- •Contents
- •Introduction
- •Contributors
- •ROLE OF BIOPSY
- •DIRECTED TREATMENTS OF DISTINCT ORBITAL INFLAMMATIONS
- •ABSTRACT
- •ACKNOWLEDGEMENTS
- •5 Future and Emerging Treatments for Microbial Infections
- •MICROBIOLOGIC DIAGNOSIS
- •EMERGING ANTIBIOTIC RESISTANCE
- •HISTORICAL PERSPECTIVE
- •CURRENT APPROACH
- •FUTURE DIRECTIONS
- •7 Non-Hodgkin’s Lymphoma
- •INCIDENCE AND EPIDEMIOLOGY
- •ETIOLOGY AND RISK FACTORS
- •DIAGNOSIS, CLASSIFICATION, AND STAGING
- •TREATMENT
- •ABSTRACT
- •INTRODUCTION
- •STEPS TOWARD TUMOR SPECIFIC THERAPY
- •CANCER SPECIFIC MOLECULAR TARGETS
- •DNA ARRAY ANALYSIS
- •WHICH MOLECULAR TARGETS?
- •CONCLUSIONS
- •10 Malignant Lacrimal Gland Tumors
- •THERAPEUTIC RECOMMENDATIONS
- •SPHENOID WING MENINGIOMAS
- •Location
- •PRESENTING SIGNS AND SYMPTOMS
- •RADIOGRAPHIC IMAGING
- •ULTRASOUND
- •HISTOPATHOLOGY
- •TREATMENT AND PROGNOSIS
- •13 Stereotactic Radiotherapy for Optic Nerve and Meningeal Lesions
- •BACKGROUND
- •DEFINITIONS
- •Precise Immobilization
- •Precise Tumor Localization
- •Conformal Treatment Planning and Delivery
- •FUTURE DEVELOPMENTS
- •SUMMARY
- •ABSTRACT
- •INTRODUCTION
- •ABSTRACT
- •INTRODUCTION
- •Enzyme-Linked Immunosorbent Assay (ELISA)
- •Prospective Study of Graves’ Disease Patients
- •DISCUSSION
- •ACKNOWLEDGEMENTS
- •ORBITAL FIBROBLASTS DISPLAY CELL-SURFACE CD40 AND RESPOND TO CD154
- •CONCLUSIONS
- •ACKNOWLEDGEMENTS
- •INTRODUCTION
- •Retina, RPE, and Choroid
- •Optic Nerve
- •ACKNOWLEDGMENT
- •INTRODUCTION
- •METHODS
- •Historical Features
- •Tempo of Disease Onset
- •Clinical Features
- •DISCUSSION
- •19 Prognostic Factors
- •PREVENTION OF GRAVES’ OPHTHALMOPATHY BY EARLIER DIAGNOSIS AND TREATMENT OF GRAVES’ HYPERTHYROIDISM?
- •CLINICAL ACTIVITY SCORE
- •ORBITAL ECHOGRAPHY
- •ORBITAL OCTREOSCAN
- •ORBITAL MAGNETIC RESONANCE IMAGING
- •URINARY GLYCOSAMINOGLYCANS
- •SERUM CYTOKINES
- •CONCLUSION
- •BACKGROUND
- •VISA CLASSIFICATION
- •Strabismus
- •Appearance=Exposure
- •DISCUSSION
- •INTRODUCTION
- •NONSEVERE GRAVES’ OPHTHALMOPATHY
- •SEVERE GRAVES’ OPHTHALMOPATHY
- •Glucocorticoids
- •Orbital Radiotherapy
- •Immunosuppressive Drugs
- •Plasmapheresis
- •Somatostatin Analogues
- •Intravenous Immunoglobulins
- •Antioxidants
- •Cytokine Antagonists
- •Colchicine
- •INTRODUCTION
- •STABLE ORBITOPATHY
- •Preferred Decompression Techniques
- •EYE MUSCLE SURGERY
- •LID PROCEDURES
- •PATHOPHYSIOLOGY OF THE DISEASE
- •MEDICAL THERAPY
- •IMPROVEMENTS IN ORBITAL DECOMPRESSION
- •IMPROVEMENTS IN EYELID SURGERY
- •STRABISMUS SURGERY
- •Michael Kazim
- •John Kennerdell
- •Daphne Khoo
- •Claudio Marcocci
- •Jack Rootman
- •Wilmar Wiersinga
- •Answer
- •Question 1 (continued)
- •Answer
- •Question 2 (from M. Potts)
- •Answer
- •Question 2 (continued)
- •Question 3
- •Answer
- •Question 3 (continued)
- •Answer
- •Question 3 (continued)
- •Answer
- •Question 3 (continued)
- •Answer
- •Question 4 (from M. Mourits)
- •Answer
- •Question 5 (from F. Buffam)
- •Answer
- •Question 6 (from F. Buffam)
- •Answer
- •Question 7 (from P. Dolman)
- •Answer
- •INTRODUCTION
- •CLINICAL MANIFESTATIONS OF DVVMs
- •INVESTIGATION OF DVVMs
- •FUTURE CONSIDERATIONS
- •CONCLUSION
- •INTRODUCTION
- •CAROTID-CAVERNOUS SINUS FISTULAS
- •ARTERIOVENOUS MALFORMATIONS
- •DISTENSIBLE VENOUS ANOMALIES
- •PREOPERATIVE EMBOLIZATION OF TUMORS
- •ANEURYSMS
- •FUTURE DIRECTIONS
- •ABSTRACT
- •INTRODUCTION
- •TECHNOLOGICAL ADVANCEMENTS
- •Advances in Medical Imaging
- •Virtual Reality Surgical Simulation
- •Surgical Robotics
- •HUMAN BODY MODELS
- •FUTURE COMPUTER-AIDED ORBITAL SURGERY
- •SUMMARY
- •ACKNOWLEDGMENTS
- •30 The Future of Orbital Surgery
- •Index
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long-term disease is indicative of a rehabilitative mode and the approaches for which are quite different.
