Lipaemia retinalis | |
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Other names | Lipemia retinalis |
Specialty | Ophthalmology, Endocrinology |
Causes | Hypertriglyceridemia |
Diagnostic method | Ophthalmoscopy |
Treatment | Diet control, Insulin treatment [1] |
Lipaemia retinalis (LR) also spelled as Lipemia retinalis is an eye disease caused by high amounts of triglycerides in the blood (hypertriglyceridemia) or Lipoprotein lipase deficiency (chylomicronemia). In this condition the retinal arteries and veins, and occasionally the entire fundus shows creamy-white to salmon red discoloration.
In early stage of lipaemia retinalis, creamy-white discoloration of retinal blood vessels occurs and in extreme cases the fundus become salmon red colour. [2] Retinal changes occur in the peripheral vessels gradually progress to entire fundus. [3] Symptoms are caused by an increase in the level of chylomicron in the retinal circulation. [4] Though visual acuity is not affected, electroretinogram amplitude may be decreased. [2]
Since acute increase in triglyceride may be asymptomatic at first, retinal signs of LR are an important clinical sign of hypertriglyceridemia. [5]
Lipaemia retinalis is a retinal disease caused by hypertriglyceridemia or chylomicronemia. [3] Depending on plasma triglyceride level, the retinal arteries, veins or occasionally the entire fundus may show creamy-white to salmon red discoloration. [6] In primary hyperlipidaemia, LR occurs only in patients with increased chylomicron levels. [6]
Lipaemia retinalis occurs commonly with familial hypertriglyceridaemia, and rarely with combined hypertriglyceridaemia. [4] Although serum triglyceride levels above 1000 mg / dL can cause eye symptoms such as xanthoma, xanthelasma and arcus senilis, lipaemia retinalis is most commonly seen when the triglyceride level is above 1500 mg / dL. [7] Changes in fundus are more pronounced when triglyceride levels exceed 2,500 mg / dL. [8]
Vinger and Sachs classified lipaemia retinalis into three grades. Early lipaemia retinalis with creamy white discoloration of peripheral retinal vessels is classified as Grade 1. [3] When the discoloration extends towards optic disc, it is graded as Grade 2 and when retina becomes salmon colored it is classified as Grade 3. [3]
When the serum triglyceride is under control by proper dietary control and or insulin, the changes in the retina and electroretinogram changes return to normal. [9] [7] [10]
According to Brunzell and Bierman, lipaemia retinalis occurs in 23% of patients with chylomicronaemia and they says it may be considered as a rare disease only because of lack of routine fundoscopic examination. [4]
In 1880, Heyl first described Lipaemia retinalis and in 1970 Vinger and Sachs introduced a grading system to describe its stages. [6]
Increase in serum triglyceride can cause lipaemia retinalis in cats also. [11]
Chylomicrons, also known as ultra low-density lipoproteins (ULDL), are lipoprotein particles that consist of triglycerides (85–92%), phospholipids (6–12%), cholesterol (1–3%), and proteins (1–2%). They transport dietary lipids, such as fats and cholesterol, from the intestines to other locations in the body, within the water-based solution of the bloodstream. ULDLs are one of the five major groups lipoproteins are divided into based on their density. A protein specific to chylomicrons is ApoB48.
Hypertriglyceridemia is the presence of high amounts of triglycerides in the blood. Triglycerides are the most abundant fatty molecule in most organisms. Hypertriglyceridemia occurs in various physiologic conditions and in various diseases, and high triglyceride levels are associated with atherosclerosis, even in the absence of hypercholesterolemia and predispose to cardiovascular disease.
Diplopia is the simultaneous perception of two images of a single object that may be displaced horizontally or vertically in relation to each other. Also called double vision, it is a loss of visual focus under regular conditions, and is often voluntary. However, when occurring involuntarily, it results from impaired function of the extraocular muscles, where both eyes are still functional, but they cannot turn to target the desired object. Problems with these muscles may be due to mechanical problems, disorders of the neuromuscular junction, disorders of the cranial nerves that innervate the muscles, and occasionally disorders involving the supranuclear oculomotor pathways or ingestion of toxins.
Hyperlipidemia is abnormally high levels of any or all lipids or lipoproteins in the blood. The term hyperlipidemia refers to the laboratory finding itself and is also used as an umbrella term covering any of various acquired or genetic disorders that result in that finding. Hyperlipidemia represents a subset of dyslipidemia and a superset of hypercholesterolemia. Hyperlipidemia is usually chronic and requires ongoing medication to control blood lipid levels.
Electroretinography measures the electrical responses of various cell types in the retina, including the photoreceptors, inner retinal cells, and the ganglion cells. Electrodes are placed on the surface of the cornea or on the skin beneath the eye to measure retinal responses. Retinal pigment epithelium (RPE) responses are measured with an EOG test with skin-contact electrodes placed near the canthi. During a recording, the patient's eyes are exposed to standardized stimuli and the resulting signal is displayed showing the time course of the signal's amplitude (voltage). Signals are very small, and typically are measured in microvolts or nanovolts. The ERG is composed of electrical potentials contributed by different cell types within the retina, and the stimulus conditions can elicit stronger response from certain components.
