Diagnosis of giant cell arteritis using clinical, laboratory, and histopathological findings in patients undergoing temporal artery biopsy.

OBJECTIVE: To identify the clinical, laboratory, and histopathological features that may predict the diagnosis of giant cell arteritis (GCA). METHODS: A retrospective chart review was performed on patients who underwent temporal artery biopsy (TAB) between January 1, 2011 and March 31, 2019. Patient demographics, clinical characteristics, laboratory features, histopathological features, and biopsy results were collected. GCA status was determined by a neuro-ophthalmologist (OOA). Stepwise logistic regression analysis was performed to identify features that predict GCA status. RESULTS: Of 101 patients who underwent TAB, 31 (31%) were diagnosed with GCA. Age was found to be statistically significant for the diagnosis of GCA (P = 0.009), with an average age of 74.4 years ( ± 8.1) in those with GCA vs. 68.9 years ( ± 10.0) in those without. The incidence of transient vision loss was higher in GCA than non-GCA patients (P = 0.005). Anterior arteritic ischemic optic neuropathy (n = 3), ophthalmic artery occlusion (n = 2), and posterior ischemic optic neuropathy (n = 1) were seen only in the GCA group. Of the 31 GCA patients, 15 had active GCA (48%), 3 (10%) had healed temporal arteritis (HTA), 8 (26%) had suggested HTA, and 5 (16%) had false negative biopsies. Of the 70 non-GCA patients, 63 (90%) had negative biopsies, 2 (3%) had HTA, and 5 (7%) had suggested HTA. Histopathological analysis revealed that CD68 staining had a sensitivity of 69% and specificity of 86%. Both presence of multinucleated giant cells (MNGC) and transmural inflammation had 100% specificity; however, sensitivity was ≤ 50%. In patients with negative TABs and complete risk factor data available (n = 66), the odds of GCA increased 2.16-fold every 5 years of age, and 1.08-fold every mg/day of oral steroid use. A biopsy result of HTA had an odds ratio of 84.7 and suggested HTA of 49.2 against a negative TAB for diagnosis of GCA. CONCLUSION: Age at time of biopsy, HTA, and suggested HTA are predictive for the diagnosis of GCA. Transient vision loss is more commonly seen in GCA, and anterior arteritic ischemic optic neuropathy, ophthalmic artery occlusion, and posterior ischemic optic neuropathy are important ophthalmic manifestations of GCA. CD68 staining is more sensitive but less specific for diagnosing GCA in comparison to other histopathologic findings such as presence of MNGC and transmural inflammation. Further work is recommended to investigate the importance of the specific histopathologic finding of CD68 staining in the diagnosis of GCA.

Terror-Related Ocular Trauma in Patients Presenting to a Tertiary Eye Center in the Middle East.

PURPOSE: Injuries caused by explosive materials are associated with severe ocular morbidity and visual impairment. This study aims to document the ophthalmic morbidity of terrorist-related severe injuries at a tertiary eye care center in the Middle East and report specific injury patterns. METHODS: In a noncomparative, interventional, retrospective case series, the visual outcomes were evaluated of 36 patients treated at a tertiary eye center in the Middle East for terrorist-related injuries from January 2003 to December 2019. Data were collected on age, gender, involved eye, initial examination of ocular injuries, associated systemic injuries, presenting visual acuity, anterior and posterior segment examinations, and the type of injury (open vs. closed globe). RESULTS: Among the 36 patients (32 males; average age, 28.1 years), 28 (77.8%) sustained trauma to one eye and 8 (22.2%) to both eyes. Despite multiple surgical procedures (average 3.1 surgeries), all patients suffered a severe visual loss. Final visual acuity of no light perception was recorded in 28 (77.8%) eyes and both eyes of 5 patients (13.9%). CONCLUSION: Ophthalmic morbidity from terrorist-related injuries may be devastating, and the vast majority of patients experience a permanent loss of vision.

Association of Disease Activity with Serum and Tear IL-2 Levels in Behçet Disease.

