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Purpose: To determine whether the corneal arcus affects sealing of the clear corneal incision (CCI) in cataract surgery. Patients and Methods: This study was a retrospective cohort study. The patients were divided into two groups based on whether stromal hydration was required to close the CCI. The corneal arcus was classified according to its degree as none, partial (if corneal arcus < 180 degrees), and circumferential (if corneal arcus ≥ 180 degrees). Multiple logistic regression was conducted to identify independent variables such as age at cataract surgery, sex, laterality, surgical time, and degree of corneal arcus associated with CCI sealing. Results: Among a total of 83 eyes of 83 patients, a partial corneal arcus was found in 34 eyes (41.0%) and a circumferential arcus in 33 eyes (39.8%). Multiple logistic regression analysis revealed that the need for stromal hydration in wound sealing increased with surgical time (OR = 1.7313, 95% CI = 1.1500– 2.6063, p = 0.0085) and decreased with severity of corneal arcus (partial, OR = 0.2901, 95% CI = 0.0451– 1.8665, p=0.1926; circumferential, OR = 0.0590, 95% CI = 0.0074– 0.4722, p = 0.0085); age was not associated (OR = 0.9790, 95% CI = 0.9121– 1.0507, p = 0.5555). Conclusion: Eyes with corneal arcus required less stromal hydration. This finding suggests that corneal arcus may increase CCI sealing in cataract surgery. Plain Language Summary: Corneal arcus is found in elderly persons, but its significance is unknown. Eyes with corneal arcus required less stromal wound hydration in cataract surgery. Corneal arcus may contribute to the closure of a clear corneal incision. [ABSTRACT FROM AUTHOR]

Abstract Background Homozygous familial hypercholesterolemia (HoFH) is an orphan metabolic disease characterized by extremely elevated low-density lipoprotein cholesterol (LDL-C), xanthomas, aortic stenosis, and premature atherosclerotic cardiovascular disease (ASCVD). In addition to LDL-C, studies in experimental models and small clinical populations have suggested that other types of metabolic molecules might also be risk factors responsible for cardiovascular complications in HoFH, but definitive evidence from large-scale human studies is still lacking. Herein, we aimed to comprehensively characterize the metabolic features and risk factors of human HoFH by using metabolic systems strategies. Methods Two independent multi-center cohorts with a total of 868 individuals were included in the cross-sectional study. First, comprehensive serum metabolome/lipidome-wide analyses were employed to identify the metabolomic patterns for differentiating HoFH patients (n = 184) from heterozygous FH (HeFH, n = 376) and non-FH (n = 100) subjects in the discovery cohort. Then, the metabolomic patterns were verified in the validation cohort with 48 HoFH patients, 110 HeFH patients, and 50 non-FH individuals. Subsequently, correlation/regression analyses were performed to investigate the associations of clinical/metabolic alterations with typical phenotypes of HoFH. In the prospective study, a total of 84 HoFH patients with available follow-up were enrolled from the discovery cohort. Targeted metabolomics, deep proteomics, and random forest approaches were performed to investigate the ASCVD-associated biomarkers in HoFH patients. Results Beyond LDL-C, various bioactive metabolites in multiple pathways were discovered and validated for differentiating HoFH from HoFH and non-FH. Our results demonstrated that the inflammation and oxidative stress-related metabolites in the pathways of arachidonic acid and lipoprotein(a) metabolism were independently associated with the prevalence of corneal arcus, xanthomas, and supravalvular/valvular aortic stenosis in HoFH patients. Our results also identified a small marker panel consisting of high-density lipoprotein cholesterol, lipoprotein(a), apolipoprotein A1, and eight proinflammatory and proatherogenic metabolites in the pathways of arachidonic acid, phospholipid, carnitine, and sphingolipid metabolism that exhibited significant performances on predicting first ASCVD events in HoFH patients. Conclusions Our findings demonstrate that human HoFH is associated with a variety of metabolic abnormalities and is more complex than previously known. Furthermore, this study provides additional metabolic alterations that hold promise as residual risk factors in HoFH population.

