Gastric antral vascular ectasia (GAVE) is an uncommon cause of chronic

gastrointestinal bleeding Gastrointestinal bleeding (GI bleed), also called gastrointestinal hemorrhage (GIB), is all forms of bleeding in the gastrointestinal tract, from the mouth to the rectum. When there is significant blood loss over a short time, symptoms may include ...

iron deficiency anemia Iron-deficiency anemia is anemia caused by a lack of iron. Anemia is defined as a decrease in the number of red blood cells or the amount of hemoglobin in the blood. When onset is slow, symptoms are often vague such as feeling tired, weak, s ...

. The condition is associated with dilated small blood vessels in the

pyloric antrum The pylorus ( or ), or pyloric part, connects the stomach to the duodenum. The pylorus is considered as having two parts, the ''pyloric antrum'' (opening to the body of the stomach) and the ''pyloric canal'' (opening to the duodenum). The ''pylori ...

, which is a distal part of the

stomach The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...

. 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 (''Citrullus lanatus'') is a flowering plant species of the Cucurbitaceae family and the name of its edible fruit. A scrambling and trailing vine-like plant, it is a highly cultivated fruit worldwide, with more than 1,000 varie ...

. 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 cause and pathogenesis are still not known. However, there are several competing hypotheses as to various causes.

Signs and symptoms

Most patients who are eventually diagnosed with watermelon stomach come to a physician complaining of

anemia Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, t ...

and blood loss. Sometimes, a patient may come to the

physician A physician (American English), medical practitioner (Commonwealth English), medical doctor, or simply doctor, is a health professional who practices medicine, which is concerned with promoting, maintaining or restoring health through th ...

because he or she notices blood in the stools—either melena (black and tarry stools) and/or hematochezia (red bloody stools).

Cause

The literature, from 1953 through 2010, often cited that the cause of gastric antral vascular ectasia is unknown. The causal connection between

cirrhosis Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue rep ...

and GAVE has not been proven. A connective tissue 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. Gastrin levels may indicate a hormonal connection.

Associated conditions

GAVE is associated with a number of conditions, including portal hypertension,

chronic kidney failure Chronic kidney disease (CKD) is a type of kidney disease in which a gradual loss of kidney function occurs over a period of months to years. Initially generally no symptoms are seen, but later symptoms may include leg swelling, feeling tired, vo ...

, and

collagen vascular disease A connective tissue disease (collagenosis) is any disease that has the connective tissues of the body as a target of pathology. Connective tissue is any type of biological tissue with an extensive extracellular matrix that supports, binds togeth ...

s. Watermelon stomach also occurs particularly with

scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas ...

, 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 polymerase marker have GAVE. In fact: The endoscopic appearance of GAVE is similar to portal hypertensive gastropathy, but is not the same condition, and may be concurrent with

cirrhosis of the liver Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue repai ...

. 30% of all patients have cirrhosis associated with GAVE. Sjögren's syndrome has been associated with at least one patient. The first case of

ectopic pancreas An ectopic pancreas is an anatomical abnormality in which pancreatic tissue has grown outside its normal location and without vascular or other anatomical connections to the pancreas. It is a congenital disease and is also known as heterotopic, ac ...

associated with watermelon stomach was reported in 2010. Patients with GAVE may have elevated gastrin levels. The Genetic and Rare Diseases Information Center (GARD) states that

pernicious anemia Pernicious anemia is a type of vitamin B12 deficiency anemia, a disease in which not enough red blood cells are produced due to the malabsorption of vitamin B12. Malabsorption in pernicious anemia results from the lack or loss of intrinsic fa ...

is one of the conditions associated with GAVE's, and one separate study showed that over three-fourths of the patients in the study with GAVE's had some kind of

Vitamin B12 deficiency Vitamin B12 deficiency, also known as cobalamin deficiency, is the medical condition in which the blood and tissue have a lower than normal level of vitamin B12. Symptoms can vary from none to severe. Mild deficiency may have few or absent symp ...

including the associated condition pernicious anemia. Intestinal permeability and diverticulitis may occur in some patients with GAVE.

Pathogenesis

GAVE is characterized by dilated capillaries in the lamina propria with fibrin

thrombi A thrombus (plural thrombi), colloquially called a blood clot, is the final product of the blood coagulation step in hemostasis. There are two components to a thrombus: aggregated platelets and red blood cells that form a plug, and a mesh of cr ...

. The main histomorphologic differential diagnosis is portal hypertension, which is often apparent from clinical findings. Research in 2010 has shown that anti- RNA polymerase III antibodies may be used as a risk marker for GAVE in

systemic sclerosis Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two ...

patients.

Diagnosis

GAVE is usually diagnosed definitively by means of an

endoscopic An endoscopy is a procedure used in medicine to look inside the body. The endoscopy procedure uses an endoscope to examine the interior of a hollow organ or cavity of the body. Unlike many other medical imaging techniques, endoscopes are insert ...

biopsy. The tell-tale watermelon stripes show up during the endoscopy. Surgical exploration of the

abdomen The abdomen (colloquially called the belly, tummy, midriff, tucky or stomach) is the part of the body between the thorax (chest) and pelvis, in humans and in other vertebrates. The abdomen is the front part of the abdominal segment of the to ...

may be needed to diagnose some cases, especially if the liver or other organs are involved.

Differential diagnosis

GAVE results in intestinal bleeding similar to duodenal ulcers and portal hypertension. The GI bleeding can result in

. It is often overlooked, but can be more common in elderly patients. It has been seen in a female patient of 26 years of age. Watermelon stomach has a different etiology and has a differential diagnosis 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.

