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In the Netherlands the general public can legally use fireworks on New Year's Eve. Despite the fact that the public is well aware of the dangers of fireworks, fireworks accidents occur in which the victims suffer a variety of injuries. In addition to burns, injuries to fingers and/or hands are most common. However, injuries to the eye, which can also involve facial trauma, frequently occur as well. Trauma mainly occurs as a result of the destructive effects of the wave of pressure released by the explosion. The best preventive measure would be to prohibit the use of fireworks by amateurs. Until such time, people should be advised to wear protective fireworks glasses when outside so that damage to the eyes from legal fireworks at least can be prevented.

Purpose: Intravenous glucocorticoids (ivGCs) given as 12-weekly infusions are the first-line treatment for moderate-to-severe and active Graves' orbitopathy (GO), but they are not always effective. In this study, we evaluated whether response at 6 weeks correlated with outcomes at 12 (end of intervention) and 24 (follow-up) weeks, particularly in patients initially unresponsive., Methods: Our database (Bartalena et al. J Clin Endocrinol Metab 97:4454-4463, 10), comprising 159 patients given three different cumulative doses of methylprednisolone (2.25, 4.98, 7.47 g) was analyzed, pooling data for analyses. Responses at 6 weeks were compared with those at 12 and 24 weeks using three outcomes: overall ophthalmic involvement [composite index (CI)]; quality of life (QoL); Clinical Activity Score (CAS). Responses were classified as "Improved", "Unchanged", "Deteriorated", compared to baseline., Results: Deteriorated patients at 6 weeks for CI (n = 8) remained in the same category at 12 weeks and 7/8 at 24 weeks. Improved patients at 6 weeks for CI (n = 51) remained in the same category in 63% and 53% of cases at 12 and 24 weeks, respectively. Unchanged patients at 6 weeks (n = 100) eventually improved in 28% of cases (CI), 58% (CAS), 32% (QoL). There was no glucocorticoid dose-dependent difference in the influence of early response on later outcomes., Conclusions: Patients who deteriorate at 6 weeks after ivGCs are unlikely to benefit from continuing ivGCs. Patients unresponsive at 6 weeks still have a significant possibility of improvement later. Accordingly, they may continue ivGC treatment, or, alternatively, possibly stop ivGCs and be switched to a second-line treatment.

The purpose of the study was to report the long-term outcome of unilateral implantation of a multifocal intraocular lens (IOL) in a pediatric cataract. This study was carried out at Tertiary Academic Ophthalmology Department, AMC, Amsterdam. This is a case report study of a 7-year-old child with a unilateral irradiation cataract, in whom an apodized diffractive multifocal IOL (SN6D3, Alcon) was implanted at the time of cataract surgery. During the follow-up period, visual acuity was preserved at logMAR -0.1; the child did not develop amblyopia. Binocular single vision was established. Few glistenings were seen on the IOL. The non-operated eye developed more myopia during the follow-up period than the multifocally implanted eye. Straylight was increased to log(s) 1.83. Patient and parents satisfaction were high. In selected cases, unilateral implantation of apodized diffractive multifocal IOLs leads to good long-term results in terms of visual acuity and patient satisfaction. No untoward effects were seen, including few glistenings on the IOL. Straylight is increased, but subjectively not disturbing.

Orbital cellulitis is a rare disease usually caused by sinusitis. Generally, the prognosis of both preseptal cellulitis and orbital cellulitis is favourable. Radiological distinction should be made between an orbital abscess and subperiosteal empyema. Theoretically, empyema is more accessible to antibiotics and, as it arises in a pre-existing anatomical space, it needs less aggressive treatment than an abscess. In contrast, the wall of an abscess created by the bacteria is scarcely permeable to antibiotics. Indications for surgical drainage should be based on clinical findings and not on Chandler's classification. Loss of vision, an unresponsive pupil or a densely packed orbit are indications for immediate surgical drainage. Drainage of an orbital abscess may speed up recovery.

Purpose: To evaluate differences in straylight between eyes implanted with a hydrophilic multifocal IOL (Seelens MF; Hanita Lenses, Hanita, Israel) and a hydrophobic multifocal IOL (SN6AD1; Alcon Laboratories, Inc., Fort Worth, TX)., Methods: In a prospective cohort study, routinely obtained straylight measurements (C-Quant; Oculus Optikgeräte, Wetzlar, Germany) 3 months after standard phacoemulsification for either cataract or refractive lens procedures were compared. Patients were implanted with either the SeeLens MF IOL or the SN6AD1 IOL. Postoperative straylight values, visual acuity, and refractive outcomes were compared., Results: The SeeLens MF IOL was implanted in 84 eyes and the SN6AD1 IOL in 79 eyes. The difference in straylight was 0.08 (P = .01), with the SeeLens MF IOL having less straylight. Postoperative CDVA was logMAR -0.03 ± 0.06 in the SeeLens MF group, and logMAR -0.02 ± 0.08 in the SN6AD1 group. Mean postoperative refraction was +0.01 ± 0.43 and +0.06 ± 0.35 D, respectively., Conclusions: The Seelens MF IOL showed a stray-light of log(s) 0.08 lower than the SN6AD1 IOL. In terms of spherical equivalent and visual acuity the lenses performed equally. More study will aid in understanding the causes and clinical impact of this difference., (Copyright 2015, SLACK Incorporated.)

The timing of orbital reconstruction is a determinative factor with respect to the incidence of potential postoperative orbital complications. In orbital trauma surgery, a general distinction is made between immediate (within hours), early (within 2 weeks), and late surgical intervention. There is a strong consensus on the indications for immediate repair, but clinicians face challenges in identifying patients with minimal defects who may actually benefit from delayed surgical treatment. Moreover, controversies exist regarding the risk of late surgery-related orbital fibrosis, since traumatic ocular motility disorders sometimes recover spontaneously and therefore do not necessarily require surgery. In this study, all currently available evidence on timing as an independent variable in orbital fracture reduction outcomes for paediatric and adult patients was systematically reviewed. Current evidence supports guidelines for immediate repair but is insufficient to support guidelines on the best timing for non-immediate orbital reconstruction., (Copyright © 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

In the 1980s, computed tomography was introduced as an imaging modality for diagnosing orbital fractures. Since then, new light has been shed on the field of orbital fracture management. Currently, most surgeons are likely to repair orbital fractures based on clinical findings and particularly on data obtained from computed tomography scans. However, an important but unresolved issue is the fracture size, which dictates the extent and type of reconstruction. In other fields of trauma surgery, an increasing body of evidence is stressing the importance of complexity-based treatment models. The aim of this study was to systematically review all articles on orbital reconstruction, with a focus on the indication for surgery and the defect size and location, in order to identify the reconstruction methods that show the best results for the different types of orbital fractures., (Copyright © 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Chronic lymphocytic leukaemia (CLL) is the most frequent form of leukaemia among adults in the Western world, presenting at a median age of 65 years. The diagnosis is usually made incidentally during routine blood examination while the disease is still in its early phase. We report a case of blindness of 24 hours due to acute sinusitis based on CLL localisation in a patient with undiagnosed CLL. Emergency endoscopic sinus surgery and intra- and extra-ocular orbital decompression were performed. The sinusitis resolved after surgery and intravenous antibiotics. Her vision improved within 24 hours and eventually recovered completely after six months. Her CLL remained in an indolent state, needing no active treatment. This case illustrates that blindness from a lymphoproliferative disorder may be treated with emergency endoscopic sinus surgery instead of conventional chemotherapy in order to salvage the vision first, even if the vision is lost for more than 24 hours.

