Disease modifying drugs in idiopathic sclerosing orbital inflammatory syndrome.

PURPOSE: Idiopathic sclerosing orbital inflammatory syndrome (ISOIS) is a rare, progressive and hard to control disease. There is a deep gap of evidence regarding application of disease-modifying drugs (DMD) regimen as a potentially effective treatment for orbital inflammatory diseases. We aimed to report the results of using DMDs and discuss the concept of applying this modality of treatment in patients with ISOIS. METHODS: This was a prospective interventional case series conducted in a tertiary university-based hospital. Biopsy proven patients with active ISOIS were included. Systematic criteria were developed to define and measure disease activity and monitor response to treatment. A DMD regimen including an anti-tumor necrosis factor alpha (anti-TNF alpha) agent plus azathioprine and low-dose corticosteroids were used. Comprehensive ophthalmic, orbital and systemic assessments were performed during each visit. RESULTS: Five eligible patients with primary ISOIS were included. Mean age was 34.20 (SD = 13.33, range 19-53) years. Three had unilateral and two had bilateral involvement. Four had diffuse orbital involvement pattern and progressive worsening of visual functions, reduced extraocular motility and proptosis. In one patient the disease was localized to extraocular muscle and lacrimal gland. Disease activity was decreased and stabilized after DMDs regimen in all patients. Mean follow up was 32.80 (SD = 30.80, range: 12-86) months. CONCLUSION: Biologic DMD (b-DMD) including anti-TNF alpha, corticosteroid and azathioprine were effective in decreasing disease activity and could change course of the disease. This study supports the concept of using b-DMD regimen in treatment of ISOIS.

Orbital Dirofilariasis Masquerading As Orbital Rhabdomyosarcoma.

Purpose: To report a 12-year-old patient with a rapid growing orbital mass and imaging findings suggestive of rhabdomyosarcoma that was found to be dirofilariasis after mass resection. Case Report: We describe a 12-year-old patient with a rapid growing orbital mass involving medial part of orbit and medial rectus muscle and imaging findings suggestive of rhabdomyosarcoma. Histopathologic examination showed the mass to be composed of granulomatous inflammation and the thread-like object to be Dirofilaria repens. The patient was well post-operation without morbidity. In this paper, we describe distinct clinical features and imaging findings of this interesting case. Conclusion: Deep orbital lesions due to dirofilariasis, as in our case, is extremely rare. It is important to add dirofilariasis to the differential diagnosis of orbital mass lesions. Attention to the imaging clues, as provided in this report, can be helpful.

Shifting to very early endoscopic DCR in acute suppurative dacryocystitis.

PURPOSE: We aimed to show the outcome of very early endoscopic dacryocystorhinostomy (VE-EDCR) in a routine pool of patients with acute dacryocystitis (AD) and abscess formation compared with the standard late external dacryocystorhinostomy L-ExDCR. METHODS: This was a prospective nonrandomized comparative study conducted from June 2013 to March 2016. Patients with AD and abscess formation were referred to our oculo-facial clinic in a university-based hospital. All patients received systemic antibiotics and were assigned to either of treatment groups. Patients in group 1 underwent late external transcutaneous DCR (L-ExDCR) and group 2 underwent EDCR within 3 days after first visit, named VE-EDCR. Primary outcome measure was success of surgery. RESULTS: Forty-one eyes of 41 patients with acute suppurative AD, were included from June 2013 to March 2016. Twenty-two patients underwent VE-EDCR and 19 underwent L-ExDCR. Mean age of patients was 43.41 (SD = 19.84, range 14-98) years. Mean follow-up was 14 (SD = 2.4) months. Anatomic, functional, and overall success in L-ExDCR and VE-EDCR groups were (89.5 and 86.4%, p = 0.99) (89.5% and 86.4%, p = 0.99) (89.5% and 81.8%, p = 0.66) respectively. Mean duration of cellulitis in VE-EDCR and L-ExDCR were 8.00 (SD = 4.63) and 16.11 (SD = 11.58) days, respectively (p = 0.027). No remarkable adverse event was found. CONCLUSIONS: Success of very early endonasal endoscopic DCR is comparable with the traditional late external DCR. Duration of cellulitis is shorter in VE-EDCR. This therapeutic approach can be considered in patients with acute suppurative dacryocystitis.

Visual Outcomes of Adding Erythropoietin to Methylprednisolone for Treatment of Retrobulbar Optic Neuritis.

