Clinical and demographic characteristics of childhood neuro-ophthalmology diseases at a tertiary eye care center.

BACKGROUND: To evaluate the demographic, etiological, and clinical properties, as well as the treatment modalities of neuro-ophthalmological diseases in childhood. METHODS: We retrospectively analyzed the clinical data of patients younger than 18 years old who were referred to the Neuro-Ophthalmology Department of Ulucanlar Eye Hospital from 2004 to 2019. RESULTS: Of 1,910 patients who presented to the Neuro-Ophthalmology Department, 128 (6.7%) were younger than 18 years old at diagnosis, and their data were analyzed. The three most common diagnoses were congenital optic disc (OD) abnormalities in 43 (33.5%), optic neuropathies in 42 (32.8%), and idiopathic intracranial hypertension in 11 (8.5%) patients. The most frequent symptoms were as follows: decreased visual acuity in 36 (28.1%), headache in 32 (25%), and no symptoms in 19 (14.8%) patients. The best visual prognosis was associated with inflammatory optic neuritis, while hereditary and compressive optic neuropathy resulted in poor visual acuity outcomes. CONCLUSIONS: Congenital OD abnormalities and optic neuropathies are the most frequently seen disorders among children with neuro-ophthalmological diseases. Clinicians should also be aware that children without any symptoms may also have neuro-ophthalmological disorders.

Real-World Clinical Experience With Idebenone in the Treatment of Leber Hereditary Optic Neuropathy.

BACKGROUND: Leber hereditary optic neuropathy (LHON) leads to bilateral central vision loss. In a clinical trial setting, idebenone has been shown to be safe and to provide a trend toward improved visual acuity, but long-term evidence of effectiveness in real-world clinical practice is sparse. METHODS: Open-label, multicenter, retrospective, noncontrolled analysis of long-term visual acuity and safety in 111 LHON patients treated with idebenone (900 mg/day) in an expanded access program. Eligible patients had a confirmed mitochondrial DNA mutation and had experienced the onset of symptoms (most recent eye) within 1 year before enrollment. Data on visual acuity and adverse events were collected as per normal clinical practice. Efficacy was assessed as the proportion of patients with either a clinically relevant recovery (CRR) or a clinically relevant stabilization (CRS) of visual acuity. In the case of CRR, time to and magnitude of recovery over the course of time were also assessed. RESULTS: At time of analysis, 87 patients had provided longitudinal efficacy data. Average treatment duration was 25.6 months. CRR was observed in 46.0% of patients. Analysis of treatment effect by duration showed that the proportion of patients with recovery and the magnitude of recovery increased with treatment duration. Average gain in best-corrected visual acuity for responders was 0.72 logarithm of the minimal angle of resolution (logMAR), equivalent to more than 7 lines on the Early Treatment Diabetic Retinopathy Study (ETDRS) chart. Furthermore, 50% of patients who had a visual acuity below 1.0 logMAR in at least one eye at initiation of treatment successfully maintained their vision below this threshold by last observation. Idebenone was well tolerated, with most adverse events classified as minor. CONCLUSIONS: These data demonstrate the benefit of idebenone treatment in recovering lost vision and maintaining good residual vision in a real-world setting. Together, these findings indicate that idebenone treatment should be initiated early and be maintained more than 24 months to maximize efficacy. Safety results were consistent with the known safety profile of idebenone.

Unilateral optic perineuritis due to nonspecific orbital inflammation: A case report.

Nonspecific orbital inflammation is an idiopathic chronic tumor-like inflammation process that usually affects the orbital tissues of both eyes. Isolated optic nerve involvement by an idiopathic inflammatory process is a rare finding. Here, we report a patient with unilateral sudden onset severe orbital pain and headache with visual loss to no light perception that could only be attributed to perineuritis. Radiological findings enabled making the correct diagnosis. Various relevant diagnoses are also discussed.

A histopathological investigation of Tenon's capsule in diabetic eyes.

The aim of this study is to investigate the histopathological features of Tenon's capsule in eyes with diabetic macular oedema and to compare them between diabetic eyes and healthy subjects. The study included 26 eyes with diabetic oedema and 17 healthy eyes as healthy controls. Tenon's capsule biopsy specimens were processed with the routine electron microscopic analysis technique. Type I and III collagen fibres were labelled immunohistochemically to determine the amounts of predominating collagen fibres. Leica Q-Win program was used to calculate the amounts of collagen fibres type I and type III and independent-t test was utilized to compare the obtained results between the groups. Statistical significance was set at p < 0.05. Demographic characteristics of both groups were similar (p > 0.05). Collagen type I and type III immunoreactivity was observed both in the control and the diabetic groups. The Amounts of collagen fibres type I and type III were significantly higher in the diabetic group than in the control group (mean collagen type I area: 13.410 ± 0.99 and mean collagen type III area: 23.692 ± 0.17 in the control group; mean collagen type I area: 25.270 ± 6.48 and mean collagen type III area: 28.192 ± 0.82 in the diabetic group. p = 0.0037 for type I and p = 0.0000 for type III). In light of the findings of this study, it can be assumed that diabetes mellitus may engender increased amounts of collagen in Tenon's capsule. This alteration affecting the success of filtration surgery should be kept in mind especially in diabetic eyes with glaucoma.

