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.

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.

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.

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.

Glaucoma and globe enlargement associated with neurofibromatosis type 1.

OBJECTIVE: To describe the features of glaucoma and globe enlargement sometimes associated with neurofibromatosis type 1 (NF1). DESIGN: Single institution, retrospective, and cross-sectional study. PARTICIPANTS: Eighty medical records of patients treated at King Khaled Eye Specialist Hospital in Saudi Arabia with NF1 were reviewed, and 46 patients were examined. METHODS: We reviewed the charts of patients with NF1 and examined available individuals, including gonioscopy, axial length, and ultrasound biomicroscopy in appropriate patients. MAIN OUTCOME MEASURES: Presence and type of glaucoma, anterior chamber angle abnormalities, globe axial length, ultrasound biomicroscopy, and visual outcome. RESULTS: Two patient groups were identified: Group 1 had 56 patients with orbito-facial NF1, and group 2 had 24 patients with NF1 but without orbito-facial involvement. Group 1 included 13 patients with glaucoma (23%), which occurred only ipsilateral to the orbito-facial involvement and generally presented before age 3 years. Glaucoma surgery was required in all of these patients, and visual prognosis was poor. In group 1, mean axial length on the side affected by NF1 was 29.8+/-4.1 mm in patients with glaucoma and 25.6+/-2.0 mm in patients without glaucoma. Patients with glaucoma (P<0.001) and without glaucoma (P<0.0001) in group 1 had significantly larger globes on the affected side. Group 2 patients had a mean axial length of 23.6+/-1.6 mm for both eyes without significant globe asymmetry. CONCLUSIONS: In this Arab population, glaucoma associated with orbito-facial NF1 occurred less often than the 50% rate that is typically cited. Glaucoma presented early in life and only in patients with ipsilateral orbito-facial involvement. Glaucoma in this setting was always associated with globe enlargement. Glaucoma required surgery, and visual prognosis was poor because of glaucoma and concurrent pathology. Globe enlargement was most severe when associated with glaucoma but also present on the side with orbito-facial involvement in patients without glaucoma. The presence of neurofibroma may induce globe enlargement on the affected side in orbito-facial NF1. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Corneoscleral necrosis after episcleral Au-198 brachytherapy of uveal melanoma.

PURPOSE: To describe the risk factors for the development of corneoscleral necrosis (CSN) and its management in patients with primary choroidal or ciliochoroidal melanoma who underwent episcleral Au-198 brachytherapy. METHODS: Clinical records of patients with symptoms of dry eye, foreign body sensation, pain, and evidence of CSN after Au-198 brachytherapy for uveal melanoma treated over a 22-year period were reviewed retrospectively. Risk factors for the development of CSN were identified and various methods of management were evaluated. The data were analyzed using multivariant analysis. A P < 0.05 was taken as a level of statistical significance. RESULTS: Of the 202 eyes of 202 patients treated with Au-198 radioactive plaque, 15 (7.4%) patients with symptomatic complaints of dry eye and pain showed evidence of CSN. First signs were noted as early as 1 month to as late as 5 years (median time 5 months) after the treatment. Risk factors for the development of CSN included tumor thickness greater than 6 mm and ciliary body involvement (P < 0.05). Associated risk factor included intraocular pressure greater than 21 mmHg. Four patients required conservative management, 11 patients required scleral patch and/or conjunctival flaps, and 6 eyes eventually required enucleation. Eyes which developed CSN were more likely to undergo enucleation compared with eyes having no evidence of CSN (P < 0.05). None of the eyes with CSN, which required enucleation because of the failed treatment, had histopathologic evidence of recurrent tumor or tumor invading sclera. CONCLUSION: Corneoscleral necrosis may occur soon or several years after Au-198 brachytherapy for uveal melanoma. Risk factors for CSN include tumor thickness greater than 6 mm, ciliary body involvement, and intraocular pressure >21 mmHg. Closer follow-up, early recognition, and timely intervention may avert serious consequences.

