Gastric antral vascular ectasia

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Gastric antral vascular ectasia
Other namesWatermelon stomach, watermelon disease
Gastric antral vascular ectasia (before and after).png
Endoscopic image of gastric antral vascular ectasia seen as a radial pattern around the pylorus before (top) and after (bottom) treatment with argon plasma coagulation
Specialty Gastroenterology   OOjs UI icon edit-ltr-progressive.svg
Symptoms Bleeding in the stomach and intestines, edema, dilated blood vessels

Gastric antral vascular ectasia (GAVE) is an uncommon cause of chronic gastrointestinal bleeding or iron deficiency anemia. [1] [2] The condition is associated with dilated small blood vessels in the gastric antrum, which is a distal part of the stomach. [1] The dilated vessels result in intestinal bleeding. [3] It is also called watermelon stomach because streaky long red areas that are present in the stomach may resemble the markings on watermelon. [1] [2] [3] [4]

Contents

The condition was first discovered in 1952, [2] and reported in the literature in 1953. [5] 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. [4] As of 2011, the cause and pathogenesis are still not known. [4] [6] However, there are several competing hypotheses as to various causes. [4]

Signs and symptoms

Most patients who are eventually diagnosed with watermelon stomach come to a physician complaining of anemia and blood loss. [7] Sometimes, a patient may come to the physician because he or she notices blood in the stools—either melena (black and tarry stools) and/or hematochezia (red bloody stools). [7]

Cause

The literature, from 1953 through 2010, often cited that the cause of gastric antral vascular ectasia is unknown. [4] [6] [7] The causal connection between cirrhosis and GAVE has not been proven. [6] A connective tissue disease has been suspected in some cases. [7]

Autoimmunity may have something to do with it, [8] as 25% of all sclerosis patients who had a certain anti-RNA marker have GAVE. [9] RNA autoimmunity has been suspected as a cause or marker since at least 1996. [8] Gastrin levels may indicate a hormonal connection. [6]

Associated conditions

Micrograph showing liver cirrhosis, a condition that often precedes hepatic encephalopathy. Trichrome stain. Cirrhosis high mag.jpg
Micrograph showing liver cirrhosis, a condition that often precedes hepatic encephalopathy. Trichrome stain.

GAVE is associated with a number of conditions, including portal hypertension, chronic kidney failure, and collagen vascular diseases. [2] [10] [11]

Watermelon stomach also occurs particularly with scleroderma, [2] [12] [13] [14] and especially the subtype known as systemic sclerosis. [2] [9] 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. [9] In fact:

Most patients with GAVE suffer from liver cirrhosis, autoimmune disease, chronic kidney failure and bone marrow transplantation. The typical initial presentations range from occult bleeding causing transfusion-dependent chronic iron-deficiency anemia to severe acute gastrointestinal bleeding.

— Masae Komiyama, et al., 2010. [10]

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. [2] [6] [15] [16] 30% of all patients have cirrhosis associated with GAVE. [6]

Sjögren's syndrome has been associated with at least one patient. [17]

The first case of ectopic pancreas associated with watermelon stomach was reported in 2010. [4]

Patients with GAVE may have elevated gastrin levels. [6]

The Genetic and Rare Diseases Information Center (GARD) states that pernicious anemia is one of the conditions associated with GAVE, [18] and one separate study showed that over three-fourths of the patients in the study with GAVE had some kind of vitamin B12 deficiency including the associated condition pernicious anemia. [19]

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. The main histomorphologic differential diagnosis is portal hypertension, which is often apparent from clinical findings.[ citation needed ]

Research in 2010 has shown that anti-RNA polymerase III antibodies may be used as a risk marker for GAVE in systemic sclerosis patients. [9]

Diagnosis

Micrograph showing gastric antral vascular ectasia. A large spherical, eosinophilic (i.e. pink) fibrin thrombus is seen off-center right. Stomach biopsy. H&E stain. Gastric antral vascular ectasia - 2 - high mag.jpg
Micrograph showing gastric antral vascular ectasia. A large spherical, eosinophilic (i.e. pink) fibrin thrombus is seen off-center right. Stomach biopsy. H&E stain.

