September 2008
Feature Story

Karen Lusky

With only a handful of ophthalmic pathologists practicing their subspecialty full time nationwide, and a waning number of training programs, eye pathologists are alerting the pathology community to what’s at stake and posing tentative solutions.

There’s a “real danger the knowledge that doesn’t make it into textbooks will be lost” as a generation of ophthalmic pathologists retire without training enough successors who will have “a supportive institutional berth” into which they can settle, says Frederick A. Jakobiec, MD, DSc. He is director of the David Glendenning Cogan Laboratory of Ophthalmic Pathology at the Massachusetts Eye and Ear Infirmary and Henry Willard Williams professor emeritus of ophthalmology and pathology at Harvard Medical School.

One issue, says Thomas J. Cummings, MD, associate professor of pathology and ophthalmology at Duke University, “is that some pathology residency programs might not have a high enough eye pathology volume, or they might not have an eye pathologist who is able to show residents the cases and teach them and get them interested in the field. The danger is that no one ends up learning it.”

The encroaching shortage may be not unlike the eye itself, known to be a window to systemic problems. Eye pathology is like the “canary in the coal mine,” says Hans E. Grossniklaus, MD, MBA, director of the L.F. Montgomery Pathology Laboratory and director of clinical eye pathology at Emory University in Atlanta. As a subspecialty that adds “luster or excellence to practices,” it’s most vulnerable and the first to signal a larger problem, he says. And that problem, in part, is low reimbursement for certain services that can skew health care delivery in unintended ways.

As Harvard University professor of clinical ophthalmology Thaddeus Dryja, MD, puts it: “Whenever you read about a shortage of any type of professional ... what’s really meant is that employers don’t want to pay what the work is worth. If you paid enough, you’d have enough nurses and high-tech engineers, for example. Eye pathology is no different. Primary care physicians are also on the list.” Dr. Dryja is global head of translational medicine in ophthalmology and molecular genetics at Novartis Institutes for Biomedical Research, Cambridge, Mass.

Though reimbursement for eye pathology has historically been low, the subspecialty has typically been a part of ophthalmology departments, which have often subsidized it. But economics has eroded that support, Dr. Jakobiec says, threatening the subspecialty’s existence and “a good deal of departmental teaching.”

Eye pathologists haven’t had a strong voice and probably have had no direct presence in working with Medicare to set pricing for its services, he adds.

To provide an example of how out of synch payment for eye pathology can be, neuropathologist Steven Bauserman, MD, who trained in eye pathology, which makes up a small part of his practice, notes that a corneal button specimen for a transplant recipient is coded as an 88304. That’s the same code used for an epidermal inclusion cyst of the skin, which requires almost no expertise—“a sophomore medical student could sign that out,” says Dr. Bauserman, of Austin (Tex.) Pathology Associates.

Most of the specimens eye pathology receives are from the cornea and conjunctiva, all of which are CPT code 88304, says Patricia Chévez-Barrios, MD, director of the ophthalmic pathology program at Methodist Hospital in Houston. “And many are really difficult cases that require special stains and even immunohistochemistry or electron microscopy,” she says. “The better codes for difficult cases are 88307 and 88309, but the only thing for which we can bill 88307 is if the entire eye was removed, whether for a simple problem or a difficult tumor. You do a lot of sectioning for an 88307.”

Additionally, says Emory’s Dr. Grossniklaus, “there aren’t that many eye pathology specimens at most institutions, so a large practice is needed in order for it to generate revenues to support the laboratory.”

What eye pathology brings to clinical practice is perhaps most evident when viewed through the Zenlike lens of what happens when the expertise is not available.

Many conditions in the body show up in the eyes. While general pathologists can say the eye has “x, y, or z,” they can’t always trace it to the cause, Dr. Chévez-Barrios says. But “pathologists are called the doctors’ doctor because they are always asked, ‘What does this mean?’ Many times, we [as eye pathologists] have to say this means this, and if you do this it will get better, or you need to do a larger excision, etc.”