Background: Optimal doses of i.v. glucocorticoids for Graves' orbitopathy (GO) are undefined., Methods: We carried out a multicenter, randomized, double-blind trial to determine efficacy and safety of three doses of i.v. methylprednisolone in 159 patients with moderate to severe and active GO. Patients were randomized to receive a cumulative dose of 2.25, 4.98, or 7.47 g in 12 weekly infusions. Efficacy was evaluated objectively at 12 wk by blinded ophthalmologists and subjectively by blinded patients (using a GO specific quality of life questionnaire). Adverse events were recorded at each visit., Results: Overall ophthalmic improvement was more common using 7.47 g (52%) than 4.98 g (35%; P = 0.03) or 2.25 g (28%; P = 0.01). Compared with lower doses, the high-dose regimen led to the most improvement in objective measurement of ocular motility and in the Clinical Activity Score. The Clinical Activity Score decreased in all groups and to the least extent with 2.25 g. Quality of life improved most in the 7.47-g group, although not reaching statistical significance. No significant differences occurred in exophthalmos, palpebral aperture, soft tissue changes, and subjective diplopia score. Dysthyroid optic neuropathy developed in several patients in all groups. Because of this, differences among the three groups were no longer apparent at the exploratory 24-wk visit. Major adverse events were slightly more frequent using the highest dose but occurred also using the lowest dose. Among patients whose GO improved at 12 wk, 33% in the 7.47-group, 21% in the 4.98-group, and 40% in the 2.25-group had relapsing orbitopathy after glucocorticoid withdrawal at the exploratory 24-wk visit., Conclusions: The 7.47-g dose provides short-term advantages over lower doses. However, this benefit is transient and associated with slightly greater toxicity. The use of a cumulative dose of 7.47 g of methylprednisolone provides short-term advantage over lower doses. This may suggest that an intermediate-dose regimen be used in most cases and the high-dose regimen be reserved to most severe cases of GO.

Graves' thyroid disease is a relatively common disorder in endocrinology and general internal medicine practice. Graves' hyperthyroidism is mediated by circulating stimulating autoantibodies. Up to 60% of patients with Graves' hyperthyroidism develop some form of Graves' orbitopathy. Immune reactivity to the thyroid-stimulating hormone receptor is also thought to play a role in the immunopathogenesis of Graves' orbitopathy. Graves' orbitopathy is characterised by a wide open eye appearance, caused by upper eyelid retraction and soft-tissue swelling that causes exophthalmus. Symptoms include photophobia, sandy feeling in the eye, painful eye movements and diplopia. Visual acuity may be reduced. In some cases emergency treatment is necessary to prevent irreversible vision loss. Smoking should be stopped. Mild to moderate Graves' orbitopathy may be an indication for corticosteroid treatment or radiotherapy. Once inflammatory signs and symptoms have waned, surgery can be performed to correct residual diplopia, exophthalmus or lid retraction. The presence of Graves' orbitopathy has consequences for the management of Graves' hyperthyroidism. Adequately controlled Graves' thyroid dysfunction is likely to improve Graves' orbitopathy, while radioactive iodine treatment can worsen the condition. Due to the wide variety in clinical presentation and the possible interference between treatment of thyroid disease and eye disease, the management of more complicated patients with Graves' orbitopathy can best be performed in combined thyroid-eye clinics, in which the patient is seen simultaneously by the ophthalmologist and the endocrinologist.

Aim: To compare the outcome of various surgical approaches of orbital decompression in patients with Graves' orbitopathy (GO) receiving surgery for disfiguring proptosis., Method: Data forms and questionnaires from consecutive, euthyroid patients with inactive GO who had undergone orbital decompression for disfiguring proptosis in 11 European centres were analysed., Results: Eighteen different (combinations of) approaches were used, the swinging eyelid approach being the most popular followed by the coronal and transconjunctival approaches. The average proptosis reduction for all decompressions was 5.0 (SD 2.1) mm. After three-wall decompression the proptosis reduction was significantly greater than after two-wall decompression. Additional fat removal resulted in greater proptosis reduction. Complications were rare, the most frequent being worsening of motility, occurring more frequently after coronal decompression. The average change in quality of life (QOL) in the appearance arm of the GO-QOL questionnaire was 20.5 (SD 24.8) points., Conclusions: In Europe, a wide range of surgical approaches is used to reduce disfiguring proptosis in patients with GO. The extent of proptosis reduction depends on the number of walls removed and whether or not fat is removed. Serious complications are infrequent. Worsening of ocular motility is still a major complication, but was rare in this series after the swinging eyelid approach.

Purpose: To describe and compare long-term (> or = 36 months) effects of patients with 86 primary pterygia treated with bare sclera extirpation (BSE) followed by Beta-RT or by sham irradiation., Methods: Prospective, multicenter, randomized, double-blind study. After BSE of their pterygium, patients were randomized to either Beta-RT or sham irradiation. In the case of Beta-RT, within 24 hours after the operation, a 90Sr eye applicator was used to deliver 2500 cGy to the sclera surface at a dose rate of between 200 and 250 cGy/min. Sham irradiation was given using the same type of applicator without the 90Sr layer. After treatment, both a masked ophthalmologist and a radiation oncologist performed follow-up examinations. These were continued until either a relapse occurred or at least 36 months had elapsed., Results: Adequate follow-up was available of 86 pterygia in 81 patients, treated between February 1998 and September 2002. Fifty-two (60%) patients were male. The mean age of the patients was 50 years (range: 24-77). After a follow-up of at least 36 months (mean: 40 months, SD:13.9 months), 5 out of 44 eyes (11%) treated with Beta-RT showed a recurrence versus 32 out of 42 eyes (76%) treated with sham-RT (after a mean follow-up of 22 months) (p<0.001). In the Beta-RT group, 80% were satisfied with the cosmetic result, whereas in the sham group this percentage was 41% (p<0.001). In the Beta-RT group, no scar or a white scar could be detected in 86% of the treated eyes, versus in 24% of the sham irradiated eyes (p<0.001). A change of keratometry (Javal) was seen in 5 patients (12%) following Beta-RT compared to 16 (38%) after sham irradiation (p=0.002). Complications were few: a granuloma was seen in three patients after sham irradiation, mild limitation of abduction in two Beta-RT patients versus in five after sham irradiation, and mild scleromalacia in one Beta-RT patient., Conclusions: Bare sclera extirpation of a pterygium without adjuvant treatment has an unacceptably high recurrence rate and therefore should be considered obsolete. Bare sclera extirpation of a primary pterygium followed by a single-dose Beta-RT is a simple, effective, and safe treatment with lasting results and very few complications.