GAVE is usually diagnosed definitively by means of an endoscopic biopsy. [6] [7] [10] [20] The tell-tale watermelon stripes show up during the endoscopy. [7]

Surgical exploration of the abdomen may be needed to diagnose some cases, especially if the liver or other organs are involved. [4]

Differential diagnosis

GAVE results in intestinal bleeding similar to duodenal ulcers and portal hypertension. [3] [6] The GI bleeding can result in anemia. [6] [7] It is often overlooked, but can be more common in elderly patients. [3] [7] It has been seen in a female patient of 26 years of age. [6]

Watermelon stomach has a different etiology and has a differential diagnosis from portal hypertension. [6] [15] In fact, cirrhosis and portal hypertension may be missing in a patient with GAVE. [6] The differential diagnosis is important because treatments are different. [3] [6] [7] [10]

Treatment

Traditional treatments

GAVE is treated commonly by means of an endoscope, including argon plasma coagulation and electrocautery. [6] [7] [21] Since endoscopy with argon photocoagulation is "usually effective", surgery is "usually not required". [7] Coagulation therapy is well tolerated but "tends to induce oozing and bleeding." [7] "Endoscopy with thermal ablation" is favored medical treatment because of its low side effects and low mortality, but is "rarely curative." [6] 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. [11]

Medications

Other medical treatments have been tried and include estrogen and progesterone therapy, [21] Corticostreoids are effective, but are "limited by their side effects." [7]

Treatment of co-morbid conditions

A transjugular intrahepatic portosystemic shunt (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. [22] [23] 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. [24] The patient, with their physician and family, must balance out a reduction in bleeding caused by TIPS with the significant risk of encephalopathy. [22] [23] [24] 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. [22]

If there is cirrhosis of the liver that has progressed to liver failure, then lactulose may be prescribed for hepatic encephalopathy, especially for Type C encephalopathy with diabetes. [24] Also, "antibiotics such as neomycin, metronidazole, and rifaximin" may be used effectively to treat the encephalopathy by removing nitrogen-producing bacteria from the gut. [24]

Paracentesis, a medical procedure involving needle drainage of fluid from a body cavity, [25] may be used to remove fluid from the peritoneal cavity in the abdomen for such cases. [23] This procedure uses a large needle, similar to the better-known amniocentesis.

Surgery

Surgery, consisting of excision of part of the lower stomach, also called antrectomy, is another option. [6] [16] Antrectomy is "the resection, or surgical removal, of a part of the stomach known as the antrum". [2] Laparoscopic surgery is possible in some cases, and as of 2003, was a "novel approach to treating watermelon stomach". [26]

A treatment used sometimes is endoscopic band ligation. [27]

In 2010, a team of Japanese surgeons performed a "novel endoscopic ablation of gastric antral vascular ectasia". [10] The experimental procedure resulted in "no complications". [10]

Relapse is possible, even after treatment by argon plasma coagulation and progesterone. [21]

Antrectomy or other surgery is used as a last resort for GAVE. [2] [6] [7] [10] [15] [16] [ excessive citations ]

Epidemiology

The average age of diagnosis for GAVE is 73 years of age for females, [3] [7] and 68 for males. [2] Women are about twice as often diagnosed with gastric antral vascular ectasia than men. [2] [7] 71% of all cases of GAVE are diagnosed in females. [3] [7] Patients in their thirties have been found to have GAVE. [6] It becomes more common in women in their eighties, rising to 4% of all such gastrointestinal conditions. [10]

5.7% of all sclerosis patients (and 25% of those who had a certain anti-RNA marker) have GAVE. [9]

Related Research Articles

<span class="mw-page-title-main">Systemic scleroderma</span> Medical condition

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 major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above, the elbows and knees with or without involvement of the face. The diffuse form also affects the skin above the elbows and knees and can also spread to the torso. Visceral organs, including the kidneys, heart, lungs, and gastrointestinal tract can also be affected by the fibrotic process. Prognosis is determined by the form of the disease and the extent of visceral involvement. Patients with limited systemic sclerosis have a better prognosis than those with the diffuse form. Death is most often caused by lung, heart, and kidney involvement. The risk of cancer is increased slightly.

<span class="mw-page-title-main">Upper gastrointestinal bleeding</span> Medical condition

Upper gastrointestinal bleeding is gastrointestinal bleeding (hemorrhage) in the upper gastrointestinal tract, commonly defined as bleeding arising from the esophagus, stomach, or duodenum. Blood may be observed in vomit or in altered form as black stool. Depending on the amount of the blood loss, symptoms may include shock.