Background: Homozygous familial hypercholesterolemia (HoFH) is an orphan metabolic disease characterized by extremely elevated low-density lipoprotein cholesterol (LDL-C), xanthomas, aortic stenosis, and premature atherosclerotic cardiovascular disease (ASCVD). In addition to LDL-C, studies in experimental models and small clinical populations have suggested that other types of metabolic molecules might also be risk factors responsible for cardiovascular complications in HoFH, but definitive evidence from large-scale human studies is still lacking. Herein, we aimed to comprehensively characterize the metabolic features and risk factors of human HoFH by using metabolic systems strategies. Methods: Two independent multi-center cohorts with a total of 868 individuals were included in the cross-sectional study. First, comprehensive serum metabolome/lipidome-wide analyses were employed to identify the metabolomic patterns for differentiating HoFH patients (n = 184) from heterozygous FH (HeFH, n = 376) and non-FH (n = 100) subjects in the discovery cohort. Then, the metabolomic patterns were verified in the validation cohort with 48 HoFH patients, 110 HeFH patients, and 50 non-FH individuals. Subsequently, correlation/regression analyses were performed to investigate the associations of clinical/metabolic alterations with typical phenotypes of HoFH. In the prospective study, a total of 84 HoFH patients with available follow-up were enrolled from the discovery cohort. Targeted metabolomics, deep proteomics, and random forest approaches were performed to investigate the ASCVD-associated biomarkers in HoFH patients. Results: Beyond LDL-C, various bioactive metabolites in multiple pathways were discovered and validated for differentiating HoFH from HoFH and non-FH. Our results demonstrated that the inflammation and oxidative stress-related metabolites in the pathways of arachidonic acid and lipoprotein(a) metabolism were independently associated with the prevalence of corneal arcus, xanthomas, and supravalvular/valvular aortic stenosis in HoFH patients. Our results also identified a small marker panel consisting of high-density lipoprotein cholesterol, lipoprotein(a), apolipoprotein A1, and eight proinflammatory and proatherogenic metabolites in the pathways of arachidonic acid, phospholipid, carnitine, and sphingolipid metabolism that exhibited significant performances on predicting first ASCVD events in HoFH patients. Conclusions: Our findings demonstrate that human HoFH is associated with a variety of metabolic abnormalities and is more complex than previously known. Furthermore, this study provides additional metabolic alterations that hold promise as residual risk factors in HoFH population. [ABSTRACT FROM AUTHOR]

Abstract Purpose To determine the age and sex-standardized prevalence of corneal arcus and its associated factors in a geriatric population. Methods This population-based cross-sectional study was conducted in 2019 in Tehran; the capital of Iran, using a multi-stage stratified random cluster sampling method. All participants underwent a detailed interview, blood pressure measurement, laboratory blood tests, and a complete ocular examination. Results Three thousand three hundred ten of 3791 invitees participated in the study (response rate: 87.31%). The mean age of the participants was 69.35 ± 7.62 years (60–97 years) and 1912 (57.76%) were female. Overall, the age and sex-standardized prevalence (95% CI) of corneal arcus was 44.28% (41.21–47.39). Based on the multiple logistic regression, the odds of corneal arcus were higher in men than in women (OR: 1.51; 95% CI: 1.14–2.00); in the age group ≥ 80 years compared to the age group 60–64 years (OR: 2.44; 95% CI: 1.68–3.53), and in retired people compared to employed individuals (OR: 2.05; 95% CI: 1.31–3.21). Conclusion The present study showed a high prevalence of corneal arcus in the geriatric population. Although various studies have reported a significant relationship between corneal arcus with blood lipid and glucose levels as well as blood pressure, these relationships were not found in the present study.