Acute endophthalmitis caused by Staphylococcus lugdunesis after intravitreal bevacizumab (Avastin) injection.

PURPOSE: To report a case of culture-positive endophthalmitis after intravitreal injection of bevacizumab (Avastin) resulting in a devastating visual outcome. METHOD: A retrospective case report of a 51-year-old diabetic women who presented with further decrease in her vision, redness, and mild pain in her eye 3 days after intravitreal injection of Avastin for macular edema due to a branch retinal vein occlusion. RESULTS: Clinical diagnosis of endophthalmitis was made and, after obtaining a vitreous tap, intravitreal antibiotics were administered. Because of worsening of the endophthalmitis, pars plana vitrectomy was undertaken followed by repeat intravitreal antibiotics injection. The patient's ocular condition improved dramatically; however, her visual acuity did not improve. The cultures from vitreous taps revealed Staphylococcus lugdunesis. CONCLUSION: Intravitreal injection of Avastin may be associated with a risk of S. lugdunesis-caused endophthalmitis which can have a devastating effect on the final visual outcome.

Outcome of treated orbital cellulitis in a tertiary eye care center in the middle East.

PURPOSE: To describe risk factors predisposing patients to orbital cellulitis and potential complications in patients treated at a tertiary eye care referral center in the Middle East. DESIGN: Noncomparative, interventional, retrospective case series. PARTICIPANTS: Patients diagnosed with orbital cellulitis. METHODS: A 15-year clinical review of patients with a diagnosis of orbital cellulitis referred to King Khaled Eye Specialist Hospital, an accredited (Joint Council on Accreditation of Healthcare Organizations, Washington, DC) tertiary care center in Riyadh, Saudi Arabia, was performed. Only those patients who had clinical signs and symptoms or radiologic evidence suggestive of orbital cellulitis were included in the study. MAIN OUTCOME MEASURES: Patient demographics, factors predisposing to orbital cellulitis, and resulting complications. RESULTS: A total of 218 patients (136 male, 82 female) fulfilling the diagnostic criteria for orbital cellulitis were identified. The average age of these patients was 25.7 years (range, 1 month-85 years). On imaging studies, there was evidence of inflammatory or infective changes to orbital structures; orbital abscesses were identified in 116 patients (53%). Sinus disease was the most common predisposing cause in 86 patients (39.4%), followed by trauma in 43 patients (19.7%). All patients received systemic antibiotic treatment before the identification of any responsible organisms. Of the 116 patients with orbital abscess, 101 patients (87%) required drainage. The results of cultures in patients in whom an orbital abscess was drained were positive for 91 patients (90%). The most common microorganisms isolated from the drained abscesses were Staphylococci and Streptococci species. Blood cultures were positive in only 4 patients from whom blood was drawn for cultures. Visual acuity improved in 34 eyes (16.1%) and worsened in 13 eyes (6.2%), including 9 (4.3%) eyes that sustained complete loss of vision, which was attributed to the delay in correct diagnosis and timely intervention (average 28 days vs. 9 days in patients with no loss of vision; P<0.05). There were 9 cases of intracranial extension of orbital abscesses that required either extended treatment with systemic antibiotics alone or in combination with neurosurgical intervention. Most patients received oral antibiotics on discharge for varying periods. There were 6 cases (2.7%) of strabismus and 4 cases (1.8%) of ptosis that persisted after treatment and resolution of orbital cellulitis. CONCLUSIONS: Untreated sinusitis and prior history of orbital trauma were the 2 major causes of orbital cellulitis in patients referred to a tertiary care eye center in the Middle East. Although rare, severe visual loss still remains a serious complication of delayed detection and intervention in most cases of orbital cellulitis.

Initial diagnosis of leprosy in patients treated by an ophthalmologist and confirmation by conventional analysis and polymerase chain reaction.

