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High school hockey star Edward “Major” Bowen almost died after a small, undetected tumor caused spinal fluid to build up in his brain, resulting in a brain herniation. The ordeal left Major in a coma that his doctors feared would be permanent. Major stunned everyone by coming out of his coma and resuming many daily activities.
The pressure from the tumor, however, had left one side of Major’s face, from eye to jaw, completely paralyzed. Although Major continued to improve physically and mentally, the “facial droop” caused by the paralysis became increasingly worse.
“Major was unable to smile or speak clearly, and the corner of his mouth shifted to the middle of his front teeth,” said Major’s father, Bill Bowen. “His facial deterioration became so consuming that he indicated to me that he no longer wanted to live.” Mr. Bowen, who has dedicated his life to bringing Major back to “120 percent” of his pre-tumor state, set out to find the best treatment.
“We visited many doctors, but each time I knew that Major wouldn’t be satisfied with the surgical results,” Mr. Bowen said. Eventually, Mr. Bowen learned about the Facial Nerve Center at the Massachusetts Eye and Ear Infirmary (MEEI). After extensive testing Major underwent hours of delicate surgeries including complicated nerve and muscle transplants. This was followed by extensive physical therapy.
While Major still faces challenges, among them short-term memory loss, he is living life fully. Mr. Bowen says, “Major has entered college and is on the Dean’s list, and he has earned a black belt in Tae Kwon Do. He also has a nearly perfect smile on his face.”
The Facial Nerve Center at MEEI, directed by Tessa A. Hadlock, M.D., treats facial nerve disorders, which typically involve facial paralysis. About 25 new patients visit the Center each month. They receive treatment from a multi-disciplinary team of renowned nerve disorder medical specialists, including neuro-otologists, facial plastic surgeons, ophthalmologists, plastic surgeons, audiologists, nurses and physical therapists.
“The deep experience within our practice enables us to deliver a level of care to facial nerve patients that is difficult to find outside this center,” said Dr. Hadlock. “We see hundreds of patients, which enables us to constantly build on our knowledge. We can quickly prioritize issues, and develop integrated treatment plans that emphasize patient quality of life.”
Dr. Hadlock joined MEEI in 1995 and completed four years of residency as well as a year of sub-specialty training in facial plastic and reconstructive surgery. At the Center, Dr. Hadlock works closely with Dr. Mack L.Cheney, MEEI’s director of Facial and Reconstructive Surgery Service.
The Center’s innovative treatment includes photo and video documentation of facial movement and electrophysiological testing of nerve function. The Center’s digital photography and videography capabilities enable a team-based assessment of patient needs.
The facial nerve carries impulses from the brain to the facial muscles, which allows facial expression and movement. The nerve also activates tear and salivary glands, tiny ear muscles, and carries taste sensations from the tongue to the brain. When impulses to the facial nerve are interrupted, any or all of these functions may be affected. Facial nerve disorders often cause significant disfigurement and may severely impair function.
The most common cause of facial nerve disorders is Bell's palsy, a rapid onset of facial paralysis on one side of the face. Many doctors believe that a virus such as herpes simplex virus (HSV) causes Bell's palsy. That the disorder often responds to antiviral and anti-inflammatory medications further supports the theory. Another leading cause of facial paralysis is nerve damage resulting from the surgical removal of tumors. Facial nerve disorders can be inherited or occur during pregnancy or birth. Serious head injuries, ear disease, or infectious and inflammatory processes, such as Lyme Disease, multiple sclerosis and diabetes, are among other causes.
Facial paralysis typically affects one side of the face, but can affect both. Side effects include difficulties with speech and eating, facial asymmetry and drooling. Patients may also be unable to close one or both eyes. The physical effects of facial paralysis are devastating, but the emotional impact on patients and their families can be even more traumatic.
“Patients may lose the ability to communicate, both verbally and nonverbally, and common activities like eating at a restaurant become something to avoid,” says Dr. Hadlock. “So it is not unusual that the patient has severe feelings of isolation and depression.”
Upon arrival at MEEI’s Facial Nerve Center, new patients undergo a complete consultation along with a physical and neurological examination. Doctors electronically record facial function information. Patients may receive evaluation from radiology and audiology specialists.
Dr. Hadlock’s team typically considers a cross-section of three treatments: medical therapy, surgery and physical therapy. Medical therapy, which is often used to treat Bell’s palsy, includes the use of steroids, antiviral medications and botulinum therapy.
“Surgical options depend on the nature and severity of the disorder and must be carefully considered,” says Dr. Hadlock. “Doctors may try to reduce nerve inflammation and pressure, or if the nerve endings are cut, doctors will try to reconnect the endings. If the nerve is injured or parts of it lost, doctors might take a nerve graft from another part of the body to connect the nerve endings and restore facial function.”
