A Powerful Synergy between Research, Teaching and Practice at the Pediatric Ophthalmology Program at Montefiore Medical Center

Friday, August 2, 2013

With specialists and subspecialists in every area of pediatric ophthalmology, the program combines leading-edge research, exceptional patient care informed by 30 years of service, and a solid commitment to understanding and working with patients and families in the Bronx and surrounding communities.

Conversation with the faculty members of the Pediatric Ophthalmology Program reveals not only a diverse set of subspecialties and academic interests, but also a shared habit of mind. These physicians hold a broad perspective, looking to treat specific eye problems in the context of individual lives. They share a dedication to the most granular of details, whether the angles of refraction of light from a single eye or the behavior of specific cells in a particular disease pathology.

It’s a model that attracts students in the program to stay on as faculty — such as Ilana Friedman, MD, FAAO, who trained at Montefiore and is now attending physician, Director of the Fellowship Training Program in Pediatric Ophthalmology and Strabismus, and Assistant Professor at Albert Einstein College of Medicine.

“Given the size of our division and the breadth of our experience, we are in some respects unique because we have so many pediatric ophthalmology subspecialties covered well. When our patients come to see us, they have the advantage of having specialists in every area of pediatric ophthalmology to consult. Whatever question or condition comes our way, we have someone here with the knowledge and expertise to figure it out.”

“At Montefiore Medical Center, we develop close relationships with families of children with pediatric eye disease because we meet and talk with them so frequently and because treatment can be challenging for the physician, family and patient. It’s a tough road for the patient, and care requires a long-term commitment and a strong, collaborative partnership between the family and the physician.”
— Ilana Friedman, MD, FAAO, attending physician, Director of the Fellowship Training Program in Pediatric Ophthalmology and Strabismus, and Assistant Professor at Albert Einstein College of Medicine

Expertise in Corneal Transplants

Recognized as a Center of Excellence, the Montefiore Einstein Center for Transplantation, in partnership with the Pediatric Ophthalmology Program, is committed to helping children with corneal diseases. The program is one of only a few on the East Coast with the capability to care for infants and newborns, which includes performing corneal transplant surgery, a procedure for which the program is widely known.

A number of conditions may give rise to the need for corneal transplant surgery, says Norman Medow, MD, FACS, Director of Pediatric Ophthalmology and Strabismus at Montefiore and Professor of Ophthalmology and Pediatrics at Einstein.

“One common condition is called Peter’s anomaly, in which the corneas do not develop properly,” says Dr. Medow. “Children cannot see clearly through the resulting ‘cloudy window,’ meaning these corneas need to be replaced. The lens may also be affected, resulting in the need for a more complex surgical procedure.”

Dr. Katkovskaya
Dr. Katkovskaya tests a patient for stereoacuity.

There are other congenital anomalies of the cornea, including congenital hereditary endothelial dystrophy (CHED), Dr. Medow explains. This recessive, inherited condition results from gene mutations and involves corneal thickening and edema within the cornea. CHED requires surgical treatment to improve vision.

“In this condition, a portion of the cornea — specifically the endothelial cells, which are supposed to keep the cornea clear by keeping a balance between fluid entering and leaving the cornea — does not function properly,” says Dr. Medow. “When that function fails, the cornea becomes cloudy.”

Trauma to the eye, common in young patients, can also result in a need for corneal surgery.

“Children around 1 year old, who are beginning to walk and run, often get poked in the eye by their siblings or fall and injure themselves,” Dr. Medow says. “In fact, trauma becomes the most common cause of corneal disorders at this age and through childhood.”

When corneas are damaged through a hereditary condition or injury, they may require replacement, often through a procedure called a penetrating keratoplasty. The first step requires obtaining suitable tissue from an eye bank.

“When the eye bank receives suitable tissue and assures us that it is not infected or damaged, we can then acquire the tissue and transplant this clear cornea into our patient’s damaged eye,” says Dr. Medow. “The entire cornea is not transplanted, but rather the central portion in which the tissue is scarred may be replaced.”

Follow-up care involves immunosuppressive medications and regular visits with a pediatric ophthalmologist, Dr. Medow says. Complications of the surgery may include rejection of the transplant and development of glaucoma.

“Children heal very rapidly, and transplant failures in children also occur very rapidly,” Dr. Medow says. “In the case of corneal transplant, the child’s stitches usually are completely removed within two to three months. Checking on children who have had cornea transplants is more time-intensive than evaluating adults, as children cannot always verbalize eye pain or decreased vision. Because of this, more frequent eye care visits are required.”

Dr. Medow and Cheng Zhang, MD, MS
Dr. Medow and Cheng Zhang, MD, MS, attending physician at Montefiore Medical Center and Assistant Professor at Albert Einstein College of Medicine, discuss a patient.

