A pan-inflammatory microRNA-cluster is associated with orbital non-Hodgkin lymphoma and idiopathic orbital inflammation.

Non-Hodgkin orbital lymphoma (NHOL) and idiopathic orbital inflammation (IOI) are common orbital conditions with largely unknown pathophysiology that can be difficult to diagnose. In this study we aim to identify serum miRNAs associated with NHOL and IOI. We performed OpenArray® miRNA profiling in 33 patients and controls. Differentially expressed miRNAs were technically validated across technology platforms and replicated in an additional cohort of 32 patients and controls. We identified and independently validated a serum miRNA profile of NHOL that was remarkably similar to IOI and characterized by an increased expression of a cluster of eight miRNAs. Pathway enrichment analysis indicated that the miRNA-cluster is associated with immune-mediated pathways, which we supported by demonstrating the elevated expression of this cluster in serum of patients with other inflammatory conditions. The cluster contained miR-148a, a key driver of B-cell tolerance, and miR-365 that correlated with serum IgG and IgM concentrations. In addition, miR-29a and miR-223 were associated with blood lymphocyte and neutrophil populations, respectively. NHOL and IOI are characterized by an abnormal serum miRNA-cluster associated with immune pathway activation and linked to B cell and neutrophil dysfunction.

Natural Course of Mild Graves Orbitopathy: Increase of Orbital Fat But Decrease of Muscle Volume With Increased Muscle Fatty Degeneration During a 4-Year Follow-Up.

PURPOSE: To describe the natural course of orbital fat volume and extraocular muscle volume in mild Graves orbitopathy during a 4-year follow-up. To describe fatty changes within the extraocular muscles. PATIENTS: Twenty-five patients with mild Graves orbitopathy, who did not require any therapeutic intervention other than supportive therapy, were followed for 4 years. METHODS: CT scans were performed in all patients at baseline and follow-up. A validated technique using Mimics (Materialise) was used to calculate fat and muscle volumes. Outcomes were compared with previously collected data. The amount of intramuscular fat was assessed on CT scans in a semi-quantitative way by two blinded observers according to a four-point scale. RESULTS: After a median follow-up of 4.3 years, the median fat to orbital volume ratio increased with 0.08 from 0.57 to 0.65 (p = 0.000), whereas the median muscle volume to orbital volume ratio decreased with 0.03 from 0.17 to 0.14 (p = 0.000). In this control group in patients between 49 and 54 years old, the changes were 0.01 and -0.002, respectively. The Clinical Activity Score decreased to zero (p = 0.000), and the median eyelid aperture decreased from 12 to 10 mm (p = 0.007). A significant increase of intramuscular fat was found in patients with Graves orbitopathy. CONCLUSIONS: The natural course of mild Graves orbitopathy, as observed over 4 years, is characterized by an increase of orbital fat volume, a decrease in muscle volume, and an increased intramuscular fatty degeneration.

Impact of superior ophthalmic vein thrombosis: a case series and literature review.

Purpose: To present nine new cases of superior ophthalmic vein thrombosis (SOVT) and compare these with the literature, and to assess the impact of SOVT for the clinician. Methods: Using the data bases of the Department of Ophthalmology of the AMC, we searched for patients with radiologically evidenced SOVT between January 2006 and December 2014. In addition, a PubMed search, using the mesh term 'superior ophthalmic vein thrombosis', was done. Results: We found nine patients with SOVT. In three patients, SOVT was related to dural arteriovenous fistulae. In one patient, it was caused by the acute reversal of warfarin by vitamin K. In two patients, an infectious cause was found. In three patients, the cause of SOVT was not found despite screening for coagulation and other disorders. All patients presented with eyelid swelling, proptosis, and/or motility impairment. We found complete recovery in four patients. Three patients had mild sequelae and two patients had severe visual impairment. In the literature, we found 60 cases reporting on SOVT with various aetiologies. Clinical presentation, treatment modalities, and outcomes were comparable to our findings. Conclusion: Our case series and literature review show that SOVT can occur simultaneously with cavernous sinus thrombosis (CST) but can also be a separate entity. Clinical presentation can mimic orbital cellulitis (OC) or CST and when no signs of OC can be found, an alternative cause for SOVT should be sought. When timely and adequate treatment is conducted, the prognosis is predominantly favourable.