Abstract Background Although the presence of physical signs [tendon xanthomas and/or corneal arcus (TX/CA)], are associated with the risk of coronary artery disease in patients with heterozygous familial hypercholesterolemia (HeFH), their relationship with genotypes and clinical characteristics has not been fully determined. This study aimed to examine the association of TX/CA with genetic mutation, lipid- and inflammation-related markers, the severity of coronary stenosis or calcification, and cardiovascular events (CVEs) in patients with HeFH. Methods LDLR, APOB, and PCSK9 genes were screened in 523 HeFH patients, and patients with TX/CA (n = 50) were 1:4 propensity score-matched to patients without TX/CA (n = 200) to adjust for age and sex. Laboratory markers (proprotein convertase subtilisin/kexin type 9 [PCSK9], lipoprotein(a) and high-sensitivity C-reactive protein [hsCRP]), computed tomography angiography, coronary angiography, and follow-up for CVEs were performed. Results Patients with physical signs had significantly higher low-density lipoprotein cholesterol levels; higher PCSK9 or hsCRP concentrations; more LDLR positive mutations; and higher prevalence of high tertiles of Gensini, SYNTAX and Jeopardy scores as well as coronary artery calcium scores than did those without. Over an average follow-up of 3.7 years, the incidence of CVEs was significantly higher in patients with TX/CA (log-rank p

Purpose: To determine the age and sex-standardized prevalence of corneal arcus and its associated factors in a geriatric population.Methods: This population-based cross-sectional study was conducted in 2019 in Tehran; the capital of Iran, using a multi-stage stratified random cluster sampling method. All participants underwent a detailed interview, blood pressure measurement, laboratory blood tests, and a complete ocular examination.Results: Three thousand three hundred ten of 3791 invitees participated in the study (response rate: 87.31%). The mean age of the participants was 69.35 ± 7.62 years (60-97 years) and 1912 (57.76%) were female. Overall, the age and sex-standardized prevalence (95% CI) of corneal arcus was 44.28% (41.21-47.39). Based on the multiple logistic regression, the odds of corneal arcus were higher in men than in women (OR: 1.51; 95% CI: 1.14-2.00); in the age group ≥ 80 years compared to the age group 60-64 years (OR: 2.44; 95% CI: 1.68-3.53), and in retired people compared to employed individuals (OR: 2.05; 95% CI: 1.31-3.21).Conclusion: The present study showed a high prevalence of corneal arcus in the geriatric population. Although various studies have reported a significant relationship between corneal arcus with blood lipid and glucose levels as well as blood pressure, these relationships were not found in the present study. [ABSTRACT FROM AUTHOR]

Lipid keratopathy (LK) is a rare ophthalmological condition characterized by a progressive reduction in visual acuity caused by corneal opacification due to central lipid accumulation. LK is characterized by lipid deposits, cholesterol clefts, and neovascularization (NV) leading to disruption in corneal optical quality. LK classification includes a primary and secondary form which depend on pre-existing corneal or systemic disorders and the evidence of NV. Secondary LK is typically associated with a prior occurrence of herpetic infection, such as herpes zoster keratitis. Patients with LK usually present with progressive vision loss and dense cream-colored corneal opacification. Treatment modalities include conservative and surgical approaches focused on corneal NV elimination. When evaluating corneal lipidosis, it is crucial to consider a range of differential diagnoses, including corneal arcus, Schnyder corneal dystrophy, and other corneal deposit conditions. We report a case of a 62-year-old male with herpes zoster keratitis complicated with LK. He presented with painless progressive vision loss and corneal scarring, which raised suspicion about LK diagnosis. This paper emphasizes the importance of correlating clinical and histological findings for accurate LK diagnosis. [ABSTRACT FROM AUTHOR]