Blepharoptosis and hypertrophic osteoarthropathy: A case report.

A 52-year-old male patient presented to our hospital with a history of secondary hypertrophic osteoarthropathy (HOA) associated with an abdominal neoplasia and blepharoptosis. He had finger clubbing, hyperhidrosis, and hypertrichosis. He also had a recent history of extensive abdominal surgery with a pathology report of myelolipoma. Routine blood work was unremarkable. Upper eyelid reconstruction with blepharoplasty, upper eyelid wedge resection, and brow suspension was performed to address his eyelid concerns. By this case report, we would like to attract notice that the eyelid involvement may be a part of HOA and to emphasize the importance of systemic and pathologic evaluation in failed blepharoptosis surgery.

Alterations in anterior chamber depth in primary open-angle glaucoma patients during latanoprost therapy.

PURPOSE: The aim of this study was to evaluate changes in the anterior chamber depth (ACD) in primary open-angle glaucoma (POAG) patients during latanoprost therapy. METHODS: We carried out a prospective study in which we enrolled 66 newly diagnosed POAG patients treated with latanoprost 0.005% (group 1) and 50 ocular hypertensive and / or glaucoma suspect cases who were given no therapy (group 2 [control]). Measurements of the ACD were performed by A-scan ultrasonography before and after cycloplegia at baseline and at 3 months of latanoprost therapy. Differences in ACD and their correlations with the ocular hypotensive effect of the agent as well as the clinical significance of changes in ACD were analysed using Student's t-test and Pearson's correlation coefficient. Statistical significance was set at p < 0.05. RESULTS: Mean baseline ACD was 3.13 ± 0.35 mm (range 2.45­3.84 mm) in group 1 and 3.14 ± 0.36 mm (range 2.54­3.80 mm) in group 2 (p = 0.89). At 1 hour after instillation of cyclopentolate 1%, mean ACD in groups 1 and 2 was 3.18 ± 0.38 mm (range 2.45­3.92 mm) and 3.19 ± 0.37 mm (range 2.56­3.91 mm), respectively (p = 0.91). After 3 months of treatment, mean ACD in group 1 both without (3.05 ± 0.36 mm, range 2.14­3.76 mm) and with (3.09 ± 0.4 mm, range 2.20­3.96 mm) cycloplegia was significantly reduced compared with baseline values (p < 0.001 for both). However, there was no significant difference between mean ACD at baseline and that at month 3 in group 2. No correlation was demonstrated between the changes in ACD and the ocular hypotensive effect of latanoprost (p = 0.96, r = ­ 0.006). There were no changes in refractive status or visual acuity. CONCLUSIONS: The overall results seem to suggest that latanoprost decreases mean ACD in patients with POAG. The clinical significance of this effect is uncertain.

Aneurysmal bone cyst of the ethmoid presenting with proptosis and epiphora.

An aneurysmal bone cyst of ethmoid is a rare and distinct clinicopathologic entity. The diagnosis is important in this localization because it requires multidisciplinary approach in its management. We report a case of a 14-year-old woman presenting to our clinic with the complaint of epiphora and proptosis in the right eye, which were the symptomatic findings of aneurysmal bone cyst of ethmoid.

Localized Cystic Enlargement of the Distal Optic Nerve Sheath after Decompression Surgery.

Cystic formation of the optic meninges may occur after optic nerve sheath decompression surgery for the management of pseudotumor cerebri. A case with a cyst-like structure of the distal optic nerve sheath at the operation site in the late surgical period after nerve sheath decompression surgery is reported.

Is there a correlation between diabetes mellitus and central corneal thickness?

