Gastric antral vascular ectasia (GAVE) is an erratic cause of chronic upper gastrointestinal bleeding. Also known as watermelon stomach, this condition is marked by dilated capillaries localized to the antrum that give the appearance of watermelon streaks. The dilated vessels render the affected areas more susceptible to bleeding. Even though the etiology is still being speculated upon, and the occurrence is rare (4% of non-variceal upper GI bleeds)1, GAVE has become a distinct entity with established co-morbid conditions.
Signs and Symptoms
Like most cases of chronic upper gastrointestinal bleeding, many patients will complain of anemia and other symptoms of blood loss.2 More uncommon is the case that presents with acute blood loss requiring revival. Occasionally, a patient may notice melena and/or hematochezia. The chronicity of the anemia also plays a role in whether the patient protests of subsequent symptoms. Typically a patient that is chronically anemic is able to tolerate a lower hemoglobin and hematocrit.
Differential diagnosis
Because many diagnoses can present in this manner, it is imperative to construct an adequate differential, as the treatments for these conditions can be vastly diverse. An adequate differential would include, but is not limited to, more common causes, like a duodenal/gastric ulcer and portal hypertension3, and more intermittent causes, like: upper gastrointestinal tumors, Dieulafoy Lesions, Gastric antral vascular ectasia, portal hypertensive gastropathy, hemobilia, hemosuccus pancreaticus, aortoenteric fistulas, and Cameron lesions.
Diagnosis
The only way to conclusively diagnose GAVE is by means of endoscopic biopsy. The classic watermelon stripes are visualized during an endoscopy. These appear as longitudinal rows of flat, erythematous stripes radiating from the pylorus into the antrum. Another presentation exists in patients with co-existing portal hypertension. These individuals may have diffuse antral angiomas in lieu of the classic radiating pattern.1 In any case, the diagnosis may be confirmed with endoscopic ultrasound, CT scan, or tagged red blood cell scan.4 Moreover, vascular ectasia, spindle cell proliferation, and fibrohyanlinosis characterizes the histopathological features of GAVE.5 There remains some evidence that suggests anti-RNA polymerase III autoantibodies may be used for risk indication in a select group of GAVE patients with co-existing systemic sclerosis.
Incidence
Women represent 71% of all cases of GAVE making the incidence roughly twice that of males.2 The average age at diagnosis is also slightly elevated for females at 73 years of age vs. 68 for men.3 Patients as early as their late twenties have been treated for GAVE; however, it is much more prevalent in those in their eighties. Ultimately in the most affected patient population, GAVE is responsible for 4% of nonvariceal upper GI bleeding.1
Pathogenesis
Since its discovery in 1952 (describe in 1953), there has been much postulation as to the etiology of watermelon stomach. Even now GAVE is thought to occur in an idiopathic manner. Many speculate there to be a significant association with cirrhosis and systemic sclerosis.5,7 However, a causal connection between cirrhosis or a connective tissue disease with GAVE has not yet been proven.8
Autoimmunity is another theory. Anti-RNA polymerase III antibodies has been documented in 25% of sclerosis patients that had GAVE.6 This finding demonstrates future potential to be used as a predictive and prognostic marker. In support of an immune-related hypothesis, in 2012 a case of antral vascular ectasia was induced after a treatment of Imatinib for GIST.9 Further, Repeat endoscopy one month after discontinuation of Imatinib showed substantial improvement in gastric inflammation.
A different notion describes a more mechanical etiology. Since the 1990s there have been records of prolapses of the stomach into the small intestine prior to the development of GAVE.10 Yet, a final theory proposes a hormonal connection with vasoactive intestinal peptide and 5-hydroxy-tryptamine.11
Associated Diseases
Gastric antral vascular ectasia is associated with a number of conditions, but perhaps the most frequently studied include portal hypertension, chronic renal failure, and connective tissue disorders.12 Watermelon stomach occurs especially in the connective tissue disease, scleroderma, with the subtype systemic sclerosis (5.7% of patients with sclerosis have GAVE, and 25% of sclerosis patients with anti-RNA III polymerase have GAVE).6 In 2010, there were reported cases of both Sjogren syndrome13 and ectopic pancreas10 co-morbid with GAVE. Strikingly, cirrhosis has been associated with 30% of all patients with GAVE.8.11 Lastly, according to the Genetic and Rare Diseases Information Center (GARD), pernicious anemia is associated with GAVE’s as an independent study showed that over three-fourths of the patients had some kind of Vitamin B12 deficiency.14,15
Treatments
In rare cases when the bleeding is acute and massive, episodic transfusions are required. When the patient is stable, endoscopic coagulation with an argon plasma coagulator, bipolar probe, or heater probe obliterates the vascular ectasia and reduces the bleeding.1 Additionally, laser therapy can be used.16 Another well reported safe and effective method is endoscopic band ligation.17,18 In fact, studies show the recurrence for GAVE is much lower for endoscopic band ligation vs. argon plasma coagulation (8.3% vs. 68.2%)19,20
Notably, portal decompression with transjugular intrahepatic portosystemic shunt (TIPS) does not consistently reduce bleeding, accentuating the uncertain relationship of portal hypertension to GAVE.21,22 Additionally, another study indicated that combination estrogen/progesterone therapy may lessen bleeding, although the ectatic vessels appear to persist.23 For patients who fail or are not candidates for endoscopic therapies, antrectomy is the treatment of choice. Although reserved for the most advanced cases, it is the only, reliably curative treatment for gastric antral vascular ectasia.
References
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14. Watermelon Stomach. Genetic and Rare Diseases Information Center. National Institution of Health.
15. Watermelon stomach and radiation proctopathy. CCS Publishing. August 1, 2011.
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18. Keohan J, Berro W, Harewood G, Murray F, Patchett S. Band ligation of gastric antral vascular ectasia is a safe and effective endoscopic treatment. Digestive Endoscopy. 2013; 25:392-6.
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21. Spahr L, Villeneuve JP, Dufresne MP, Tasse D, Bui B, Willems B, Fenyves D, Pomier-Layrargues G. Gastric antral vascular ectasia in cirrhotic patients: absence of relation with portal hypertension. Gut. 1999;44(5):739.
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This original text was submitted by permission of Joseph K. Wray, MS4.