Impact of duration of macula-off retinal detachment on visual outcome: a systematic review and meta-analysis of literature.

PURPOSE: To systematically review the influence of the lag time between macula-off retinal detachment and surgical intervention on postoperative visual acuity as main outcome measure. METHODS: Systematic review and meta-analysis of articles published from 1995 to October 2013 of patients with macula-off retinal detachment and treated with scleral buckling or pars plana vitrectomy. Eligible data were pooled in a meta-analysis, analyzing the odds ratio between different durations of ≤ 3, ≤ 4, ≤ 7, and ≤ 10 days, comparing a final visual acuity of ≤ 0.4 logMAR with >0.4 logMAR, using a random-effects model. Last, the number needed to treat was calculated. RESULTS: Fourteen articles were eligible, of which 9 studies contained data that were suitable for meta-analysis. Patients who were operated with scleral buckling (n = 602) within 3 days since macular detachment had a statistically significant better chance of reaching a final visual acuity of 0.4 logMAR or better compared with a longer duration of macular detachment, with an odds ratio for ≤ 3 days versus 4 days to 7 days of 2.86 (95% confidence interval, 1.37-5.99) and an odds ratio for ≤ 3 days versus >3 days of 3.09 (95% confidence interval, 1.56-6.12), and with a number needed to treat of 4. For pars plana vitrectomy, the limited amount of data precluded a meta-analysis with substantial results. CONCLUSION: This meta-analysis suggests that scleral buckling for macular detachment must preferably be performed within 3 days to optimize visual outcome.

Cataract surgery in patients with nanophthalmos.

PURPOSE: To report the visual outcomes and complications of cataract surgery in a large series of patients with nanophthalmos. DESIGN: Retrospective, interventional case series. PARTICIPANTS: Forty-three eyes with an axial length ≤ 20.5 mm of 32 adult patients who underwent cataract surgery in a tertiary clinic. METHODS: Medical records of patients undergoing cataract surgery between 1994 and 2010 were reviewed. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA) at 6 months after surgery and postoperative complications occurring during the entire follow-up period. RESULTS: Forty-three eyes of 32 patients (aged 19-87 years; median, 69 years) were included. Cataract surgery resulted in improvement of ≥ 3 Snellen lines in 19 eyes (44.2%). Two eyes (4.7%) lost ≥ 3 Snellen lines because of corneal decompensation in one and angle-closure glaucoma in the other. During the entire follow-up period, complications occurred in 12 eyes (27.9%). The most frequent complications were uveal effusion (9.3%) and cystoid macular edema (CME) (7.0%). CONCLUSIONS: Cataract surgery in patients with nanophthalmos remains a surgical challenge, and complications often occur in these high-risk eyes.

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.

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.

On selecting quality indicators: preferences of patients with breast and colon cancers regarding hospital quality indicators.

BACKGROUND: There is an increasing number of quality indicators being reported publicly with aim to improve the transparency on hospital care quality. However, they are little used by patients. Knowledge on patients' preferences regarding quality may help to optimise the information presented to them. OBJECTIVE: To measure the preferences of patients with breast and colon cancers regarding publicly reported quality indicators of Dutch hospital care. METHODS: From the existing set of clinical quality indicators, participants of patient group discussions first assessed an indicator's suitability as choice information and then identified the most relevant ones. We used the final selection as attributes in two discrete choice experiments (DCEs). Questionnaires included choice vignettes as well as a direct ranking exercise, and were distributed among patient communities. Data were analysed using mixed logit models. RESULTS: Based on the patient group discussions, 6 of 52 indicators (breast cancer) and 5 of 21 indicators (colon cancer) were selected as attributes. The questionnaire was completed by 84 (breast cancer) and 145 respondents (colon cancer). In the patient group discussions and in the DCEs, respondents valued outcome indicators as most important: those reflecting tumour residual (breast cancer) and failure to rescue (colon cancer). Probability analyses revealed a larger range in percentage change of choice probabilities for breast cancer (10.9%-69.9%) relative to colon cancer (7.9%-20.9%). Subgroup analyses showed few differences in preferences across ages and educational levels. DCE findings partly matched with those of direct ranking. CONCLUSION: Study findings show that patients focused on a subset of indicators when making their choice of hospital and that they valued outcome indicators the most. In addition, patients with breast cancer were more responsive to quality information than patients with colon cancer.