Prognosis of septic cavernous sinus thrombosis remarkably improved: a case series of 12 patients and literature review.

PURPOSE: Septic cavernous sinus thrombosis (CST) is a rare complication of infections in the head and neck area. CST is notorious for its bad prognosis, with high mortality and morbidity rates described in literature. However, these rates are based on old series. We question whether the prognosis of CST is currently still as devastating. The primary purpose of this study is to assess the mortality and morbidity of CST. METHODS: Using the databases of all relevant specialties in our tertiary referral hospital, we collected all the patients treated for CST in the period 2005-2017. In addition, a PubMed search, using the mesh term 'cavernous sinus thrombosis', was performed. RESULTS: We found 12 patients with CST in the study period. Of the 12 patients, 11 survived and 9 recovered without any permanent deficits. Seven patients were treated with anticoagulation, and in none of the patients we saw hemorrhagic complications. In literature, older articles describe higher mortality rates (14-80%), but more recent articles report mortality and morbidity rates similar to our results. CONCLUSIONS: The prognosis of CST nowadays is more favorable than previously described. Anticoagulation seems to be a safe addition to antibiotic and surgical treatment, at least in patients without central nervous system infection.

Vernal shield ulcers treated with frequently installed topical cyclosporine 0.05% eyedrops.

PURPOSE: To report on the beneficial results of an intense regimen of 0.05% cyclosporine eye drops, eight times a day in patients with therapy resistant vernal shield ulcers. METHODS: Case cohort of four eyes of three male children with vernal keratoconjunctivitis complicated by shield ulcers, who were treated with frequent cyclosporine 0.05% eye drops and observed for up to 5 years. RESULTS: Quick resolution of the shield ulcers and complete re-epithelialization within 14-25 days was observed after adding intensive treatment with cyclosporine 0.05% to regular anti-inflammatory, histamine blocking, and surgical therapy. In one patient, additional scraping of the bottom of the ulcer was needed. CONCLUSIONS: In patients with vernal shield ulcers, frequent installation of low-concentration cyclosporine eye drops seems to have a promising therapeutical value.

Topical bevacizumab treatment in aniridia.

PURPOSE: To report the results of long-term topical treatment with bevacizumab (Avastin) 5 mg/mL eyedrops in a case of aniridia-related neovacularization of the cornea. METHODS: Interventional case report. A female patient with aniridia had a decrease in the best corrected visual acuity from 0.32 to 0.02 in the OS over the course of 4 years, secondary to central corneal neovascularization and epithelial breakdown. Vision in the OD was 0.2. In 2008, at age 28, a shared decision was made to start off-label treatment with bevacizumab eyedrops 0.5% in both eyes. After 9 years, the visual acuity in the OD remained stable, with stability of the macropannus and maintenance of central corneal clarity. In the OS, the central corneal neovascularization regressed, the epithelium regained its clarity, and after cataract surgery visual acuity was regained to 0.32. RESULTS: After 9 years of treatment with topical bevacizumab, vision acuity is comparable to the situation of 12 years previously: Visual acuity remained stable in the ODS. CONCLUSION: In a young patient with progressive corneal neovascularization secondary to aniridia, stability of central corneal neovascularization was obtained and corneal clarity was preserved by adding a daily drop of bevacizumab 5 mg/mL. No adverse events occurred. Vessel growth was inhibited, and as such, the progression of the natural history of the patient's disease was halted. More clinical study with longer follow-up is needed to investigate the applicability of treatment with topical VEGF inhibitors in aniridia.

True abscess formation is rare in bacterial orbital cellulitis; consequences for treatment.