PURPOSE: To investigate the association of Behçet disease activity with serum and tear interleukin-2 levels. METHODS: The study was designed as a prospective case control study. The study population consisted of 45 patients with Behçet disease and 24 age and sex-matched healthy participants. Behçet disease patients were classified as active (24 patients) or inactive (21 patients) according to disease activity. Serum and tear interleukin-2 levels were determined using the enzyme-linked immunosorbent assay method. RESULTS: The mean serum and tear interleukin-2 levels of the active disease, inactive disease, and control groups were 17.04 ± 5.02 and 32.61 ± 16.53 pg/mL; 15.20 ± 4.68 and 29.61 ± 8.30 pg/mL; and 14.22 ± 4.18 and 28.89 ± 8.73 pg/mL, respectively. There was no statistically significant difference between the groups with respect to all measured data. CONCLUSIONS: There was no significant difference in serum or tear IL-2 levels between Behçet patients and controls; there was no association of disease activity with serum and tear IL-2 levels.

Ocular motility abnormalities in orbitofacial neurofibromatosis type 1.

PURPOSE: To evaluate the causes of ocular motility disturbances in a group of patients with orbitofacial neurofibromatosis (OFNF) with neurofibromas on the lid, brow, face, or in the orbit from infancy or early childhood. METHODS: The medical records of patients with OFNF from one institution were retrospectively reviewed; selected patients were reexamined. RESULTS: A total of 45 patients with unilateral OFNF and 4 with bilateral OFNF were included. Of these, 14 had no strabismus and relatively good vision, with no ductional abnormalities on either side despite large globes, sphenoid dysplasia, and neurofibromas in the orbit and/or cavernous sinus in many. The 8 patients with comitant strabismus also had no ductional abnormalities with a similar constellation of anatomic abnormalities, but these patients all had poor vision in at least one eye. The 27 patients with incomitant strabismus all had downward displacement of the globe and limited ductions. CONCLUSIONS: The pathologic anatomic changes associated with OFNF do not always cause ocular motility abnormalities: strabismus generally was not present when ocular motility was full and visual acuity was good. Comitant strabismus occurred in the setting of full ocular motility with reduced vision in at least one eye. Incomitant strabismus was always accompanied by reduced vision and a ductional abnormality in one or both eyes due to anatomic abnormalities of the orbit and skull.

Ocular malignant tumors. Review of the Tumor Registry at a tertiary eye hospital in central Saudi Arabia.

OBJECTIVE: To present the epidemiologic profile and magnitude of ocular malignant tumors (MT) representative of the Saudi population from the Tumor Registry (TR) at King Khaled Eye Specialist Hospital (KKESH). METHODS: This study evaluated the demographic information, clinical features including tumor laterality, ocular tissue of origin, and diagnosis of patients from the TR registry between 1983 and 2012 at KKESH, Riyadh, Kingdom of Saudi Arabia. The incidence of MT among Saudi adults (>/= 15 years old), and children (<15 years old) was estimated. RESULTS: The TR recorded 4,146 neoplasms (2,509 [60.5%] benign tumors, and 1,637 [39.5%] MT). The incidence of MT in children was 3.6 per million/year (M/Y), and 2.4/M/Y for adults. Retinoblastoma (Rb) (n=763, 91%) was the most common ocular malignancy in children. In adults, the most common MT was squamous cell carcinoma (SCC) (n=363, 45.8%), basal cell carcinoma (BCC) (n=186, 23%), uvealmelanoma (n=94, 11.9%), sebaceous gland carcinoma (n=54, 6.8%), lymphomas (orbital, adnexal) (n=46, 5.8%), and others (n=53, 6.8%). The Rb (7.7/M/Y in <5 years old Saudi children) was less frequent than that reported in some Gulf countries, but higher than that reported from the West. The SCC was less frequent in countries with comparable sun exposure than in other continents, but the incidence remained unchanged over 3 decades. There was a significant increase in BCC between 1983-1992 and 2003-2012. CONCLUSION: The rates of all cancers remained stable over 3 decades except BCC, which showed a significant rise.

Acute, unilateral transient blepharoptosis of unknown etiology: a review.

