“You can’t see the ceiling, can you?” the man asked his 31-year-old wife. She grimaced, then shook her head. She lay in bed, looking at the familiar shadows and shapes of the winter morning sun. But she couldn’t see them. It was as if a thick white mist lay between her and those daily shifting patterns. Squeezing didn’t help. Opening her eyes as wide as she could, didn’t do it either. All her life she had perfect vision. It was a secret source of pride. She had never even seen an ophthalmologist. But that morning everything changed.
Six months earlier, she first noticed the trouble in her eyes. A professional violinist and teacher, she took her students to Italy that summer to experience the sacred music and art. As she looked up at the frescoes that adorned the ceiling of a favorite cathedral, a glittering shape with jagged, irregular edges appeared out of nowhere. The dots seemed to twinkle as the star-like image slowly expanded. Within the glittering outline, the colors were jumbled, like the crystals in a kaleidoscope. It was beautiful and terrifying. She lowered her head, closed her eyes, and rubbed her aching neck.
When she opened her eyes, the star with its glittering edges still burst there, distorting everything beyond. It got so big that it was almost all she could see. Then it slowly started to fade; after nearly half an hour, the world began to regain its familiar look and shape. There had been similar, albeit less severe, experiences: every now and then, if she stood up quickly after sitting or lying down, she would feel an intense pressure in her head, and when it let go, everything looked faded and pale for a moment before return to normal tones. These spells only lasted a few seconds and only occurred a handful of times in recent years. She blamed it on fatigue or stress. After that day in Italy, those glittering star bursts appeared weekly and then daily.
Stranger still, straight lines developed strange bumps and bumps as she looked at them out of the corner of her eye. Doorways, curbs, and table edges seemed to wobble, with bulges and potholes. When she faced the object fully, it straightened obediently, but resumed its aberration as soon as it was back on the sidelines.
Days after her morning outage, the young woman went to see an optometrist in nearby Fort Lee, NJ, Dr. Paul Shahinian. If the star bursts were of concern to the young woman, Shahinian’s reaction to her exam was terrifying. She needed to see a neuro-ophthalmologist, he told her — an eye and brain specialist — and she needed to see one soon. All of the information collected by vision is sent to the brain through a thick cable of fibers at the back of the eye called the optic nerve, the doctor explained, and the nerve in her left eye was extremely swollen. While sitting in his office, Shahinian called the specialists herself. The first two offices he called had the same answer: She couldn’t make an appointment for months. He then contacted a neuro-ophthalmologist new to the area, Dr. Kaushal Kulkarni, and, after explaining the urgency, arranged for the patient to be seen the following week.
Communication failure
Kulkarni listened to the patient describe her strange visual abnormalities. Although her vision was still 20/20, the intermittent star bursts and the curved lines in her peripheral vision — a phenomenon known as metamorphopsia — suggested there was something wrong with the way the brain received and processed her visual information. Kulkarni shone a narrow bright light into the young woman’s right eye. As expected, both pupils constricted. He moved the light slightly to the left and both pupils dilated immediately. Moving it to the right made her pupils constrict again; returning to the left, they suddenly widened again. The signal on the left clearly didn’t get through. The swelling cut off the flow of information from the eye to the brain. However, it seemed to be a one-way problem: The fact that the left pupil narrowed when the light shone into the right eye indicated that information from the brain was still coming through.
There are many causes of this type of optic nerve injury. Shahinian had thought that, given the patient’s age, this was likely multiple sclerosis, an autoimmune disease in which the immune system mistakenly attacks nerve fibers that connect the brain to the body. Kulkarni agreed that was a possibility and ordered an MRI to look for evidence of those or other abnormalities. Was this a tumor? Or a stroke? Other autoimmune diseases also needed to be considered. It can also be the result of an infection: Lyme disease can cause it; also cat scratch disease, an infection caused by the bacteria Bartonella henselae; even syphilis, often called the great imitator because of its ability to manifest itself in so many ways, can cause this type of injury.
He sent the patient to the laboratory for examination. The results of the blood test came in quickly. It wasn’t Lyme or Bartonella or syphilis. None of the inflammatory markers indicative of autoimmune disease were elevated. It was the MRI that contained the answer. Kulkarni didn’t see the bright white patches of dots and dashes that would indicate MS. Instead, a large round object, a mass the size of a plum, dominated the center of the left side of her brain.
Kulkarni called the patient and told her that the MRI was abnormal. A few days later she came back. He couldn’t tell her what kind of tumor she had based on the pictures. The most common would be a meningioma, a tumor of the tissue that lines the brain. An acoustic neuroma was rarer but also possible. This is a slow-growing tumor that develops in the tissue that connects the ear to the brain. She was still a bit young for that; these tumors usually affect men and women over the age of 40, and they usually cause problems with hearing and balance rather than vision. Whatever it was, the tumor was so large that it blocked the circulation of spinal fluid through the brain, causing the nerve to swell. It should be removed.
Occupational hazard?
Kulkarni knew the patient had no health insurance. He called the neurosurgeons he knew and tried to figure out how to give this woman the care she needed. The only answer seemed to be that she would come in through the emergency department. Thanks to a law called the Emergency Medical Treatment and Labor Act, all emergency departments are required to provide stabilizing treatment to every patient who comes in, regardless of insurance status or ability to pay. The care provided is not free; patients are billed, but they must be treated.
She went to the emergency department at NewYork-Presbyterian Hospital/Columbia University Medical Center and the mass was removed. The pathologist confirmed that the tumor was an acoustic neuroma. The patient read about this type of growth and noted that hearing loss is a common symptom. She had hearing loss in her left ear, but she hadn’t made a connection between that problem and the one with her eyes. She thought that the constant exposure to the sound of her violin might have done a little damage. She thought it was just the price of doing what she loved. After the surgery, her hearing did not change, but the strange star outbursts completely disappeared. However, straight lines still tend to kink in her peripheral vision.
Before her hair had even grown enough to hide the scar, the bills started rolling in. The numbers were even more terrifying than the images of the tumor. The final accounting was about $650,000. She and her husband were afraid they would have to go bankrupt. They had some money — they were saving for a rainy day, but this was a tsunami. The rescue came unexpectedly when a friend of a friend asked if they had spoken to the hospital about financial aid. It turned out that Columbia Presbyterian had a financial aid program. In fact, all non-profit hospitals are required to provide financial aid to those in need; it is mandated by the Affordable Care Act. They didn’t have to go bankrupt themselves. They are grateful that the ordeal ended well. And two years after surgery, they’re starting to rebuild their savings – because you never know when it’s going to rain again.
Lisa Sanders, MD, is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a resolved case to share, write to [email protected].