PURPOSE: To compare the short-term visual function results and safety of erythropoietin as an add-on to the standard corticosteroid therapy in retrobulbar optic neuritis (RON). METHODS: In this prospective pilot study, adult patients with isolated RON with less than 10 days of onset were enrolled. Patients were consecutively assigned to standard intravenous methylprednisolone treatment either in combination with intravenous erythropoietin (20,000 units/day for three days) (group-1) or intravenous methylprednisolone alone (group-2). Primary outcome measure was best-corrected visual acuity (BCVA), which was assessed up to 120 days from the day the treatment was begun. Systemic evaluations were performed during and after treatment. RESULTS: Sixty-two patients with RON (mean age = 26.6 ± 5.77 years; range = 18-40 years) were enrolled into the study (group-1, n = 35; group-2, n = 27). BCVA three months after the treatment was 0.19 ± 0.55 logMAR and 0.11 ± 0.32 logMAR in group-1 and group-2, respectively (95% CI: - 0.61 - 0.16; P = 0.62). Change in BCVA after three months was 2.84 ± 3.49 logMAR in group-1 and 2.46 ± 1.40 logMAR in group-2 (95% CI: - 0.93 - 1.91; P = 0.57). Pace of recovery was not significantly different between the groups. No complications were detected among patients. CONCLUSION: Intravenous erythropoietin as an add-on did not significantly improve the visual outcome in terms of visual acuity, visual field, and contrast sensitivity compared to traditional intravenous corticosteroid. This pilot study supports the safety profile of intravenous human recombinant erythropoietin, and it may help formulate future investigations with a larger sample size.

Erythropoietin in Treatment of Methanol Optic Neuropathy.

BACKGROUND: Methanol poisoning can cause an optic neuropathy that is usually severe and irreversible and often occurs after ingestion of illicit or homemade alcoholic beverages. In this study, we evaluated the potential neuroprotective effect of erythropoietin (EPO) on visual acuity (VA) in patients with methanol optic neuropathy. METHODS: In a prospective, noncomparative interventional case series, consecutive patients with methanol optic neuropathy after alcoholic beverage ingestion were included. All patients initially received systemic therapy including metabolic stabilization and detoxification. Treatment with intravenous recombinant human EPO consisted of 20,000 units/day for 3 successive days. Depending on clinical response, some patients received a second course of EPO. VA, funduscopy, and spectral domain optical coherence tomography were assessed during the study. Main outcome measure was VA. RESULTS: Thirty-two eyes of 16 patients with methanol optic neuropathy were included. Mean age was 34.2 years (±13.3 years). The mean time interval between methanol ingestion and treatment with intravenous EPO was 9.1 days (±5.56 days). Mean follow-up after treatment was 7.5 months (±5.88 months). Median VA in the better eye of each patient before treatment was light perception (range: 3.90-0.60 logMAR). Median last acuity after treatment in the best eye was 1.00 logMAR (range: 3.90-0.00 logMAR). VA significantly increased in the last follow-up examination (P < 0.0001). Age and time to EPO treatment after methanol ingestion were not significantly related to final VA. No ocular or systemic complications occurred in our patient cohort. CONCLUSIONS: Intravenous EPO appears to improve VA in patients with methanol optic neuropathy and may represent a promising treatment for this disorder.

Spontaneous Rupture of Lacrimal Gland Pleomorphic Adenoma: Pivotal Role in Masquerading Orbital Cellulitis.

The authors aim to report a 68-year-old patient presenting with acute swelling and redness of orbital area diagnosed initially as orbital cellulitis. Surgical excision was decided based on clinical and imaging findings with the diagnosis of pleomorphic adenoma of the lacrimal gland. Lacrimal gland pleomorphic adenoma usually presents with painless gradual swelling of upper eyelid. In an exceptionally rare circumstance, this case showed acute orbital inflammation, and imaging findings of orbital inflammation mostly focused around the cystic space of the lacrimal gland tumor. Histologic evaluation confirmed a ruptured cystic space of pleomorphic adenoma with foreign body-type inflammatory reaction. The authors propose spontaneous rupture of cystic space in the lacrimal gland pleomorphic adenoma as underlying mechanism for acute presentation of this tumor. Imaging and clinical characteristics and also prognostic implication of this phenomenon are discussed.

Validation of Farsi Translation of the Ocular Surface Disease Index.

PURPOSE: To develop and validate a Farsi version of Ocular Surface Disease Index (OSDI) for the Iranian population. METHODS: This study was a translation and cross-cultural adaptation and validation of Farsi version of OSDI. Four bilingual (English-Persian) individual including three physicians and one native English teacher were asked to translate the original English OSDI questionnaire in Farsi. Following back and forth translation, integration and pilot check, the translation team came to consensus on translation. Consecutive patients visited in ophthalmology clinic, underwent comprehensive general ophthalmology exam and specific assessments for dry eye including non-anesthetic Schirmer's test, fluorescein tear break-up time, Fluorescein and Rose Bengal staining and Farsi OSDI (F-OSDI). F-OSDI was again rechecked within 2-7 days after the examination. RESULTS: Forty-four participants were enrolled into study. Thirty-two (72.7%) were male and 12 (27.3%) female. Mean age of participants was 45.5 (SD = ±15.97, range = 18-80) years. Twenty five percent were less than 31 years old and 10% percent older than 65. The cronbach's alpha for the questionnaire was 0.807. Questions number 7, 8 showed excellent, and question12 showed good internal consistency, respectively. There was a significant correlation between all pre measures and post assessments. CONCLUSION: The obtained F-OSDI showed acceptable internal consistency and test-retest reliability. This F-OSDI could be used for assessment of dry eye, ocular surface discomfort and quality of life in Iranian and Farsi speaking populations.