As Dr. Gorman often plays the role of the coordinator of the patient’s care, the next stage at this point is to outline from the beginning to end what are the expected sequential steps in the patient’s total rehabilitation so that the patient has the entire perspective on what is required for total rehabilitation. The key element of this is free communication of a consistent message from the ophthalmologist and endocrinologist and a firm commitment to the patient that the team will be a part of this process.
Once these steps are in place, the approach to mild, moderate, and severe disease falls into place. If the disease is severe and vision threatening, it is typically a short course of steroids. Failure to respond to steroids leads to the offer of orbital decompression. If the disease is mild or moderate and stable, the approach is with the methods that have been described in this symposium, and that is primarily a surgical approach with muscle surgery first followed by lid surgery.
Michael Kazim
Dr. Kazim identified a few differences between his practice and that of Dr. Gorman, specifically the nature of the referral, which leads to a different type of patient being seen. He believed that his patients present very early in the course of their disease and therefore tend not to be stable-phase patients but rather acute-phase, which he considers to be a medical as opposed to a surgical condition.
Like Dr. Gorman, it is his opinion that the patient’s first visit provides the specialist with the opportunity to set the tone for the rest of the patient encounter, which can last from 1 to 4 years. A typical first visit is about 1–1.5 hr—however long it takes—for Dr. Kazim to understand the patient’s disease status and needs and for the patient to understand the complete approach he is offering. If patients somehow do not ‘‘buy into’’ his approach, they will have to convince him that their condition or their relationship to their
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condition, be it special functional or cosmetic needs, would alter the way he would normally proceed.
In his experience, the approach to mild, acute-phase disease is virtually 100 percent supportive. In the cases where it may be a little more congestive, some form of nonsteroidal anti-inflammatory drug may be used. At one point of time, he had studied nonsteroidal anti-inflammatories and based on particular characteristics of the drugs, chose Voltaren. (He notes that he did not study the outcome of treatment of the drugs.) Patients receive Voltaren 75 mg twice a day. Steroids are not routinely used, although occasionally a patient will present already on steroids but this happens less frequently. If patients are on steroids without compressive neuropathy, the steroids are withdrawn, since he does not believe that the drug will make a lot of difference in the long term. Only in cases of optic neuropathy will he proceed with surgical intervention, which is where his approach differs from that of Dr. Gorman’s. Dr. Kazim’s institution has a great deal of experience and success with a combination of radiation and steroids—radiation to limit the time frame of the disease and steroids to eliminate the optic neuropathy. They proceed surgically only where the steroids and=or radiotherapy are contraindicated or fail. All other patients fall into stablephase disease and are treated surgically, as was described by other speakers.
John Kennerdell
Dr. Kennerdell noted that management of thyroid orbitopathy had been extensively covered during the symposium, most of which was in keeping with his approach. One important factor of management is to ensure that the approach was individualized to the patient. Another important factor is flexibility: individual treatment of the patient based on having available all the modalities. In his practice, all modalities are used, including, steroids, radiotherapy, chemotherapy, and various types of decompression. With muscle surgery, adjustable sutures are preferred, and a limbal approach is used, as in his opinion, there is a need to recess the conjunc-
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tiva but cover the insertion site. Another lid surgery technique is the upper and lower tarsal transplant, which is a simple procedure and the rejection rate is practically 0. It is a useful technique for resolving a 2.0–2.5 mm lower lid retraction. In his experience, he has only one buckle.
