Mystery Diagnosis: Chairman’s Challenge

One of the cool things about being an ophthalmic imager is the teamwork that can develop with the ophthalmologists you work for. They rely heavily on the images you provide to help diagnose various eye conditions. I’ve been fortunate to work with physicians that rely not only on my imaging expertise, but also my experience in recognizing clinical features of unusual conditions. Recently, one of our medical retina specialists (my boss) challenged my diagnostic skills with an unusual case.

challenge stickyThe photo request came through as a post-it note that simply listed the patient’s name and the words: “Fundus photos & OCT”. There was no diagnosis listed or specific instructions given. I called the patient in to the imaging suite and it was a teenage girl accompanied by her mother. I asked if the doctor had given them any paperwork for me and the patient’s mother said, “No. Doctor Q said he wanted to see if you could guess the diagnosis”. Dr. Q and I have worked closely together for twenty years and I can often anticipate exactly what type of images he needs without much direction. But this was unusual. The lack of information was clearly deliberate on his part, which got me thinking:

Is he testing me?

Or does he need my help in making the dx? Yeah that’s it.

Wait… He’s Chairman of the Department, a sub-specialist in medical retina, has written a textbook/atlas of retinal diseases….

He’s clearly testing me, but it must be a rare condition. Hmmm….

Aha! He probably wants images for his new book.

These thoughts are swirling through my mind as I start with an OCT of the right eye. The IR fundus image shows a large oval area that is dark from elevation. It’s suggestive of a serous retinal detachment, but the OCT shows no fluid. The retinal pigment epithelium is pushed forward suggesting a choroidal lesion.

OsteomaA

I start going through a differential diagnosis process in my mind as I continue to capture images:

Nope, not a serous detachment. There’s increased choroidal reflectivity and thickening which suggests a choroidal tumor or nevus of some type. Could it be a melanoma or an osteoma?

Osteomas are pretty rare. I’ve seen one case in the past 30 years. Probably not…

I move to the fellow eye and the OCT shows significant pigmentary changes, subretinal fluid, and what looks like a choroidal neovascular membrane.

OsteomaB

Hmmm…. The findings are more dramatic in the left eye. Looks like maybe an exudative process in a young patient … could it be Coat’s Disease?

I move the patient to the fundus camera and peer through the eyepiece.

osteomaR

Yikes! I wasn’t expecting that. Maybe I was right to think Coat’s.

Coat’s disease is an idiopathic developmental retinal vascular abnormality in children.

coats1

Characteristic findings include telangiectatic vessels with aneurysmal dilation and exudative detachments.

But Coat’s doesn’t quite fit in the right eye. Maybe Vogt Koyanagi Harada disease (VKH)?

VKH1

Features of VKH include: choroidal thickening, hyperemic optic disc, multi-lobed serous detachments and de-pigmentation and clumping of the RPE late in disease.

Although some of the features fit, the characteristic serous detachments of VKH aren’t present. Both Coat’s and VKH are unusual, but not rare. At least not rare enough for Dr. Q to challenge me like this.

I shifted the camera to the left eye to take photos. Once again I was surprised at the clinical appearance.

osteomaL

At that point I must have smiled a little because the patient’s mom asked if I knew the condition. I said yes. Although it looked somewhat like Coat’s, I was almost certain it was a case of bilateral choriodal osteomas. A moment later, Dr. Q came into the imaging suite and confirmed the diagnosis as choroidal osteoma. Sure enough, he wanted good photos for his next book!

osteoma5

Choriodal osteomas are benign ossifying tumors of the choroid composed of mature bone elements. They often demonstrate a thin plaque-like yellow-tan lesion in the macula with sharp, scalloped borders. They are usually unilateral, but can be bilateral. Symptoms include metamorphopsia, scotoma and blurred vision. Vision loss may be due to direct tumor involvement or secondary to choroidal neovascularization with subretinal fluid, lipid, or hemorrhage.

Now that the mystery was over and I had passed the test, Dr Q. handed me the patient’s chart as I finished up the photo session. Her clinical findings were listed:

  • 15 y.o. female
  • VA: 20/25 OD 6/200 OS
  • IOP: 15/10
  • Pupils: Left APD (afferent pupillary defect)
  • SLE: WNL OU
  • DFE:
    • OD: Orange placoid elevated lesion
    • OS: Yellow-orange lesion with well-defined borders. Pigmented CNVM w/ subretinal hemorrhage
  • B-scan ultrasonography demonstrated classic highly reflective plaque-like structures with an acoustically empty region behind the tumors.

osteoma8

This case prompted me to look up the previous case I had encountered nearly twenty years ago. It was before the advent of OCT, but the osteoma was well documented with multi-spectral monochromatic imaging, color fundus photography, fluorescein angiography and B-scan ultrasonography. Dr. Q included that case in his retinal atlas and I had used it lectures on multi-modality imaging. The clinical appearance was quite striking and unforgettable.

osteoma12

It’s great when physicians challenge their staff to take an active role in a team approach to eye care. I’m fortunate to work with physicians like Dr. Q, who challenge me to not only capture high quality images, but to recognize the clinical features of routine and rare cases. Luckily, I passed this test!