In some cases, doctors may bring neurons into the facial nerve from a nearby nerve to restore facial movement. This approach, called reinnervation, requires cutting a different motor nerve, like that to the tongue or to the shoulder, which creates an entirely new motor problem.
Dr. Hadlock’s team rarely uses reinnervation. “This technique treats the entire face as one unit, which can cause speech impairment and other problems,” said Dr. Hadlock. “We divide the face into zones to correct problems locally, yet as part of the whole. This enables us to restore function in one area without affecting other functions of the face.”
After more than one year, paralyzed face muscles tend to undergo atrophy and scarring. If this occurs, Dr. Hadlock’s team may transfer muscle into the face from the head and neck, or from elsewhere in the body. Contraction of the newly transferred muscle imitates the native facial muscles to permit smiling, eye closure, and movement of the lower lip.
In addition to preventing certain functions, facial nerve disorders can freeze features on the paralyzed side. A patient’s mouth may droop on one side, the nose might collapse, or the space between the upper and lower eyelids might widen, making one eyebrow lower than the other and causing the loss of the blink reflex. To correct these problems, Dr. Hadlock’s team works closely with patients to determine the best option. An eyebrow can be repositioned to match the other brow’s height, for example, during rehabilitation of a paralyzed eye; doctors can widen the nasal cavity using small cartilage grafts; or, “static sling” techniques can reposition corners of the mouth, without a muscle transfer.
After surgery, physical therapy is key in recovery. MEEI boasts one of the world’s most highly accredited physical therapists, Mara Robinson. “Although neuromuscular retraining can’t restore perfect function, it helps with facial muscle control and increases self-confidence,” says Dr. Hadlock. She added, “If a doctor stops with surgery, the patient’s quality of life may fall short.”
In keeping with MEEI’s strong commitment to research and education, the Facial Nerve Center engages in extensive laboratory and clinical research. This results in innovations in facial nerve grafting, muscle and nerve transfers and other areas central to treating facial nerve disorders. MEEI urges physicians to focus on reconstructive plastic surgery to treat complex problems like facial paralysis, ear deformities in children, and severe burns. Because these surgeries require extensive subspecialty training, MEEI is establishing a fellowship program to train new specialists.
Under this program, a physician who has completed general ENT residency training will spend up to two years in specialty training in facial nerve and reconstructive surgery, with a clinical or research component. The fellow will be exposed to the most complex and challenging cases, both in the MEEI operating room, and during MEEI humanitarian missions to repair pediatric deformities. Dr. Hadlock and Dr. Cheney travel annually to Ecuador to use reconstructive surgery to help children with microtia, a congenital ear deformity.
Mr. Bowen says, “We almost went down the wrong road in terms of surgery before discovering MEEI. Tessa Hadlock diagnosed my son accurately, which was crucial to developing the right treatment plan.”
He continued, “Dr. Hadlock, Dr. Cheney and their team literally wrote the book on this surgery. I believe that MEEI has done more of these procedures than anyone in the country, and they’ve perfected this surgery. They did a wonderful job! It’s an honest to God success story.”
For more information, contact the Development office at 617-573-3345.
Why is the cornea of the eye free of blood vessels? This medical puzzle has intrigued physicians and scientists for centuries. The mystery was solved recently by a team of
Massachusetts Eye and Ear Infirmary (MEEI) researchers led by Reza Dana, M.D., M.S.c, M.P.H. Not only does this critical discovery offer hope for preventing and curing
blinding eye disease, this knowledge can also be applied to illnesses such as cancer, in which blood vessels grow abnormally and uncontrollably to feed the tumor.
The findings were published in the July 25 issue of the prestigious journal The Proceedings of the National Academy of Sciences.
The cornea is the transparent, dome-shaped window that covers the front of the eye. Extremely sensitive, it is one of a few tissues in the body that is free of blood vessels; cartilage is another. The cornea is also a powerful refracting surface, providing most of the eye's focusing power. Comparable to a watch crystal, the cornea gives people a clear window to look through. The epithelium is the thin membrane covering the cornea. Without a clear cornea, vision is impaired.
"I noted in my clinical work that the epithelium that covers the cornea displays properties that suppress inflammation and angiogenesis [growth of blood vessels]. But we didn’t know the reasons why," said Dr. Dana, head of the MEEI Cornea Service, a Senior Scientist at the Schepens Eye Research Institute and an Associate Professor of Ophthalmology at Harvard Medical School. "If there are blood vessels in the cornea, vision is impaired. Corneal blindness is a major cause of blindness worldwide, accounting for the second most common cause of blindness after cataract. Between 10 and 20 million people can be afflicted. Scarring from blood vessels is a very common problem in the cornea. We wanted to determine why the healthy cornea is free of blood vessels and why the epithelium can stop the growth of blood vessels."