PURPOSE: To describe 5 patients with transient, acute, unilateral blepharoptosis of unknown etiology. METHODS: Medical records of 5 patients, which were seen in 5 different clinics between 1998 and 2011, were reviewed. RESULTS: The ages of the patients were 7, 7, 32, 44, and 75 years (2 women and 3 men). Four patients had partial (1.5-3.5 mm) and 1 patient had total ptosis. Levator function was normal in 2 patients, and 3 patients had only 2 to 3 mm decreased levator function on the affected side. None of the patients had limited motility or any pupil abnormalities. All patients had a history of flu-like illness preceding the development of blepharoptosis. The ptosis completely resolved in 3 to 14 days in all patients and did not recur during the follow-up period (range: 2 months-7 years). CONCLUSIONS: Transient, acute, unilateral blepharoptosis is a very rare condition. Patients presenting with acute ptosis should be questioned about a recent history of flu-like syndrome, and if workup is negative with this history, the clinicians should suspect this rare condition.

Congenital ectropion uvea and mechanisms of glaucoma in neurofibromatosis type 1: new insights.

OBJECTIVE: To describe the clinicopathologic features of congenital ectropion uvea associated with glaucoma in neurofibromatosis-1 (NF-1). DESIGN: Retrospective case series. PARTICIPANTS AND CONTROLS: Five cases of NF-1 associated with glaucoma, from which enucleated eyes were available, and 2 eye bank eyes used as controls. METHODS: The clinical features and courses of these patients were reviewed. Formalin-fixed, paraffin-embedded eyes were examined by light and electron microscopy. Immunohistochemistry using antineurofibromin, anti-glial fibrillary acidic protein, and antivimentin was performed in 3 patients. Gene expression of the mitogen-activated protein kinase (MAPK) signaling pathway was examined in corneal endothelial cells in 1 patient. MAIN OUTCOME MEASURES: Cause of glaucoma in patients with ectropion uvea and NF-1. RESULTS: The age of patients at the time of glaucoma diagnosis ranged from birth to 13 years. Four of the 5 patients had megalocornea and buphthalmos at presentation. Ectropion uvea was noted clinically in 2 patients, but was demonstrated histopathologically in all 5 patients. On histopathologic examination, all patients had varying degrees of angle closure secondary to endothelialization of the anterior chamber angle. Uveal neurofibromas were noted in all patients; anteriorly displaced ciliary processes were noted in 4 of 5 patients who demonstrated ciliary body involvement with neurofibromas. Absence of Schlemm's canal was observed. The endothelial cells lining the closed angle demonstrated positive stain results with the vimentin antibody. Positive antineurofibromin immunolabeling was detected in normal control corneal endothelium, but was absent in corneal endothelium in patients with endothelialization of the angle. Upregulation of genes from the MAPK signaling pathway was demonstrated in the corneal endothelial cells isolated from the NF-1 eyes. CONCLUSIONS: Ectropion uvea in NF-1 glaucoma is secondary to endothelialization of the anterior chamber angle and is associated commonly with severe pediatric glaucoma in NF-1 patients. The endothelial cell proliferation may be related to overexpression of the Ras (Rat sarcoma)-MAPK genes in these eyes.

The hot orbit: orbital cellulitis.

Orbital cellulitis is an uncommon condition previously associated with severe complications. If untreated, orbital cellulitis can be potentially sight and life threatening. It can affect both adults and children but has a greater tendency to occur in the pediatric age group. The infection most commonly originates from sinuses, eyelids or face, retained foreign bodies, or distant soources by hematogenous spread. It is characterized by eyelid edema, erythema, chemosis, proptosis, blurred vision, fever, headache, and double vision. A history of upper respiratory tract infection prior to the onset is very common especially in children. In the era prior to antibiotics, vision loss from orbital cellulitis was a dreaded complication. Currently, imaging studies for detection of orbital abcess, the use of antibiotics and early drainage have mitigated visual morbidity significantly. The purpose of this review is to describe current investigative strategies and management options in the treatment of orbital cellulitis, establish their effectiveness and possible complications due to late intervention.

