Gastric antral vascular ectasia
Encyclopedia
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Gastric antral vascular ectasia (GAVE) is an uncommon cause of chronic gastrointestinal bleeding
Gastrointestinal bleeding
Gastrointestinal bleeding or gastrointestinal hemorrhage describes every form of hemorrhage in the gastrointestinal tract, from the pharynx to the rectum. It has diverse causes, and a medical history, as well as physical examination, generally distinguishes between the main forms...
or iron deficiency anemia
Iron deficiency anemia
Iron-deficiency anemia is a common anemia that occurs when iron loss occurs, and/or the dietary intake or absorption of iron is insufficient...
. The condition is associated with dilated small blood vessels in the antrum
Antrum
In biology, antrum is a general term for a cavity or chamber which may have specific meaning in reference to certain organs or sites in the body.In vertebrates, it may mean for example:...
, or the last part of the stomach
Stomach
The stomach is a muscular, hollow, dilated part of the alimentary canal which functions as an important organ of the digestive tract in some animals, including vertebrates, echinoderms, insects , and molluscs. It is involved in the second phase of digestion, following mastication .The stomach is...
. The dilated vessels result in intestinal bleeding. It is also called watermelon stomach because streaky long red areas that are present in the stomach may resemble the markings on watermelon
Watermelon
Watermelon is a vine-like flowering plant originally from southern Africa. Its fruit, which is also called watermelon, is a special kind referred to by botanists as a pepo, a berry which has a thick rind and fleshy center...
.
The condition was first discovered in 1952, and reported in the literature in 1953. Watermelon disease was first diagnosed by Wheeler et al. in 1979, and definitively described in four living patients by Jabbari et al. only in 1984. As of 2011, the etiology and pathogenesis are still not known. However, there are several competing hypotheses as to various etiologies.
Signs and symptoms
Most patients who are eventually diagnosed with watermelon stomach come to a physician complaining of anemiaAnemia
Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...
and blood loss. Sometimes, a patient may come to the medical doctor because he or she notices blood in the stools — either melena
Melena
In medicine, melena or melaena refers to the black, "tarry" feces that are associated with gastrointestinal hemorrhage. The black color is caused by oxidation of the iron in hemoglobin during its passage through the ileum and colon.-Melena vs...
(black and tarry stools) and/or hematochezia
Hematochezia
Hematochezia is the passage of fresh blood per anus, usually in or with stools . Hematochezia is commonly associated with lower gastrointestinal bleeding, but may also occur from a brisk upper GI bleed...
(maroon stools).
Differential diagnosis
GAVE results in intestinal bleeding similar to duodenal ulcers and portal hypertensionPortal hypertension
In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...
. The GI bleeding can result in anemia
Anemia
Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...
, as well as occult blood in the stool. It is often overlooked, but can be more common in elderly patients. It has been seen in patients as young as 38 years of age.
Watermelon stomach has a different etiology and has a differential diagnosis
Differential diagnosis
A differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...
from portal hypertension. In fact, cirrhosis and portal hypertension may be missing in a patient with GAVE. The differential diagnosis is important because treatments are different.
Diagnosis
GAVE is usually diagnosed definitively by means of an endoscopicEndoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...
biopsy. The tell-tale watermelon stripes show up during the endoscopy.
Surgical exploration of the abdomen
Abdomen
In vertebrates such as mammals the abdomen constitutes the part of the body between the thorax and pelvis. The region enclosed by the abdomen is termed the abdominal cavity...
may be needed to diagnose some cases, especially if the liver or other organs are involved.
Pathologic features
GAVE is characterized by dilated capillaries in the lamina propriaLamina propria
The lamina propria is a constituent of the moist linings known as mucous membranes or mucosa, which line various tubes in the body ....
with fibrin
Fibrin
Fibrin is a fibrous, non-globular protein involved in the clotting of blood. It is a fibrillar protein that is polymerised to form a "mesh" that forms a hemostatic plug or clot over a wound site....
thrombi. The main histomorphologic differential diagnosis is portal hypertension
Portal hypertension
In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...
, which is often apparent from clinical findings.
Research in 2010 has shown that anti-RNA polymerase
RNA polymerase
RNA polymerase is an enzyme that produces RNA. In cells, RNAP is needed for constructing RNA chains from DNA genes as templates, a process called transcription. RNA polymerase enzymes are essential to life and are found in all organisms and many viruses...