As one example of what can occur when a general pathologist with little background does ophthalmic pathology, Dr. Jakobiec points to pathologic diagnosis of the common chalazion, an aseptic inflammation of the meibomian gland deep within the eyelid structure. “Under the microscope, it is a granulomatous inflammation and looks like it could be a fungal infection or perhaps a systemic granulomatous disorder,” he says. “If you show such a lesion to a general pathologist, he or she might think it’s caused by a fungus, leprosy, tuberculosis, or even Wegener’s granulomatosis. However, I’ve never seen those infections in the tarsus of the eyelid.”

Dr. Chévez-Barrios finds that pathologists usually make the correct diagnosis of retinoblastoma. But to provide an accurate prognosis, the pathologist has to examine the optic nerve to see if the tumor goes through the optic nerve or is within a certain distance of the nerve. “That information determines whether the patient has to have adjuvant treatment or not,” she says. Yet “many of the specimens we get don’t have the sections [required to make that determination] because the pathologist or the technician cutting the eye doesn’t know how to obtain this area consistently or what to look for.”

Dr. Grossniklaus recently evaluated a case at Emory that wasn’t treated properly because of a series of errors in clinical and pathologic diagnoses. “An area where this occurs is corneas,” he says. Some patients have bad outcomes after refractive surgery because their corneas were too thin for LASIK, putting them at risk for developing keratectasia, which requires corneal transplantation. And the eye pathologist is “suited to detect the pathologic findings in keratectasia” when examining such corneas, he says, thereby providing useful information for surgeons who are choosing patients for refractive surgery.

Other conditions that can be misdiagnosed by those without eye pathology training include:

• Primary intraocular lymphoma. Dr. Grossniklaus notes that one needs ophthalmology training to know how to interpret clinical tests and findings to diagnose this condition, which presents as subretinal pigment epithelium infiltrates, vitreous cells, and a leopard spot appearance on a fluorescein angiogram. “The patient presents with blurred or decreased vision.”



• Sebaceous carcinoma of the eye. Older data show that sebaceous carcinoma of the eye is usually misdiagnosed by general pathologists as basal cell or squamous cell carcinomas more than 50 percent of the time on the initial biopsy, Dr. Grossniklaus says. But sebaceous cell carcinoma is more aggressive than the aforementioned cancers and usually requires more extensive surgery than a basal cell carcinoma, he notes. Sebaceous carcinoma tends to get into the conjunctival epithelium and replace it, says Ralph C. Eagle Jr., MD, director of the Department of Pathology at the Wills Eye Institute, Philadelphia. “You may have to do map biopsies to evaluate the extent of conjunctival involvement.”



• Acanthamoeba infection causing keratitis. This condition is something that an eye pathologist tends to see enough of to diagnose from scrapings, Dr. Grossniklaus says. “Usually, patients with this condition have a history of wearing contact lenses and presenting with a painful corneal ulcer. The acanthamoeba comes from nonsterile cleaning solutions or people cleaning their contact lenses using tap water or well water.” Numerous antimicrobial drugs are needed to treat the condition. “Untreated or inadequately treated, it can lead to blindness and the need for corneal transplantation or even enucleation.”

Without education and the availability of ophthalmic pathologists, ophthalmology may also lose touch with how to submit specimens and interpret a pathology report, and with quality assurance controls such as the refractive surgery question and evaluating new ophthalmic procedures, warns Dr. Grossniklaus.

For example, eye pathologists figured out that a new outpatient procedure to treat uveal melanomastranspupillary thermotherapy using a laser to treat the melanoma—was sometimes failing. Emory and a few other centers realized this when they examined enucleated eyes and saw melanoma cells in the sclera that had been insulated from the thermotherapy and thus spread to adjacent structures, Dr. Grossniklaus says. “The sclera goes all around to the back of the optic nerve.” And you can’t clinically examine the back of the eye. The person with uveal melanoma in this instance should have the eye removed or have a radioactive plaque put on top of the sclera, which is done only in surgery, he says.

There’s another danger to the decline of eye pathology, and that is losing the connection between basic science research and clinical ophthalmology care. It was eye pathologists, for example, who discovered that certain drugs have a bad effect on the eye, Dr. Grossniklaus says. “One thing our lab found [via experimental studies] is that if you inject gentamicin in the eye, it can result in retinal toxicity because of a gravitational effect.” Thus, patients who receive drug injections in the eye need to remain upright until the drug settles.