Muscle surgery is delayed for at least 6 months for stability, longer if possible. Lid surgery is delayed for a year. Both surgeries occur after the decompression has been done.
Like Dr. Gorman, most of his time is spent counseling the patient. This involves explaining in detail the whole course of the disease and what they may expect. Two brochures that summarize the disease are also provided. By and large, it is a counseling job with the exception of the more severe cases.
Thank you.
Daphne Khoo
Dr. Khoo is an endocrinologist in Singapore whose clinic sees a large number of Graves’ disease patients. They see approximately 1500–2000 referrals of Graves’ disease per year, and of these about 400 will have at least some form of thyroid eye disease. Very few of these cases actually end up with the ophthalmologist because of the volume, unfortunately. At one stage, a proposal had been put forth by the ophthalmologists that the thyroid eye disease patients be sent to them, but after 2 months they said they could not cope with the volume. Generally, about 10% the cases will eventually go to the ophthalmologists and obviously these will be the most symptomatic patients.
One unusual factor of their population base, relative to the Caucasian populations, is a very low prevalence of smoking. The majority of patients are Chinese females and of this group, only 8% are smokers, and this may be the reason why patients with far less severe ophthalmopathy are seen in their clinic. This has led to some question as to the applicability of data from Caucasian studies to their studies.
In terms of optic neuropathy, most patients end up with the ophthalmologist rather than with us, since they present most of the time with visual disturbances. With these
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patients, the management is quite clear-cut. High-dose pulsed methylprednisolone is administered for a couple of days and if there is no marked improvement, the patients undergo decompression surgery.
The other situations are more difficult because they have found that there is a great reluctance on the part of their patients to agree to decompression surgery for indications other than optic neuropathy. Despite careful explanation that the decompression is just the first of multiple surgeries, most patients do not give consent, which may explain the low numbers that were presented earlier by Dr. Seah, my colleague in ophthalmology from Singapore.
With regard to orbital irradiation, the problem there is a reluctance amongst the therapeutic radiologists in Singapore to give orbital radiation, largely because the incidence of nasopharyngeal carcinoma (NPC) is fairly high in the Chinese population. They have found with NPC that about 10 years after irradiation, it is very common to develop hypopituitarism, although patients may be fine for the first 10 years. Given the bad experiences with NPC, the therapeutic radiologists are very concerned with whether the shielding is currently adequate to prevent the onset of optic nerve damage or damage to other parts of the eye many years down the line. These fears of course may well be unfounded.
For the patients who have moderately severe disease but do not want either decompression or orbital irradiation, the course of management is often pulsed steroids, which is used frequently by their group. They have had good results with octreotide; unfortunately, the cost of this treatment modality is a big factor, as it is not subsidized by the government. Other treatments have been tried, including cyclosporine, pentoxyfylline and cox-2 inhibitors, all with varying degrees of success. In their experience, pulsed steroids and octreotide have been effective.
Claudio Marcocci
Dr. Marcocci’s center is one of the major referral centers in Italy. They see a large number of patients with Graves’
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disease and Graves’ ophthalmopathy and work side-by-side with the ophthalmologists. Once the patient has been evaluated, a great deal of time is spent discussing with the patient the natural history of the disease, what can be done for them, and what their expectations are.
For a patient with mild, active eye disease, they believe that it is important to restore and maintain euthyroidism with antithyroid drugs. Once the patient has reached euthyroidism, the choice of treatment for hyperthyroidism is not influenced by the presence of ophthalmopathy. Particular care is taken to avoid hypothyroidism that can be induced by excess in antithyroid therapy. They usually suggest the use of supporting measures until the disease becomes inactive, the so-called ‘‘wait-and-see’’ policy. Once the disease is inactive, rehabilitative surgery may be suggested if necessary.
In patients with moderate and active ophthalmopathy, it is necessary to have a rapid and permanent control of hyperthyroidism. For this purpose, once euthyroidism is reached with antithyroid drugs, the patient is treated with either radioiodine or thyroidectomy according to goiter size and sometimes at the request of the patient. When radioiodine is used, the patient also receives a shortened course of glucocorticoids. Particular attention is taken once again to avoid post-treatment hypothyroidism. Supportive measures are also suggested and once the disease is over in terms of activity, rehabilitative surgery is offered along with, in certain cases, orbital decompression.
For severe orbital disease, unless there is a risk of loss of vision, once control of thyrotoxicosis is obtained with permanent treatment through radioiodine or thyroidectomy, the use of total thyroid ablation is considered to get rid of all orbital antigens. Again, surgery is delayed until the disease is inactive.
In patients with sight-threatening ophthalmopathy, their approach differs according to whether the patient has already been treated elsewhere. Orbital decompression is usually offered as the initial treatment, if previous treatment from another institution has been noted. In cases where the