III antibodies may be used as a risk marker for GAVE in systemic sclerosis patients.
Incidence
The average age of diagnosis for GAVE is 73 years of age for females, and 68 for males. Women are about twice as often diagnosed with gastric antral vascular ectasia than men. 71% of all cases of GAVE are diagnosed in females. Patients in their thirties have been found to have GAVE. It becomes more common in women in their eighties, rising to 4% of all such gastrointestinal conditions.5.7% of all sclerosis patients (and 25% of those who had a certain anti-RNA marker) have GAVE.
Etiology or pathogenesis
The literature, from 1953 through 2010, often cited that the etiology or pathogenesis of watermelon disease are unknown.Gender has some relationship to the etiology. 65% of patients with both cirrhosis and GAVE are male, but a total of 30% have both conditions. The causal connection between cirrhosis and GAVE has not been proven.
A connective tissue
Connective tissue
"Connective tissue" is a fibrous tissue. It is one of the four traditional classes of tissues . Connective Tissue is found throughout the body.In fact the whole framework of the skeleton and the different specialized connective tissues from the crown of the head to the toes determine the form of...
disease has been suspected in some cases.
Autoimmunity may have something to do with it, as 25% of all sclerosis patients who had a certain anti-RNA marker have GAVE. RNA autoimmunity has been suspected as a cause or marker since at least 1996.
One theory current since the 1990s focuses on a history of prolapse
Prolapse
Prolapse literally means "to fall out of place", from the Latin prolabi meaning "to fall out". In medicine, prolapse is a condition where organs, such as the uterus, fall down or slip out of place. It is used for organs protruding through the vagina or the rectum or for the misalignment of the...
of the stomach into 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...
.
Gastrin
Gastrin
In humans, gastrin is a peptide hormone that stimulates secretion of gastric acid by the parietal cells of the stomach and aids in gastric motility. It is released by G cells in the antrum of the stomach, duodenum, and the pancreas...
levels may indicate a hormonal connection. This may be due to a "vasoactive intestinal peptide and 5-hydroxy-tryptamine…".
It is also possible that infection by H. pylori can cause it.
Associated conditions
Portal hypertension
In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...
, chronic renal failure
Chronic renal failure
Chronic kidney disease , also known as chronic renal disease, is a progressive loss in renal function over a period of months or years. The symptoms of worsening kidney function are unspecific, and might include feeling generally unwell and experiencing a reduced appetite...
, and collagen vascular diseases.
Watermelon stomach also occurs particularly with scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
, and especially the subtype known as systemic sclerosis. A full 5.7% of persons with sclerosis have GAVE, and 25% of all sclerosis patients who had a certain anti-RNA marker have GAVE. In fact:
The endoscopic appearance of GAVE is similar to portal hypertensive gastropathy
Portal hypertensive gastropathy
Portal hypertensive gastropathy refers to changes in the mucosa of the stomach in patients with portal hypertension; by far the most common cause of this is cirrhosis of the liver. These changes in the mucosa include friability of the mucosa and the presence of ectatic blood vessels at the surface...
, but is not the same condition, and may be concurrent with cirrhosis of the liver. 30% of all patients have cirrhosis associated with GAVE.
Sjögren's syndrome
Sjögren's syndrome
Sjögren's syndrome , also known as "Mikulicz disease" and "Sicca syndrome", is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands that produce tears and saliva....
has been associated with at least one patient.
The first case of ectopic pancreas associated with watermelon stomach was reported in 2010.
Patients with GAVE may have elevated gastrin
Gastrin
In humans, gastrin is a peptide hormone that stimulates secretion of gastric acid by the parietal cells of the stomach and aids in gastric motility. It is released by G cells in the antrum of the stomach, duodenum, and the pancreas...
levels.
Traditional treatments
GAVE is treated commonly by means of an endoscope, including argon plasma coagulationArgon plasma coagulation
Argon plasma coagulation or APC is a medical endoscopic procedure used primarily to control bleeding from certain lesions in the gastrointestinal tract, and also sometimes to debulk tumours in the case of patients for whom surgery is not recommended...
and electrocautery. Since endoscopy with argon photocoagulation is "usually effective", surgery is "usually not required." Coagulation therapy is well-tolerated but "tends to induce oozing and bleeding." "Endoscopy with thermal ablation" is favored medical treatment because of its low side effects and low mortality, but is "rarely curative."