Could advances in eye imaging pick up some of the slack caused by a shortage of eye pathologists? Duke’s Dr. Cummings says that while modern imaging techniques are good, they are not absolute. He points to a recent case in which a patient with a known systemic cancer had an orbital/optic nerve lesion believed clinically to be metastatic disease or a meningioma. Yet the biopsy showed the lesion was caused by an inflammatory condition. As a result, the patient received the correct therapy and showed marked clinical improvement.

To address gaps in eye pathology training, a few academic institutions are turning to cyber solutions.

Currently, the University of Illinois at Chicago is providing this form of “distributive” graduate medical education to several universities and medical centers in addition to its own, says Robert Folberg, MD, department head of pathology and professor of ophthalmology and visual science. (He left his position at UIC Sept. 15 to become dean of the Oakland University William Beaumont School of Medicine in Michigan.) The technologies used in the program include bidirectional videoconferencing, Web-based and interactive CD-ROM-based tools, and virtual microscopy. “The program has been approved by the ophthalmology residency review committee as an acceptable means of training people,” Dr. Folberg says.

Emory University offers the Internet-Based Eye Pathology Teaching Initiative, which is geared toward ophthalmology and pathology residents and medical students. The site is available to those who sign up for it and pay a user fee to help maintain it. It is updated quarterly with new unknown cases and includes an archive. The initiative can be part of a rotation in the L.F. Montgomery Ophthalmic Pathology Laboratory with hands-on training, though any ophthalmology or pathology department can register to use it, Dr. Grossniklaus says.

Duke University hosts the EyePathologist (www.eyepathologist.com), which, as the Web site says, is set up to “emulate one-to-one interaction with an expert eye pathologist.” The site, which requires only registration to use, includes a database of more than 5,000 diseases that affect the eye and vision, as well as other tissues, and almost 4,000 images. There’s also a glossary of more than 6,000 definitions that the user can access.

The author of the site is Gordon K. Klintworth, MD, PhD, professor of pathology and Joseph A.C. Wads­worth research professor of ophthalmology at Duke University Medical Center. An anatomic pathologist and neuropathologist who delved into eye pathology in the early 1960s, Dr. Klintworth developed the site, in part, using the teaching materials he collected over a period of more than 40 years. Financing for the interactive site came from unrestricted funds, as well as his own personal funds. Dr. Klintworth believes that the 24/7 availability of the site helps ophthalmology and pathology residents, most of whom are extremely busy to the point that they cannot easily attend conferences and seminars even if someone offers them.

Dr. Klintworth also developed the site for international use, noting that in many parts of the world, there’s not a single ocular pathologist on the entire continent. “Currently, there are 9,080 registered users from 158 countries,” he says.

As for educational requirements for practicing eye pathology moving forward, Duke’s Dr. Cummings says that one issue is how much training a physician needs to be competent and, based on ophthalmology and pathology guidelines, to be given the authority to sign out cases.

Dr. Jakobiec, who, like a small number of eye pathologists (among them Drs. Grossniklaus, Chévez-Barrios, and Folberg), is double boarded in pathology and ophthalmology, believes board certification in one of the two specialties, combin­ed with an appropriate supplemental program in the other specialty, may be sufficient. “The general way people get to acquire adequate experience has been through an eye pathology fellowship,” says Dr. Jakobiec, who adds there are probably still a handful of institutions offering fellowships in the United States where a “center of excellence” standard can be met.

Credentialing is one way to judge competency apart from conferring automatic privileges by virtue of becoming boarded, says Dr. Jakobiec. Each institution has to have its own way of addressing that.

There could also be a qualifying exam in eye pathology that’s less than a full board certification, he says. “It could be a form of national credentialing. The American Association of Ophthalmic Pathologists [AAOP], with now over 100 members and its own governing body, could provide the aegis for such a function.” The AAOP currently supports the oversight of ophthalmic pathology fellowships by having them approved by the Association of University Professors of Ophthalmology Fellowship Compliance Committee.

Opinions differ about where ophthalmic pathologists should be housed—the pathology or ophthalmology department—to best support and promote the subspecialty and its practitioners.

Dr. Folberg is encouraging a colleague who is double boarded in pathology and ophthalmology and others to practice in the context of a pathology department where, in addition to eye pathology, they teach and do general surgical pathology work to supplement their income.