Untangling the Roots of Congenital Corneal Disease

In addition to treating young patients, physicians in the Pediatric Ophthalmology Program are working hard to uncover the tangled genetic roots of eye conditions resulting from heredity or mutation. One such problem, congenital stromal corneal dystrophy (CSCD), is the research focus of Cheng Zhang, MD, MS, attending physician at Montefiore and Assistant Professor at Einstein.

Clouding the corneas of both eyes, CSCD is dominantly an inherited condition caused by mutation of the decorin gene.

“Now that the gene in question has been identified, we are trying to correlate the genetic mutation with the pathology of the disease,” Dr. Zhang says. “We are finding that the gene mutation of these patients causes significant protein structural changes in the eye. These changes affect the collagen fibril formation of the cornea, causing it to become cloudy and resulting in vision problems. Normal collagen structure and arrangement is the key to maintaining clear corneas.”

For the moment, research is focused on studying the pathogenic mechanism of the disease. The payoff of such research, Dr. Zhang says, will eventually be a gene-based treatment for CSCD.

“This single gene causes a 33 amino acid deletion at the C-terminal end, resulting in decorin protein changes leading to collagen fibril malformation,” he says. “With this knowledge, we will now be able to understand other corneal diseases involving collagen abnormality as well as how protein changes affect collagen structure and lead to cloudy corneas.”

Although CSCD is rare, Dr. Zhang says, conclusions that can be easily drawn from this single-mutation disease may be translated to aid in the understanding and treatment of related conditions.

“It’s easier to investigate a disease involving a mutation of a single gene,” he says. “This is one of the very few studies on pathogenic transmission of congenital corneal disease, and it has broad implications for our understanding of other related conditions.”

Childhood Glaucoma, Retinal Detachment and Cataracts

Another rare condition seen with some frequency at a tertiary care center such as Montefiore is childhood glaucoma.

“Children can be born with glaucoma or develop it,” Dr. Medow says. “We provide care for this and many other conditions until our young patients are 18 years old, just as other pediatricians do.”

Resulting from a variety of factors — including defaults in the eye’s draining system, diabetes, injury and extremes in blood pressure — glaucoma is characterized by an increase in intraocular pressure. This increase in pressure can injure the cornea and cause vision problems, in addition to excessive blinking, tearing and sensitivity to light.

For young patients especially, surgical management of glaucoma is preferable to medical management due to the need to prevent blindness. At Montefiore, surgeons use endoscopy to place a tube shunt, moving fluids out of the eye and reducing pressure within the eyeball. With surgery and care, more than 80 percent of pediatric glaucoma patients can go on to have normal vision.

Greater risk of developing glaucoma, along with other eye conditions, is becoming part of the sequelae of the growing epidemic of Type 2 diabetes in childhood. As this disease becomes increasingly common in young people, Dr. Friedman says, many eye disorders associated with adult diabetes are seen more frequently in child patients.

Ilana Friedman, MD, FAAO
Ilana Friedman, MD, FAAO, attending physician, Director of the Fellowship Training Program in Pediatric Ophthalmology and Strabismus, and Assistant Professor at Albert Einstein College of Medicine, examining a child with the slit lamp biomicroscope

“Complications of adult diabetes, such as cataracts and retinal detachment, are now becoming possible for younger patients due to the prevalence of Type 2 diabetes in the pediatric population,” Dr. Friedman says. “In children with Type 1 diabetes, standard procedure is to screen for eye disorders three to five years into the disease process, whereas in adults with Type 2 diabetes, we screen immediately upon diagnosis. It’s not clear what direction the screening path for pediatric patients with Type 2 diabetes will take — whether they will follow the adult Type 2 model or the Type 1 model.

“This is a challenging time, with a new population of pediatric patients whom we didn’t treat previously for diabetes-related eye disorders. It will be interesting to see what happens and how we can develop the best practices in treating and managing these children.”

Dr. Friedman adds: “We are also considering looking at compliance in terms of how often and at what point in the disease process pediatricians of patients with Type 2 diabetes or any juvenile diabetes are referring these children for eye examinations. We want to focus on education at the patient level, and also at the level of our physician partners, helping them get these young people into pediatric ophthalmology practices as soon as possible so we can monitor and manage them going forward.”

A Nuanced Touch: Strabismus Surgery

Another procedure performed frequently in Montefiore’s Pediatric Ophthalmology Program is surgery to correct strabismus. While the procedure for this misalignment of the eyes is not new, the surgery requires precision, and, as Dr. Zhang points out, every physician’s technique is slightly different.