Background: The use of punctal plugs in the treatment of dry eyes is well established. Anophthalmic patients have less tears in the anophthalmic socket in comparison to their normal side, due to an absent corneal reflex (Lee & Elsie, 1981a,b). Many of those patients complain of dry eye symptoms, even when they are treated with tear replacement therapy. The authors wished to examine whether they could improve their dry socket complaints with punctal plugs., Purpose: To evaluate the clinical efficacy and tolerability of punctal plug (Smart Plugs) insertion in anophthalmic patients with symptoms of dry eye., Patients and Methods: Interventional case series. Fifteen anophthalmic patients with dry eye symptoms, a Schirmer test of less than 3 mm and an open lacrimal passage were examined before and after insertion of punctal plugs. The patients were asked for their subjective evaluation of the treatment and were examined to evaluate the change. Schirmer tests were compared. Bacterial cultures were taken at both visits., Results: Schirmer results of less than 3 mm in the anophthalmic socket were obtained in 75% of patients with dry eyes symptoms; 87% of patients in whom punctal plugs were inserted reported an improvement in dry eye sensation. More than half of the patients demonstrated less discharge (p < or = 0.05). Schirmer tests improved from 1.4 mm to 1.9 mm (p < or = 0.05). Patients with a Schirmer outcome of 2 mm or more tended to have less pathogenic bacterial cultures (p = 0.11)., Conclusion: Punctal plugs improve the symptoms and signs of dry socket. Punctal plugs seem to reduce the pathogenic bacterial growth in the anophthalmic socket.

Objective: To determine management patterns among clinicians who treat patients with Graves' orbitopathy (GO) in Europe., Design and Methods: Questionnaire survey including a case scenario of members of professional organisations representing endocrinologists, ophthalmologists and nuclear medicine physicians., Results: A multidisciplinary approach to manage GO was valued by 96.3% of responders, although 31.5% did not participate or refer to a multidisciplinary team and 21.5% of patients with GO treated by responders were not managed in a multidisciplinary setting. Access to surgery for sight-threatening GO was available only within weeks or months according to 59.5% of responders. Reluctance to refer urgently to an ophthalmologist was noted by 32.7% of responders despite the presence of suspected optic neuropathy. The use of steroids was not influenced by the age of the patient, but fewer responders chose to use steroids in a diabetic patient (72.1 vs 90.5%, P<0.001). Development of cushingoid features resulted in a reduction in steroid use (90.5 vs 36.5%, P<0.001) and increase in the use of orbital irradiation (from 23.8% to 40.4%, P<0.05) and surgical decompression (from 20.9 to 52.9%, P<0.001). More ophthalmologists chose surgical decompression for patients with threatened vision due to optic neuropathy, who were intolerant to steroids than other specialists (70.3 vs 41.8%, P<0.01)., Conclusion: Deficiencies in the management of patients with GO in Europe were identified by this survey. Further training of clinicians, easier access of patients to specialist multidisciplinary centres and the publication of practice guidelines may help improve the management of this condition in Europe.

Objective: Only a small percentage of Graves' ophthalmopathy (GO) patients develop optic neuropathy with impending loss of visual acuity. Therapy with methylprednisolone pulses is the treatment of first choice in severe and active GO patients. When the effect is insufficient, patients are usually treated with surgical decompression. We investigated whether surgery could become the first-line treatment, thus preventing treatment with steroids., Design and Subjects: We performed a randomized trial in 15 patients with very active GO and optic neuropathy. Six patients were treated with surgical decompression, and nine with methylprednisolone i.v. pulses for 2 weeks, followed by oral prednisone for 4 months. The primary outcome was determined by changes in visual acuity. If the eye disease deteriorated despite treatment or did not improve sufficiently, patients were switched to the other treatment arm., Results: The severity and activity of GO in both groups were similar at baseline. The Clinical Activity Score (CAS) was 6.3+/- 0.8 in the surgical group vs. 6.0+/- 0.5 in the steroids group and the Total Eye Score was 24+/- 6 vs. 25+/- 6. In the surgery group, 5/6 patients (82%) did not respond because of insufficient improvement in vision (n=3) or persistent chemosis (n=2), and all needed further immunosuppression. In the steroids group, 4/9 patients (45%) did not improve in visual acuity (P=0.132 vs. surgery group), and these needed decompressive surgery. All patients in whom therapy failed were switched to the other treatment arm and visual acuity improved in almost all patients. Visual acuity improved from 0.36 (0.02--0.40) to 0.90 (0.63--1.0) in the surgery group and from 0.50 (0.32--0.63) to 0.75 (0.32--1.0) in the steroids group at 52 weeks. At long-term follow-up in the surgery group 3/6 patients required squint surgery and 5/9 patients in the steroids group. Eyelid surgery was performed in 5/6 patients in the surgery group and in 4/9 patients in the steroids group., Conclusion: Immediate surgery does not result in a better outcome and therefore methylprednisolone pulse therapy appears to be the first-choice therapy.

Aim: The purpose of this study was to test the reliability of an exophthalmometer commonly used in the Netherlands; to determine the exophthalmometry value distribution with this instrument and to assess the upper exophthalmometry limits of normal in a healthy, adult, Caucasian, Dutch population. Furthermore, to assess the effects of gender and age on exophthalmometry readings in this group and in a group of Graves' patients by comparing healthy, adult, Caucasian, Dutch individuals with adult, Caucasian, Dutch Graves' patients., Methods: To test the reliability of our Hertel exophthalmometer, we determined the interobserver variation between two observers by measuring 160 eyes in healthy, adult, Caucasian, Dutch females and males (10 females and 10 males in each decade between 20 and 60 years of age). These data were also used for the assessment of the Hertel value distribution and for defining the upper limits of normal in these individuals by logistic regression analysis. The effects of disease, age and gender were established using these data plus data of a retrospective study of 393 adult, Caucasian, Dutch females (n=294) and males (n=99) with Graves' orbitopathy in whom Hertel values were measured with the same exophthalmometer., Results: Exophthalmometry using an Hertel exophthalmometer appeared reliable (Pearson correlation coefficient for interobserver variation 0.89; 96% of the Hertel values measured by two observers were within the limits (of 2 mm) of agreement). Hertel values usually show a normal distribution in healthy individuals and in Graves' patients and are sex- and age-dependent, but there was no dependence on age in this small series in adults. Logistic regression analysis revealed an upper limit of normal of 16 mm in females and 20 mm in males in our group, using the exophthalmometer described., Conclusions: Exophthalmometry is reliable and absolute measurement of proptosis is feasible. International standardization of Hertel exophthalmometry is required in order to compare exophthalmometry data in the literature reliably.

Objective: To assess the results of lacrimal duct probing in young children with epiphora, due to a congenital nasolacrimal duct obstruction., Design: Retrospective, descriptive., Method: Data were collected from medical dossiers on the results of lacrimal duct probing in children (0-48 months) with epiphora that was done in the period from January 1, 1997 to December 31, 2001 at the University Medical Centre in Utrecht, the Netherlands. The percentage of eyes that showed complete resolution of symptoms three months after the final probing was calculated., Results: Of the 89 children who had undergone lacrimal duct probing, seven were excluded and in three children (six eyes) the data could not be retrieved. In 96 of the remaining 116 eyes (83%), the symptoms disappeared: this included 96% of the age group 0-12 months (n = 25), 85% of the 13-24 months-olds (n = 55), 77% of the 25-36 months-olds (n = 22) and 57% of the 37-48 months-olds (n = 14). No complications of probing were seen., Conclusion: In most children in the various age groups, epiphora disappeared. Thus, children do not need to be probed in the first year of life out of fear of failure at an older age. Whether or not probing shortens the duration of epiphora cannot be determined on the basis of either this study or the literature.