The role of biopsy in diagnosing patients suspected of idiopathic orbital inflammation.

PURPOSE: To provide a framework when to biopsy patients suspected of idiopathic orbital inflammation (IOI). MATERIALS AND METHODS: One-hundred seventeen patients were selected with signs of orbital inflammation in whom after history and physical examination, no definite diagnosis was made. In this cohort, the role of biopsy in making a diagnosis was evaluated and compared to a therapeutic trial of corticosteroids. RESULTS: In 67 of 117 patients, a diagnosis was evident after imaging and laboratory testing. In 50 remaining patients, a decision had to be made to biopsy or administer a therapeutic trial of corticosteroids. Thirty-nine patients underwent biopsy as the next step. Nine patients underwent a therapeutic trial of corticosteroids of which one was subsequently subjected to biopsy. Two patients spontaneously resolved. No patients with a malignancy were inadvertently treated with corticosteroids. One patient experienced a biopsy related complication but did not lose vision. CONCLUSIONS: This study suggests using a therapeutic trial of corticosteroids only in patients with low suspicion of malignancy with muscular and apical mass localizations, or with optic-nerve compression. This framework was demonstrated to be safe in not delaying diagnosis of malignancies and efficient in providing a rapid diagnosis.

Treatment of severe idiopathic orbital inflammation with intravenous methylprednisolone.

BACKGROUND: Prednisone pulse therapy is used to treat active non-infectious orbital inflammatory disease to attain faster clinical improvement and to shorten the duration of prednisone treatment. This study addresses the use of intravenous methylprednisolone (IVMP) pulse therapy, in addition to oral prednisone (OP), in the treatment of severe idiopathic orbital inflammation (IOI). METHODS: This was a multicentre retrospective cohort study. Patients with severe IOI treated with IVMP pulse and OP therapy (IVMP+OP) were compared with patients with IOI who were treated only with OP. Main outcome measures were duration of prednisone treatment, symptom-free outcome and complications. RESULTS: Between 2000 and 2007, 12 patients with severe IOI were treated with IVMP+OP and 15 patients were treated with OP only. The median treatment duration was 160 (range 34-680) days in the IVMP+OP group and 110 (range 27-730) days in the OP-only group. In patients who had severe IOI, 73% in the IVMP+OP group and 87% in the OP-only group were symptom-free after treatment. No patients developed complications related to prednisone therapy. CONCLUSION: In our study there was no advantage of treating patients with severe IOI with IVMP+OP in terms of shortened treatment duration, lower cumulative dose or decrease in persistent symptoms. We suggest that the indication of IVMP in the treatment of severe IOI is limited to speeding symptom relief and recovery from optic nerve dysfunction.

Hearing disability in patients with Fuchs' endothelial corneal dystrophy: unrecognized co-pathology?

PURPOSE: To investigate a possible association between Fuchs' endothelial corneal dystrophy (FECD) and hearing disability. METHODS: A cross-sectional observational study was performed at the University Medical Center Utrecht. Cases and controls were patients who were treated by a cornea specialist between 2004 and 2008. FECD patients had either already undergone or were planned for a keratoplasty procedure. All controls were patients treated for cataract without any corneal pathology. Cases and controls were matched by age group and gender. A structured telephone interview combined with a validated self-perceived hearing function test (the Hearing Handicap Inventory for the Elderly, screening version; HHIE-S) was used for data collection. RESULTS: Hearing disability was reported in 33 patients (45.8%) in the FECD-group (n = 72, average age: 73 years) versus 50 patients (34.7%) in the control group (n = 144, average age: 73 years). Hearing disability was significantly associated with FECD after adjustment for age, noise exposure, and diabetes mellitus (odds ratio 1.97 95% confidence interval 1.04-3.75). CONCLUSION: This case-control study reports a significant association between FECD and hearing disability. The causal relation was not evaluated in this study, though mutations in the SLC4A11 gene could have played an important role. This gene encodes for an ion transporter, which has been found in the cornea and inner ear. With the lack of examination with a pure tone or speech audiometry in this study, further studies need to be performed in order to support the association and should include a complete ENT examination with audiometry and genetic research.

