Collaboration Leads to Solution for Patient with Complex Diagnosis

— Mira Sachdeva, M.D., Ph.D., in the retina division; Bryn Burkholder, M.D., in the uveitis division; and Ashley Campbell, M.D., an expert in orbital tumors

 Bryn Burkholder, M.D. and Ashley Campbell, M.D. with Dana Cole.

On a Sunday in May 2023, Dana Cole stood on her deck in Annapolis gazing at her boat on the water. Two days before, she could only see a “fuzzy blob” when she looked at the vessel. But in this moment, she could see her boat clearly for the first time in years. “It makes me emotional thinking about it,” she says. 

For three years, Cole had tried to find treatment for the deteriorating eyesight in her right eye. She arrived at Wilmer Eye Institute, Johns Hopkins Medicine in late 2022 with a detached retina but few answers as to what caused it or how to move forward. At Wilmer, three ophthalmologists — Mira Sachdeva, M.D. Ph.D.in the retina division; Bryn Burkholder, M.D., in the uveitis division; and Ashley Campbell, M.D., an expert in orbital tumors — put together the pieces of the puzzle to identify a mass behind Cole’s right eye. It was the beginning of Cole getting a long-sought diagnosis and the care she needed to improve her vision.

Cole’s vision challenges began in 2020, when she first saw wavy lines with her right eye when looking at the panes of a window, or at a doorjamb, but her vision continued to decline over the next year. By then, she says, “I couldn’t see close. I couldn’t see far away. I just saw everything wavy with my right eye.” Cole went to an ophthalmologist near her home, who diagnosed her with central serous chorioretinopathy, which leads to fluid building up under the retina, and said her vision likely wouldn’t improve because of this disease.

At this point, Cole was heavily relying on her left eye in her work as an advertising producer. Reading became a struggle. She also began to feel the strain physically. Normally a high-energy person, Cole was tired all the time, and she was getting headaches.

“My left eye was working so hard that I was in pain from trying to focus all day,” she says. “I couldn’t go from having vision to having no vision in a year and a half with zero hope. I thought it was time for a second opinion.” 

Cole found that hope through Tamara Fackler, M.D., a former Wilmer fellow, at her practice in Annapolis. Fackler diagnosed Cole with a serous retinal detachment. This can happen when fluid builds up behind the retina, causing the retina to separate from the back of the eye. However, Fackler thought there was something atypical that had caused her retina to detach. She referred Cole to Wilmer, telling her that the institute has the technology and expertise to help.

At Wilmer, Cole firstsaw Sachdeva, a retina specialist, who determined that Cole didn’t have a tear, the typical cause of a retinal detachment. However, Sachdeva noticed inflammation, so she referred Cole to Burkholder, a specialist in uveitis, or inflammation of the eye. 

It appeared that Cole had posterior scleritis, which is caused by inflammation of the sclera and can lead to retinal detachment. But an ocular ultrasound revealed an unusual amount of swelling and inflammation behind Cole’s right eyeball — a finding that made Burkholder question whether Cole actually had this disease. “She wasn’t in any pain, and the appearance of the eye was not typical for posterior scleritis,” she says. However, Burkholder observed a mass behind Cole’s eyeball. She ordered an MRI to confirm this, then referred Cole to Campbell for potential biopsy. 

The mass was likely either the result of an inflammatory process — or it was lymphoma. Burkholder and Campbell collaborated to determine if a biopsy could be performed safely. The location of the mass presented a challenge: It was growing around Cole’s optic nerve, and any damage to the optic nerve during biopsy would cause irreversible vision loss. Another option was to treat the mass with steroids. But if it was lymphoma, the disease would respond to them initially and then likely come back. “We’d be masking the condition and delaying her diagnosis,” Burkholder says. With Cole’s agreement, they decided that Campbell would perform the biopsy. 

Before starting the procedure, Campbell had to think carefully about how to access the area. “It was tricky enough that I did talk to a number of colleagues and mentors to get their opinion,” she says. Campbell ultimately decided to detach the medial rectus muscle, one of four muscles in the eye that are responsible for eye movement, to create more room for her to access the back of the eyeball. Then, she says, “We hugged along the eyeball until we got to the mass. Ms. Cole did really well with the surgery and didn’t have any vision loss or double vision.” 

The biopsy showed that Cole had a low-grade lymphoma, and a PET scan revealed that the disease had not spread to the rest of her body. For treatment, Campbell referred Cole to Jeffrey Meyer, M.D., M.S., a radiation oncologist at Johns Hopkins, where Cole received two sessions of low-dose radiation to the affected area of her eye. The radiation shrank the tumor, which took pressure off of Cole’s optic nerve. This allowed the fluid to reabsorb from under the retina, and Cole’s vision in her right eye was restored.

Today, Cole is monitored by Meyer and Campbell with follow-up MRI imaging, and she continues to see Burkholder for her regular ophthalmology care. Her vision remains clear.

“It’s now been a year-plus of clarity that I haven’t had for years,” she says. “Last year was a tough year, and I’m happy to have come out on the other side in a much better position. I’m so grateful, and I have such appreciation for the level of care and expertise at Wilmer and Johns Hopkins.”