Ophthalmic Photography: Where Science Meets Art

Ophthalmic photography has long played an important role in the documentation and diagnosis of ocular diseases. Ocular photography is used to record medical conditions, track disease progression, and create illustrations for publication and education. The primary role of ophthalmic imaging however goes well beyond documentation in its ability to aid in diagnosis of a broad range of eye conditions. Treatment plans derived from the diagnostic information provided by ophthalmic images have benefited countless patients, as ophthalmologists use images for decision making purposes on a daily basis, and in some cases, rely on photographs as a “road map” to guide therapy.

The history of ocular photography dates back to the late 1800’s when Jackman and Webster described a technique for photographing the retina of a living human subject. The next fifty years witnessed a slow advancement in instrumentation and techniques. Photographic results were mostly inadequate due to slow film speeds, long exposures, and inconsistent light sources. In the 1950s electronic flash and 35mm cameras were adapted to ophthalmic instruments and modern ophthalmic photography was born.

slit1

There is a compelling connection between this photographic discipline and our subject, the human eye; a connection that goes beyond the obvious parallels between eye and camera, cornea and lens, iris and aperture, retina and film. We use the visual art of photography to identify problems in another visual system – the eye itself. Optical instruments used to examine or photograph the eye often utilize the optics of the subject eye to help in visualizing the target pathology.

CarterR4

Ophthalmic photography can at times be simple or incredibly complex. Ocular tissues can be opaque, translucent, or transparent and may require different strategies to record these structures photographically. Enhancement of anatomic features is sometimes necessary, and can be achieved by using fluorescent dyes, monochromatic light, or specialized optical devices and techniques to adequately document subtle pathology that would otherwise not be visible.

Bennett Cataract

Creativity and aesthetics have their place in this field and many ophthalmic images transcend their scientific purpose and achieve artistic merit, but it can be heartbreaking when the most photogenic or aesthetically pleasing subjects present themselves because a patient’s vision is severely compromised. Enthusiasm for creative imaging can suffer in the context of tragic loss of vision. This is balanced however, against those times when an imager captures great images that help preserve a patient’s vision. Unfortunately not all images will be of sufficient quality to warrant public display or publication. The challenge for ophthalmic photographers is to provide consistent clinical images of adequate diagnostic quality, even under adverse conditions.

RD Barr

By providing support for education, research, and clinical eye care activities, ophthalmic photographers have been an integral part of the professional eye care community for decades. As new diagnostic imaging and treatment modalities are developed, the role of the ophthalmic imager will evolve and continue to play an important role in the preservation of sight.

bennett slideshow17

The Eye is Like a Camera

“The eye is like a camera.”

I first heard this expression many years ago when a retina specialist was explaining the results of a fluorescein angiogram to a patient and her family. He went on to explain that her retina represented the “film” in the camera and that it was swollen with fluid and wrinkled, which was the cause of her blurred vision. Everyone present easily understood the analogy, as 35mm cameras were commonplace at the time. The idea of warped or wrinkled film resonated as an analogy.    

Although it was my first time hearing this comparison, it was not a new or unique concept. The compelling connection between the eye and camera dates as far back as the 16th century when Leonardo DaVinci drew a comparison between the camera obscura and the human eye. In the next century, Della Porta, Cigoli, and Descartes also made a similar connection in their writings on optics and vision.

With the introduction of practical photographic methods in 1839, the analogy soon extended beyond the optical comparison between eye and camera obscura to include the comparison between vision and imaging. As photography and cameras quickly grew in popularity, scientists, physicians and ophthalmologists such as Helmholtz and LeConte advanced the analogy between photographic camera and the eye.​

“The further explanation of the wonderful mechanism of the eye is best brought out by a comparison with some optical instrument. We select for this purpose the photographic camera. The eye and the camera: the one a masterpiece of Nature’s, the other of man’s work.”

            Joseph LeConte

​From the earliest days of photography, several investigators sought to use the camera to document the condition of the eye. Due to the technical limitations of available photosensitive materials and the difficulty in illuminating the interior of the eye, fundus photography became the “holy grail” of medical imaging. Over the next several decades there were incremental advances in optical instrumentation and photographic processes, all moving steadily towards practical examination and photography of the eye. The most notable of these milestones was the 1851 introduction of the ophthalmoscope by Helmholtz.

