Taking a fresh look

A historical case offers some friends the chance to give a speculative diagnosis

Taking a fresh look
Leigh Spielberg
Leigh Spielberg
Published: Monday, April 1, 2019
So, do we have any theories on how he went blind? No one knows for sure how it happened. A diagnosis was never made,” I said to everyone but no one in particular. We were watching Ray, the biopic about the legendary jazz pianist Ray Charles. It’s a spectacularly moving film in which his struggles as a blind musician in an unforgiving world are given a great deal of attention. It was a unique moment to have this discussion. The eight of us were back in the Alps for our yearly ski-trip reunion of Rotterdam Eye Hospital ophthalmology graduates. With six subspecialists and two general ophthalmologists, it became a lively discussion. We were particularly interested in the scene that depicts the seven-year-old Ray Charles suffering rapidly progressive visual loss. “The way his disease is depicted here, it looks like congenital glaucoma,” said Theo Nieuwendijk, a glaucoma specialist in the Rotterdam Eye Hospital. “The blurry vision, the tearing eyes and the rapid visual loss look a lot like the presentation of high IOP at birth. Except he was apparently seven years old by then, and juvenile glaucoma presents differently. And he didn’t have the typical buphthalmos appearance that would have made for an easy diagnosis, even later in life.” “That’s a pretty significant detail. Sudden-onset glaucoma in a seven-year-old is pretty rare, certainly if it’s primary glaucoma,” said Gertjan Hoddenbach, also a glaucoma specialist in South Holland. “Maybe he had bilateral uveitis? Possibly in combination with secondary glaucoma? Like in the context of juvenile arthritis?” I wondered. “Juvenile cataract is a possibility, with subsequent amblyopia,” suggested Roger Cals, a phaco specialist in North Holland. “But he probably would’ve been operated on at some point thereafter, once he had access to medical care.” We all pulled out our smartphones and started searching for information, but there was little to be found. As far as we could determine, Ray Charles not only never had access to an ophthalmologist during his childhood, but apparently never received a diagnosis later in life, when he could certainly afford such care. And we decided to not use the clinical presentation in the film as a necessarily accurate portrayal of his symptomatology. “How about trachoma?” mused Tom van Goor, a cornea specialist in the Rotterdam Eye Hospital. “That could explain the bilateral loss of vision, the irritation and the discharge. But in trachoma, visual loss occurs at the end stage of the disease, due to corneal scarring by the conjunctival changes.” A quick search online yielded some information that supported this theory: trachoma was responsible for 10% of the cases of blindness in a school for the blind nearby where Ray Charles grew up. Niels Hoevenaars, a strabismus surgeon in South Holland and a jazz pianist himself, recalled a presentation he had given when we were all in training. It concerned the extraordinary auditory faculties of people who lose their vision at an early age. “The neuronal plasticity allows the brain to expand the capabilities of the auditory cortex in order to expand the total sensory capabilities,” a point which was made clear in the film. Ray Charles was said to have had excellent hearing. “Stevie Wonder lost his sight due to retinopathy of prematurity, but that’s probably not the case here,” I said. “Late-onset RDs in ROP are unlikely, since he was known to have been able to see in his early childhood years,” said Yashna van Kinderen, a VR surgeon in North Holland. “FEVR is a possibility in a young male with bilateral visual loss,” said Peter van Etten, VR surgeon in the Rotterdam Eye Hospital. Retinitis pigmentosa was discounted, considering its rarity and Charles’s young age at onset, as were bilateral detachments due to Stickler’s syndrome. After considering the combination of the poor, rural setting of Charles’s upbringing, the sudden, bilateral loss of vision and the irritation and discharge said to have been present, we finally settled on trachoma as the most likely cause of his blindness. The next day, we ventured out on to the slopes, where we encountered a total whiteout, a combination of low-hanging clouds and a blizzard that rendered the eight of us effectively blind for several hours. The stress caused by low vision was readily apparent in the group. Skiing slowed to a crawl and we stayed together. Visibility was only a few metres, and losing someone from the group could lead to a tricky situation. Despite the considerable ophthalmological skill set in the group, we had no solution for this universal epidemic of sudden-onset, bilateral blindness. So we did the only thing we could think of: we went inside for lunch and waited for the skies to clear. Dr Leigh Spielberg is a vitreoretinal and cataract surgeon at Ghent University, Belgium
Latest Articles
Simulators Benefit Surgeons and Patients

Helping young surgeons build confidence and expertise.

Read more...

How Many Surgeries Equal Surgical Proficiency?

Internet, labs, simulators, and assisting surgery all contribute.

Read more...

Improving Clinical Management for nAMD and DME

Global survey data identify barriers and opportunities.

Read more...

Are Postoperative Topical Antibiotic Drops Still Needed?

Cataract surgeons debate the benefits of intracameral cefuroxime prophylaxis.

Read more...

Emerging Technology for Detecting Subclinical Keratoconus

Brillouin microscopy shows promise in clinical studies.

Read more...

Knowing Iris Repair: Modified Trifold Technique

Part eight of our series covers the modified trifold technique for large iris defects.

Read more...

It’s All About Biomechanics!

Increasing the pool of patients eligible for refractive surgery.

Read more...

Uncovering More Safe and Quick Options

Different strategies, such as PresbyLASIK, can offer presbyopes good outcomes.

Read more...

Topography-Guided PRK for Keratoconus

Improving visual acuity in patients with keratoconus.

Read more...

Defining AMD Treatment Protocol

Treatments trending to fewer injections for better results.

Read more...