Dr. Folberg reports seeing more “cross-dialogue” between eye pathologists and the larger community of pathologists in the U.S. and even overseas. Most major pathology journals, he says, are now accepting and encouraging submission of articles on ophthalmic pathology. And sessions dedicated to ophthalmic pathology are offered now at pathology meetings.

He predicts, in fact, that eye pathology will eventually become a full-fledged subspecialty under pathology, albeit a small one. “Until a few years ago, there was skepticism whether pathology was interested” in this subspecialty, he says, “but now we see they are, which is even more impetus to drive this toward pathology.” For the subspecialty to be taken back by pathology, “not as a reaction to something bad but as the natural evolution of the discipline of pathology, is a beautiful solution moving forward.”

But Harvard’s Dr. Jakobiec maintains that “having eye pathologists under pathology alone would make eye pathology the minority activity for the person. It’s better than no arrangement at all, but if the eye pathologist is in the ophthalmology department, his or her primary focus will be on eye pathology. The eye department would have someone at its beck and call to do diagnostic work [and] be available for teaching and collaborations in science.”

He also thinks that ophthalmology department chairs have perhaps been too “shortsighted” in viewing eye pathology as a financial burden that needs to be dealt with. “The eye department is the main place that ophthalmologists, trainees, and ophthalmic surgeons learn about eye diseases and morbid ocular and adnexal tissue changes as taught by eye pathologists. Without that expertise coupled with surgical training, the residents and staff are half a step away from being an optometrist,” says Dr. Jakobiec, who says he is speaking as the former chairman of ophthalmology at Harvard Medical School and former chief of ophthalmology at Massachusetts Eye and Ear Infirmary.

“My successor in the ophthalmology department at Harvard and the MEEI has embraced eye pathology, which has been a tradition here, but I know not every institution is going to embrace it in this way.”

Wherever eye pathology resides, Dr. Jakobiec thinks ophthalmology departments should have an intimate relationship with pathology departments on a political and support level to ensure the best outcomes. For example, the eye pathology laboratory, of which he is director, is under both the Massachusetts Eye and Ear Infirmary ophthalmology department and Massachusetts General Hospital pathology department. The eye pathology facility resides at the infirmary, where it’s connected to Massachusetts General by a walkway.

The hospital does the lab’s tissue processing, which it bills to Medicare and third-party payers. The slides come to the eye pathology unit at the infirmary. There Dr. Jakobiec, another eye pathologist, and a neuropathologist who is associate director of the eye pathology lab and in the pathology department at MGH, sign out the cases with rotating residents and fellows in attendance and bill for the professional component. Dr. Jakobiec reports primarily to the ophthalmology department chair and secondarily to the pathology chair.

“This structure has been in place for the past year and a half, and all parties are progressively benefiting from it,” he says.

“Every week or two I see a case with features that I’ve never observed before and turn to my resident, fellow, or attending and say, ‘Let’s write this up or present it at a conference.’ That’s only going to happen in a university with great eye departments and dedicated individuals with a comparatively vast experience—and with the backup and help that can be supplied by a pathology department for sophisticated subspecialty consultations and special stains, etc.”

Dr. Grossniklaus, too, believes eye pathology needs to remain aligned with ophthalmologists. “They are the customers,” he says of ophthalmologists. “The person directly responsible for the patient’s care is the one who should know that the case needs to go to someone trained in eye pathology.”

Of course, there’s still the issue of resources and funding, which put eye pathology on its downward trajectory. In Dr. Jakobiec’s view, the ophthalmology and pathology departments both have “to pony up financial support for facilities and salaries if they really value eye pathology.”

Dr. Grossniklaus’ laboratory, on the other hand, is what he calls “a model profit center” in the Department of Ophthalmology. “This is because it is a freestanding, independently licensed laboratory that evalutates a high volume of specimens, focusing on quality of service and operational efficiences.” Dr. Grossniklaus uses endowments and grants to support the research and teaching mission of the lab.

When people ask whether we need eye pathology, Dr. Grossniklaus says, “the answer is that the general pathologist does a pretty good job in many cases. But without eye pathologists, there are going to be patients falling through the cracks. What if we miss patients with deadly [or blinding] diseases? To me, it’s not worth going backward and losing the expertise we have gained with eye pathology.”