Lucien Howe, one of the early pioneers in fundus photography echoed the idea of eye as camera in his 1887 article in which he described having captured the first recognizable fundus photograph:

“We know that the eye itself is a camera, and when placed behind an ophthalmoscope it has pictured upon the retina an image of an eye observed.”

Lucien Howe

The analogy was taken yet a step further by Mann who presented a paper utilizing animal eyes as an actual camera by placing photographic film against an aperture cut in the posterior pole of the globe.

“The main object has been to demonstrate that the eye can actually be used as a camera, a fact which is of interest chiefly because of the frequent comparison between the two.”

William Mann


From that point on there were several milestones in the evolution of both photography and ophthalmic photography that culminated in reflex-free fundus cameras equipped with electronic flash by the 1950’s. The modern fundus camera revolutionized ophthalmic photography and provided the platform for the important development of fluorescein angiography techniques by Novotny and Alvis in 1959. Optical coherence tomography has further revolutionized ophthalmic imaging, but fundus photography and angiography remain vital tools in the diagnosis and treatment of ocular disease.

In today’s wireless and digital age, the analogy of eye as camera may not be as universally accepted as it was a generation ago. Most 35mm cameras had a spring-loaded pressure plate on the film door to ensure the film was held flat and firmly in place. It was easy to envision the concept of a piece of film (or a retina) being wrinkled, torn, or warped.

Today’s digital cameras do not open to reveal the fixed sensor inside so the analogy doesn’t resonate as well. Yet the optical comparison between the eye and camera lens still rings true today, even with sophisticated scanning instruments such as SLO and OCT.

“The eye is like a camera.”…. Since the days of DaVinci, this idea has been echoed countless times by philosophers, artists, ophthalmologists and photographers. As an ophthalmic photographer, I consider myself fortunate to have spent a career photographing the human eye: one camera being used to preserve the capabilities of the other.

“The camera exists because men were intrigued by the function of the eye and wished to be able to reproduce on a permanent record that which the eye enabled the brain to record. How appropriate then that ophthalmology has turned the camera into a valuable tool for recording the structures of the eye.”

R. Hurtes

“The eye is like a camera.”

References

Wade NJ, Finger S. The eye as optical instrument: from camera obscura to Helmholtz’s perspective. Perception 2001; 30:1157-1177

LeConte J. The Eye as an Optical Instrument. from Sight: An Exposition of the Principles of Monocular and Binocular Vision. 1897

Howe L. Photography of the interior of the eye. Trans Amer Ophth Soc. 23:568-71 July 1887

Mann WA. Direct utilization of the eye as a camera. Trans Am Ophthalmol Soc 42:495-508, 1944

Novotny HR, Alvis DL. A method of photographing fluorescence in circulating blood of the human retina. Circulation. 24:82, 1961

Meyer-Schwickerath, G. Ophthalmology and photography. AJO 66:1011, 1968

Bennett TJ. Ophthalmic imaging – an overview and current state of the art. Journal of Biocommunication, 32(2):16-26, 2007.

Bennett TJ. Ophthalmic imaging – an overview and current state of the art, part II. Journal of Biocommunication, 33(1):3-10, 2007.

Hurtes R. Evolution of Ophthalmic Photography. International Ophthalmology Clinics. 1976; 16(2):1-22

 

 

Me, Myself, and Eye

Although it may seem like a recent phenomenon, the photographic self-portrait has been with us since the dawn of photography. Perhaps the earliest known “selfie” was taken by Hippolyte Bayard (Portrait of a Drowned Man), a Frenchman who claimed to have invented a photographic process prior to the Daguerrotype. The same can be said of William Henry Fox-Talbot (The Reading Establishment).

In recent years, the photographic self-portrait has exploded in popularity into a global phenomenon, fueled by social media sites such as Facebook, Instagram, Twitter, Snapchat, and others. It is estimated that over one million “selfies” are taken every day. A recent search of Instagram returned over 211 million photos with the hashtag “#selfie”. The term “selfie” is believed to have originated in Australia and has been elevated from internet slang to our common vernacular and even inclusion in several formal English dictionaries. In fact, selfie was Oxford Dictionary’s word of the year for 2013!

Everybody seems to be getting into the act including celebrities, politicians, and even the Pope! Selfies are even popular in space.  Astronauts have shared several spacewalk selfies online, and last year, NASA promoted a global selfie project to celebrate Earth Day. They solicited over 36,000 selfies from around the world and created an interactive composite image that can be viewed on their website.