Purpose: To study possible causes of persistent pain in patients who underwent enucleation of the globe and in whom all other noninvasively detectable causes of pain had been ruled out., Methods: Twenty patients were studied, 10 with intractable pain (score >5 on a 0-to-9 self-reporting pain scale) persisting for more than 6 months after enucleation (for various reasons) and 10 without pain (score <4) at least 6 months after enucleation. Magnetic resonance imaging (MRI) with dynamic color mapping (MRI-DCM) was used to quantify the motion of the optic nerve in millimeters per degree of gaze, 2 to 3 mm behind the implant. Histopathologic study of biopsy specimens was used to verify imaging findings., Results: The optic nerve was attached to the implant in almost all (19/20) patients. On average, the motion was significantly less in patients with persistent intractable pain (0.04 mm/deg) than in patients without pain (0.08 mm/deg; normal orbit, 0.13 mm/deg). A biopsy specimen was available in 5 of 10 patients with persistent pain, and in 4 of those 5, microscopic neuroma was found close to the optic nerve-implant junction., Conclusions: In the enucleated orbit, the optic nerve is usually attached to the implant and soft tissue motion is decreased. In patients who have persistent pain after enucleation, motion is decreased even more, and a high percentage of microscopic amputation neuromas are found. Increased stiffness of orbital soft tissue and optic nerve attachment after enucleation are detectable using MRI-DCM, and may play a role in susceptible patients in the development of microscopic amputation neuroma and pain.

A simple method for medical canthal wiring reconstruction with the help of a homolaterally fixed osteosynthesis plate and a metal wire is presented. This avoids transnasal wiring and gives superior control when correcting the position of the lacerated Omedial canthus.

Objective: The Graves' ophthalmopathy quality of life questionnaire (GO-QOL) is the first instrument available to measure health-related quality of life (HRQL) of patients with Graves' ophthalmopathy. The main objective of this study was to define a minimal clinically important difference (MCID) in score on the GO-QOL that can be considered an important improvement in HRQL by examining changes in GO-QOL scores in patients who subjectively report improvement from their treatment. A secondary objective was to test the longitudinal validity of the GO-QOL, using prespecified hypotheses about expected treatment effects., Design: A prospective cohort study., Patients: We included 164 patients who were scheduled for radiotherapy (23), orbital decompression (10 for sight loss, 38 for exophthalmos), eye muscle surgery (31), eyelid lengthening (43) or blepharoplasty (19)., Measurements: Patients completed the GO-QOL and three general HRQL questionnaires, before and three or six months after treatment, depending on the performed procedure. Clinical characteristics were collected from the medical records. Mean changes in GO-QOL scores and effect sizes were calculated after different treatments, and in subgroups of responders and nonresponders according to clinical characteristics and according to the patients themselves., Results: A clinical response to treatment was associated with a change in GO-QOL scores of approximately 10--20 points after major treatments (radiotherapy or decompression), and with a change of approximately 3--10 points after minor surgery (eye muscle surgery, eyelid lengthening, blepharoplasty). Changes in GO-QOL scores of about 6--10 points were considered important improvements by the patients themselves. The direction and amount of change in GO-QOL scores after different treatments were in accordance with our prespecified hypotheses about treatment effects. Effect sizes in the GO-QOL subscales were generally higher than effect sizes of the general HRQL subscales, supporting the longitudinal validity of the GO-QOL., Conclusions: As a general guideline, one could consider a mean change of at least 6 points on one or both subscales an important change in daily functioning for patients. For more invasive therapies, a change of at least 10 points is recommended as a minimal clinically important difference.

Purpose: To determine prospectively the cataract-free interval (latency time) after total body irradiation (TBI) and bone marrow transplantation (BMT) and to assess accurately the final severity of the cataract., Methods and Materials: Ninety-three of the patients who received TBI as a part of their conditioning regimen for BMT between 1982 and 1995 were followed with respect to cataract formation. Included were only patients who had a follow-up period of at least 23 months. TBI was applied in one fraction of 8 Gy or two fractions of 5 or 6 Gy. Cataract-free period was assessed and in 56 patients, who could be followed until stabilization of the cataract had occurred, final severity of the cataract was determined using a classification system. With respect to final severity, two groups were analyzed: subclinical low-grade cataract and high-grade cataract. Cataract-free period and final severity were determined with respect to type of transplantation, TBI dose, and posttransplant variables such as graft versus host disease (GVHD) and steroid treatment., Results: Cataract incidence of the analyzed patients was 89%. Median time to develop a cataract was 58 months for autologous transplanted patients. For allogeneic transplanted patients treated or not treated with steroids, median times were 33 and 46 months, respectively. Final severity was not significantly different for autologous or allogeneic patients. In allogeneic patients, however, final severity was significantly different for patients who had or had not been treated with steroids for GVHD: 93% versus 35% high-grade cataract, respectively. Final severity was also different for patients receiving 1 x 8 or 2 x 5 Gy TBI, from patients receiving 2 x 6 Gy as conditioning therapy: 33% versus 79% high-grade cataract, respectively. The group of patients receiving 2 x 6 Gy comprised, however, more patients with steroid treatment for GVHD. So the high percentage of high-grade cataract in the 2 x 6 Gy group might also have been caused to a significant extent by steroid treatment. The percentage of patients with high-grade cataract was lower in allogeneic transplanted patients without steroid treatment for GVHD than in autologous transplanted patients: 35% versus 48%. An explanation for this could be pretransplant therapy containing high-dose steroids., Conclusions: After high-dose-rate TBI in one or two fractions, steroids for GVHD influence latency time of a cataract and are of great importance for the severity the cataract finally attains. Although a cataract will develop in all patients, a clinically important high-grade cataract is relatively infrequent in patients not treated with steroids. Pretransplant therapy might also influence final severity of cataract.

Purpose: To investigate both feasibility and clinical potential of magnetic resonance imaging-dynamic color mapping (MRI-DCM) in measuring the motion of soft tissues in the orbit and in the diagnosis of orbital disorders by detecting changes in motion., Methods: Sequences of MRI scans were acquired (acquisition time, 5 seconds) in a shoot-stop manner, while the patient fixated at a sequence of 13 gaze positions (8 degrees intervals). Motion was quantified off-line (in millimeters per degree of gaze change) using an optical flow algorithm. The motion was displayed in a color-coded image in which color saturation of a pixel shows the displacement and the hue the displacement's orientation. Six healthy volunteers and four patients (two with an orbital mass and two with acrylic ball implant after enucleation) were studied., Results: The technique was found to be clinically feasible. For a gaze change of 1 degrees, orbital tissues moved between 0.0 and 0.25 mm/deg, depending on the type of tissue and location in the orbit. In the patients with an orbital mass, motion of the mass was similar to that of the medial rectus muscle, suggesting disease of muscular origin. In the enucleated orbits, soft tissue motion was decreased. One eye showed attachment of the optic nerve to the implant, which could be verified by biopsy., Conclusions: MRI-DCM allows noninvasive and quantitative measurement of soft tissue motion and the changes in motion due to pathologic conditions. In cases in which the diagnosis of a tumor in the apex is in doubt, it may reduce the need for biopsy. In contrast to static computed tomographic (CT) scans and MRIs, it can differentiate between juxtaposition and continuity and may be a new and promising tool in the differential diagnosis of intraorbital lesions.