Background: Although the presence of physical signs [tendon xanthomas and/or corneal arcus (TX/CA)], are associated with the risk of coronary artery disease in patients with heterozygous familial hypercholesterolemia (HeFH), their relationship with genotypes and clinical characteristics has not been fully determined. This study aimed to examine the association of TX/CA with genetic mutation, lipid- and inflammation-related markers, the severity of coronary stenosis or calcification, and cardiovascular events (CVEs) in patients with HeFH.Methods: LDLR, APOB, and PCSK9 genes were screened in 523 HeFH patients, and patients with TX/CA (n = 50) were 1:4 propensity score-matched to patients without TX/CA (n = 200) to adjust for age and sex. Laboratory markers (proprotein convertase subtilisin/kexin type 9 [PCSK9], lipoprotein(a) and high-sensitivity C-reactive protein [hsCRP]), computed tomography angiography, coronary angiography, and follow-up for CVEs were performed.Results: Patients with physical signs had significantly higher low-density lipoprotein cholesterol levels; higher PCSK9 or hsCRP concentrations; more LDLR positive mutations; and higher prevalence of high tertiles of Gensini, SYNTAX and Jeopardy scores as well as coronary artery calcium scores than did those without. Over an average follow-up of 3.7 years, the incidence of CVEs was significantly higher in patients with TX/CA (log-rank p < 0.001). Patients with physical signs and mutation positivity had threefold higher risks of CVEs (adjusted hazard ratio 3.34, 95% confidence interval 1.04-10.72, p = 0.024).Conclusions: Physical signs were associated with genotypes and phenotypes, and worse outcomes in patients with HeFH, suggesting that these signs may help in risk stratification in these patients. [ABSTRACT FROM AUTHOR]

We report here a case of an 11-year-old adolescent boy who presented with asymptomatic multiple yellowish skin lesions and corneal arcus. A family history of similar complaints was present in the elder male sibling who died with underlying coronary artery disease. Laboratory findings and family history were consistent with homozygous familial hypercholesterolemia. He was started on lipid-lowering drugs and is under regular follow-up. Early diagnosis and timely initiation of treatment helped to save the affected individual and the other family members.

• Familial hypercholesterolemia (FH) is underdiagnosed and is associated with high risk of premature coronary artery disease. • Genetic diagnosis of FH, although strongly recommended, is not always feasible. • Tendon xanthomas and corneal arcus <45 years are highly diagnostic of heterozygous FH. • Physicians should be able to recognize physical signs suggestive of FH. • Diagnosis of FH necessitates early lipid-lowering therapy and family screening. Familial hypercholesterolemia (FH) is a common, inherited disorder of cholesterol metabolism characterized by very high plasma concentrations of low-density lipoprotein cholesterol. It is crucial to diagnose and treat this disorder early since if left untreated it increases the risk for coronary artery disease (CAD) at least by 10-fold. Although genetic testing for FH, when available and affordable, should ideally be offered to most individuals with clinical phenotype suggestive of FH, it is underutilized in most countries. Therefore, FH diagnosis in the majority of cases is made by combining cholesterol levels and clinical characteristics of the patient leaving the need for genetic testing usually in equivocal cases. The presence of some cutaneous and ocular signs can raise the suspicion or even lead to the diagnosis of FH among usually "healthy" individuals. These physical signs comprise cutaneous lesions such as tendon xanthomas or the less specific xanthelasmata and ocular signs, such as corneal arcus in individuals under the age of 45 years. The presence of these signs should prompt the physician to request lipid tests and use clinical scores to diagnose FH. If the diagnosis of FH is likely, aggressive lipid-lowering therapy should be initiated to reduce the risk of CAD and a cascade screening of family members should also be requested. [ABSTRACT FROM AUTHOR]

A 48-year-old male with a history of familial hypercholesterolemia treated with evolocumab subcutaneous since 2017 developed recurrent episodes of acute uveitis. The most common diseases associated with uveitis were ruled out. Physical examination revealed an arcus senilis in both eyes. To the best of our knowledge, there have been no cases of uveitis reported with the use of evolocumab. We discuss a possible mechanism for the development of uveitis in this case. We conclude that acute anterior uveitis is a possible side effect related to the use of this medication. [ABSTRACT FROM AUTHOR]