Other treatments
Other medical treatments have been tried and include estrogenEstrogen
Estrogens , oestrogens , or œstrogens, are a group of compounds named for their importance in the estrous cycle of humans and other animals. They are the primary female sex hormones. Natural estrogens are steroid hormones, while some synthetic ones are non-steroidal...
and progesterone
Progesterone
Progesterone also known as P4 is a C-21 steroid hormone involved in the female menstrual cycle, pregnancy and embryogenesis of humans and other species...
therapy, and anti-fibrinolytic drugs such as tranexamic acid
Tranexamic acid
Tranexamic acid is a drug used to treat or prevent excessive blood loss during surgery and in certain other conditions...
. Corticostreoids are effective, but are "limited by their side effects
Side Effects
Side Effects is an anthology of 17 comical short stories written by Woody Allen between 1975 and 1980, all but one of which were previously published in, variously, The New Republic, The New York Times, The New Yorker, and The Kenyon Review. It includes Allen's 1978 O...
."
Cryotherapy
Cryotherapy
Cryotherapy is the local or general use of low temperatures in medical therapy or the removal of heat from a body part. The term "cryotherapy" comes from the Greek cryo meaning cold and the word therapy meaning cure...
ablation is another possibility, which has been used for esophageal cancer
Esophageal cancer
Esophageal cancer is malignancy of the esophagus. There are various subtypes, primarily squamous cell cancer and adenocarcinoma . Squamous cell cancer arises from the cells that line the upper part of the esophagus...
, and Barrett's esophagus
Barrett's esophagus
Barrett's esophagus refers to an abnormal change in the cells of the inferior portion of the esophagus. A positive diagnosis generally requires observing specific macroscopic and microscopic changes...
. While used successfully for both esophageal dysplasia
Dysplasia
Dysplasia , is a term used in pathology to refer to an abnormality of development. This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells. Dysplasia is often indicative of an early neoplastic process...
and neoplasia
Neoplasia
Neoplasm is an abnormal mass of tissue as a result of neoplasia. Neoplasia is the abnormal proliferation of cells. The growth of neoplastic cells exceeds and is not coordinated with that of the normal tissues around it. The growth persists in the same excessive manner even after cessation of the...
, it has not yet been reported for the treatment of GAVE. The U.S. Food and Drug Administration
Food and Drug Administration
The Food and Drug Administration is an agency of the United States Department of Health and Human Services, one of the United States federal executive departments...
has approved such cryotherapy only for esophagus cancer and Barrett's with dysplasia
Dysplasia
Dysplasia , is a term used in pathology to refer to an abnormality of development. This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells. Dysplasia is often indicative of an early neoplastic process...
.
Treatment of co-morbid conditions
A transjugular intrahepatic portosystemic shuntTransjugular intrahepatic portosystemic shunt
A Transjugular Intrahepatic Portosystemic Shunt is an artificial channel within the liver that establishes communication between the inflow portal vein and the outflow hepatic vein...
(TIPS or TIPSS) procedure is used to treat portal hypertension when that is present as an associated condition. Unfortunately, the TIPSS, which has been used for similar conditions, may cause or exacerbate hepatic encephalopathy
Hepatic encephalopathy
Hepatic encephalopathy is the occurrence of confusion, altered level of consciousness and coma as a result of liver failure. In the advanced stages it is called hepatic coma or coma hepaticum...
. TIPSS-related encephalopathy occurs in about 30% of cases, with the risk being higher in those with previous episodes of encephalopathy, higher age, female sex, and liver disease due to causes other than alcohol. The patient, with his or her physician and family, must balance out a reduction in bleeding caused by TIPS with the significant risk of encephalopathy
Encephalopathy
Encephalopathy means disorder or disease of the brain. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of global brain dysfunction; this syndrome can be caused by many different illnesses.-Terminology:...
. Various shunts have been shown in a meta-study of 22 studies to be effective treatment to reduce bleeding, yet none have any demonstrated survival advantage.
If there is cirrhosis of the liver that has progressed to liver failure
Liver failure
Acute liver failure is the appearance of severe complications rapidly after the first signs of liver disease , and indicates that the liver has sustained severe damage . The complications are hepatic encephalopathy and impaired protein synthesis...