His research on corneal anti-angiogenesis began approximately four years ago using mouse models and human tissues. "Most of our work was done with mice because they are genetically identical," he explained. "We also examined human tissue in the laboratory." According to Dr. Dana, the key to maintaining a blood-vessel free cornea is vascular endothelial growth factor receptor-3 (VEGFR-3), a protein that is naturally present in large amounts on a healthy corneal epithelium. “VEGFR-3 acts as a kind of ‘sink.’ This protein neutralizes the growth factors sent by the body to stimulate blood vessel growth,” he noted. "In most other tissues of the body, blood vessel growth occurs in response to a need for increased blood flow to heal an injured or infected area. The immune system sends in growth factors such as vascular endothelial growth factors (VEGF) to bind with a protein receptor called VEGFR-2 on blood vessels to trigger growth. Three forms of VEGF -- A, C, and D -- bind with this receptor. Two of them, C and D, also bind with VEGFR-3, which is usually found on cells lining lymphatic vessels and sprouting new blood vessels, to stimulate their growth.”
To test this theory Dr. Dana and his team conducted experiments using corneas with and without epithelial layers that were injured and discovered that the corneas without the layers developed blood vessels. When a substitute for VEGFR-3 was added to corneas without epithelial layers, blood vessel growth continued to be suppressed. When intact corneas were exposed to an agent that blocked VEGFR-3, blood vessels began to grow, showing that the epithelial suppression of blood vessel growth was due to its high expression of VEGFR-3.
"These tests confirmed our belief that VEGFR-3, which is present in abundance on healthy corneal epithelium, is the major factor in preventing the formation and growth of blood vessels in the cornea," said Dr. Dana. "One can surmise that the presence of VEGFR-3 in the cornea also prevents scarring and vascularization after LASIK surgery."
He added, "By knowing how the cornea works normally or physiologically, we can understand what can interfere with it pathologically. We can apply our knowledge to the cornea, the retina, or to cancers in other parts of the body. Breast cancer is one example. Cancer cells need blood vessels to proliferate. If we can stop the blood vessel growth, we can potentially stop the cancer."
Dr. Dana has been on the MEEI medical staff since 1994. He conducts research in his lab at the Schepens Eye Research Institute, treats an average of 50 to 60 patients a week with corneal and ocular inflammatory problems, and performs surgeries, primarily corneal and ocular surface transplantation. The Infirmary’s mission meshes with Dr. Dana’s reasons for entering the field of ophthalmology. "Science is profoundly important -- the foundation of history," he said. "Ophthalmology is a field where you can help and, sometimes, even cure people.”
He described the Infirmary’s environment as stimulating, because clinician-scientists are given the resources to conduct research, see patients, perform surgery, and apply new technologies that define the cutting edge of modern medicine. “In many other facilities, clinicians only get to see patients or perform research," he said.
MEEI is also home to an Angiogenesis Laboratory, where a team of clinical researchers including Joan W. Miller, M.D., chief of ophthalmology at MEEI, and Evangelos S. Gragoudas, M.D., the director of the Retina Service at MEEI, are conducting experiments that complement Dr. Dana’s work. Dr. Dana’s collaborators include Claus Cursiefen, M.D., Lu Chen, M.D., Ph.D., Magali Saint-Geniez, Ph.D., Pedram Hamrah, M.D., Yiping Jin, M.D., Ph.D., Saadia Rashid, M.D., and the late J. Wayne Streilein, M.D., all from the Schepens Eye Research Institute; and Kris Persaud, Bronislaw Pytowski, and Yan Wu from ImClone Systems, Inc. in New York.
According to Dr. Dana, the next stage of his research will focus on developing drugs that use VEGFR-3 to heal corneas that have suffered trauma or help shrink tumors that are fed by the rapid growth of blood vessels. "After we have worked out the processes in the laboratory, we can then move to the clinic," he said. "I’m confident that we’ll be able to restore the sight of many individuals or, even better, prevent the medical problems from developing in the first place."
In addition to this avenue of investigation, Dr. Dana would like to establish a first-of-its-kind Ocular Surface Center at MEEI. One potential project would grow cells from the lining of a patient’s mouth and use them to reconstruct the corneas of patients for whom surgery is no longer an option. He explained, "This technique would help two types of patients. Individuals with two bad eyes would have a new and safe source of stem cells that could be used to reconstruct the corneal surface. These stem cells could also be used on patients with one bad eye and eliminate the risk of damage to the good eye during the harvesting process."