BACKGROUND: Pre- or retroseptal bacterial orbital cellulitis (pOC/rOC) is not an uncommon orbital disease. Treatment consists of antibiotics with or without surgical drainage. Several questions regarding course, complications and outcome of treatment are unanswered and the indication for surgery is not well defined. The aim of this study is to: 1. describe the outcome of orbital cellulitis (OC) in a large cohort, 2. assess the significance of Chandler's classification, 3. assess the incidence of abscess formation in OC, and 4. redefine criteria for surgery. METHODS: Retrospective case series of patients with OC seen between 1-1-2007 and 1-1-2014 in a tertiary referral center. RESULTS: Sixty-eight patients presented with (presumed) bacterial pOC. Two out of these 68 developed rOC. All 68 patients had a full recovery. Forty-eight patients presented with rOC. Four out of 48 (8%) had intracranial extension of the infection at the time of admission. No admitted patient developed distant seeding. Only four (8%) patients with rOC had a true orbital abscess. In the other 92% we found a diffuse orbital inflammation or a subperiosteal empyema. Forty-four (92%) patients with rOC had a full recovery. CONCLUSIONS: 1. The prognosis of both pOC and rOC nowadays is generally favorable. 2. Chandler's classification is of little use. 3. True abscess formation in OC is rare. 4. The indication for surgical intervention must be based on the clinical presentation and the assessment of true orbital abscess formation.

LASIK flap breakthrough in Nd:YAG laser treatment of epithelial ingrowth.

PURPOSE: To present two cases with complications after Nd:YAG laser treatment of epithelial ingrowth. METHODS: Case reports. RESULTS: Dense central recurrent epithelial ingrowth was treated with a Nd:YAG laser directed at the epithelial nests in the LASIK flap interface in one case. Misalignment of the aiming beam after movement resulted in perforation of the LASIK flap, followed by renewed epithelial ingrowth through the new defect. The epithelial ingrowth receded and became more translucent as a result of the treatment, but the area of the perforation remained irregular. In another case, use of the Nd:YAG laser to treat recurrent epithelial ingrowth adjacent to the flap edge created a cavitation bubble that broke through the flap edge, creating a new epithelial channel through which ingrowth recurred. CONCLUSIONS: Surface breakthrough and renewed epithelial ingrowth is a possible complication of Nd:YAG laser treatment of epithelial ingrowth.

Outcome of inferior and superior rectus recession in Graves' orbitopathy patients.

PURPOSE: To evaluate the surgical effect of unilateral inferior rectus recession (IR-group) with or without a recession of contralateral superior rectus (IR-SR-group) on squint angle and motility restrictions in Graves' Orbitopathy (GO) patients. DESIGN: Retrospective case series. MATERIALS AND METHODS: Primary outcome parameters were the changes of squint angle 3 months and 6-12 months postoperatively. As in a previous study, success was defined as a postoperative vertical squint angle of ≤3° in primary position and on downgaze. Secondary outcome parameters were the influence of surgery on duction range and influence of muscle size on dose-effect response. RESULTS: Fifty-six patients were included in the study; 31 patients in the IR-group and 25 patients in the IR-SR-group. The amount of (fixed suture) recession ranged from 2 mm to 7 mm. Vertical deviations in primary position changed from 8.0° [95% CI 6.6-9.7°] to 1.0° [95% CI -0.4-6.5°] in the IR-group and from 17.0° [95% CI 15.7-20.0°] to 1.5° [95% CI 0.8-2.9°] in the IR-SR-group. The success rate was 74% in the IR-group and 64% in the IR-SR-group. Elevation significantly improved in both groups (IR-group p = 0.007; IR-SR- group p = 0.000). The volume of vertical rectus muscles as assessed on CT-scans did not influence the dose-effect response. CONCLUSIONS: The highest success rate and highest reduction of depression was found in the IR-group. The total duction range remained stable after strabismus surgery (IR-group) or improved (IR-SR-group). Both squint angle and cyclodeviation remained stable during long time follow-up (6-12 months after surgery).

Selenium and the course of mild Graves' orbitopathy.