Treatment

Traditional treatments

GAVE is treated commonly by means of an endoscope, including

argon plasma coagulation Argon plasma coagulation (APC) is a medical endoscopic procedure used to control bleeding from certain lesions in the gastrointestinal tract. It is administered during esophagogastroduodenoscopy or colonoscopy. Medical use APC involves the use o ...

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." Treatment of GAVE can be categorized into endoscopic, surgical and pharmacologic. Surgical treatment is definitive but it is rarely done nowadays with the variety of treatment options available. Some of the discussed modalities have been used in GAVE patients with another underlying disease rather than SSc; they are included as they may be tried in resistant SSc-GAVE patients. Symptomatic treatment includes iron supplementation and blood transfusion for cases with severe anemia, proton pump inhibitors may ameliorate the background chronic gastritis and minute erosions that commonly co-existed in biopsy reports.

Medications

Other medical treatments have been tried and include

estrogen Estrogen or oestrogen is a category of sex hormone responsible for the development and regulation of the female reproductive system and secondary sex characteristics. There are three major endogenous estrogens that have estrogenic hormonal ac ...

and progesterone therapy, Corticostreoids are effective, but are "limited by their

side effects In medicine, a side effect is an effect, whether therapeutic or adverse, that is secondary to the one intended; although the term is predominantly employed to describe adverse effects, it can also apply to beneficial, but unintended, consequence ...

Treatment of co-morbid conditions

transjugular intrahepatic portosystemic shunt Transjugular intrahepatic portosystemic shunt (TIPS or TIPSS) is an artificial channel within the liver that establishes communication between the inflow portal vein and the outflow hepatic vein. It is used to treat portal hypertension (which is of ...

(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 (HE) is an altered level of consciousness as a result of liver failure. Its onset may be gradual or sudden. Other symptoms may include movement problems, changes in mood, or changes in personality. In the advanced stage ...

. 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 (; from grc, ἐνκέφαλος "brain" + πάθος "suffering") means any disorder or disease of the brain, especially chronic degenerative conditions. In modern usage, encephalopathy does not refer to a single disease, but r ...

. Various shunts have been shown in a meta-study of 22 studies to be effective treatment to reduce variceal bleeding, yet none have any demonstrated survival advantage. If there is cirrhosis of the liver that has progressed to liver failure, then

lactulose Lactulose is a non-absorbable sugar used in the treatment of constipation and hepatic encephalopathy. It is used by mouth for constipation and either by mouth or in the rectum for hepatic encephalopathy. It generally begins working after 8–12 ...

may be prescribed for hepatic encephalopathy, especially for '' Type C encephalopathy'' with

diabetes Diabetes, also known as diabetes mellitus, is a group of metabolic disorders characterized by a high blood sugar level ( hyperglycemia) over a prolonged period of time. Symptoms often include frequent urination, increased thirst and increased ...

. Also, "antibiotics such as

neomycin Neomycin is an aminoglycoside antibiotic that displays bactericidal activity against gram-negative aerobic bacilli and some anaerobic bacilli where resistance has not yet arisen. It is generally not effective against gram-positive bacilli and ...

metronidazole Metronidazole, sold under the brand name Flagyl among others, is an antibiotic and antiprotozoal medication. It is used either alone or with other antibiotics to treat pelvic inflammatory disease, endocarditis, and bacterial vaginosis. It i ...

, and

rifaximin Rifaximin, is a non-absorbable, broad spectrum antibiotic mainly used to treat travelers' diarrhea. It is based on the rifamycin antibiotics family. Since its approval in Italy in 1987, it has been licensed in over more than 30 countries for th ...

" may be used effectively to treat the encephalopathy by removing nitrogen-producing bacteria from the gut.

Paracentesis Paracentesis (from Greek κεντάω, "to pierce") is a form of body fluid sampling procedure, generally referring to peritoneocentesis (also called laparocentesis or abdominal paracentesis) in which the peritoneal cavity is punctured by a needl ...

, a medical procedure involving needle drainage of fluid from a

body cavity A body cavity is any space or compartment, or potential space, in an animal body. Cavities accommodate organs and other structures; cavities as potential spaces contain fluid. The two largest human body cavities are the ventral body cavity, and ...

, may be used to remove fluid from the

peritoneal cavity The peritoneal cavity is a potential space between the parietal peritoneum (the peritoneum that surrounds the abdominal wall) and visceral peritoneum (the peritoneum that surrounds the internal organs). The parietal and visceral peritonea are la ...

in the

for such cases. This procedure uses a large needle, similar to the better-known

amniocentesis Amniocentesis is a medical procedure used primarily in the prenatal diagnosis of genetic conditions. It has other uses such as in the assessment of infection and fetal lung maturity. Prenatal diagnostic testing, which includes amniocentesis, is n ...

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

antrum ''This is a disambiguation page for the biological term. For the 2018 horror movie, see Antrum (film)'' 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 bod ...

".Surgery Encyclopedia website page on Antrectomy
Accessed September 29, 2010.

Laparoscopic surgery Laparoscopy () is an operation performed in the abdomen or pelvis using small incisions (usually 0.5–1.5 cm) with the aid of a camera. The laparoscope aids diagnosis or therapeutic interventions with a few small cuts in the abdomen.Medli ...

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 Japanese surgeons performed a "novel endoscopic

ablation Ablation ( la, ablatio – removal) is removal or destruction of something from an object by vaporization, chipping, erosive processes or by other means. Examples of ablative materials are described below, and include spacecraft material for a ...

of gastric antral vascular ectasia". The experimental procedure resulted in "no complications". Relapse is possible, even after treatment by argon plasma coagulation and progesterone. Antrectomy or other surgery is used as a last resort for GAVE.

Epidemiology

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.

References

External links

{{DEFAULTSORT:Gastric Antral Vascular Ectasia Stomach disorders Diseases of liver