Introduction: Corneal arcus caused by lipid deposition around the cornea has been proposed as an early marker for several metabolic disorders and even cardiovascular events. Non-alcoholic fatty liver (NAFLD) is a common health problem diagnosed by elevated liver enzymes and abdominal ultrasound findings. This study was conducted to assess the prevalence of corneal arcus in patients with NAFLD in Gorgan, northeast of Iran.Materials and Methods: This cross-sectional study was performed on 200 NAFLD patients younger than 50 years of age who were referred to hospitals of Gorgan between March 2014 and February 2015. Diagnosis of NAFLD was based on liver function test and abdominal ultrasound examination. Presence of corneal arcus was evaluated by slit-lamp examination. Results: Overall, corneal arcus was found in 91 patients (45.5%). Presence of corneal arcus was significantly correlated with smoking, opium consumption and family history of NAFLD. After controlling the confounding variables )diabetes, hypertension, smoking, hyperlipidemia, age, gender, ethnicity and body mass index(, we found that corneal arcus is significantly associated with history of ischemic heart disease and family history of fatty liver disease.Conclusions: Presence of corneal arcus in patients younger than 50 years with NAFLD is significantly associated with having a history of ischemic heart disease and family history of fatty liver disease.

We report here a case of an 11-year-old adolescent boy who presented with asymptomatic multiple yellowish skin lesions and corneal arcus. A family history of similar complaints was present in the elder male sibling who died with underlying coronary artery disease. Laboratory findings and family history were consistent with homozygous familial hypercholesterolemia. He was started on lipid-lowering drugs and is under regular follow-up. Early diagnosis and timely initiation of treatment helped to save the affected individual and the other family members. [ABSTRACT FROM AUTHOR]

The corneal arcus is probably a useful clinical marker to identify patients with coronary artery disease.To evaluate the independent association between the corneal arcus and coronary artery disease as well as the possibility of a relation betweenthe magnitude of the sing and the severity of the disease; and it`s efficiency as a diagnostic marker, conditioned by this association.A descriptive transverse study was conducted at the Cardiology Service of Hermanos Ameijeiras Hospital’s from February 2009 until June2010, to assess independent association between corneal arcus and coronary artery disease, the possibility of a relationship between thesign magnitude and severity of the disease; and its effectiveness as a diagnostic marker, conditioned by this association. The sample wasformed by 176 patients with coronary angiography, divided into two groups: 93 with coronary artery disease and 83 with normal coronaryarteries to determine if there was corneal arcus.A correlation between corneal arcus and coronary heart disease was obtained (p

A 23-year-old female patient applied to our clinic with the complaints of color changes in both of her corneas. Ophthalmological examination revealed 20/20 vision in both eyes. Intraocular pressure was 14 mmHg on the right eye and 12 mmHg on the left eye. Eye movements were normal and fundus examination revealed normal optic disc, macula, and peripheral retina. Biomicroscopic examination revealed diffuse haze in both of her corneas with circumferential arcus at the limbus and normal conjunctivae. Biochemical parameters were normal except for high-density lipoprotein values below 5 mg/ml, which was confirmed on repetitive tests. Together with ocular manifestations and low high-density lipoprotein values, a diagnosis of lecithin-cholesterol acyltransferase deficiency was made. In this article, a young patient who had low high density lipoprotein serum levels, peripheral corneal arcus, and corneal haze not affecting the visual acuity is presented. (Turk J Ophthalmol 2014; 44: 319-21)