BACKGROUND: Oxygen free radicals and cytokines play a pathogenic role in Graves' orbitopathy. METHODS: We carried out a randomized, double-blind, placebo-controlled trial to determine the effect of selenium (an antioxidant agent) or pentoxifylline (an antiinflammatory agent) in 159 patients with mild Graves' orbitopathy. The patients were given selenium (100 µg twice daily), pentoxifylline (600 mg twice daily), or placebo (twice daily) orally for 6 months and were then followed for 6 months after treatment was withdrawn. Primary outcomes at 6 months were evaluated by means of an overall ophthalmic assessment, conducted by an ophthalmologist who was unaware of the treatment assignments, and a Graves' orbitopathy-specific quality-of-life questionnaire, completed by the patient. Secondary outcomes were evaluated with the use of a Clinical Activity Score and a diplopia score. RESULTS: At the 6-month evaluation, treatment with selenium, but not with pentoxifylline, was associated with an improved quality of life (P<0.001) and less eye involvement (P=0.01) and slowed the progression of Graves' orbitopathy (P=0.01), as compared with placebo. The Clinical Activity Score decreased in all groups, but the change was significantly greater in the selenium-treated patients. Exploratory evaluations at 12 months confirmed the results seen at 6 months. Two patients assigned to placebo and one assigned to pentoxifylline required immunosuppressive therapy for deterioration in their condition. No adverse events were evident with selenium, whereas pentoxifylline was associated with frequent gastrointestinal problems. CONCLUSIONS: Selenium administration significantly improved quality of life, reduced ocular involvement, and slowed progression of the disease in patients with mild Graves' orbitopathy. (Funded by the University of Pisa and the Italian Ministry for Education, University and Research; EUGOGO Netherlands Trial Register number, NTR524.).

Indications for and outcomes of tertiary referrals in refractive surgery.

PURPOSE: To review the spectrum of disease, symptomatology, and management offered to patients referred for a second opinion after refractive surgery. METHODS: A prospective cohort study was done on all patients referred from October 1, 2006, to September 30, 2011, to a tertiary eye clinic after refractive surgery of any kind (ie, corneal laser surgery, conductive keratoplasty, radial keratotomy, phakic implants, refractive lens exchanges, or any combination thereof). Data analysis was performed on all demographic and clinical aspects of this cohort, including the initial complaint, type of referral, number of complaints, procedure previously performed, diagnosis at our center, type of advice given, and rate and type of surgical intervention. RESULTS: One hundred thirty-one eyes (69 patients) were included. Corneal refractive surgery was performed in 82% (108 eyes), and 11% (14 eyes) were seen after phakic intraocular lens (PIOL) implantation and 7% (9 eyes) after refractive lens exchange. The most common diagnoses were tear film dysfunction (30 eyes, 23%), residual refractive error (25 eyes, 19%), and cataract (20 eyes, 15%). Most patients (42 patients, 61%) were treated conservatively. In 27 patients (39%), 36 eyes (28%) were managed surgically. Severe visual loss was seen in 1 eye. CONCLUSIONS: No major problems were found in most second opinions after refractive surgery referral. Dry eyes, small residual refractive error, or higher-order aberrations were the most common complaints. Surgical intervention was needed in 36 eyes (28%), almost half of which were cataract extractions. Severe visual loss was seen in 1 eye with a PIOL. There was no incidence of severe visual loss in keratorefractive and refractive lens exchange procedures.

What is the aim of immunosuppressive treatment in patients with graves' orbitopathy?