Unilateral eyelid swelling, proptosis and diplopia as initial manifestation of acute myeloid leukemia.

Myeloid sarcoma is a tumor of immature myeloid cells occurring in many extramedullary sites, orbit being one of them where the tumor may occur prior to or after the diagnosis of underlying disease. We report a case of a 17-year-old male who presented with upper eyelid swelling, proptosis and diplopia after presumed blunt trauma without any other clinical signs and symptoms. Initial imaging suggested possibility of subperiosteal hematoma. Magnetic resonance imaging studies demonstrated a solid tumor. Complete excision of the tumor and histopathologic diagnosis revealed evidence of acute myeloid leukemia (AML). There were no other sites indicating any tumoral process; however, bone marrow aspirate revealed an evidence of systemic disease. After chemotherapy and allogenic bone marrow transplant, the patient had complete remission of his disease. An episode of graft vs host reaction resulting in severe dry eyes along with skin eruptions was treated successfully by immunosuppression and topical lubrication without any visual or systemic sequela. This case emphasizes on the need for an aggressive work-up for any unusual orbital lesion in the absence of any explainable etiology. Further, AML may be suspected in the cases of orbital tumors even in the absence of systemic manifestations of leukemia at any age.

Unilateral optic neuritis, scleritis and exudative retinal detachment due to recurrent orbital pseudotumor.

Idiopathic orbital inflammation, also known as orbital pseudotumor, describes a spectrum of idiopathic, non-neoplastic, non-infectious, space-occupying orbital lesions without identifiable local or systemic cause. The condition occurs mainly in young adults who may present with acute pain, proptosis, chemosis and limitations of extraocular movements. Decreased vision due to optic nerve infiltration and macular edema as a result of scleritis is less common sequel of orbital pseudotumor. Herein, we present a case of unilateral orbital pseudotumor in a young male who presented with chief complaints of painful decreased vision which was attributed to optic neuritis and macular edema as a result of scleritis. Imaging studies were helpful in the establishment of the correct diagnosis and patient's complaints improved with administration of systemic corticosteroids.

Analysis and comparison of proteomic profiles of tear fluid from human, cow, sheep, and camel eyes.

PURPOSE: To investigate the tear proteome profiles of human, cow, sheep, and camel comparatively and to explore the difference of tear protein profiles among different species. METHODS: Tears were collected from both eyes of 25 clinically healthy volunteers, 50 cows, 25 sheep, and 50 camels. Pooled tear protein samples were separated by SDS-PAGE and two-dimensional electrophoresis. Protein spots of differential expression were excised and subjected to in-gel digestion and identification by matrix assisted laser desorption/ionization-time-of-flight/time-of-flight mass spectrum analysis. Because of the incomplete genomic data of cow, sheep, and camel, a combined strategy of de novo sequencing and BLAST (Best Local Alignment Search Tool) homology searching was also used for protein identification. The differentially expressed proteins were validated by Western blot analysis. RESULTS: On comparison with human tears (182 ± 6 spots), 223 ± 8, 217 ± 11, and 241 ± 3 well-resolved protein spots were detected in triphenylmethane dye-stained gels of cow, sheep, and camel tears, respectively. Similar high-abundant proteins (lactoferrin, lysozyme, etc.) were found in all tear fluids. Tear lipocalins have been identified in cow and sheep tears. BLAST searching revealed a 21-kDa protein, identical with human vitelline membrane outer layer protein 1 (VMO1) homolog, in camel tears. The Western blot confirmed that VMO1 homolog was present in both camel and sheep tears but not in human and cow tears. CONCLUSIONS: The comparative proteomic analyses of tears from healthy humans, cows, sheep, and camels were first reported. Differential protein expression existed in the tear among species, offering useful information for further study on tear proteins and the related ocular diseases.

Comparison of camel tear proteins between summer and winter.