Purpose: To investigate the association between senile corneal arcus and atherosclerosis risk factors in patients with recent acute myocardial infarction. Methods: In this cross sectional study, atherosclerosis risk factors including fasting blood sugar, total cholesterol and triglyceride levels were measured in 165 patients with recent (less than three months′ duration) acute myocardial infarction. Slitlamp examination was performed to detect corneal arcus. Associations between senile corneal arcus and atherosclerosis risk factors were assessed. Results: Overall, 165 patients including 100 male and 65 female subjects with mean age of 62±10.3 years were evaluated. In 122 patients (74%), variable degrees of corneal arcus were observed. The presence of corneal arcus was significantly associated with age (P = 0.03) and high levels of total cholesterol (over 200 mg/dl, P < 0.01). After adjusting for age, arcus was not associated with sex (P = 0.10), hypertriglyceridemia (P = 0.09), fasting blood sugar (P = 0.06), or systemic hypertension (P = 0.08). Conclusion: Our study revealed that corneal arcus is associated with age and hypercholesterolemia in patients with recent acute myocardial infarction. No association was detected with sex, fasting blood sugar, hypertension, and hypertriglyceridemia.

Background: Cranial autonomic dysregulation is a common symptom of patients suffering from cluster headache or migraine. The peripheral vascular dysfunction may increase the risk for ischemic or hemorrhagic strokes, myocardial infarction, retinal vasculopathy, cardiovascular mortality, and peripheral artery diseases. Furthermore, it may also manifest with ocular symptoms, e.g., increased lacrimation, conjunctival injection, and facial swelling., Case Presentation: We here report a case of a patient with migraine and ocular signs of a vascular dysregulation that have led to persisting changes of conjunctival vessels and to a corneal arcus., Conclusions: Autonomic vascular dysregulation may not only cause headaches but also persisting changes of ocular tissues, e.g., conjunctival vessel alterations and a corneal arcus.

Background: H syndrome is an autosomal recessive histiocytosis with multisystemic involvement caused by mutations in theSLC29A3gene. The term H syndrome was coined to denote the major clinical findings which includehyperpigmentation,hypertrichosis,hearing loss,hepatosplenomegaly,hypogonadism,hyperglycemia/diabetes mellitus andhallux valgus/flexion contractures. Almost 100 individuals affected with this disorder have been reported, however, a thorough evaluation of the ophthalmologic features of H syndrome has not yet been performed. Materials and Methods: Ophthalmic examination of a 50-year-old male with H syndrome. Mutation analysis ofSLC29A3was also performed in this patient. Results: Ophthalmic findings included; shallow orbits with exorbitism, bilateral pterygium, limbal thickening, corneal arcus and cortical cataract. We also review ophthalmologic findings in previously reported H syndrome patients. Conclusions: The presence of dilated lateral scleral vessels, corneal arcus and shallow orbits should raise the suspicion of H syndrome, especially when seen in young age. [ABSTRACT FROM AUTHOR]