Hematemesis is the vomiting of blood. It can be confused with hemoptysis or epistaxis (nosebleed), which are more common. The source is generally the upper gastrointestinal tract, typically above the suspensory muscle of duodenum. It may be caused by ulcers, tumors of the stomach or esophagus, varices, prolonged and vigorous retching, gastroenteritis, ingested blood, or certain drugs.

Coffee ground vomitus refers to a particular appearance of vomit. Within organic heme molecules of red blood cells is the element iron, which oxidizes following exposure to gastric acid. This reaction causes the vomitus to look like ground coffee.

<span class="mw-page-title-main">Angiodysplasia</span> Medical condition

In medicine (gastroenterology), angiodysplasia is a small vascular malformation of the gut. It is a common cause of otherwise unexplained gastrointestinal bleeding and anemia. Lesions are often multiple, and frequently involve the cecum or ascending colon, although they can occur at other places. Treatment may be with colonoscopic interventions, angiography and embolization, medication, or occasionally surgery.

<span class="mw-page-title-main">Esophagogastroduodenoscopy</span> Diagnostic endoscopic procedure

Esophagogastroduodenoscopy (EGD) or oesophagogastroduodenoscopy (OGD), also called by various other names, is a diagnostic endoscopic procedure that visualizes the upper part of the gastrointestinal tract down to the duodenum. It is considered a minimally invasive procedure since it does not require an incision into one of the major body cavities and does not require any significant recovery after the procedure. However, a sore throat is common.

<span class="mw-page-title-main">Esophageal varices</span> Medical condition

Esophageal varices are extremely dilated sub-mucosal veins in the lower third of the esophagus. They are most often a consequence of portal hypertension, commonly due to cirrhosis. People with esophageal varices have a strong tendency to develop severe bleeding which left untreated can be fatal. Esophageal varices are typically diagnosed through an esophagogastroduodenoscopy.

<span class="mw-page-title-main">Portal hypertension</span> Abnormally increased portal venous pressure

Portal hypertension is defined as increased portal venous pressure, with a hepatic venous pressure gradient greater than 5 mmHg. Normal portal pressure is 1–4 mmHg; clinically insignificant portal hypertension is present at portal pressures 5–9 mmHg; clinically significant portal hypertension is present at portal pressures greater than 10 mmHg. The portal vein and its branches supply most of the blood and nutrients from the intestine to the liver.

<span class="mw-page-title-main">Dieulafoy's lesion</span> Medical condition

Dieulafoy's lesion is a medical condition characterized by a large tortuous artery most commonly in the stomach wall (submucosal) that erodes and bleeds. It can present in any part of the gastrointestinal tract. It can cause gastric hemorrhage but is relatively uncommon. It is thought to cause less than 5% of all gastrointestinal bleeds in adults. It was named after French surgeon Paul Georges Dieulafoy, who described this condition in his paper "Exulceratio simplex: Leçons 1-3" in 1898. It is also called "caliber-persistent artery" or "aneurysm" of gastric vessels. However, unlike most other aneurysms, these are thought to be developmental malformations rather than degenerative changes.

<span class="mw-page-title-main">Gastrointestinal bleeding</span> Medical condition

Gastrointestinal bleeding, 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 vomiting red blood, vomiting black blood, bloody stool, or black stool. Small amounts of bleeding over a long time may cause iron-deficiency anemia resulting in feeling tired or heart-related chest pain. Other symptoms may include abdominal pain, shortness of breath, pale skin, or passing out. Sometimes in those with small amounts of bleeding no symptoms may be present.

<span class="mw-page-title-main">Gastric varices</span> Medical condition

Gastric varices are dilated submucosal veins in the lining of the stomach, which can be a life-threatening cause of bleeding in the upper gastrointestinal tract. They are most commonly found in patients with portal hypertension, or elevated pressure in the portal vein system, which may be a complication of cirrhosis. Gastric varices may also be found in patients with thrombosis of the splenic vein, into which the short gastric veins that drain the fundus of the stomach flow. The latter may be a complication of acute pancreatitis, pancreatic cancer, or other abdominal tumours, as well as hepatitis C. Gastric varices and associated bleeding are a potential complication of schistosomiasis resulting from portal hypertension.

<span class="mw-page-title-main">Gastrointestinal disease</span> Medical condition

Gastrointestinal diseases refer to diseases involving the gastrointestinal tract, namely the esophagus, stomach, small intestine, large intestine and rectum, and the accessory organs of digestion, the liver, gallbladder, and pancreas.