PURPOSE: Proteins in the tear fluid have positive effects on maintaining the integrity and stabilization of the tear film, which is affected by several environmental factors. The aim of this study is to investigate seasonal variation of protein patterns in camel tears collected during the summer and winter season. METHODS: Tears from both eyes of 50 clinically normal camels (Camelus dromedarius) were collected in the summer (June-July) and in the winter (December-January) respectively. Pooled tear protein samples from two seasons were separated by SDS-PAGE and two-dimensional electrophoresis (2-DE). Protein spots of differential expression in two season gels were excised and subjected to in-gel digestion and identification by matrix assisted laser desorption/ionization-time of flight/time of flight-mass spectrum (MALDI-TOF/TOF-MS) analysis. Two differentially expressed proteins, lactoferrin (LF) and vitelline membrane outer layer protein 1 homolog (VMO1 homolog), were validated by western blotting. RESULTS: Thirteen well resolved bands were detected in SDS-PAGE gels of both summer and winter camel tears. By band densitometry, significantly higher intensities of band 6, 7, 11, and lower intensity of band 13 were observed in the summer group compared to the winter group. In 2-DE profiles of camel tears, four protein spots were found expressed differentially in two seasons. Further protein identification by MALDI-TOF/TOF-MS and confirmation by western blotting indicated that there was a significant decrease in LF (p=0.002) and an increase in VMO1 homolog (p=0.042) in tears in the summer compared to the winter. CONCLUSIONS: The seasonal variation of camel tear fluids has been found in the composition of proteins, including LF and VMO1 homolog. This result will expand our knowledge of physiologic characteristics of tear fluids and establish a foundation for the mechanistic studies and clinical practices on ocular surface disorders.

Bilateral infectious keratitis after implantation of intrastromal corneal ring segments.

PURPOSE: To report the development of bilateral infectious keratitis after implantation of intrastromal corneal ring segments (ICRSs). DESIGN: Retrospective case report. PARTICIPANTS: A 20-year-old woman presented with photophobia, decreased vision, and pain 11 days after uncomplicated implantation of ICRSs for keratoconus in both eyes. Bilateral corneal stromal infiltrates were noted at the site of ICRSs implantation. INTERVENTION: The patient was started on frequent topical fortified antibiotics in both eyes. Despite aggressive medical management, stromal infiltrates progressed, necessitating removal of ICRSs from both corneas to control infectious keratitis and melting of cornea. METHODS: Retrospective case report of a 20-year-old woman who developed bilateral severe infectious keratitis 11 days after uncomplicated implantation of ICRSs for keratoconus. RESULTS: Cultures obtained at the time of initial presentation yielded Streptococcus viridans. Patient responded well to the treatment and was left with stromal scars in both corneas. CONCLUSIONS: Although rare, simultaneous implantation of ICRSs may carry a risk of severe bilateral infectious keratitis. Early recognition of infection, aggressive treatment with antibiotics, and, in some cases, removal of ICRSs may be necessary to prevent serious sight-threatening complication of this refractive procedure.

Eradicating blinding trachoma: What is working?

Trachoma remains the leading cause of preventable corneal blindness in developing countries. The disease is contracted in early childhood by repeated infection of the ocular surface by C. trachomatis. Initial clinical manifestation is a follicular conjunctivitis which if not treated on timely basis, may lead to conjunctival and eyelid scarring that may eventually result in corneal scarring and loss of vision. Over the past two decades, a remarkable reduction in the prevalence of active trachoma has occurred due to the World Health Organization's (WHOs) program GET 2020 for the elimination of trachoma with adoption of the SAFE strategy incorporating Surgery, Antibiotic treatment, Facial cleanliness and Environmental hygiene. However, patients who already had infection at young age may present with adnexal-related complications of trachomatous scarring that may cause corneal scarring and visual loss. These patients may present with evidence of trichiasis/entropion as well as eyelid retraction. Lacrimal complications may include nasolacrimal-duct obstruction, dacryocystitis and canaliculitis requiring intervention. In addition to the increased risk for corneal scarring, trichiasis/entropion may further increase the risks for microbial keratitis in patients who may have unrecognized dacryocystitis and canaliculitis. Female patients may have more trachomtous-related complications and may present at an early age. Available evidence indicates that SAFE strategy may be effective and on the right track towards achieving GET 2020 goal for the eradication of trachoma.