Purpose: To determine the prevalence of corneal arcus, its risk factors, and its relationship to ocular and visual indices. Methods: In this cross-sectional study, 300 clusters were randomly selected from Shahroud in the north of Iran, using multistage sampling. A total of 20 people were invited to participate from each cluster. After enrollment, all optometric, biometric and ophthalmic exams were conducted on site. Results: Of 6311 people invited, 5190 (82.2%) participated in the study. The prevalence of corneal arcus was 23.3% (95% confidence interval, CI, 22.1-24.6), and 98.4% were bilateral cases. The prevalence of corneal arcus was higher in men (odds ratio, OR, 2.02, 95% CI 1.8-2.3, p < 0.001) and increased with age (OR 1.1/year, p < 0.001). In a multivariable-adjusted regression model, age (OR 1.1/year, p = 0.006), male sex (OR 1.30, p = 0.001), diabetes (OR 0.7, p < 0.001), smoking (OR 1.5, p = 0.003), outdoor activity (OR 1.4, p = 0.006), systolic blood pressure (OR 1.01, p = 0.012), and diastolic blood pressure (OR 0.99, p = 0.016) were significantly correlated with corneal arcus. Including biometric components in another model, corneal thickness (OR 0.99, p < 0.001), anterior chamber depth (OR 0.68, p < 0.001) and corneal radius of curvature (OR 1.59, p < 0.001) were significantly correlated with corneal arcus. Conclusion: This study adds valuable information to the epidemiology of corneal arcus in Iran and the Middle East. In people aged over 60 years, nearly 50% of the study population had corneal arcus. Older age, male sex, smoking, and systolic hypertension were risk factors for corneal arcus. Corneal arcus was also associated with thin and flat corneas and shallow anterior chamber depth. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the association between senile corneal arcus and atherosclerosis risk factors in patients with recent acute myocardial infarction. Methods: In this cross sectional study, atherosclerosis risk factors including fasting blood sugar, total cholesterol and triglyceride levels were measured in 165 patients with recent (less than three months' duration) acute myocardial infarction. Slitlamp examination was performed to detect corneal arcus. Associations between senile corneal arcus and atherosclerosis risk factors were assessed. Results: Overall, 165 patients including 100 male and 65 female subjects with mean age of 62±10.3 years were evaluated. In 122 patients (74%), variable degrees of corneal arcus were observed. The presence of corneal arcus was significantly associated with age (P = 0.03) and high levels of total cholesterol (over 200 mg/dl, P < 0.01). After adjusting for age, arcus was not associated with sex (P = 0.10), hypertriglyceridemia (P = 0.09), fasting blood sugar (P = 0.06), or systemic hypertension (P = 0.08). Conclusion: Our study revealed that corneal arcus is associated with age and hypercholesterolemia in patients with recent acute myocardial infarction. No association was detected with sex, fasting blood sugar, hypertension, and hypertriglyceridemia. [ABSTRACT FROM AUTHOR]

Abstract: Corneal arcus is a lipid-rich and predominantly extracellular deposit that forms at the corneoscleral limbus. It represents the most common peripheral corneal opacity and is not associated with tissue breakdown but rather with the deposition of lipids. The deposition of cholesterol in the peripheral cornea and arterial wall are similar in that both are accelerated by elevated serum levels of atherogenic lipoproteins, such as low-density lipoproteins (LDL). Corneal arcus is more prevalent in men than in women and in Blacks than in Whites. Its prevalence increases with advancing age. It has been associated with hypercholesterolemia, xanthelasmas, alcohol, blood pressure, cigarette smoking, diabetes, age, and coronary heart disease. Nevertheless, it is not clear whether or not corneal arcus is an independent risk factor for coronary heart disease (CHD). The present systematic review examines the relationship of corneal arcus and CHD to determine if corneal arcus is an independent CHD risk factor. We conclude that there is no consensus that corneal arcus is an independent risk factor. The presence of corneal arcus in a young person should prompt a search for lipid abnormalities. Also, because corneal arcus represents physical evidence of early lipid deposition, its presence suggests the need for aggressive lipid therapy. [Copyright &y& Elsevier]

We report the case of a 12-year-old male who developed corneal arcus and multiple skin lesions with a 10-year history of xanthomas. The lesions appeared over his fingers, hands, elbows, knees, buttocks and feet. Laboratory studies showed a total serum cholesterol level of 752.1 mg/dL; a triglyceride level of 96.6 mg/dL; a low-density lipoprotein cholesterol level of 661.3 mg/dL. Findings were consistent with homozygous familial hypercholesterolemia. To our knowledge, this is the first such case to be reported from China.

A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child presented with reduced level of consciousness and bilateral hypoacusis. The patient was lethargic. Ophthalmic examination showed branch retinal artery occlusion (BRAO). This finding was crucial to the diagnosis of Susac′s syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Magnetic resonance imaging of the brain also showed typical features. Thorough blood investigations did not reveal any other abnormality. Patient was treated with immunosuppressive to prevent her from developing severe sequelae of this disease. The child showed dramatic improvement in her systemic condition within 48 h of starting the treatment. This is the youngest ever and first case report from India.

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