PURPOSE: The EUGOGO criteria and the clinical activity score (CAS) have been used as outcome measures to report response rates for patients after pulsed intravenous methylprednisolone (PIM) therapy. This study compares the results after PIM for both criteria and evaluates the number of rehabilitative surgeries performed in relation to treatment outcome. METHODS: This was a retrospective review of patients with active moderate-to-severe Graves' orbitopathy (GO) treated with PIM (cumulative dose of 4.5 to 5 g). The EUGOGO criteria or improvement in CAS (≥2 points) was used as the primary outcome measure. Baseline characteristics were examined to evaluate any determinants of treatment response. Additional immunosuppressive therapy after PIM and rehabilitative surgical procedures for all patients within a 2-year period were also recorded. RESULTS: Thirty-two patients were identified. Using the EUGOGO criteria, an improvement was seen in 38%, no change (stabilization) in 47%, and worsening of the disease in 15%. The response rate using the CAS was 63%. According to the EUGOGO criteria and the CAS, 20% (4/20) and 33% (4/12) of nonresponders required additional immunosuppressive treatment after PIM. None of the responders required additional immunosuppressive therapy. There was a reduction of 0.5 surgeries per patient for responders using either outcome measures. CONCLUSIONS: In this study, PIM alone stabilizes active GO in 85% and reduces the severity of GO in 38% of the patients. Despite incongruent response rates obtained using the EUGOGO criteria and the CAS, both outcome measures were good predictors for additional immunosuppressive treatment and additional rehabilitative surgeries in nonresponders.

Comparison of a hydrophilic and a hydrophobic apodized diffractive multifocal intraocular lens.

UNLABELLED: To compare outcomes between a new design apodized diffractive hydrophilic multifocal intraocular lens (IOL) (Seelens MF; study group), and a well-known apodized diffractive hydrophobic multifocal IOL (SN6AD1; control group). A comparative case series comparing refractive and visual outcomes at distance and near. Patient satisfaction with a validated questionnaire, dysphotopsia and straylight measurement scores were recorded at 3 months post-operatively. The study group comprised 48 eyes and the control group 37 eyes. At 3 months post-operatively the mean uncorrected distance visual acuity (UDVA) was not statistically significant different between the study group and the control group (0.02 ± 0.07 logMAR [SD] vs 0.04 ± 0.09 logMAR). Corrected distance visual acuity (CDVA) was statistically significantly better with the study lens (-0.04 ± 0.05 logMAR vs -0.01 ± 0.04 logMAR (p < 0.019). There was no clinical or statistical significant difference at the 40 cm distance (0.09 ± 0.12 logMAR vs 0.08 ± 0.09 logMAR). The study group had statistically significant better uncorrected near acuity at 50 and 60 cm distances (p < 0.03 and p < 0.007, respectively). In terms of satisfaction the lenses performed equally. Halos were seen less often with the study lens. Straylight, as a parameter for visual quality, was significantly less with the study lens. CONCLUSION: The Seelens MF performs equally as well as the well-known SN6AD1 for UCDA and CDVA. The Seelens MF performs better at intermediate distance, and seems to allow for better depth of focus, and increased visual quality. More study is needed to corroborate the last finding.

Intraocular pressure after descemet stripping endothelial keratoplasty (DSEK).

To assess the incidence of elevated intraocular pressure (IOP) after descemet stripping endothelial keratoplasty (DSEK). Retrospective case series. From the start of the technique in our hospital in 2003 until the middle of 2010, 73 eyes underwent DSEK, of which 66 (90.5 %) had a clear graft 1 year after surgery. IOP data pre- and postoperatively were analyzed. Follow-up was 1-5 years (mean 3.8, SD 1.3). Mean age was 71 years (SD 11, range 43-89). Three eyes (4.5 %) had a history of glaucoma before DSEK. Mean pre-operative IOP was 12.4 mmHg (SD ± 4.3). Mean IOP 1 day post-operatively was 16.4 mmHg (±9.2). Mean IOP after 1 and 6 months, and 1, 3, and 5 years were, respectively, 13.9 (±5.3), 14.7 (±5.6), 14.2 (±5.4), 13.8 (±3.7), and 12.6 (±3.5). Seventeen percent (11 eyes) had a postoperative rise in IOP which needed medical or surgical intervention: 8 % (5 eyes) had a rise in IOP 1 day after DSEK, of which 1 underwent a trabeculectomy 7 months after DSEK surgery; 6 % (4 eyes) had a rise in IOP later in the postoperative period, which could all be managed medically; and 3 % (2 eyes) had increased IOP 1 day after DSEK and later in the postoperative period, both eyes underwent a trabeculectomy 5 and 24 months, respectively, after DSEK surgery. An increase in IOP was encountered in 17 % of DSEK patients. Checking the IOP in the short and long postoperative period is necessary.