The selfie is so ubiquitous in today’s pop culture that cell phones and digital cameras often include built-in selfie-friendly apps and features such as extra wide angle lenses, articulating screens or front facing screens that facilitate the selfie pose.  You can also purchase selfie sticks to extend the camera to a better vantage point.  These popular items go well beyond the simple self-timer found on many cameras of yesteryear. Selfies are often purposely self-deprecating, campy, cheesy, or irreverent. They are meant to be spontaneous and fun and are not usually taken very seriously. The selfie craze has even spawned the infamous “duck face” pose.

Despite the fun and seemingly harmless spirit behind them, there is a belief that taking selfies can be a sign of narcissism rather than simple self-expression. There is also some concern it can be addicting and unhealthy. But there is a growing trend in telemedicine where patients can take and forward selfies to their doctors to help diagnose or triage the urgency of their condition.  So maybe there are some legitimate uses for selfies.

As a life-long photographer, I’ve taken my share of selfies over the years. I’ve even attempted a few with the equipment I use for diagnostic ophthalmic photography. Some are goofy, and in the spirit of social media selfies. Some are more practical.

Most ophthalmic imaging devices are not what you’d normally consider selfie friendly, at least not in terms of taking a photo of one’s own eyes. Because the optics of these devices are designed for photographing curved surfaces found in the interior of the eye, they usually create distortion when backed up an appropriate distance to take a facial portrait. The effect of this distortion eliminates the need for a goofy facial expression if your goal is just to post a unique selfie on the internet!

But what about useful diagnostic or artistic photos of your own eyes, photos that go beyond distorted face selfies? It’s not only possible, but surprisingly good images can be obtained with some devices.  Non-mydriatic instruments with a monitor that can be pivoted toward the patient/photographer lend themselves to self-imaging, while those with an optical viewfinder (fundus camera) or fixed monitor position (Cirrus) do not. I’ve been able to obtain eye selfies with the Zeiss Stratus, Heidelberg HRT, Heidelberg HRA/OCT, Clarity RetCam, Tomey specular microscope and various handheld external cameras. But, you might ask, “So what?” or “Why?”

Well, there have been times when I needed to check a device during maintenance or a software upgrade and it was convenient to use myself as the patient. Sometimes while training staff to use a device, I’ll demonstrate the procedure on myself. Other times, I’ve needed a quick example of a “normal” eye for a lecture like those above.

Surprisingly, the ability to take eye “selfies” has helped me identify and track pathology in my own eyes. Two years ago I suffered an idiopathic retinal tear with avulsed bridging vessel and persistent vitreous hemorrhage. This was successfully treated with vitrectomy. Like many patients, I developed a cataract after the vitrectomy. I also began to notice some distortion that corresponded to progression of an epiretinal membrane (ERM) in the same eye.

Any time I noticed a change in vision I would repeat an OCT on myself. Over the course of six months I tracked an increase in thickness of about 100 microns. The cataract also progressed and I was scheduled for cataract surgery. Two weeks prior to surgery I noticed a very subtle change in vision and sat down at the OCT like I’d done several times in the past. The OCT detected some cystoid macular edema (CME) from the ERM. Picking up the CME prior to cataract surgery was very beneficial. Preexisting CME can be exacerbated by cataract surgery, so my surgeon began a course of treatment that reduced the edema. My OCT selfies likely helped us avoid more severe or persistent edema by catching it in advance.

Cataract surgery went as planned, but within a few hours of my procedure I began to notice a new visual abnormality: a paracentral gray scotoma. Upon arriving at the clinic the next day for my post-operative check, I immediately did an SLO/OCT selfie and identified an unusual finding that corresponded directly to the scotoma.

SD-OCT demonstrated an area of hyper-reflectivity in the middle retinal layers just temporal to the fovea (green arrows) and the IR reflectance image showed a distinct dark gray lesion. Fortunately, the scotoma began to fade within a few days and so did the lesion. The jury is still out on the exact cause of the lesion but the selfies have enabled us to track improvement of my condition and possibly publish a case report. We believe it may be a case of paracentral acute middle maculopathy (PAMM), a recently described variant of acute macular neuroretinopathy (AMN). It’s rare enough, that I was able to present it at the OPS Rare Case Symposium in Ann Arbor.

As you can see, image quality can be quite good with a little practice. So good in fact that I’ve received a bill from my institution for OCT images that I’ve performed on myself! Here is a double selfie video of a Spectralis IR fundus image showing how easy it is to capture my own epiretinal membrane.

I’m beginning to think maybe I should stop taking selfies of my own eyes. After all I keep finding abnormalities! But there is a growing trend in telemedicine where patients can take and forward selfies to their doctors to help diagnose or triage the urgency of their condition.

Ophthalmic Photographers taking diagnostic selfies: obsessive, silly, or beneficial?