Purpose: To compare artificial eye amplitudes in patients who randomly received either a hydroxyapatite or an acrylic, scleral-covered spherical implant after enucleation., Design: Randomized, controlled trial., Participants: Thirty-four consecutive patients who underwent enucleation because of an intraocular melanoma and 21 healthy control participants from the hospital staff., Methods: Eligible patients randomly received a hydroxyapatite or an acrylic, scleral-covered spherical orbital implant. Fourteen patients were fitted with a hydroxyapatite implant, and 16 were fitted with an acrylic implant. We measured horizontal and vertical saccadic amplitudes of both the artificial eye and the healthy eye. Measurements were performed with the magnetic search coils technique. Saccadic amplitudes of the artificial eye were compared with the healthy eye of the patient. The amplitudes of the healthy eyes were compared with saccadic amplitudes of control participants. The interval from surgery to measurements was at least 3 months in all patients. Saccadic gain (artificial eye and eye amplitude divided by target amplitude) and saccadic symmetry (artificial eye amplitude divided by healthy eye amplitude) were calculated., Main Outcome Measures: Saccadic gain and saccadic symmetry., Results: The gain in the healthy eyes of the patients was comparable with the gain of the control eyes. Saccadic symmetry was 1.0 in control participants. In patients, it was 0.334 in horizontal saccades and 0.577 in vertical saccades. However, saccadic symmetry did not differ significantly between the acrylic group and the hydroxyapatite group (P: > 0.1 for any saccadic direction). Equivalence was detectable with a power more than 90% for horizontal saccades and more than 80% for vertical saccades. Curvilinearity was rejected for both patient groups and for all saccadic directions (P: > 0.5)., Conclusions: When no motility peg is placed, acrylic and hydroxyapatite spherical implants yield comparable saccadic amplitudes of the artificial eye. Artificial eye amplitudes were markedly more restricted horizontally than vertically. In all saccadic directions, the relation between target amplitude and artificial eye amplitude was linear.

Objective: To determine the long-term outcome in patients with a lesion of a single lacrimal canaliculus without surgical correction., Design: Prospective., Method: In the period August 1995-August 1999 16 patients were seen with a monocanalicular trauma. No attempt was made to reconstruct the canaliculus; only the superficial layers were closed (skin and M. orbicularis oculi). At follow-up the patients were asked about epiphora (score of Munk) and the test of Anel and the fluorescein dye disappearance test were carried out., Results: At follow-up a mean of 13.7 months (range: 3-33) after the trauma the injured canaliculus was totally blocked in all patients. In spite of this, none of the patients experienced epiphora either indoors or outdoors. Tear drainage of the ipsilateral unharmed canaliculus was functioning normally in such a way that epiphora was prevented., Conclusion: In patients with a monocanalicular lesion repair of only the superficial layers of the eyelid without reanastomosis of the torn ends of the canaliculus is sufficient to maintain normal tear drainage. This procedure is safe and simple.

Background: The best treatment (steroids, irradiation, or both) for moderately severe Graves' orbitopathy, a self-limiting disease is not known. We tested the efficacy of external beam irradiation compared with sham-irradiation., Methods: In a double-blind randomised clinical trial, 30 patients with moderately severe Graves' orbitopathy had radiotherapy (20 Gy in ten fractions), and 30 were assigned sham-irradiation (ten fractions of 0 Gy). Treatment outcome was measured qualitatively by changes in major and minor criteria and quantitatively in several ophthalmic and other variables, such as eyelid aperture, proptosis, eye movements, subjective eye score, and clinical-activity score at 24 weeks., Findings: The qualitative treatment outcome was successful in 18 of 30 (60%) irradiated patients versus nine of 29 (31%) sham-irradiated patients at week 24 (relative risk [RR]=1.9 [95% CI 1.0-3.6], p=0.04). This difference was caused by improvements in diplopia grade, but not by reduction of proptosis, nor of eyelid swelling. Quantitatively, elevation improved significantly in the radiotherapy group, whereas all other variables remained unchanged. The field of binocular single vision was enlarged in 11 of 17 patients after irradiation compared with two of 15 after sham-irradiation. Nevertheless, only 25% of the irradiated patients were spared from additional strabismus surgery., Interpretation: In these patients with moderately severe Graves' orbitopathy, radiotherapy should be used only to treat motility impairment.

Objective: To evaluate the success rate of a simple surgical method for the treatment of a monocanalicular lacrimal lesion., Design: Retrospective noncomparative case series., Participants: Thirteen consecutive patients with monocanalicular trauma who were seen from August 1995 to March 1998. In six patients, the canaliculus was lacerated as a result of an external injury and in seven patients as a result of tumor removal (iatrogenic injury)., Intervention: Reapproximation of the orbicularis muscle and skin overlying the torn canaliculus without reanastomosis of the lacerated canaliculus. In those patients in whom the canaliculus was sacrificed as part of the removal of an eyelid tumor, no attempt was made to reconstruct the canaliculus., Main Outcome Measures: Symptomatology, patency of the lacrimal passage, fluorescein dye disappearance test, and patient satisfaction., Results: In all patients the injured canaliculus was totally blocked, but despite this none of the patients complained of inconvenient tearing either indoors or outdoors. The ipsilateral unharmed canaliculus was functioning normally in such a way that the fluorescein dye instillation test showed residual dye in six patients after 2 minutes and in none of the patients after 5 minutes. All patients were satisfied with the functional and cosmetic result., Conclusion: Nonrepair of a monocanalicular lesion is a valid approach that results in little or no morbidity.

Aims: To assess the prevalence of dysthyroid optic neuropathy (DON) in patients with diabetes mellitus (DM) and Graves' orbitopathy (GO) and to investigate the complications of surgery for GO in these patients., Methods: The records of 482 consecutive patients with GO referred in a 5 year period were studied. Those patients who also had DM were selected for further study. The prevalence of insulin dependent diabetes mellitus (IDDM) and non-insulin dependent diabetes mellitus (NIDDM) was registered, as well as the prevalence and course of DON. In the patients who underwent surgery for GO the postoperative complications were recorded., Results: Out of 482 patients with GO, 15 (3.1%) also had DM. Eight (1.7%) had IDDM, 7 (1.4%) had NIDDM. Five patients (33.3%) three with IDDM and two with NIDDM developed DON with 50% improvement of visual acuity after treatment, whereas in the whole population of 482 GO patients 19 had DON (3.9%), showing 69.4% improvement of vision after treatment. 10 patients with GO and DM were operated for GO; in one of them an optic atrophy developed as a result of a postoperative haemorrhage directly after a three wall orbital decompression by coronal approach. No other postoperative complications occurred., Conclusions: The prevalence of IDDM in patients with GO is higher than in the normal population. DON occurs much more frequently in patients with GO and DM than in the total group of GO patients and seems to have a worse visual prognosis.

Background: Multifocal fibrosclerosis (MF) is a rare disease that may be misdiagnosed as Graves' orbitopathy. The combination of localisations of MF presented here has not been reported before., Case Report: A 44-year-old man was referred with progressive bilateral exophthalmos. CT of his chest and abdomen revealed an intrathoracic and retroperitoneal peri-aortal soft-tissue process with hydronephrosis. Histological examination of orbital masses showed a fibrous process with low inflammatory activity. Later the sella was found to be involved as well. A diagnosis of MF was made., Conclusion: MF should be considered in the differential diagnosis of bilateral exophthalmos.