Oguchi disease is an autosomal recessive form of congenital stationary night blindness associated with fundus discoloration and abnormally slow dark adaptation.
Lipoprotein lipase deficiency is a genetic disorder in which a person has a defective gene for lipoprotein lipase, which leads to very high triglycerides, which in turn causes stomach pain and deposits of fat under the skin, and which can lead to problems with the pancreas and liver, which in turn can lead to diabetes. The disorder only occurs if a child acquires the defective gene from both parents. It is managed by restricting fat in diet to less than 20 g/day.
Charcot–Bouchard aneurysms are aneurysms of the brain vasculature which occur in small blood vessels. Charcot–Bouchard aneurysms are most often located in the lenticulostriate vessels of the basal ganglia and are associated with chronic hypertension. Charcot–Bouchard aneurysms are a common cause of cerebral hemorrhage. Charcot–Bouchard aneurysm rupture might be linked to senile plaque formation in the Alzheimer's disease.
Angioid streaks, also called Knapp streaks or Knapp striae, are small breaks in Bruch's membrane, an elastic tissue containing membrane of the retina that may become calcified and crack. Up to 50% of angioid streak cases are idiopathic. It may occur secondary to blunt trauma, or it may be associated with many systemic diseases. The condition is usually asymptomatic, but decrease in vision may occur due to choroidal neovascularization.
Blurred vision is an ocular symptom where vision becomes less precise and there is added difficulty to resolve fine details.
Intraocular hemorrhage is bleeding inside the eye. Bleeding can occur from any structure of the eye where there is vasculature or blood flow, including the anterior chamber, vitreous cavity, retina, choroid, suprachoroidal space, or optic disc.
Familial hypertriglyceridemia is a genetic disorder characterized by the liver overproducing very-low-density lipoproteins (VLDL). As a result, an affected individual will have an excessive number of VLDL and triglycerides on a lipid profile. This genetic disorder usually follows an autosomal dominant inheritance pattern. The disorder presents clinically in patients with mild to moderate elevations in triglyceride levels. Familial hypertriglyceridemia is typically associated with other co-morbid conditions such as hypertension, obesity, and hyperglycemia. Individuals with the disorder are mostly heterozygous in an inactivating mutation of the gene encoding for lipoprotein lipase (LPL). This sole mutation can markedly elevate serum triglyceride levels. However, when combined with other medications or pathologies it can further elevate serum triglyceride levels to pathologic levels. Substantial increases in serum triglyceride levels can lead to certain clinical signs and the development of acute pancreatitis.
Bonnet–Dechaume–Blanc syndrome, also known as Wyburn-Mason syndrome, is a rare congenital disorder characterized by arteriovenous malformations of the brain, retina or facial nevi. The syndrome has a number of possible symptoms and can, more rarely, affect the skin, bones, kidneys, muscles, and gastrointestinal tract. When the syndrome affects the brain, people can experience severe headaches, seizures, acute stroke, meningism, and progressive neurological deficits due to acute or chronic ischaemia caused by arteriovenous shunting.
Branch retinal vein occlusion is a common retinal vascular disease of the elderly. It is caused by the occlusion of one of the branches of central retinal vein.
Microperimetry, sometimes called fundus-controlled perimetry, is a type of visual field test which uses one of several technologies to create a "retinal sensitivity map" of the quantity of light perceived in specific parts of the retina in people who have lost the ability to fixate on an object or light source. The main difference with traditional perimetry instruments is that, microperimetry includes a system to image the retina and an eye tracker to compensate eye movements during visual field testing.
Autoimmune retinopathy (AIR) is a rare disease in which the patient's immune system attacks proteins in the retina, leading to loss of eyesight. The disease is poorly understood, but may be the result of cancer or cancer chemotherapy. The disease is an autoimmune condition characterized by vision loss, blind spots, and visual field abnormalities. It can be divided into cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR). The condition is associated with retinal degeneration caused by autoimmune antibodies recognizing retinal proteins as antigens and targeting them. AIR's prevalence is extremely rare, with CAR being more common than MAR. It is more commonly diagnosed in females in the age range of 50–60.
Photic retinopathy is damage to the eye's retina, particularly the macula, from prolonged exposure to solar radiation or other bright light, e.g., lasers or arc welders. The term includes solar, laser, and welder's retinopathy and is synonymous with retinal phototoxicity. It usually occurs due to staring at the Sun, watching a solar eclipse, or viewing an ultraviolet, Illuminant D65, or other bright light.[1]
Photostress recovery time (PSRT) is the time taken for visual acuity to return to normal levels after the retina has been bleached by a bright light source. Photostress recovery time measurement procedure is known as photostress test. Normal recovery time is about 15–30 seconds.
Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.
Polypoidal choroidal vasculopathy (PCV) is an eye disease primarily affecting the choroid. It may cause sudden blurring of vision or a scotoma in the central field of vision. Since Indocyanine green angiography gives better imaging of choroidal structures, it is more preferred in diagnosing PCV. Treatment options of PCV include careful observation, photodynamic therapy, thermal laser, intravitreal injection of anti-VEGF therapy, or combination therapy.