Inflammatory myofibroblastic tumor of the orbit: A clinico-pathological study of 25 cases.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma, which typically occurs in children or young adults. The IMT is considered generally a benign lesion, although about 20% of cases may experience recurrence, and most rarely develop metastasis. Herein, we present the largest series of primary orbital IMT ever reported. PATIENTS AND METHODS: The clinical records of 25 patients, collected between the 1995 and 2015, with biopsy-proven diagnosis of orbital IMT were retrospectively reviewed to determine demographic, clinical, radiologic and pathological features, management, and outcome. RESULTS: The study included 13 females and 12 male patients, age ranged from 5 to 76 years. Disease onset was in all cases unilateral (25/25), with posterior location (10/25) or extending anterior to posterior (7/25). The most common signs and symptoms were: proptosis (19/25), ptosis (18/25), diplopia (10/25), periocular swelling (9/25), pain (8/25), redness (7/25). All patients underwent to incisional biopsy which included total or subtotal tumor resection avoiding arming of the adjacent structure, followed by systemic steroid therapy (22/25) or radiotherapy (3/25). The disease recurred in 6 (24%) patients who responded to the subsequent therapy. No one developed metastasis or died because of the disease. CONCLUSION: IMT is a distinct entity which may occur in the orbit primarily. It should be considered in differential diagnosis in all orbital masses, particularly with onset of acute or subcronic inflammation. Surgical biopsy associated to a partial debulcking of the tumor, avoiding to damage adjacent vital structure may contribute to improve the outcome. Steroid therapy, seems to be the suitable as first line medical therapy, although, as reported in literature, not all cases respond to this treatment regimen. Radiotherapy, may be considered as an alternative therapy. Recurrences occurred in 24% of patients and may be treated with additional surgical resection and a new course of steroid or radiotherapy. No specific pathological features which may correlate with the prognosis have been found in this series.

Evaluation of ischemic diabetic maculopathy with Fourier-domain optical coherence tomography and microperimetry.

OBJECTIVE: To evaluate the efficacy of high-speed Fourier-domain optical coherence tomography (FD-OCT) and fundus microperimetry (MP-1) in identifying the anatomic and functional features of ischemic diabetic maculopathy. DESIGN: Prospective noninterventional study. PARTICIPANTS: Forty-two consecutive eyes (23 patients) with ischemic diabetic maculopathy and 40 normal eyes (25 control subjects) were included in this study. METHODS: Best corrected visual acuity, ganglion cell complex (GCC) thickness measured with FD-OCT, and central light sensitivity recorded with MP-1 were evaluated. RESULTS: GCC thickness and light sensitivity were significantly reduced in all affected eyes versus control eyes. logMAR BVCA was significantly correlated with mean macular sensitivity (R=0.783, R(2)=0.611). CONCLUSIONS: GCC thickness and microperimetry integrated with fluorescein angiography could be a marker of retinal vascular abnormalities that is useful for the diagnosis of ischemic diabetic maculopathy.

Choroidal thickness in open-angle glaucoma measured by spectral-domain scanning laser ophthalmoscopy/optical coherence tomography.

AIMS: To measure macular choroidal thickness in healthy and glaucomatous eyes using spectral-domain scanning laser ophthalmoscopy/optical coherence tomography. METHODS: Choroidal thickness was measured in 21 healthy eyes and 16 glaucomatous eyes. Choroidal thickness was measured under the fovea and at 500-µm intervals from the foveal center to 4 mm in the nasal and temporal directions. Vessels were counted in a macular area of 8,000 µm(2). Only choroidal vessels with a horizontal diameter of at least 155 µm and a vertical diameter of at least 50 µm were considered. RESULTS: The mean choroidal thickness was greatest below the fovea, i.e. 343.8 µm (SD ± 29.06) in controls and 411.56 µm (SD ± 33.60; p < 0.001) in the glaucomatous group. The mean vertical diameter was significantly greater (p = 0.008) in glaucomatous eyes (112.18 ± 37.64 µm) than in healthy eyes (103.98 ± 25.67 µm), whereas the luminal area of vessels was significantly larger (p = 0.3) in glaucomatous eyes (39,157.34 ± 21,657.23 µm(2)) than in healthy eyes (43,779.84 ± 22,874.87 µm(2)). CONCLUSIONS: We show that choroidal thickness is increased in glaucomatous eyes due to an increase in both the vertical diameter and the luminal area of the vessels. This seems to implicate the choroid in the pathogenesis of open-angle glaucoma.