<span class="mw-page-title-main">Transjugular intrahepatic portosystemic shunt</span> Artificial channel within the liver

Transjugular intrahepatic portosystemic shunt 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 frequently leads to intestinal bleeding, life-threatening esophageal bleeding and the buildup of fluid within the abdomen (ascites).

<span class="mw-page-title-main">Distal splenorenal shunt procedure</span> Medical procedure

In medicine, a distal splenorenal shunt procedure (DSRS), also splenorenal shunt procedure and Warren shunt, is a surgical procedure in which the distal splenic vein is attached to the left renal vein. It is used to treat portal hypertension and its main complication. It was developed by W. Dean Warren.

<span class="mw-page-title-main">Argon plasma coagulation</span> Medical procedure

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.

<span class="mw-page-title-main">Double-balloon enteroscopy</span>

Double-balloon enteroscopy, also known as push-and-pull enteroscopy, is an endoscopic technique for visualization of the small bowel. It was developed by Hironori Yamamoto in 2001. It is novel in the field of diagnostic gastroenterology as it is the first endoscopic technique that allows for the entire gastrointestinal tract to be visualized in real time.

<span class="mw-page-title-main">Portal hypertensive gastropathy</span> Changes in the mucosa of the stomach in patients with portal hypertension

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. Patients with portal hypertensive gastropathy may experience bleeding from the stomach, which may uncommonly manifest itself in vomiting blood or melena; however, portal hypertension may cause several other more common sources of upper gastrointestinal bleeding, such as esophageal varices and gastric varices. On endoscopic evaluation of the stomach, this condition shows a characteristic mosaic or "snake-skin" appearance to the mucosa of the stomach.

Therapeutic endoscopy is the medical term for an endoscopic procedure during which treatment is carried out via the endoscope. This contrasts with diagnostic endoscopy, where the aim of the procedure is purely to visualize a part of the gastrointestinal, respiratory or urinary tract in order to aid diagnosis. In practice, a procedure which starts as a diagnostic endoscopy may become a therapeutic endoscopy depending on the findings, such as in cases of upper gastrointestinal bleeding, or the finding of polyps during colonoscopy.

<span class="mw-page-title-main">Cirrhosis</span> Chronic disease of the liver, characterized by fibrosis

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 to the liver leads to repair of liver tissue and subsequent formation of scar tissue. Over time, scar tissue can replace normal functioning tissue, leading to the impaired liver function of cirrhosis. The disease typically develops slowly over months or years. Early symptoms may include tiredness, weakness, loss of appetite, unexplained weight loss, nausea and vomiting, and discomfort in the right upper quadrant of the abdomen. As the disease worsens, symptoms may include itchiness, swelling in the lower legs, fluid build-up in the abdomen, jaundice, bruising easily, and the development of spider-like blood vessels in the skin. The fluid build-up in the abdomen may develop into spontaneous infections. More serious complications include hepatic encephalopathy, bleeding from dilated veins in the esophagus, stomach, or intestines, and liver cancer.Stages of cirrhosis include compensated cirrhosis and decompensated cirrhosis.

<span class="mw-page-title-main">Antrectomy</span> Type of gastric resection surgery

Antrectomy, also called distal gastrectomy, is a type of gastric resection surgery that involves the removal of the stomach antrum to treat gastric diseases causing the damage, bleeding, or blockage of the stomach. This is performed using either the Billroth I (BI) or Billroth II (BII) reconstruction method. Quite often, antrectomy is used alongside vagotomy to maximise its safety and effectiveness. Modern antrectomies typically have a high success rate and low mortality rate, but the exact numbers depend on the specific conditions being treated.