PURPOSE: To report the initial diagnosis of leprosy in patients seeking treatment from an ophthalmologist in a tertiary eye care center, its confirmation by histopathologic and polymerase chain reaction analysis, and review of literature. DESIGN: Noncomparative retrospective case series. PARTICIPANTS: Patients with no known history of leprosy who were selected based on either a clinical suspicion or a histopathologic diagnosis and were found to have ocular or periocular lesions suggestive of leprosy. METHODS: Review of clinical records of patients with no known history of leprosy seen by an ophthalmologist at a tertiary eye care referral center. MAIN OUTCOME MEASURES: Patient demographics, presenting symptoms and signs, diagnostic studies, complications, and treatment. RESULTS: Among the 6 patients (5 women and 1 man; average age, 55 years), only 2 were found to have leprosy from clinical examination alone. Histopathologic characteristics or demonstration of acid-fast bacilli, suggestive of leprosy, were found in 5 patients. Definite confirmation of leprosy was made by polymerase chain reaction performed on formalin-fixed, paraffin-embedded tissues of 4 patients having suspected leprosy based on clinicohistopathologic examination results. CONCLUSIONS: The diagnosis of leprosy relies on the clinical symptom complex, epidemiologic factors, and demonstration of acid-fast bacilli in the tissue sample. Considering that the global leprosy population is 12 million, a patient with leprosy may be found anywhere. The ophthalmologist may be the first one to encounter such patient, in which case suspicion and detection of ocular findings may lead to early treatment of the infection. Polymerase chain reaction may be a new tool in the definite diagnosis of leprosy when suspicion of the diseases is raised by clinicohistopathologic studies.

Epidemiology of giant-cell arteritis in an Arab population: a 22-year study.

OBJECTIVE: To study the epidemiology of biopsy-proven giant-cell arteritis (GCA) in an Arab population with clinical features suggestive of GCA. METHODS: Clinical records of 102 patients who underwent temporal artery biopsy (TAB) at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia from January 1983 to December 2004 were reviewed. RESULTS: Among the 56 men and 46 women who had TAB, 7 (6.8%) had biopsy-proven GCA. The mean (SD) age of the biopsy-positive group (71.7 (8.6) years) was higher than in the biopsy-negative group (65 (9.8) years; p = 0.005). None of the untreated patients with a negative TAB developed additional symptoms that are typical of GCA. CONCLUSION: The small absolute number of biopsy-proven cases of GCA implies a relatively low incidence of GCA in Arab population, confirming previous observations that GCA occurs primarily in the Caucasian population.

Current indications and resultant complications of evisceration.

PURPOSE: Evisceration is an alternative treatment modality to enucleation for many end-stage eye diseases. No study has addressed the indications for evisceration of eyes in Saudi Arabia. The aim of this study was to determine the current clinical indications for evisceration in patients at a tertiary eye care center and attempt clinicopathological correlation. METHODS: Clinical records of patients who had undergone evisceration at a tertiary eye care center over a 4-year period were reviewed retrospectively. The patients' demographic data and clinical indications for evisceration were studied, and the results from histopathological findings were correlated with the clinical diagnosis. RESULTS: Evisceration of the eyes was performed in 187 patients. Males outnumbered females in a ratio of 1.3:1 (105 males and 82 females). Blind painful eye was the primary presenting symptom in 117 (62.6%) patients and unsightly eye in 38 (20.3%) patients. Clinical indications for evisceration included endophthalmitis in 85 (45.5%), phthisis bulbi in 38 (20.3%), traumatic injury in 36 (19.2%), and glaucoma in 14 (7.5%) patients. Sixty-three patients (33.7%) had prior history of cataract surgery, penetrating keratoplasty, glaucoma surgery, or retina surgery. Clinicopathological correlation was 100% in cases with definite clinical diagnosis of endophthalmitis. CONCLUSION: Blind painful eye, endophthalmitis, phthisis bulbi, severe traumatic injury, and glaucoma were the major indications for eviscerations in a tertiary eye care center.

L-carnitine protects human retinal pigment epithelial cells from oxidative damage.