Hepatitis C virus infection in ocular adnexal lymphomas.

OBJECTIVE: To assess the influence of hepatitis C virus (HCV) infection on disease appearance and outcome of ocular adnexal non-Hodgkin lymphoma (ONHL). DESIGN: Retrospective comparative study (from January 1, 1992, through December 31, 2006). METHODS: The medical records of 129 patients with ONHL were retrospectively reviewed. All the patients were tested serologically for the presence of HCV infection. Patients were divided into 2 groups according to the presence or absence of HCV infection. MAIN OUTCOME MEASURES: Prevalence of HCV infection, staging to evaluate the extent of disease at the onset, and clinical outcome data on overall and disease-free survival. RESULTS: The prevalence of HCV infection among the patients with ONHL was 17.8%. Seropositivity for HCV infection was significantly associated with extraorbital lymphoma at the onset (P = .006). High prevalence of mucosa-associated lymphoid tissue disease (79.8%) was registered. Protocol therapy included radiotherapy and chemotherapy, depending on the stage of the disease. Complete remission was achieved in 99 patients (76.7%). A total of 23.6% of patients with HCV-seronegative status and 21.7% of those with HCV-seropositive status experienced relapse of the lymphomatous disease. No significant differences in the 5-year overall survival and disease-free survival between the 2 groups were observed. CONCLUSIONS: Prevalence of HCV infection in patients with ONHL is a relevant issue, accounting for 17.8% of the examined patients. Infection with HCV may influence the initial appearance of ONHL because it is associated with more widespread disease at the onset. However, the overall and disease-free survival of the infected patients are not statistically different than that of patients who are not infected.

Intravitreal triamcinolone, bevacizumab and pegaptanib for occult choroidal neovascularization.

PURPOSE: To evaluate best-corrected visual acuity (BCVA) and foveal thickness (FT) changes in occult subfoveal choroidal neovascularization (CNV) from age-related macular degeneration (AMD) after intravitreal bevacizumab (IVB, 1.25 mg/0.05 ml), pegaptanib (IVP, 0.3 mg/0.09 ml) and triamcinolone acetonide (IVTA, 4 mg/0.1 ml) injected on an as needed basis. METHODS: Retrospective, interventional, comparative study. BCVA (Early Treatment Diabetic Retinopathy Study LogMAR) and FT by optical coherence tomography (OCT) were evaluated during 12 months from first treatment. Patients were retreated if signs of neovascular activity were still present on angiography or OCT. RESULTS: Forty-eight eyes received IVB, 43 eyes received IVP, 52 eyes received IVTA. BCVA and FT at baseline were 1.22 ± 0.49 LogMAR and 410.2 ± 41.83 µm in the IVB group, 1.25 ± 0.43 LogMAR and 452.3 ± 44.83 µm in the IVP group and 1.31 ± 0.4 LogMAR and 456.6 ± 48.27 µm in the IVTA group. BCVA and FT improved in the three groups during follow-up. A significantly greater improvement of BCVA was present at month-3, month-6 and at month-12 in the IVB and IVP groups (p = 0.01). Improvement of FT was greater in the IVTA group at month-3 (p = 0.02), while it was greater in the anti-Vascular Endothelial Growth Factor (VEGF) groups at month-6 and month-12 (p = 0.01). A postoperative increase of intraocular pressure was detected in 9/52 (17.3%) eyes treated with IVTA, and in two cases it was resistant to topical therapy. CONCLUSION: Intravitreal injection of anti-VEGF drugs administered on an as needed basis for AMD-related occult CNVs provided functional and anatomic improvement during 12 months of follow-up.