PURPOSE: To evaluate the association of central corneal thickness (CCT) with diabetes mellitus and compare it with age and sex-matched healthy controls. MATERIALS AND METHODS: This study included 245 eyes of 245 subjects (one eye per subject). One hundred diabetic patients constituted the study group and 145 were healthy controls. The study group was subdivided into 3 subgroups: subgroup 1 (no diabetic retinopathy), subgroup 2 (nonproliferative diabetic retinopathy), and subgroup 3 (proliferative diabetic retinopathy). CCT was determined with ultrasonic pachymeter. None of the patients had glaucoma, retinal laser treatment, and history of ocular surgery. Statistical analyses were performed by analysis of variance and Kruskall-Wallis tests. Correlation analysis was performed to assess the association between disease duration and glycosylated hemoglobin levels among subgroups. RESULTS: Demographic characteristics of study and control groups were similar (P>0.05). The mean CCT was significantly greater in study group (564±30 µm) compared with control group (538±35 µm) (P=0.001). In addition, mean CCT was found to be greater in subgroup 3 (582±23 µm) compared with subgroups 1 (565±32 µm) and 2 (558±31 µm); but the difference did not reach statistical significance (P=0.056). Also, there was no significant correlation in respect to the level of glycosylated hemoglobin and disease duration among the subgroups. CONCLUSIONS: We found that the central cornea of diabetic patients is thicker when compared with nondiabetic patients. Thicker central cornea associated with diabetes mellitus should be taken into consideration while obtaining accurate intraocular pressure measurements in diabetics.

Combined cilioretinal artery and partial central retinal vein occlusion in the postpartum period.

PURPOSE: To report a combined cilioretinal artery and partial central retinal vein occlusion shortly after delivery in a twin-pregnant woman. METHODS: A 25-year-old woman presented to our clinic with the complaint of blurred vision in the right eye 1 week after delivery. She underwent detailed ophthalmic and systemic investigations. RESULTS: The patient had an uncomplicated twin pregnancy. Ocular examination showed combined cilioretinal artery and partial central retinal vein occlusion in the right eye. Systemic diseases were excluded. Laboratory evaluations revealed increased plasma d-dimer level (1.64 mg/mL). Spontaneous recovery occurred without treatment 1 month after delivery. CONCLUSION: Twin pregnancy may lead to exaggerated hypercoagulability and increased d-dimer level in pregnant women. This situation may be a risk factor for retinochoroidal vascular occlusions.

Cavernous sinus syndrome as the initial presentation of childhood non-hodgkin lymphoma.

A 10-year-old boy with unilateral ophthalmoplegia, ptosis, and proptosis underwent diagnostic examination. Symptoms of headache, nausea, dyspnea, fatigue, weakness, and loss of appetite began 14 days after the onset of ocular manifestations and 7 days before he was examined. Complete blood count showed an increased white blood cell count with 64% blast cells, anemia, and thrombocytopenia. The patient was transferred to a pediatric hematology unit, where he underwent bone marrow aspiration biopsy. Bilateral ocular inflammatory findings and left-sided mild proptosis became evident. He was diagnosed as having B-cell non-Hodgkin lymphoma infiltrating the bilateral cavernous and sphenoid sinuses.

Retinopathy in patients with diabetic ophthalmoplegia.

PURPOSE: To compare patients with palsy of the 3rd, 4th or 6th cranial nerve due to diabetes mellitus (DM) with controls matched for age, sex and disease duration in terms of the presence and severity of diabetic retinopathy. PATIENTS AND METHOD: Thirty-three diabetic patients with cranial nerve palsies and 33 age- and sex-matched controls with the same duration of disease were included in this case-control study. Groups were evaluated in terms of the type of DM, presence and level of diabetic retinopathy and coexisting systemic diseases. Outcomes were compared by chi(2) and paired-sample tests. RESULTS: Eleven patients had diabetic retinopathy in the study group. In the control group, 23 patients had diabetic retinopathy. Differences in the presence and severity of diabetic retinopathy between the two groups were found to be statistically significant (p = 0.028 and p = 0.018, respectively). CONCLUSION: Presence and level of diabetic retinopathy are significantly lower in diabetics with cranial nerve palsy than in the age-, sex- and disease-duration-matched controls.

Keratoconjunctivitis sicca in juvenile rheumatoid arthritis.

PURPOSE: To compare the symptoms, signs, and results of objective tests for keratoconjunctivitis sicca (KCS) in patients with juvenile rheumatoid arthritis (JRA) and controls. METHODS: Sixty-four patients with JRA and 64 age- and sex-matched controls were compared in terms of symptoms, signs, and results of objective tests for KCS. Relation between tear film breakup time (TBUT), Schirmer test results, and JRA-related variables such as age of onset, duration, and type of JRA; presence of antinuclear antibodies (ANAs); and history of uveitis were evaluated. Analysis of variance, multivariate regression analysis, Kruskall-Wallis, Student t tests, and chi tests were used for statistical analysis. RESULTS: Twelve and a half percent of patients with JRA complained of dry eye symptoms compared with 1.5% of the controls (P = 0.031). Dry eye signs were detected in 10.9% of patients with JRA compared with 1.5% of controls (P = 0.038). TBUT and Schirmer test results were lower in the JRA group than in controls (P = 0.032 and P = 0.029, respectively). Seven patients (10.9%) had definite and 1 (1.5%) had probable diagnosis of KCS in the JRA group compared with no children in the control group (P = 0.034). Within the JRA group, Schirmer test and TBUT results were significantly lower in male patients and ones with longer duration of disease. CONCLUSIONS: The prevalence of symptoms, signs, and definite diagnosis of KCS is higher and basal tear secretion and tear film stability are lower in children with JRA than in controls. Among children with JRA, male sex and longer duration of disease are independent risk factors for having decreased basal tear secretion and tear film stability.