Effect of Nd:YAG laser capsulotomy on refraction in multifocal apodized diffractive pseudophakia.

PURPOSE: To evaluate the effect on refraction of neodymium:YAG (Nd:YAG) laser posterior capsulotomy for posterior capsule opacification (PCO), and to evaluate the correlation between automated and subjective refraction in multifocal apodized diffractive pseudophakia. METHODS: A retrospective study of 75 pseudophakic eyes (50 patients) with multifocal apodized diffractive pseudophakia, treated for PCO with Nd:YAG laser posterior capsulotomy, was performed. Pre- and postintervention values of refractive and visual parameters were compared. The outcomes of autorefraction and subjective refraction were also compared. RESULTS: Uncorrected and corrected distance visual acuity improved significantly after Nd:YAG capsulotomy (P<.001). No significant changes were noted in defocus equivalent, astigmatic power vectors J(0) and J(45), and overall blurring strength in subjective refraction and autorefraction. Spherical equivalent changed significantly in autorefraction (P=.008), but not in subjective refraction. Autorefraction and subjective refraction were highly correlated in spherical equivalent, defocus equivalent, and blurring strength (r(2)>0.59). In approximately 7% of eyes, a change of more than 0.50 diopters in spherical equivalent in subjective refraction occurred. CONCLUSIONS: In most cases, Nd:YAG laser capsulotomy in patients with multifocal pseudophakia did not result in a change in refraction. However, 7% of eyes experienced a significant change in subjective refraction. Autorefraction correlated well with subjective refraction in apodized diffractive multifocal IOLs.

A short history of contemporary oculoplastic surgery (and the need for RCTs): excerpts from the Mustardé lecture 2011.

A short review of some contemporary developments in oculoplastic surgery (OPS), in particular of lacimal surgery, of orbital implants and of Graves' orbitopathy, discloses the influence of different sorts of research and underlines (according to the author) the need for (more) randomized clinical trials in nowadays OPS.

Evaluation of classification systems for nonspecific idiopathic orbital inflammation.

PURPOSE: To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. METHODS: A systematic literature search was conducted to find all studies that described and applied a classification system for IOI. Classification categories used in more than two studies were extracted, and criteria for these categories were defined using common descriptors. Using patient data, these newly defined classification systems were evaluated. Reliability was tested by inter- and intrarater agreement of two raters and distinction tested by evaluating clinical differences among classification categories. Feasibility, face validity, and content validity were qualitatively tested. RESULTS: The most frequently encountered IOI classification systems were based on onset (acute, chronic), histopathology (classic, granulomatous, sclerosing), or localization (diffuse, extraocular muscle, lacrimal gland, sclera, optic nerve). Systems based on histopathology and localization showed good reliability (κ values range 0.74-0.89), were easy to apply (feasibility), and described the biologic process (face validity). Because of static sampling, histopathology-based systems had moderate content validity and moderate distinction between classification categories. Being a static measure, localization had moderate content validity, but good distinction. It was found that content validity was improved by combining histopathology and localization into a two-dimensional classification system. CONCLUSIONS: This combined histopathology and localization-based classification system provides a repeatable, easy to use, plausible, and complete classification system that can be used to further advance the research of IOI.

Measured Corneal Astigmatism Versus Pseudophakic Predicted Refractive Astigmatism in Cataract Surgery Candidates.