Azathioprine and prednisone combination treatment for adult periocular and orbital xanthogranulomatous disease.

PURPOSE: To report the authors' experience with azathioprine and prednisone combination for adult periocular and orbital xanthogranulomatous disease. METHODS: We identified 13 adults with histology-proven periocular or orbital xanthogranuloma in two tertiary referral orbital centres from 1984 to 2008. Patient records were reviewed and data collected on orbital localization, immune dysfunction, applied treatment and outcome. RESULTS: Five patients with periocular or orbital xanthogranulomatous disease were fully treated with prednisone and azathioprine combination, which resulted in stabilization in two and regression in three. Two other patients had to discontinue azathioprine because of side-effects. Of the non-fully treated prednisone/azathioprine patients, four out of eight progressed. CONCLUSION: In adult periocular and orbital xanthogranuloma, combined treatment with prednisone and azathioprine yields adequate immunosuppression, often for a prolonged period of time.

Risk factors for idiopathic orbital inflammation: a case-control study.

OBJECTIVE: To identify risk factors involved in the development of idiopathic orbital inflammation (IOI). METHODS: Case-control study of 69 adults who had had a first episode of IOI and 296 adult controls with rhegmatogenous retinal detachment (RD) selected from three orbital centres in The Netherlands between 2000 and 2006. Participants filled out a questionnaire on demographic factors, medical history, health status and exposures for the 2 years prior to disease presentation. In addition, women were questioned about previous or current pregnancies and their hormonal status. ORs and accompanying 95% CIs for IOI in relation to potential risk factors such as body mass index (BMI), bisphosphonates and autoimmune disease were estimated. ORs were adjusted for age, sex, socio-economic status, smoking and blunt orbital trauma using logistic regression. Analyses were carried out both with and without multiple imputation of missing values. RESULTS: The risk of IOI was increased in participants who had a higher BMI (third vs first tertile: OR, 2.88; 95% CI 1.32 to 6.32) and in participants who used bisphosphonates (OR 8.68; 95% CI 1.16 to 65.0). The risk was decreased in participants with a higher socio-economic status (third vs first tertile: OR 0.38; 95% CI 0.17 to 0.84) and in women who were older at first childbirth (third vs first tertile: OR 0.14; 95% CI 0.03 to 0.64). An almost significant association was found for IOI and autoimmune disease (OR 2.56; 95% CI 0.93 to 7.05). CONCLUSIONS: IOI is associated with lower socio-economic status, higher BMI and use of oral bisphosphonates. In women, IOI is also associated with younger age at first childbirth.

Proliferative vitreoretinopathy in human immunodeficiency virus-infected patients in the era of highly active antiretroviral therapy.