Background: Several lengthening techniques have been proposed for upper eyelid retraction in patients with Graves' orbitopathy and variable rates of success have been reported. Most authors recommend different procedures for different degrees of retraction, but cannot prevent residual temporal retraction in a significant number of cases. The modified levator aponeurosis recession described by Harvey and colleagues, in which the lateral horn is cut completely, seems to be an exception to this rule, but was evaluated in a limited number of cases only., Method: The authors further modified Harvey's technique by dissecting the aponeurosis together with Müller's muscle of the tarsus and the conjunctiva medially only to the extent necessary to achieve an acceptable position and contour of the eyelid in upright position. They also used an Ethilon 6.0 suture, instead of Vicryl, on a loop. It is placed between the tarsal plate and the detached aponeurosis to prevent spontaneous disinsertion. This modification was used in 50 Graves' patients (78 eyelids) with a upper lid margin-limbus distance ranging from 1 to 7 mm and evaluated using strict criteria., Results: A perfect or acceptable result was obtained in 23 of 28 patients (82%) with bilateral retraction and in 18 of 22 patients (82%) with unilateral retraction. Seven eyelids were overcorrected (too low) and three undercorrected, necessitating reoperation. All other eyelids had an almond-like contour and a lid crease of 10 mm or less. No complications except subcutaneous haematomas were seen. Two patients showed a recurrence of lid retraction 9 months after the operation., Conclusion: This technique is safe and efficacious and can be used for all degrees of eyelid retraction.

Aims: To investigate the prevalence and to discuss the necessity of treating elevated intraocular pressures (IOP) in patients with Graves' orbitopathy (GO). In addition, to study the effects of orbital decompression and extraocular muscle surgery on IOP., Methods: The records of consecutive patients with GO referred in a 5 year period were studied and those selected, in which glaucoma medication had been prescribed, or a diagnosis of primary open angle glaucoma (POAG) or of ocular hypertension (> or = 22 mm Hg) (OH) had been made. The necessity of treating these patients with glaucoma medication was questioned and the effects of corticosteroids, orbital decompression, and extraocular muscle surgery on the IOP were evaluated., Results: Of 482 patients with GO, 23 (4.8%) met the inclusion criteria. Four patients (0.8%) had POAG, four had elevated IOPs and visual field defects consistent with dysthyroid optic neuropathy, and 15 (3.1%) had only elevated IOPs. Five patients with OH showed a permanent drop of IOP after orbital decompression, two had a marked decrease of their IOP after recession of the inferior rectus muscle., Conclusions: POAG has the same prevalence in the general Dutch population as in the GO subgroup. The combination of elevated IOPs and visual field defects in GO patients may be attributed to other mechanisms than obstructed aqueous outflow in the trabecular meshwork and should be treated accordingly. Orbital decompression and extraocular muscle surgery may lower the IOP in patients with GO.

Purpose: Firstly, to assess the incidence of induced diplopia following orbital decompression in patients with Graves' orbitopathy. Secondly, to assess patient satisfaction after orbital decompression. Thirdly, to determine the factors that contribute to the variable reported incidence of diplopia complicating decompression surgery., Methods: We present a retrospective analysis of the alterations of ocular motility in a consecutive series of 81 patients with Graves' orbitopathy who underwent orbital decompression by either a coronal or a translid approach. We assessed patient satisfaction by a telephone survey, and we reviewed the literature., Results: Eleven patients underwent decompressive surgery for dysthyroid optic neuropathy (DON); 5 of them had a three-wall coronal decompression, the other 6 had a two-wall translid decompression. One of the 5 (20%) coronal versus 2 of the 6 (33%) traslid patients experienced worsening of their existing diplopia. Seventy patients underwent surgery for disfiguring proptosis; 41 of them had a coronal decompression and 29 had a translid decompression. Eight of the 41 coronal patients (20%) and 4 of the 29 translid patients (14%) experienced aggravation of their motility impairment. There was no statistically significant difference between these percentages (chi-squared, p > 0.05). Three of 26 coronal patients (12%) without pre-operative motility impairment developed diplopia in all directions. Twenty-five per cent needed strabismus surgery (9% multiple times). High satisfaction scores were noted after both types of orbital decompression. Through a review of the literature, several factors that may add to heterogeneous results were identified, including definition of diplopia, inclusion criteria and type of surgery., Conclusions: Induced diplopia is seen after any type of orbital decompression (19% overall), and its incidence is determined by various factors. To facilitate comparative studies between decompression techniques, a standardised protocol for orthoptic evaluation should be developed.

Aims: To present three patients with a syringomatous carcinoma (SC). SC is a rare cutaneous neoplasm, most frequently situated on the face and scalp and histologically characterised by an infiltrative pattern of basaloid or squamous cells, a desmoplastic stromal reaction, keratin filled cysts, and granular structures., Methods: The clinical histories of the patients with a SC were investigated retrospectively., Results: Patient 1 had a benign appearing tumour of the lower eyelid. Five tumour excisions were necessary to remove the SC completely. Patient 2 had a tumour on the lateral part of the lower eyelid and in the medial canthal area. The histopathological findings revealed a squamous cell carcinoma, later revised as a SC. In spite of two excisions and one microscopically controlled excision, a recurrence occurred. An exenteration orbitae was recommended. Patient 3, known to have a history of multiple malignant skin tumours after kidney transplantation and use of cyclosporin, presented with a firm mass in the eyebrow region and in the nasal area of the orbit. The pathological diagnosis of this adnexal tumour was difficult. An exenteration was recommended., Conclusions: SC is a benign appearing but extremely invasive, locally destructive, slowly growing adnexal tumour, derived from eccrine sweat glands. It is often mistaken, both clinically and microscopically, for other benign and malignant entities. The tumour recurrence is high due to extensive perineural invasion, but regional or distant metastases are rare. The local aggressive nature of the tumour and the high recurrence rate may necessitate mutilating procedures. Optimal treatment consists of a complete microscopically controlled surgical excision with clear surgical margins.

Objective: Approximately 35% of patients with Graves' ophthalmopathy do not respond to immunosuppressive treatment. A possible explanation for this finding is that only patients with active ophthalmopathy respond to immunosuppressive treatment, whereas patients with fibrotic end stage disease do not. To distinguish between these two groups and to predict the outcome of immunosuppressive treatment, we developed a clinical activity score (CAS) based on four of the five classical signs of inflammation and tested its efficacy in a double-blind, prospective study., Design, Patients and Measurements: The CAS was determined by an opthalmologist before, on the day of, and after the start of either oral prednisone or retrobulbar irradiation in 43 patients with moderate to severe Graves' ophthalmopathy. The therapeutic outcome was determined by a second ophthalmologist unaware of the CAS stores given. Success of treatment was defined as an improvement in NOSPECS class or grade., Results: Responders (22) and non-responders (21) did not differ in age, sex, duration or severity of their Graves' ophthalmopathy. The pretreatment CAS, however, was significantly higher in responders than in non-responders. Twelve of 22 responders and three of 21 non-responders had a CAS > or = 4 (55% vs 14%; P < 0.01) [corrected]. Using this CAS cut-off point, the accuracy of CAS in predicting the therapeutic outcome was: specificity 86%, sensitivity 55%, positive predictive value 80%, negative predictive value 64%. Patients with a CAS > or = 4 had a similar duration of Graves' ophthalmopathy as patients with a CAS < 4., Conclusions: The clinical activity score has a high predictive value for the outcome of immunosuppressive treatment in Graves' ophthalmopathy. Disease activity, and not disease duration, is the prime determinant of therapeutic outcome.