To learn more about Dr. Dana’s work, please contact Melissa Paul at 617-573-4168.
The Massachusetts Eye and Ear Infirmary is the first institution in the United States to offer the newest FDA-approved LASIK technology. The new technology will allow delivery of faster vision correction treatments and enhance patient comfort. Read more.
Since joining the Massachusetts Eye and Ear Infirmary’s (MEEI) staff, Assistant Surgeon and Glaucoma Specialist Dr. Douglas Rhee has devoted the majority of his day to treating patients with glaucoma and researching new and better ways to treat them. His dedication to finding a cure for this blinding disease doesn’t leave him with much personal time. “I don’t have very many hobbies, but that’s OK, because I love my job. My job is my hobby” says Dr. Rhee, as he explains how much he truly enjoys his role at the MEEI.
After choosing ophthalmology as his specialty, Dr. Rhee explains the reason he decided to further sub-specialize in glaucoma. “Patients diagnosed with glaucoma often require long-term follow up. They often need someone to help them through dealing with a disease that does not currently have a cure,” says Dr. Rhee. He enjoys the ongoing clinical relationship that develops as a result.
“My patients are a constant reminder of the need for more research,” he says. “While clinical care allows me to help one person at a time, teaching and research provide the opportunity to increase that ability exponentially,” says Dr. Rhee.
During his research, Dr. Rhee searches for the underlying causes of glaucoma, which is the second leading cause of blindness in the United States. Though he loves the relationships he now develops with his patients, Dr. Rhee hopes that discoveries through ongoing research will one day lead to a cure for this blinding disease.
With the winter months approaching, the dangers of accidental carbon monoxide poisoning increase. Read more on how to protect yourself and your family from falling victim to this odorless, poisonous gas.
Calling all runners! For the second year in a row, the Massachusetts Eye and Ear Infirmary (MEEI) is convening Team Eye and Ear to run in the Boston Marathon. MEEI is thankful for the continued generosity of John Hancock, which has provided official race numbers for 50 runners who will have the opportunity of a lifetime to run in the world-renowned Boston Marathon and to raise funds for an important cause: to preserve vision, hearing, voice and balance. Last year’s team raised more than $180,000 in funds to support the MEEI’s mission. We hope this year’s team will be just as successful, if not more so. MEEI welcomes people of all backgrounds and abilities to consider joining the team. People who participated last year ranged from first-time marathon runners to triathlon athletes!
If you'd like to join MEEI Team Eye and Ear or know someone who would, please contact Special Events and Research Coordinator Courtney Hudson at 617-573-3347 or email Courtney at:courtney_hudson@meei.harvard.edu.
Dr. Louis Pasquale published a study in the Journal of Ophthalmology on July 25 that found that women with Type 2 diabetes have a higher risk of having glaucoma. The
results of the study were also posted on UPI, MSN, Daily India, MedPage Today and Newswise.
Read the full story.
Drs. Roland D. Eavey and Michael J. Cunningham of the Massachusetts Eye and Ear Infirmary Pediatric Otolaryngology Service, in conjunction with other medical schools,
found that reducing the amount of work hours alone for surgical residents does not appear to improve quality of patient care. The study was published in the October
issue of the Journal of Surgical Research.
Read more about the study.
Boston Herald reporter and radio talk show host Margery Eagan details her experience receiving a mini-facelift from facial and cosmetic surgeon Dr. Mack Cheney of
Massachusetts Eye and Ear Infirmary’s Facial and Cosmetic Surgery Center in the October issue of Boston Magazine.
View the entire article.
Researchers at the Massachusetts Eye and Ear Infirmary (MEEI), including Johanna Seddon, M.D., director of MEEI’s epidemiology unit, found that individuals who smoke
or have a history of smoking had an increased risk of developing age-related macular degeneration (AMD) as compared to those who never smoked. Those who ate fish and had
diets higher in Omega-3 fatty acids reduced their risk of AMD.
Read the full article.
The Massachusetts Eye and Ear Infirmary continues the fight to preserve and restore sight, hearing, voice and balance. Support this important cause by making your tax-deductible gift. There are many ways that you can contribute. Every gift, no matter the size, is important and is deeply appreciated.
The 2007 Guys for Eyes calendar is now available. Suzi Reynolds, the calendar's creator, was inspired by the care her mother received at the Massachusetts Eye and Ear Infirmary. The 2007 calendar features a variety of men and raises money for research into the prevention and cause of blinding diseases macular degeneration and diabetic retinopathy.
Visit guys4eyes.org to purchase a copy.
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