarlov disease: Johns Hopkins University in Baltimore, Maryland, Georgetown University in Washington, DC, and the University of California, San Francisco (UCSF). But Karen* got lucky. Neurosurgeon G. Edward Vates, MD, PhD, had just joined the University of Rochester staff, and he had trained at UCSF with Tarlov disease expert Philip R. Weinstein, MD. Karen* saw Dr. Vates right away. The first thing he needed was to find a way to update the images of her cysts.

The best way to look at Tarlov cysts, especially small ones, is with an MRI, but she couldn’t have one because she still had the ANS neurostimulator. No one who has metal inside the body, such as a neuromodulation device, can undergo an MRI, which uses powerful magnets. So she had to have a special kind of CT, called a CT myelogram, in which doctors inject a dye that will show up on the images.

The key finding was that the S3 cyst emptied when she was flat and filled back up when she was upright. Her pain depended on her position in the same way—eased when she was flat and worse when she was upright. That clinched the diagnosis of a symptomatic Tarlov cyst as the cause of her pain. After six long years, the search for the root cause of her IC was over. In a delicate, four-hour operation, Dr. Vates opened her cyst, packed it with fat, and closed it up.

Four days later, when she was allowed to stand, she had no pain from standing up. She did have difficulty with a urinary tract infection after surgery, but that cleared up. Today, her pain is down from the 8 to 9 that nearly drove her to suicide to a manageable 3 to 5.  

Pain experts believe that longstanding chronic pain leaves an imprint on the nervous system that keeps perpetuating the pain, even when the original source of the pain is gone. She still has to take painkillers and medications for neuropathic pain, such as the antiepileptic drugs Lamictal (lamotrigine), Topamax (topiramine), and Keppra (levetiracetam). But if her Tarlov cyst had been treated in 1998,  she believes, she might be pain free today. Nevertheless, she told the ICA Update, “The biggest blessing is that my activity levels have gone up. I’m much sharper, my mood is so much better, and I’m filled with hope.”

The story also has some lessons. One is for doctors to suspect a symptomatic Tarlov cyst when a patient with pelvic pain and bladder symptoms has a telltale history—usually a trauma, such as a fall; pain that gets worse when the patient is upright and does things that increase abdominal pressure, such as bending; and pain that eases when the patient lies down. Radiologists, orthopedists, and pain medicine and rehabilitation specialists are much more aware of the potential of Tarlov cysts to cause problems, said Karen*, but the awareness is still low among physicians who take care of women, the very group that has the highest rate of Tarlov disease.

Another lesson is to do an MRI for a patient with these telltale symptoms before implanting any metal device, such as a neurostimulator. Does that mean that everyone slated for a neurostimulator should get an MRI? After all, the incidence of symptomatic cysts is still unknown and the cost of an MRI is high. That’s a question for healthcare economists to consider, said Karen*, but she pointed out that she might not have needed an implant or all these medications if doctors had understood the importance of the cysts that showed up on the MRI that she did get in 1998. Just implanting the neurostimulator cost $80,000, and medications can cost some $5,000 a month—a cost that’s likely to continue and stay fairly high because of her ongoing nerve pain. All that money—and her career—might have been saved.

Is another lesson that patients who have IC symptoms because of nerve pain don’t really have IC? Probably not. Most IC experts believe that IC may have many causes. Many theorize that longstanding bladder pain puts an imprint on the nervous system that perpetuates the pain, even if the original source of the pain is gone. For example, some IC patients who have had their bladder removed still have IC pain. But the nervous system is a two-way street, and it could be that damage to the nervous system, such as from a problem Tarlov cyst, can produce damage in the tissues the nerves go to. Many pelvic pain experts believe that inflammation in the bladder may be neurogenic, that is, generated by nerves. And Karen’s* bladder did show glomerulations.

A lesson may also be that the best treatments for IC and pelvic pain are the ones that address the cause of the symptoms. But are the lessons only for a small group of patients who have these cysts and their physicians? Probably not. Symptoms similar to these can point physicians to damage to the pudendal nerve, which can cause some IC and chronic prostatitis/chronic pelvic pain symptoms. But the telltale history of patients with pudendal nerve pain is a little different. They usually get relief, not only when they lie down, but also when they sit on a toilet seat or a cutout cushion that doesn’t put pressure on the perineal area.

But the biggest lesson may be to listen to the whole story. 

*Pseudonym has been used for privacy.