PURPOSE: To compare standard and total corneal astigmatism measurements to the predicted pseudophakic (nontoric) refractive astigmatism in candidates for cataract surgery. DESIGN: A retrospective, cross-sectional study. METHODS: A single-center analysis of consecutive eyes measured with a swept-source optical coherence tomography biometer at a large tertiary medical center between February 2018 and June 2020. Corneal astigmatism was calculated based on standard keratometry astigmatism (KA), total corneal astigmatism (TCA), and predicted refractive astigmatism (PRA) for a monofocal nontoric intraocular lens (IOL) implantation calculated by the Barrett toric calculator using the predicted posterior corneal astigmatism (PRA(Predicted-PCA)) and the measured posterior corneal astigmatism (PRA(Measured-PCA)) options. Separate analyses were performed for each eye. SETTING: Ophthalmology Department, Shaare Zedek Medical Center, Jerusalem, Israel. RESULTS: In total, 8152 eyes of 5320 patients (4221 right eyes [OD] and 3931 left eyes [OS], mean age 70.6±12.2 years, 54.2% females) were included in the study. The mean vector values (centroid) for KA, TCA, PRA(Predicted-PCA), and PRA(Measured-PCA) were 0.07 diopters [D] at 19.5°, 0.27 D at 7.5°, 0.44 D at 2.9°, and 0.43 D at 179.3°, respectively (P < .01), for OD and 0.02 D at 150.3°, 0.23 D at 169.7°, 0.40 D at 179.4°, and 0.42 D at 169.5°, respectively (P < .01), for OS. More than 73% of eyes had a PRA >0.5 D. CONCLUSIONS: Standard and total corneal astigmatism measurements differ significantly from the PRA by the Barrett toric calculator. The PRA, rather than the KA or TCA, should be used as the reference guide for astigmatism correction with toric IOL implantation.

Do subtypes of graves' orbitopathy exist?

PURPOSE: To describe the prevalence of fat and muscle volume (MV) increase in Graves' orbitopathy (GO) patients, calculated from computed tomography scans, and the associated ophthalmic and endocrine characteristics. DESIGN: Consecutive, observational case series. PARTICIPANTS: Ninety-five consecutive Caucasian GO patients attending the thyroid eye clinic. METHODS: Volumetry using age-specific reference values in untreated GO patients who had been rendered euthyroid. MAIN OUTCOME MEASURES: Subgroups in GO and main characteristics. RESULTS: Four subgroups could be distinguished: Group 1, no fat volume (FV) or MV increase (n = 24); group 2, only FV increase (n = 5); group 3, only MV increase (n = 58); and group 4, both FV and MV increase (n = 8). Patients with an increase of MV were older and had higher thyroid-stimulating hormone-binding inhibitory immunoglobulin TBII, more proptosis, and more impaired ductions than those without MV increase. Patients with an increase of FV differed from those without FV increase only in having more proptosis. The clinical activity score did not differ between the 4 groups. CONCLUSIONS: Of these GO patients, 25% have orbital fat and MVs within an age-specific reference range. An increase of the FV is seen in only 14% of GO patients and characterized by proptosis. Muscle enlargement occurs in 70% of patients and is associated with older age, higher TBII values, more proptosis, and impaired motility.

Orbital mass as manifestation of Wegener's granulomatosis: an ophthalmologic diagnostic approach.

OBJECTIVES: Orbital manifestation of Wegener's granulomatosis is diverse and diagnosis is often difficult. This study aims to improve the diagnostic strategy in orbital Wegener. METHODS: A review of the diagnostic process in patients in whom a diagnosis of orbital WG was considered. RESULTS: Thirty-three patients were analysed, consisting of 15 patients with orbital WG, 11 with idiopathic orbital inflammation, 6 with orbital sarcoidosis and one with aspergillosis. Diagnostic findings indicating orbital WG were ear/nose/throat involvement, multiple organ system involvement, a positive ANCA, and on histology vasculitis, whereas granulomatous inflammation without signs of vasculitis was more indicative of another orbital disease. CONCLUSIONS: The diagnostic process of orbital WG should include CT scanning of the orbit and sinuses, ANCA blood testing, consultation of a rheumatologist, an ophthalmologist, and an ear-nose-throat specialist, and biopsy of an easily accessible, active inflammatory lesion.