PURPOSE: To determine the efficacy of L-carnitine (LC) against oxidative changes in human retinal pigment epithelium (RPE) cells. METHODS: The RPE cells from human donor eyes were cultured in Hams F-10 medium. The effect of LC on H2O2-induced morphologic changes in the RPE cells was analyzed by light microscopy. Reduction in cell death after the impact of LC treatment on H2O2-treated cells was analyzed by MTT [3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide] assays. In addition, the effect of H2O2 on the activity of RPE-antioxidant enzymes, glutathione (GSH) and superoxide dismutase (SOD), and LC-induced protection was also determined. RESULTS: LC protected the RPE cells by inhibiting the peroxide-induced cytopathic effect from 50% to 10%. Nuclear condensation observed in 40% of the H2O2-treated cells decreased to 20% after LC treatment. The MTT assays demonstrated that 100 microM oxidant caused appreciable cell death, which was reduced by LC treatment; however, 100% protection was not achieved. Significant peroxide-induced cell death was seen within 5 hr of H2O2 treatment, and a quantifiable reduction was observed after LC treatment for a similar time period. The change in the antioxidant potential of the RPE induced by oxidative stress was restored by LC treatment, as demonstrated by an increase in GSH and SOD activities. CONCLUSIONS: LC is capable of protecting the RPE cells from H2O2-induced oxidative damage, implying that micronutrients can have a positive effect and can play an important role in the treatment of oxidation-induced ocular disorders. Further studies are needed to understand the mechanism of LC-induced protection to the RPE cells.

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.

Severe ocular injuries from pointed door handles in children.

OBJECTIVE: To report severe ocular injuries caused by pointed door handles in children. DESIGN: Noncomparative, interventional, retrospective case series. METHODS: Clinical records of 15 patients who sustained severe ocular injuries from door handles were reviewed for place of trauma, presenting symptoms and signs, surgical procedures performed, and final outcome. MAIN OUTCOME MEASURES: Trauma caused by complications from the door handle injury, such as loss of vision and ocular and adnexal injuries, and treatment rendered. RESULTS: There were 12 boys and 3 girls with an average age of 7.8 years (range, 6-10 years). Place of trauma was at home in 12 children and in school in 3 children. Presenting visual acuity (VA) was 4/200 in 1 patient, light perception (LP) in 5 patients, and no light perception (NLP) in 9 patients. There were 9 eyelid or canalicular lacerations, 4 ruptured globes, and 14 optic nerve avulsions. All ruptured globes were repaired initially and eyelid and canalicular lacerations were repaired primarily within 24 hours of presentation. Average follow-up was 21 months (range, 1 month-8 years). Final VA was 20/30 in 1 patient, LP in 1 patient, and NLP in 13 patients. Eight eyes required enucleation for painful blind eye or to achieve optimal cosmesis. CONCLUSIONS: Pointed door handles installed at current heights may pose a significant risk of ocular and periocular injuries among young children.

Juvenile xanthogranuloma of the corneoscleral limbus: case report and review of the literature.

Juvenile xanthogranuloma is a benign histiocytic cutaneous disorder mainly occurring in infants which may affect the eye. Ocular manifestations usually present in the form of iris lesions with secondary hyphema and glaucoma, but juvenile xanthogranuloma may also present as a corneoscleral limbal mass. We present the case of an 18-month-old female infant with ocular juvenile xanthogranuloma occurring as a corneoscleral limbal mass without associated cutaneous or systemic findings. The limbal mass lesion was excised by lamellar dissection and histopathological studies revealed histiocytes admixed with lymphocytes, plasma cells, and eosinophils; foreign body and Touton giant cells present throughout the specimen confirmed the diagnosis of juvenile xanthogranuloma. At 14-month follow-up, there was no recurrence of the limbal mass. The natural history of this uncommon ocular condition is discussed and the relevant literature reviewed. Juvenile xanthogranuloma should be considered in the differential diagnosis of any corneoscleral limbal mass lesion, particularly in children.

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.