, then lactulose
Lactulose
Lactulose is a synthetic, non-digestible sugar used in the treatment of chronic constipation and hepatic encephalopathy, a complication of liver disease. It is a disaccharide formed from one molecule each of the simple sugars fructose and galactose...
may be prescribed for hepatic encephalopathy, especially for Type C encephalopathy with diabetes. Also, "antibiotics such as neomycin
Neomycin
Neomycin is an aminoglycoside antibiotic that is found in many topical medications such as creams, ointments, and eyedrops. The discovery of Neomycin dates back to 1949. It was discovered in the lab of Selman Waksman, who was later awarded the Nobel Prize in Physiology and medicine in 1951...
, metronidazole
Metronidazole
Metronidazole is a nitroimidazole antibiotic medication used particularly for anaerobic bacteria and protozoa. Metronidazole is an antibiotic, amebicide, and antiprotozoal....
, and rifaximin" may be used effectively to treat the encephalopathy by removing nitrogen-producing bacteria from the gut.
Paracentesis
Paracentesis
Paracentesis is a medical procedure involving needle drainage of fluid from a body cavity, most commonly the peritoneal cavity in the abdomen.A related procedure is thoracocentesis, which is needle drainage of the chest cavity...
, a medical procedure involving needle drainage of fluid from a body cavity
Body cavity
By the broadest definition, a body cavity is any fluid-filled space in a multicellular organism. However, the term usually refers to the space located between an animal’s outer covering and the outer lining of the gut cavity, where internal organs develop...
, may be used to remove fluid from the peritoneal cavity
Peritoneal cavity
The peritoneal cavity is a potential space between the parietal peritoneum and visceral peritoneum, that is, the two membranes that separate the organs in the abdominal cavity from the abdominal wall...
in the abdomen
Abdomen
In vertebrates such as mammals the abdomen constitutes the part of the body between the thorax and pelvis. The region enclosed by the abdomen is termed the abdominal cavity...
for such cases. This procedure uses a large needle, similar to the better-known amniocentesis
Amniocentesis
Amniocentesis is a medical procedure used in prenatal diagnosis of chromosomal abnormalities and fetal infections, in which a small amount of amniotic fluid, which contains fetal tissues, is sampled from the amnion or amniotic sac surrounding a developing fetus, and the fetal DNA is examined for...
.
Surgery
Surgery, consisting of excision of part of the lower stomach, also called antrectomy, is another option. Antrectomy is "the resection, or surgical removal, of a part of the stomach known as the antrumAntrum
In biology, antrum is a general term for a cavity or chamber which may have specific meaning in reference to certain organs or sites in the body.In vertebrates, it may mean for example:...
." Laparoscopic surgery
Laparoscopic surgery
Laparoscopic surgery, also called minimally invasive surgery , bandaid surgery, or keyhole surgery, is a modern surgical technique in which operations in the abdomen are performed through small incisions as opposed to the larger incisions needed in laparotomy.Keyhole surgery makes use of images...
is possible in some cases, and as of 2003, was a "novel approach to treating watermelon stomach."
A treatment used sometimes is endoscopic band ligation.
In 2010, a team of Japan
Japan
Japan is an island nation in East Asia. Located in the Pacific Ocean, it lies to the east of the Sea of Japan, China, North Korea, South Korea and Russia, stretching from the Sea of Okhotsk in the north to the East China Sea and Taiwan in the south...
ese surgeons performed a "novel endoscopic ablation
Ablation
Ablation is removal of material from the surface of an object by vaporization, chipping, or other erosive processes. This occurs in spaceflight during ascent and atmospheric reentry, glaciology, medicine, and passive fire protection.-Spaceflight:...
of gastric antral vascular ectasia." The experimental procedure resulted in "no complications."
Relapse is possible, even after treatment by argon plasma coagulation and progesterone. In such cases of relapse, surgery may be the only option; in one case that involved "Endoscopic mucosal resection
Segmental resection
Segmental resection is a surgical procedure to remove part of an organ or gland. It may also be used to remove a tumor and normal tissue around it. In lung cancer surgery, segmental resection refers to removing a section of a lobe of the lung.- External links :* entry in the public domain NCI...
".
Antrectomy or other surgery
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
is used as a last resort for GAVE. The risks of surgery should be considered. It is said that "surgery is the only cure" for GAVE.