The purpose of this retrospective study is to investigate the influence of 45 Gy megavoltage external beam radiotherapy on the occurrence of second primary tumours in hereditary- and non-hereditary retinoblastoma patients. Eighty-seven hereditary and 19 non-hereditary patients were irradiated for retinoblastoma. The follow-up of the hereditary patients ranged from 4-23 years (mean 12.4 years), of the non-hereditary patients from 6-23 years (mean 12 years). In the hereditary group 4 patients developed a second primary tumour (2 rhabdomyosarcoma, 1 osteosarcoma, 1 malignant histiocytoma), and 5 patients developed a pineoblastoma. Three second primary tumours were situated inside the radiation field. The latency period ranged from 1.5 to 18 years (mean 4.8 years). None of the non-hereditary patients developed a second primary tumour. The actuarially calculated probability of being free from second primary tumours was 96.9% at 10 years and 89.4% at 20 years (pineoblastoma excluded). The survival was 91.2% at 10 years and 84.2% at 20 years (pineoblastoma excluded). From this study it is suggested that external beam irradiation has a potentiating effect in patients, with the genetic predispopsition for retinoblastoma and who are more susceptible to second primary malignancies.

Aims: To determine the effectiveness and safety of three wall orbital decompression by the coronal approach in Graves' ophthalmopathy., Methods: The records of 125 patients with Graves' ophthalmopathy, who had undergone three wall orbital decompression by coronal approach between April 1984 and October 1993, were studied retrospectively. Special attention was paid to proptosis reduction, changes in ocular motility, and complications., Results: The preoperative Hertel values ranged from 15 to 30 mm (mean 22.43 mm). The mean proptosis reduction was 4.34 mm (range 0-10 mm). Proptosis reduction in patients with preoperative Hertel values higher than 27 mm was significantly more than in patients with preoperative values between 25 and 27 mm (p < 0.05). This last group showed significantly more proptosis reduction than patients with preoperative Hertel values of 23 and 24 mm (p < 0.01). Postoperatively, 3.2% of the patients showed new diplopia in primary and/or reading position. In 4% of the patients with normal ocular motility preoperatively, diplopia in the extreme directions of gaze developed. In 4% of the patients, preoperative motility disturbances decreased or disappeared postoperatively., Conclusion: Three wall orbital decompression by coronal approach is a safe and effective technique, to achieve proptosis reduction in patients with Graves' ophthalmopathy, with fewer complications than other techniques thus far described.

Aims/background: In Graves' ophthalmopathy the increase in volume of intraocular muscles and fat will cause elevated intraorbital pressure. In order to investigate the pressure levels involved, intraorbital pressure, or retrobulbar pressure (RBP) was measured continuously in orbits of patients with Graves' ophthalmopathy during surgical decompression., Methods: Retrobulbar pressure was measured before and during surgical decompression using an intraorbitally applied pressure transducer., Results: In eight patients with dysthyroid optic neuropathy (DON) RBPs between 17 and 40 mm Hg were recorded. At the end of the surgical procedure the mean RBP was reduced from 28.7 mm Hg to 18.7 mm Hg, the decrease ranging from 8 to 12 mm Hg, which showed a high correlation with the starting pressures (p < 0.001). In two cases without DON, pressures were 11 and 9 mm Hg. Forces exerted by spatula manipulation usually resulted in a RBP level of more than 70 mm Hg., Conclusions: This study shows that RBPs are markedly elevated in Graves' ophthalmopathy and that surgical decompression can result in a significant reduction in the intraorbital pressure. Optic nerve dysfunction in Graves' ophthalmopathy may not be caused exclusively by the direct pressure of swollen extraocular muscles upon the optic nerve, but also by a raised RBP. It is hypothesised that the damage inflicted upon the optic nerve can be caused in consequence by RBP induced incarceration of the nerve, compressed by surrounding periosteal lined orbital fat bulging posteriorly into the entrance of the optic canal.

Purpose: The late side effects of external beam irradiation in patients with retinoblastoma such as orbital bony growth retardation, are a serious problem in adolescence. Therefore, a quantitative study was performed to investigate the late effects of irradiation on orbital growth in patients with retinoblastoma., Methods: The orbits of 68 patients with retinoblastoma, 52 bilateral and 16 unilateral, were divided into two treatment groups: radiotherapy alone, 77 orbits; and radiotherapy + enucleation, 43 orbits. Follow-up time was 12 to 240 months (mean, 95 months) in group 1 and 27 to 216 months (mean, 97 months) in group 2. These groups were subdivided further into age groups at which radiotherapy was given. The morphometric measurements of these groups were compared., Results: The authors showed that irradiation causes a significant growth retardation when compared with the growth of nonirradiated orbits (P<0.001). They also demonstrated that radiotherapy in children younger than 6 months of age is more damaging to the orbital growth than at an older age (P<0.01). Finally, the authors showed that secondary enucleation does not have an additive growth-retarding effect., Conclusion: Orbital growth retardation in patients with retinoblastoma after radiotherapy is influenced mainly by the age at which irradiation is given and is defined at 6 months. Theoretically, it would be desirable to postpone irradiation in children until they are older than 6 months of age if possible. The irradiation effect on these orbits is not enhanced by enucleation.

Background: The current grading of class IV (extraocular muscle involvement) of the NO SPECS classification for Graves ophthalmopathy is subjective and, in practice, virtually limited to three grades (grade c rarely occurs). The subjectiveness results in a high degree of observer disagreement, as illustrated by kappa values of 0.48 to 0.64 for the agreement between the authors in grading of class IV changes in 13 patients., Methods: The authors investigated the significance of a quantitative approach in assessing eye motility and modified a hand perimeter to measure the monocular eye movements in degrees., Results: The mean maximal excursions in the four main directions of gaze, obtained with this device in 40 healthy control subjects (5 men and 5 women from each decade between 20 and 60 years of age), were as follows: elevation = 34 degrees, depression = 58 degrees, adduction = 48 degrees, and abduction = 46 degrees. The ductions showed an age-related decline. The intraobserver and interobserver variations were assessed in 12 and 18 patients with Graves ophthalmopathy, respectively, by calculating the coefficients of repeatability, which varied from 5 degrees to 8 degrees. The authors compared the current grading of NO SPECS class IV with quantitative measurements of elevation in 11 patients by calculating the kappa values for agreement. These were found to be 0.57 and 0.80, respectively., Conclusions: The quantitative measurement of eye movements with a modified perimeter appears to be precise and reproducible. In addition, it should replace the current grading of the extraocular muscle movements of Graves ophthalmopathy patients in the NO SPECS classification, which is less reliable, as evidenced by a higher degree of observer disagreement.

Contrast sensitivity function was measured by a computer automated method on 38 eyes with dysthyroid optic neuropathy and 34 eyes with Graves' ophthalmopathy only. The results were compared with 74 healthy control eyes. Disturbances of contrast sensitivity functions were found in both groups when compared with controls. The eyes affected with dysthyroid optic neuropathy showed pronounced loss of contrast sensitivity in the low frequency range, which facilitates differentiation between the two groups.

