Lymphangiectasia
Encyclopedia
Lymphangiectasia is a pathologic dilation of lymph vessels. When it occurs in the intestine
s of dogs, and more rarely humans, it causes a disease known as "intestinal lymphangiectasia." This disease is characterized by lymphatic vessel dilation, chronic diarrhea
and loss of protein
s such as serum albumin
and globulin
. It is considered to be a chronic form of protein-losing enteropathy.
It is also known as "lymphangiectasis".
of the small intestine
shows dilation of the lacteal
s of the villi and distension of the lymphatic vessel
s. Reduced lymph
flow leads to a malabsorption
syndrome of the small intestine, especially of fat
and fat-soluble vitamin
s. Rupture of the lymphatics causes protein loss into the intestines.
The most common cause of lymphangiectasia is congenital malformation of the lymphatics. Secondary lymphangiectasia may be caused by granuloma
s or cancer causing lymphatic obstruction, or increased central venous pressure
(CVP) causing abnormal lymph drainage. Increased CVP can be caused by pericarditis
or right-sided heart failure. Inflammatory bowel disease
can also lead to inflammation of the lymphatics and lymphangiectasia through migration of inflammatory cells through the lymphatics.
), which causes low oncotic pressure
. These signs include ascites
, pleural effusion
, and edema
of the limbs and trunk. Weight loss is seen with long-term disease.
s, and decreased cholesterol
support the diagnosis. Hypocalcaemia
(low calcium
) is also seen due to poor absorption of vitamin D
and calcium, and secondary to low protein binding of calcium. Medical ultrasonography
may show striation
s in the intestinal mucosa indicating dilated lacteals.
) oil and/or the drug Octreotide
. In dogs, fat soluble vitamins (A
, D
, E
, and K
) should be supplemented. Corticosteroid
treatment may be required for life. Antibiotic
s can be used to treat bacterial overgrowth. With a very low serum albumin
, transfusion
with blood plasma
or an infusion of hetastarch may be necessary to treat the signs until the diet can take effect. Lymphangiectasia is rarely cured but can remain in remission for a long time. It can be fatal when unresponsive to treatment.
There is a potential cure for Intestinal Lymphangiectasia that is new and easy for a GI doctor to administer. All patients with this serious disease should ask their doctors to investigate. It is an argon laser treatment, used to burn the affected area and the tissue can grow back normally, potentially sealing the intestinal leaks and preventing the loss of proteins.[ Source?]
, Soft-Coated Wheaten Terrier
, Norwegian Lundehund
, Basenji
, and Yorkshire Terrier
.
Intestine
In human anatomy, the intestine is the segment of the alimentary canal extending from the pyloric sphincter of the stomach to the anus and, in humans and other mammals, consists of two segments, the small intestine and the large intestine...
s of dogs, and more rarely humans, it causes a disease known as "intestinal lymphangiectasia." This disease is characterized by lymphatic vessel dilation, chronic diarrhea
Diarrhea
Diarrhea , also spelled diarrhoea, is the condition of having three or more loose or liquid bowel movements per day. It is a common cause of death in developing countries and the second most common cause of infant deaths worldwide. The loss of fluids through diarrhea can cause dehydration and...
and loss of protein
Protein
Proteins are biochemical compounds consisting of one or more polypeptides typically folded into a globular or fibrous form, facilitating a biological function. A polypeptide is a single linear polymer chain of amino acids bonded together by peptide bonds between the carboxyl and amino groups of...
s such as serum albumin
Serum albumin
Serum albumin, often referred to simply as albumin is a protein that in humans is encoded by the ALB gene.Serum albumin is the most abundant plasma protein in mammals. Albumin is essential for maintaining the osmotic pressure needed for proper distribution of body fluids between intravascular...
and globulin
Globulin
Globulin is one of the three types of serum proteins, the others being albumin and fibrinogen. Some globulins are produced in the liver, while others are made by the immune system. The term globulin encompasses a heterogeneous group of proteins with typical high molecular weight, and both...
. It is considered to be a chronic form of protein-losing enteropathy.
It is also known as "lymphangiectasis".
Cause
BiopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
of the small intestine
Small intestine
The small intestine is the part of the gastrointestinal tract following the stomach and followed by the large intestine, and is where much of the digestion and absorption of food takes place. In invertebrates such as worms, the terms "gastrointestinal tract" and "large intestine" are often used to...
shows dilation of the lacteal
Lacteal
A lacteal is a lymphatic capillary that absorbs dietary fats in the villi of the small intestine.Triglycerides are emulsified by bile and hydrolyzed by the enzyme lipase, resulting in a mixture of fatty acids and monoglycerides. These then pass from the intestinal lumen into the enterocyte, where...
s of the villi and distension of the lymphatic vessel
Lymphatic system
The lymphoid system is the part of the immune system comprising a network of conduits called lymphatic vessels that carry a clear fluid called lymph unidirectionally toward the heart. Lymphoid tissue is found in many organs, particularly the lymph nodes, and in the lymphoid follicles associated...