Refractive errors in neurofibromatosis type 1 and type 2.

OBJECTIVE: To document the prevalence of refractive errors in patients with neurofibromatosis type 1 (NF1) and type 2 (NF2) and to compare it with that of age- and sex-matched controls. METHODS: 82 patients with NF1, 21 patients with NF2 and 103 age- and sex-matched controls were evaluated in this prospective observational case-control study. Cycloplegic autorefraction and dilated fundus examination were performed. Myopia was defined as the spherical equivalent refraction of at least -0.50 diopters (D), hyperopia as the spherical equivalent refraction of at least 2.0 D and astigmatism as the cylinder of at least 1.0 D. Main outcome measures were refractive error, IQ, years of education, height, weight and body mass index (BMI). RESULTS: The prevalence of myopia was 23.1% in patients with NF1, 23.8% in patients with NF2 and 16.5% in age- and sex-matched controls. These differences were significant (p<0.03, p<0.03), and adjusting for intelligence, education, height, weight and BMI increased the significance of this finding (p<0.001, p<0.001). The prevalences of astigmatism and hyperopia were similar in both groups. CONCLUSION: A high prevalence of myopia seems to be an additional feature of NF1 and NF2.

Graves' ophthalmopathy: comparison of the Doppler sonography parameters with the clinical activity score.

PURPOSE: Our aim was to compare the Doppler ultrasonography (US) parameters with clinical activity score (CAS) in patients with Graves' ophthalmopathy. METHODS: Blood flow parameters in the ophthalmic artery (OA), superior ophthalmic vein (SOV), central retinal artery (CRA) and central retinal vein (CRV) were measured by Doppler US in 118 patients with Graves' disease in 3 groups (A, B, and C) and 20 control subjects. Group A included 25 patients with CAS value "0"; group B 55 patients with CAS value "1 or 2"; group C 38 patients with CAS value "3 or above." RESULTS: Peak systolic velocities (PSV) and end-diastolic velocities (EDV) in the OA, PSV, and in the CRA calculated for group C were significantly higher than for group A, group B, and the control group. Resistance index in the OA and maximal velocity (MV) in the SOV calculated for group C were significantly lower than those calculated for group A, group B, and the control group. CONCLUSION: Doppler parameters of MV in SOV, PSV and EDV in OA, and PSV in CRA seem to be helpful in the differentiation of active and inactive phases of Graves' ophthalmopathy.

Projected color slides as a method for mass screening test for color vision deficiency (a preliminary study).

This article compared the efficiency of the mass screening test with projected color slides in detecting color-blindness with the authentic classic method of Ishihara. The study was conducted in a randomly selected lecture room with 104 students aged between 19-25 years (median 21). Using Ishihara projected slides, performed mass screening test. Re-testing was done individually with printed Ishihara plates. Six male and one female with color-blindness were detected. The frequency of color-blindness was 13.6% among males, with a total incidence of 6.7%. The results of two testing methods were compared statistically. Sensitivities and specificities of both tests were 100%. Using projected slides of Ishihara plates instead of the authentic method is an effective and timesaving method for detecting color-blindness. This method can be suggested as a mass-screening test and might be beneficial in detecting color-blindness in large populations such as students, soldiers, and so on.

Congenital color blindness in young Turkish men.

We investigated a healthy population of men from different regions of Turkey for the presence of congenital red-green color blindness. Using Ishihara pseudoisochromatic plates, 941 healthy men from the Turkish army were tested for congenital red-green color blindness. The prevalence of red-green color blindness was 7.33 +/- 0.98% (5.10% protans and 2.23% deutans). These ratios were higher than other reported samples from Mediterranean Europe. Higher percentages of color blindness were found in regions with a lower education level and more consanguineous marriages.

Monocular temporal hemianopia in a young patient.

A 12-year-old girl presented with a history of sudden visual hemi-field loss in the left eye. The visual field defect was a clear-cut temporal hemianopia in the left eye; eye was normal. Complete eye examination and neuro-imaging of brain and opto-chiasmal region revealed normal structural findings. The visual field defect was suspected to be non-organic. This assumption was proven to be the diagnosis when simultaneous binocular fields showed the same pattern, although the contralateral eye's nasal hemi-field was intact. This symptom was alleviated by reassurance and placebo treatment.