From the Editor-in-Chief

With all the unpredictability in our lives, one would imagine we would avoid adding additional uncertainty. Not in the least—we seem inherently drawn to mysteries, secrets, and puzzles. Millions eagerly embrace a crossword, spelling challenge, or number puzzle every day, and new game crazes repeatedly erupt and race across the world with viral speed. Indeed, our attraction to solving problems, even artificially created ones, must be hardwired in us—a distinctive attribute largely responsible for our evolutionary success.

Some famous puzzles span years, centuries, or even millennia before solution, dangling in our minds and taunting us. There is Kryptos, a sculpture created in 1990 by Jim Sanborn containing 4 large, coded alphabet panels in front of the US Central Intelligence Agency in Langley, Virginia. Three have been decoded, but the fourth remains unbroken after 3 decades, despite being passed by dozens of professional code breakers as they enter their workplace every day.

Valentin’s ¹ illustrated book, Sur La Traces De La Chouette d’Or (On the Trail of the Golden Owl), published in 1993, contains cryptic riddles that purportedly lead to a bronze owl statue buried somewhere in France. It has yet to be found after almost 30 years, despite hundreds of thousands of attempts by chouetteurs—determined seekers who are organized into various societies, host conventions, share clues on websites, etc.—and the extremely valuable gold-and-silver version to be awarded to the finder remains unclaimed. This puzzle has survived the death of its creator as well as acrimonious lawsuits over the ownership of the precious avian prize.

Fermat’s last theorem tormented mathematicians for over 3 and a half centuries before a proof was found by Andrew Wiles in 1994. Wiles’ proof came in at 129 pages of highly complex math, simultaneously proving both Fermat’s theorem and his assertion that the “truly marvelous proof” he claimed to possess was too large to fit in the margin.

Perhaps the most famous puzzle of all is the Phaistos Disc, a 6-inch clay plate with 45 distinct pictorial symbols or tokens used 242 times to inscribe a beautiful but seemingly chaotic spiral sequence on both sides. The disc, dating from the Minoan Bronze age and found on Crete in 1908, immediately became the subject of intense research and speculation. Though scores have claimed a solution, not one has been proven or accepted. Interested—often obsessed—investigators debate the purpose of the disc, the reading direction of the spirals, the tokens’ relation to other Minoan scripts, and (with understandable frustration) whether the disc itself is an outright fraud.

The present issue of JVRD contains an enduring retinal mystery in plain sight: West African Crystalline Maculopathy. Drs. Obinna Umunakwe, Mohsin Ali, Naina Bains, John Matthews, and Sharon Fekrat present an asymptomatic 71-year-old Igbo man with excellent visual acuity bilaterally who displayed multiple yellow-green refractile crystals near the fovea in each eye. Multimodal imaging documented the inner-retinal location of the crystals, and no abnormalities were noted on fluorescein angiography or autofluorescent photography.

West African Crystalline Maculopathy was first described by Sarraf et al. ² They presented 6 unrelated patients—4 females and 2 males with ages ranging from 54 to 69—who displayed tiny refractile perifoveal yellow-green crystals that were visually harmless to their best determination. All these initial patients were from the Igbo tribe and had spent the most of their lives in eastern Nigeria.

Dr. Sarraf and colleagues wondered about a link to kola nut chewing, common among the Igbo and other groups in West Africa. Later reports would document that such patients are not rare nor are they confined to the Igbo or admitted kola nut chewers.

A high prevalence of diabetes and other vascular diseases in many affected patients has been confirmed since the initial description, implicating vascular leakage as a factor in chronic retinal accumulation. Despite careful inquiries by many authors for the usual causes of crystalline retinopathy as well as genetic, nutritional, environmental, or other factors, the crystals remain a mystery.

Kola nuts are produced by evergreen trees of the genus Cola, and of course have become world famous as the eponymous ingredient in Coca Cola. Kola extract originally contributed both flavor and caffeine to the beverage, but apparently it was replaced with other ingredients quite recently. It is impossible to say for sure because the formula for Coca Cola is, well, another secret!

What can be said regarding the possible link between kola nuts and West African Crystalline Maculopathy? It remains a mystery that may be solved by the study of histologic specimens and innovative in vivo research. Nevertheless, a lifelong exposure to a nutritional item like the kola nut in patients with retinal vasculopathy remains an attractive hypothesis.

The chewing of kola nuts is widespread throughout West Africa, where the nut carries many societal, cultural, and religious connotations; it is prominently included in many important life ceremonies and has been used as currency. In the end, we can certainly say that the kola nut punches far above its weight: denominating one of the world’s most famous brands, anchoring vibrant traditions for many peoples, and offering retina specialists their own hidden-in-plain-sight mystery in West African Crystalline Maculopathy. What more could a nut hope for?

Donald J. D’Amico
Editor-in-Chief
Journal of VitreoRetinal Diseases