s. Reduced lymph
Lymphatic system
The lymphoid system is the part of the immune system comprising a network of conduits called lymphatic vessels that carry a clear fluid called lymph unidirectionally toward the heart. Lymphoid tissue is found in many organs, particularly the lymph nodes, and in the lymphoid follicles associated...
flow leads to a malabsorption
Malabsorption
Malabsorption is a state arising from abnormality in absorption of food nutrients across the gastrointestinal tract.Impairment can be of single or multiple nutrients depending on the abnormality...
syndrome of the small intestine, especially of fat
Fat
Fats consist of a wide group of compounds that are generally soluble in organic solvents and generally insoluble in water. Chemically, fats are triglycerides, triesters of glycerol and any of several fatty acids. Fats may be either solid or liquid at room temperature, depending on their structure...
and fat-soluble vitamin
Vitamin
A vitamin is an organic compound required as a nutrient in tiny amounts by an organism. In other words, an organic chemical compound is called a vitamin when it cannot be synthesized in sufficient quantities by an organism, and must be obtained from the diet. Thus, the term is conditional both on...
s. Rupture of the lymphatics causes protein loss into the intestines.
The most common cause of lymphangiectasia is congenital malformation of the lymphatics. Secondary lymphangiectasia may be caused by granuloma
Granuloma
Granuloma is a medical term for a tiny collection of immune cells known as macrophages. Granulomas form when the immune system attempts to wall off substances that it perceives as foreign but is unable to eliminate. Such substances include infectious organisms such as bacteria and fungi as well as...
s or cancer causing lymphatic obstruction, or increased central venous pressure
Central venous pressure
Central venous pressure describes the pressure of blood in the thoracic vena cava, near the right atrium of the heart...
(CVP) causing abnormal lymph drainage. Increased CVP can be caused by pericarditis
Pericarditis
Pericarditis is an inflammation of the pericardium . A characteristic chest pain is often present.The causes of pericarditis are varied, including viral infections of the pericardium, idiopathic causes, uremic pericarditis, bacterial infections of the precardium Pericarditis is an inflammation of...
or right-sided heart failure. Inflammatory bowel disease
Inflammatory bowel disease
In medicine, inflammatory bowel disease is a group of inflammatory conditions of the colon and small intestine. The major types of IBD are Crohn's disease and ulcerative colitis.-Classification:...
can also lead to inflammation of the lymphatics and lymphangiectasia through migration of inflammatory cells through the lymphatics.
Signs and symptoms
Chronic diarrhea is almost always seen with lymphangiectasia, but most other signs are linked to low blood protein levels (hypoproteinemiaHypoproteinemia
Hypoproteinemia is a condition where there is an abnormally low level of protein in the blood.One common cause is due to excess protein in the urine , which can be a medical sign of nephrotic syndrome....
), which causes low oncotic pressure
Oncotic pressure
Oncotic pressure, or colloid osmotic pressure, is a form of osmotic pressure exerted by proteins in blood plasma that usually tends to pull water into the circulatory system.Throughout the body, dissolved compounds have an osmotic pressure...
. These signs include ascites
Ascites
Ascites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver...
, pleural effusion
Pleural effusion
Pleural effusion is excess fluid that accumulates between the two pleural layers, the fluid-filled space that surrounds the lungs. Excessive amounts of such fluid can impair breathing by limiting the expansion of the lungs during ventilation.-Pathophysiology:...
, and edema
Edema
Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
of the limbs and trunk. Weight loss is seen with long-term disease.
Diagnosis
Diagnosis is through biopsy. The presence of hypoproteinemia, decreased blood lymphocyteLymphocyte
A lymphocyte is a type of white blood cell in the vertebrate immune system.Under the microscope, lymphocytes can be divided into large lymphocytes and small lymphocytes. Large granular lymphocytes include natural killer cells...
s, and decreased cholesterol
Cholesterol
Cholesterol is a complex isoprenoid. Specifically, it is a waxy steroid of fat that is produced in the liver or intestines. It is used to produce hormones and cell membranes and is transported in the blood plasma of all mammals. It is an essential structural component of mammalian cell membranes...
support the diagnosis. Hypocalcaemia
Hypocalcaemia
In medicine, hypocalcaemia is the presence of low serum calcium levels in the blood, usually taken as less than 2.1 mmol/L or 9 mg/dl or an ionized calcium level of less than 1.1 mmol/L or 4.5 mg/dL. It is a type of electrolyte disturbance...
(low calcium
Calcium
Calcium is the chemical element with the symbol Ca and atomic number 20. It has an atomic mass of 40.078 amu. Calcium is a soft gray alkaline earth metal, and is the fifth-most-abundant element by mass in the Earth's crust...