Corticosteroids are usually given for management of Graves' ophthalmopathy, but they have many and serious side-effects. By comparison, retrobulbar irradiation is well tolerated, although its efficacy has been evaluated only in uncontrolled studies. Therefore, we did a double-blind randomised trial, in which 28 patients with moderately severe Graves' ophthalmopathy were treated with a 3-month course of oral prednisone and sham irradiation, and 28 received retrobulbar irradiation (20 Gy) and placebo capsules. Therapeutic outcome, assessed twenty-four weeks after the start of treatment, was determined by the change in the highest NOSPECS class. A successful outcome was observed in 14 prednisone-treated and in 13 irradiated patients. Responders to treatment (but not nonresponders) in both groups showed improvements in total and subjective eye score and a decrease in eye-muscle volume. Response to either treatment was due largely to changes in soft-tissue involvement and eye-muscle motility. Mean elevation in responders to radiotherapy increased from 18.5 degrees (95% CI 14.8-22.2) at baseline to 21.8 degrees (18.6-25.0) at week twenty-four (p = 0.003), but did not change in prednisone responders. Side-effects were more frequent and severe during prednisone than during radiotherapy. Radiotherapy and oral prednisone appear to be equally effective as initial treatment in patients with moderately severe Graves' ophthalmopathy. In view of its better tolerability, radiotherapy should be considered the treatment of first choice.

Purpose: Clinical data indicate that extraocular muscles have different susceptibilities for some orbital immune disorders depending on their anatomic location. The resident immunocompetent cells may be important mediators in the local pathogenesis of such disorders so the distribution of these cells was studied in extraocular muscles obtained from normal human donors. For comparison skeletal muscles were studied., Methods: The cell distributions were analyzed quantitatively in cryostat cross-sections subjected to a two-step immunoperoxidase method using monoclonal antibodies against T cells, B cells, macrophages and several other markers for cell differentiation or activation. The macrophage distribution was analyzed in more detail using on-line semiautomatic image analysis equipment (VIDAS, Kontron, Elektronik GmbH, Eching, Germany)., Results: Extraocular muscles contain numerous macrophages, fewer human leukocyte antigenD-related (HLA-DR) positive cells and T cells, whereas B cells are absent. The numeric density of all cell types, and macrophages in particular, is much higher in extraocular muscles than in skeletal muscles. In extraocular muscles the majority of T cells are positive for the CD8 antigen (suppressor/cytotoxic), in skeletal muscle CD4 positive T cells (helper) predominate., Conclusions: Extraocular muscles contain many more CD8-positive cells and macrophages per square millimeter than skeletal muscles. Of all the cell types studied, only the macrophage distribution differs significantly among the normal extraocular muscles: the medial and inferior recti muscles contain about twice as many macrophages as the lateral rectus and superior oblique muscles. Their mean sizes (area) or shape distributions however, appear to be similar.

Purpose: To determine whether the internal reflectivity of the eye muscles on A-mode ultrasonography serves as indicator for disease activity in Graves ophthalmopathy, thereby predicting response to immunosuppressive treatment., Methods: Eye muscle reflectivity, expressed as percentage of the initial scleral spike, was measured in the superior, medial, and lateral recti, and normal values were determined in 23 healthy controls. These were compared with values in 16 consecutive patients with untreated, moderately severe Graves ophthalmopathy. The accuracy of the measurement was assessed by calculating the intra- and interobserver coefficient of repeatability. Baseline eye muscle reflectivity in the 16 patients was correlated to the therapeutic outcome 24 weeks after start of immunosuppressive therapy. The response to treatment served as indicator for pretreatment disease activity., Results: Eye muscle reflectivity could be measured reliably, with an intraobserver coefficient of repeatability of 8.2% in controls and 10.2% in patients. Patients had lower reflectivity than controls (54.6 +/- 17.4% versus 70.7 +/- 6.6%; P < 0.001). Reflectivity in eye muscles with the lowest echogenicity was lower in responders than in nonresponders (25.8 +/- 8.4% versus 40.6 +/- 13.6%; P = 0.02). From the individual data, a cut-off value of 40% was derived, which appeared as an accurate predictor of outcome (positive predictive value 73%; 95% confidence interval 39%-94%)., Conclusions: Measuring eye muscle reflectivity in Graves ophthalmopathy appears to be a reliable new method to determine disease activity, with a promising accuracy in predicting therapeutic outcome of immunosuppressive treatment.

Diagnosing and monitoring of Graves' Orbitopathy (GO) can be supported by use of Ultrasonography (USG) and Computerized Tomography (CT); they provide supplementary information. In this retrospective study we describe 107 clinical GO patients evaluated by B-scan USG and 27 clinical GO patients evaluated by CT scan. Analysis of 236 B-scan USG included measurements of medial, inferior and lateral rectus muscles. The presence of muscle enlargement and increased orbital fat were noted by the radiologist on 27 CT scans. Sensitivity of both B-scan USG and CT scan were calculated. We suggest that B-scan USG has a high sensitivity, which is equal or better than CT scan sensitivity in diagnosing GO. Furthermore USG A and B scan combination is an effective, accurate tool in diagnosing GO, and optic neuropathy, but it also provides essential information about the GO disease activity.

In various autoimmune diseases circulating levels of soluble IL-2 receptor (sIL-2R) seem to be related to disease activity. Because reliable parameters of disease activity in Graves' ophthalmopathy are lacking, we measured sIL-2R levels in 47 patients with this disorder. The patients had Graves' disease, but no other immune-mediated diseases, had not yet received specific treatment for their ophthalmopathy and were euthyroid during the entire study period. Twenty-one of the 47 patients (45%) had sIL-2R values above the upper normal limit of 650 U/ml, as established in 20 healthy controls. There were no differences between patients with normal (median 469, range 280-644 U/ml) and elevated (median 946, range 678-1588 U/ml) sIL-2R levels regarding duration or severity of the eye disease (as assessed clinically from the total eye score). However, patients with severely enlarged eye muscles had higher sIL-2R values than patients with less severely enlarged eye muscles on CT scan. Patients with elevated sIL-2R tended to have a higher response rate (71%) to a 3-month course of prednisone, than those with normal levels (46%; P = 0.081). Since a successful outcome of prednisone treatment might be representative for disease activity, the elevated sIL-2R levels seem to reflect active inflammation. Although the practical relevance of this finding in individual patients is limited, it underscores the importance of cell-mediated immune responses in this thyroid-related eye disease.

Classification of the eye changes of Graves' disease may have clinical use in the description of the present eye state, in the assessment of treatment results, and in the choice of therapy. Requirements for any classification system should include simplicity, clinical nature (i.e., easily carried out by any physician equipped with ordinary noninvasive techniques), reproducibility and meaningfulness (i.e., objective and of clinical relevance to the patient). The NOSPECS system (although it can be improved) is suitable for pure description. It is not very helpful in the assessment of treatment results. Therapeutic outcome probably is best described by measuring quantitatively changes in visual acuity, eye muscle motility, and proptosis. Such an approach, however, does not solve the problem of whether or not the overall outcome is a success in the patient with improvement in one aspect but worsening in another item. A recently developed clinical activity score may be helpful in the selection of therapy, especially for deciding whether or not immunosuppressive therapy should be instituted.