“The layman’s description of strabismus is crossed or lazy eyes — esotropia and exotropia,” Dr. Zhang says. “Surgery involves modifying the eye muscle so the eyes are aligned. While many think of this as a cosmetic procedure, its main purpose is relief of stereopsis in children and to improve the functional and communicative ability of the eye.”

During a typical strabismus surgery, Dr. Zhang says, the patient is put under general anesthesia.

“We make a small incision on the conjunctiva, that is, the outermost layer of the eyeball,” Dr. Zhang explains. “Then the muscle that controls eye movement, which is also outside the eyeball, is relocated. We adjust the length of the muscle to weaken or strengthen it, bringing the eye into perfect alignment. After attaching the muscle, we suture the incision, leaving virtually no visible scarring.”

The trick to this surgery, Dr. Zhang says, is the process of determining how much of the muscle should be removed before reattaching the muscle to the eye.

“Before surgery, we use a tool called prism lenses to measure the patient’s angle of deviation,” he says. “This technique was initially developed by Dr. Marshall Parks, [MD,] the father of pediatric ophthalmology, several decades ago. The surgeon uses the measurement to determine the length of muscle to be cut or moved backward using the prism table. However, each surgeon modifies the table based on his or her own personal experience and technique.”

Pediatric Ophthalmology Program
The Pediatric Ophthalmology Program at Montefiore Medical Center comprises: (L-R): Irina Katkovskaya, OD; Ilana Friedman, MD; Norman Medow, MD, FACS; Erin Walsh, MD; Cheng Zhang, MD; and (not pictured) Jamie Rosenberg, MD.

Technology Brings Pediatricians and Pediatric Ophthalmologists Together

While each Montefiore pediatric ophthalmologist applies a personalized touch to treatment planning and delivery, his or her expertise is complemented by that of colleagues in pediatrics. Technology such as RetCam enables pediatric ophthalmologists to collaborate with pediatricians in any location and help diagnose and treat pediatric eye diseases. Montefiore is one of the few medical centers in the nation to possess both stationary and mobile RetCam units for use in the neonatal intensive care unit (NICU), the operating room or the office setting.

“RetCam is a video camera for the imaging of infant retinal disorders,” Dr. Zhang says. “It is moveable, so you can take pictures of the retinas of premature babies quite easily in the NICU. We know premature infants may have problems with the retinal vessels not developing well; with this technology, we can document changes in infants’ retinas for many weeks after birth and determine which babies will develop severe eye problems requiring laser or medical treatment.”

Dr. Zhang looks forward to a time when this technology will streamline the diagnosis of retinal problems in premature infants.

“In the Internet age, RetCam can be used in a NICU, and the images can be forwarded to a pediatric ophthalmologist or pediatric retinal specialist who is an expert in deciphering them,” he says. “While the process has not been standardized yet, it offers great hope for the diagnosis and prompt treatment of these infants, preventing even more cases of blindness.”

Dr. Walsh consults with Dr. Friedman
Dr. Walsh consults with Dr. Friedman on a patient case.

Synergy in Practice

Combining research and teaching with regular clinical work sharpens the faculty’s approach to each discipline.

“Research has a strong impact on our day-to-day work in the clinical setting,” Dr. Friedman says. “We apply what we learn on a regular basis to our patients. Both research and clinical work gives us the opportunity to educate the next round of residents, who will eventually become colleagues.”

Dr. Friedman is principal investigator in the National Institutes of Health Pediatric Eye Disease Investigators Group (PEDIG), which is focused on determining the best treatment approaches to different childhood eye disorders.

“The group has researched whether treating amblyopia with part-time patching is as effective as full-time patching,” says Dr. Friedman. “Contrary to popular belief, six hours of patching can achieve the same results as full-time patching, the study revealed. That insight has changed how we treat patients and has improved compliance because children can now patch in the privacy of their homes. This groundbreaking study is directly impacting patient’s lives.”

Support Staff
The program’s support staff at the Henkind Eye Institute at Montefiore Medical Center

An International Collaboration

One of the most unique aspects of Montefiore’s Pediatric Ophthalmology Program is the combination of world-class medical expertise with embedded, local commitment to care.

The program’s faculty published 13 papers and presented 14 lectures between January 2012 and May 2013. Other scholarly outreach includes a planned collaborative educational summit between Montefiore’s Department of Pediatric Ophthalmology and China’s Association for Pediatric Ophthalmology, an effort spearheaded by Dr. Zhang.

“Through outstanding clinical care, world-class research and innovative partnerships, we are dedicated to improving and maintaining patients’ vision,” says Dr. Medow. “With therapies and technologies improving all the time, we envision a bright future for children’s eye care, and Montefiore will help to lead these efforts.”

To learn more about the Pediatric Ophthalmology Program at Montefiore, please visit www.montefiore.org/eyes.