) is also seen due to poor absorption of vitamin D
Vitamin D
Vitamin D is a group of fat-soluble secosteroids. In humans, vitamin D is unique both because it functions as a prohormone and because the body can synthesize it when sun exposure is adequate ....
and calcium, and secondary to low protein binding of calcium. Medical ultrasonography
Medical ultrasonography
Diagnostic sonography is an ultrasound-based diagnostic imaging technique used for visualizing subcutaneous body structures including tendons, muscles, joints, vessels and internal organs for possible pathology or lesions...
may show striation
Striation
Striations means a series of ridges, furrows or linear marks, and are used in several ways* Glacial striation* Striation , a striation as a result of a geological fault* In medicine, striated muscle...
s in the intestinal mucosa indicating dilated lacteals.
Treatment
Treatment is multifactorial. A diet very low in fat and high in high quality protein is essential. Treatment of humans can also involve the use of MCT (medium chain triglyceridesMedium chain triglycerides
Medium-chain triglycerides are medium-chain fatty acid esters of glycerol.MCTs passively diffuse from the GI tract to the portal system without requirement for modification like long-chain fatty acids or very-long-chain fatty acids. In addition, MCTs do not require bile salts for digestion...
) oil and/or the drug Octreotide
Octreotide
Octreotide is an octapeptide that mimics natural somatostatin pharmacologically, though it is a more potent inhibitor of growth hormone, glucagon, and insulin than the natural hormone...
. In dogs, fat soluble vitamins (A
Vitamin A
Vitamin A is a vitamin that is needed by the retina of the eye in the form of a specific metabolite, the light-absorbing molecule retinal, that is necessary for both low-light and color vision...
, D
Vitamin D
Vitamin D is a group of fat-soluble secosteroids. In humans, vitamin D is unique both because it functions as a prohormone and because the body can synthesize it when sun exposure is adequate ....
, E
Vitamin E
Vitamin E is used to refer to a group of fat-soluble compounds that include both tocopherols and tocotrienols. There are many different forms of vitamin E, of which γ-tocopherol is the most common in the North American diet. γ-Tocopherol can be found in corn oil, soybean oil, margarine and dressings...
, and K
Vitamin K
Vitamin K is a group of structurally similar, fat soluble vitamins that are needed for the posttranslational modification of certain proteins required for blood coagulation and in metabolic pathways in bone and other tissue. They are 2-methyl-1,4-naphthoquinone derivatives...
) should be supplemented. Corticosteroid
Corticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
treatment may be required for life. Antibiotic
Antibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
s can be used to treat bacterial overgrowth. With a very low serum albumin
Serum albumin
Serum albumin, often referred to simply as albumin is a protein that in humans is encoded by the ALB gene.Serum albumin is the most abundant plasma protein in mammals. Albumin is essential for maintaining the osmotic pressure needed for proper distribution of body fluids between intravascular...
, transfusion
Blood transfusion
Blood transfusion is the process of receiving blood products into one's circulation intravenously. Transfusions are used in a variety of medical conditions to replace lost components of the blood...
with blood plasma
Blood plasma
Blood plasma is the straw-colored liquid component of blood in which the blood cells in whole blood are normally suspended. It makes up about 55% of the total blood volume. It is the intravascular fluid part of extracellular fluid...
or an infusion of hetastarch may be necessary to treat the signs until the diet can take effect. Lymphangiectasia is rarely cured but can remain in remission for a long time. It can be fatal when unresponsive to treatment.
There is a potential cure for Intestinal Lymphangiectasia that is new and easy for a GI doctor to administer. All patients with this serious disease should ask their doctors to investigate. It is an argon laser treatment, used to burn the affected area and the tissue can grow back normally, potentially sealing the intestinal leaks and preventing the loss of proteins.[ Source?]
Affected breeds
Breeds commonly affected by lymphangiectasia and/or protein-losing enteropathy include the PapillonPapillon
Papillon may refer to:* Papillon , a memoir by Henri Charrière about his imprisonment at a penal colony in French Guiana** Papillon , a 1973 film based on the book starring Steve McQueen...
, Soft-Coated Wheaten Terrier
Soft-Coated Wheaten Terrier
The Soft-Coated Wheaten Terrier is a breed of dog originating from Ireland. There are four coat varieties: Traditional Irish, Heavy Irish, English, and American...
, Norwegian Lundehund
Norwegian Lundehund
The Norwegian Lundehund is a small dog breed of the Spitz type that originates from Norway. Its name is composed of the prefix Lunde, from the Norwegian lundefugl , and the suffix hund, meaning dog...
, Basenji
Basenji
The Basenji is a breed of hunting dog that was bred from stock originating in central Africa. Most of the major kennel clubs in the English-speaking world place the breed in the Hound Group; more specifically, it may be classified as belonging to the sighthound type...
, and Yorkshire Terrier
Yorkshire Terrier
The Yorkshire Terrier is a small dog breed of terrier type, developed in the 19th century in the county of Yorkshire, England to catch rats in clothing mills. The defining features of the breed are its size, to , and its silky blue and tan coat...
.