Ward R. Bijlsma

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.

Ocular firework trauma: a systematic review on incidence, severity, outcome and prevention

Aim To provide a systematic review on ocular firework trauma with emphasis on incidence and patient demographics, the extent of ocular trauma and visual function loss, and firework regulation effects on injury rates. Methods A literature search was performed using predetermined inclusion and exclusion criteria. Demographic characteristics of ocular firework casualties were obtained and incidence rates of sustained trauma and vision loss calculated. Results Twenty-six relevant articles were suitable for calculation of trauma incidence and patient demographics, of which 17 articles could be used for calculating trauma severity and vision loss. Victims were male (77%), young (82%) and often bystander (47%). Most of the trauma was mild and temporary. Penetrating eye trauma, globe contusions and burns accounted for 18.2%, with a 3.9% enucleation rate. Mean visual acuity was >10/20 in 56.8%, with severe vision loss (<10/200) in 16.4%. Countries using restrictive firework legislation show 87% less eye trauma (p<0.005). Conclusions One in six ocular firework traumas show severe vision loss, mostly in young males. Bystanders are as frequently injured. Firework traumas are a preventable cause of severe ocular injury and blindness because countries using restrictive firework legislation have remarkable lower trauma incidence rates.

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.

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.

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.

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.

Idiopathic Orbital Inflammation and Graves Ophthalmopathy

Idiopathic orbital inflammation (IOI) is a poorly understood disease entity in which an orbital inflammatory process is found with, by definition, no identifiable local or systemic cause. Graves ophthalmopathy (GO) is often mentioned as a disease to exclude in the diagnosis of IOI. In the Orbital Clinic of the University Medical Center Utrecht, we have encountered 4 patients in whom diagnoses of both IOI and GO were made at different times. In this case series, we describe the clinical and diagnostic features of these patients, show that both IOI and GO can occur at different times in the same patient, and demonstrate the ways the diseases can be differentiated.

Identification of infectious entities in idiopathic orbital inflammation biopsies

Idiopathic orbital inflammation (IOI) is a poorly understood disease that can cause symptoms of pain, proptosis and diplopia. The condition is considered of non-infectious origin and a diagnosis is only made after exclusion of a neoplasm, primary infection and systemic disorders. However, a recent history of upper respiratory tract infection prior to development of IOI is not uncommon.1 It has been postulated that IOI is caused by an auto-immune response that develops after an infectious trigger.2 We investigated whether IOI may have an infectious origin by using molecular techniques to identify genetic material of a panel of viruses and bacteria in tissue biopsies of patients with IOI and controls. After ethical review board approval, 48 orbital biopsy specimens were collected at orbital clinics in Amsterdam, Maastricht, Rotterdam …

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.