References

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  2. 1 2 3 4 5 6 7 8 9 10 11 Surgery Encyclopedia website page on Antrectomy. Accessed September 29, 2010.
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  8. 1 2 Valdez, BC; Henning, D; Busch, RK; Woods, K; Flores-Rozas, H; Hurwitz, J; Perlaky, L; Busch, H (1996). "A nucleolar RNA helicase recognized by autoimmune antibodies from a patient with watermelon stomach disease". Nucleic Acids Research. 24 (7): 1220–4. doi:10.1093/nar/24.7.1220. PMC   145780 . PMID   8614622.
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  10. 1 2 3 4 5 6 7 8 Komiyama, Masae; Fu, K; Morimoto, T; Konuma, H; Yamagata, T; Izumi, Y; Miyazaki, A; Watanabe, S (2010). "A novel endoscopic ablation of gastric antral vascular ectasia". World Journal of Gastrointestinal Endoscopy. 2 (8): 298–300. doi: 10.4253/wjge.v2.i8.298 . PMC   2999147 . PMID   21160630.
  11. 1 2 El-Gendy, Hala; Shohdy, Kyrillus S.; Maghraby, Gehad G.; Abadeer, Kerolos; Mahmoud, Moustafa (2017-02-01). "Gastric antral vascular ectasia in systemic sclerosis: Where do we stand?". International Journal of Rheumatic Diseases. 20 (12): 2133–2139. doi:10.1111/1756-185X.13047. ISSN   1756-185X. PMID   28217887. S2CID   24009655.
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  17. ↑ Krstić, M; Alempijević, T; Andrejević, S; Zlatanović, M; Damjanov, N; Ivanović, B; Jovanović, I; Tarabar, D; Milosavljević, T (2010). "Watermelon stomach in a patient with primary Sjögren's syndrome". Vojnosanitetski Pregled. Military-medical and Pharmaceutical Review. 67 (3): 256–8. doi: 10.2298/VSP1003256K . PMID   20361704.
  18. ↑ "Watermelon Stomach" Archived 2012-01-01 at the Wayback Machine Genetic and Rare Diseases Information Center (GARD), National Institution of Health.
  19. ↑ "Watermelon Stomach and Radiiation Proctopathy CCS Publishing, August 1, 2011
  20. ↑ Gilliam, John H.; Geisinger, Kim R.; Wu, Wallace C.; Weidner, Noel; Richter, Joel E. (1989). "Endoscopic biopsy is diagnostic in gastric antral vascular ectasia". Digestive Diseases and Sciences. 34 (6): 885–8. doi:10.1007/BF01540274. PMID   2721320. S2CID   25328326.
  21. 1 2 3 Shibukawa, G; Irisawa, A; Sakamoto, N; Takagi, T; Wakatsuki, T; Imamura, H; Takahashi, Y; Sato, A; et al. (2007). "Gastric antral vascular ectasia (GAVE) associated with systemic sclerosis: relapse after endoscopic treatment by argon plasma coagulation". Internal Medicine (Tokyo, Japan). 46 (6): 279–83. doi: 10.2169/internalmedicine.46.6203 . PMID   17379994.
  22. 1 2 3 Khan S, Tudur Smith C, Williamson P, Sutton R (2006). "Portosystemic shunts versus endoscopic therapy for variceal rebleeding in patients with cirrhosis". The Cochrane Database of Systematic Reviews. 2006 (4): CD000553. doi:10.1002/14651858.CD000553.pub2. PMC   7045742 . PMID   17054131.
  23. 1 2 3 Saab S, Nieto JM, Lewis SK, Runyon BA (2006). "TIPS versus paracentesis for cirrhotic patients with refractory ascites". The Cochrane Database of Systematic Reviews. 2010 (4): CD004889. doi:10.1002/14651858.CD004889.pub2. PMC   8855742 . PMID   17054221.
  24. 1 2 3 4 Sundaram V, Shaikh OS (July 2009). "Hepatic encephalopathy: pathophysiology and emerging therapies". Med. Clin. North Am. 93 (4): 819–36, vii. doi:10.1016/j.mcna.2009.03.009. PMID   19577116.
  25. ↑ "paracentesis" at Dorland's Medical Dictionary
  26. ↑ Sherman, V; Klassen, DR; Feldman, LS; Jabbari, M; Marcus, V; Fried, GM (2003). "Laparoscopic antrectomy: a novel approach to treating watermelon stomach". Journal of the American College of Surgeons. 197 (5): 864–7. doi:10.1016/S1072-7515(03)00600-8. PMID   14585429.
  27. ↑ Wells, C; Harrison, M; Gurudu, S; Crowell, M; Byrne, T; Depetris, G; Sharma, V (2008). "Treatment of gastric antral vascular ectasia (watermelon stomach) with endoscopic band ligation". Gastrointestinal Endoscopy. 68 (2): 231–6. doi:10.1016/j.gie.2008.02.021. PMID   18533150.

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