PURPOSE: To assess the prevalence of proliferative vitreoretinopathy (PVR) and prognosis of cytomegalovirus (CMV) retinitis-related retinal detachment (RD) surgery in the era of highly active antiretroviral therapy (HAART). DESIGN: Retrospective interventional cohort study. METHODS: Thirty-five human immunodeficiency virus (HIV)-positive patients with CMV retinitis-related RD who underwent surgical repair were assessed for PVR, CD4-positive T cell counts, and use of HAART. Main outcome measures included anatomic and functional outcomes of RD surgery as well as the presence of PVR and CD4-positive T cell counts. RESULTS: PVR was present in 10 of 35 patients (29%) at the time of the first surgery. The presence of PVR was associated with worse preoperative and postoperative visual acuity (P = .017 and P = .009, respectively), with the CD4-positive T cell counts above 200 cells/microL (P = .054), and with a longer interval between the diagnosis of RD and surgery (P = .025). The odds ratio for development of PVR in patients with CD4-positive T cells above 200 cells/microL was 11.3 (95% confidence interval 1.01-125). PVR was not associated with age, gender, or duration of HIV infection. Anatomic reattachment was obtained in 31 patients (89%), though the functional outcomes were limited. The central location of CMV retinitis was associated with postoperative visual acuity (VA) of less than 0.1 (P = .000). Postoperative logMAR VA was associated with preoperative logMAR VA (P < .001) and development of PVR (P = .009). CONCLUSION: PVR was present in 29% of CMV retinitis-related RD and was associated with higher CD4-positive T cell counts and longer interval between the diagnosis of RD and surgery.

Optical coherence tomography findings for nanophthalmic eyes.

PURPOSE: To describe the optical coherence tomography (OCT) findings for nanophthalmic eyes. METHODS: : In this retrospective, single-center, observational case series, 15 patients (28 eyes) with axial lengths of

Methylprednisolone pulse therapy for patients with moderately severe Graves' orbitopathy: a prospective, randomized, placebo-controlled study.

OBJECTIVE: To assess whether methylprednisolone (MP) pulse therapy is efficacious in the treatment of moderately severe Graves' orbitopathy (GO). DESIGN: Prospective, placebo (PL)-controlled, double-blind, randomized study. METHODS: Fifteen previously untreated patients with active, moderately severe GO participated in the study; 6 patients received MP and 9 patients a PL. Moderately severe disease was defined using the NOSPECS classification of clinical signs of GO . Activity was measured with the clinical activity score (CAS). A dose of 500 mg MP or only solvent was administered intravenously, over three consecutive days, in four cycles at 4 weekly intervals (6 g of MP in total). Qualitatively, a successful treatment outcome was defined as an improvement in one major and/or two minor criteria in the worst eye at week 48. The major criteria were: improvement in diplopia grade; improvement in eye movement; a decrease in CAS of three points. The minor criteria were: decrease of eyelid retraction; decrease of proptosis; improvement in grade of soft tissue swelling; a decrease in CAS of two points. RESULTS: The qualitative treatment outcome was successful at the end of the trial in five out of six (83%) patients receiving MP and in one out of nine (11%) patients given the PL (relative risk=7.5; (95% confidence interval 1.1-49.3), P=0.005). The treatment was well tolerated. CONCLUSIONS: In spite of the small number of patients, a significant difference in outcome was observed between MP- and PL-treated patients. We conclude that MP pulse therapy appears to be an effective treatment for active, moderately severe GO.

Radiologic measurement of extraocular muscle volumes in patients with Graves' orbitopathy: a review and guideline.

OBJECTIVE: To evaluate and compare techniques for extraocular muscle (EOM) volume measurement and to provide guidelines for future measurements. DESIGN: Systematic review. RESULTS: Existing techniques used to measure extraocular muscle volumes on radiologic scans can be divided into manual outlining, computer assisted and automated segmentation. Both computed tomography (CT) and magnetic resonance (MR) image datasets can be used. On CT scans, one best measures muscle volume using region grow segmentation, accepting an overestimation of true volume by inevitable inclusion of non-muscular tissue. On high resolution MRI scans, single muscles can be outlined manually, but measurements include only part of the muscle due to poor tissue contrast at the orbital apex. Measurement errors can be reduced 3.5% by exact horizontal repositioning. A measured volume change of at least 6-17% is required to demonstrate a significant difference. CONCLUSION: Currently the best choice for EOM volume measurements on CT images is computer assisted grey value segmentation and on MRI images is manual outlining of individual muscles. Because of the time required and the complexity of the measurements, present EOM volume measurement is as yet only suitable for research purposes.