Gastric antral vascular ectasia

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Gastric antral vascular ectasia
Classification and external resources
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
ICD-10 K31.88
ICD-9 537.82
DiseasesDB 29505
MeSH D020252

Gastric antral vascular ectasia (GAVE) is an uncommon cause of chronic gastrointestinal bleeding or iron deficiency anemia.[1][2][3] The condition is associated with dilated small blood vessels in the antrum, or the last part of the stomach.[1] The dilated vessels result in intestinal bleeding.[4] 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][5]

The condition was first discovered in 1952,[2] and reported in the literature in 1953.[6] 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.[5] As of 2011, the etiology and pathogenesis are still not known.[3][5][7] However, there are several competing hypotheses as to various etiologies.[3][5]

Signs and symptoms[edit]

Most patients who are eventually diagnosed with watermelon stomach come to a physician complaining of anemia and blood loss.[8] Sometimes, a patient may come to the medical doctor because he or she notices blood in the stools — either melena (black and tarry stools) and/or hematochezia (maroon stools).[8]

Differential diagnosis[edit]

GAVE results in intestinal bleeding similar to duodenal ulcers and portal hypertension.[3][4][7] The GI bleeding can result in anemia,[7][8] as well as occult blood in the stool.[3] It is often overlooked, but can be more common in elderly patients.[3][4][8] It has been seen in patients as young as 34 years of age.[7]

Watermelon stomach has a different etiology and has a differential diagnosis from portal hypertension.[7][9] In fact, cirrhosis and portal hypertension may be missing in a patient with GAVE.[7] The differential diagnosis is important because treatments are different.[3][4][7][8][10]

Diagnosis[edit]

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.[7][8][10][11] The tell-tale watermelon stripes show up during the endoscopy.[8]

Surgical exploration of the abdomen may be needed to diagnose some cases, especially if the liver or other organs are involved.[5]

Pathologic features[edit]

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.

Research in 2010 has shown that anti-RNA polymerase III antibodies may be used as a risk marker for GAVE in systemic sclerosis patients.[12]

Incidence[edit]

The average age of diagnosis for GAVE is 73 years of age for females,[4][8] and 68 for males.[2] Women are about twice as often diagnosed with gastric antral vascular ectasia than men.[2][8] 71% of all cases of GAVE are diagnosed in females.[4][8] Patients in their thirties have been found to have GAVE.[7] 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.[12]

Etiology or pathogenesis[edit]

The literature, from 1953 through 2010, often cited that the etiology or pathogenesis of watermelon disease are unknown.[5][7][8]

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.[3] The causal connection between cirrhosis and GAVE has not been proven.[7]

A connective tissue disease has been suspected in some cases.[8]

Autoimmunity may have something to do with it,[13] as 25% of all sclerosis patients who had a certain anti-RNA marker have GAVE.[12] RNA autoimmunity has been suspected as a cause or marker since at least 1996.[13]

One theory current since the 1990s focuses on a history of prolapse of the stomach into the small intestine.[3][5]

Gastrin levels may indicate a hormonal connection.[7] This may be due to a "vasoactive intestinal peptide and 5-hydroxy-tryptamine…".[3]

It is also possible that infection by H. pylori can cause it.[citation needed]

Associated conditions[edit]

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 renal failure, and collagen vascular diseases.[2][10]

Watermelon stomach also occurs particularly with scleroderma,[2][14][15][16] and especially the subtype known as systemic sclerosis.[2][12] 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.[12] In fact:

Most patients with GAVE suffer from liver cirrhosis, autoimmune disease, chronic renal 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][7][9][17] 30% of all patients have cirrhosis associated with GAVE.[3][7]

Sjögren's syndrome has been associated with at least one patient.[18]

The first case of ectopic pancreas associated with watermelon stomach was reported in 2010.[5]

Patients with GAVE may have elevated gastrin levels.[7]

The Genetic and Rare Diseases Information Center (GARD) states that pernicious anemia is one of the conditions associated with GAVE's,[19] 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 including the associated condition pernicious anemia. [20]

Intestinal permeability and diverticulitis may occur in some patients with GAVE.

Treatment and prognosis[edit]

Treatments for GAVE have proliferated in recent years.

Traditional treatments[edit]

GAVE is treated commonly by means of an endoscope, including argon plasma coagulation and electrocautery.[7][8][21] Since endoscopy with argon photocoagulation is "usually effective", surgery is "usually not required."[8] Coagulation therapy is well-tolerated but "tends to induce oozing and bleeding."[8] "Endoscopy with thermal ablation" is favored medical treatment because of its low side effects and low mortality, but is "rarely curative."[7]

Other treatments[edit]

Other medical treatments have been tried and include estrogen and progesterone therapy,[21] and anti-fibrinolytic drugs such as tranexamic acid.[citation needed] Corticostreoids are effective, but are "limited by their side effects."[8]

Cryotherapy ablation is another possibility, which has been used for esophageal cancer,[22][23][24] and Barrett's esophagus.[25] While used successfully for both esophageal dysplasia and neoplasia,[26] it has not yet been reported for the treatment of GAVE. The U.S. Food and Drug Administration has approved such cryotherapy only for esophagus cancer and Barrett's with dysplasia.[22]

Treatment of co-morbid conditions[edit]

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.[27][28] 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.[29] 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.[27][28][29] 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.[27]

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.[29] Also, "antibiotics such as neomycin, metronidazole, and rifaximin" may be used effectively to treat the encephalopathy by removing nitrogen-producing bacteria from the gut.[29]

Paracentesis, a medical procedure involving needle drainage of fluid from a body cavity,[30] may be used to remove fluid from the peritoneal cavity in the abdomen for such cases.[28] This procedure uses a large needle, similar to the better-known amniocentesis.

Surgery[edit]

Surgery, consisting of excision of part of the lower stomach, also called antrectomy, is another option.[7][17] 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."[31]

A treatment used sometimes is endoscopic band ligation.[32]

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] In such cases of relapse, surgery may be the only option; in one case that involved "Endoscopic mucosal resection".[33]

Antrectomy or other surgery is used as a last resort for GAVE.[2][3][7][8][9][10][17][33] The risks of surgery should be considered.[8][33] It is said that "surgery is the only cure" for GAVE.[3]

References[edit]

  1. ^ a b c Suit, PF; Petras, RE; Bauer, TW; Petrini Jr, JL (1987). "Gastric antral vascular ectasia. A histologic and morphometric study of "the watermelon stomach"". The American journal of surgical pathology 11 (10): 750–7. doi:10.1097/00000478-198710000-00002. PMID 3499091. 
  2. ^ a b c d e f g h i j k Surgery Encyclopedia website page on Antrectomy. Accessed September 29, 2010.
  3. ^ a b c d e f g h i j k l m n Leventoğlu, Sezai; Güliter, Sefa; Akyürek, Nusret; Menteş, Bülent; Yakaryilmaz, Fahri; Dursun, Ayşe; Bayoğlu, Yeşim; Görgül, Ahmet (2005). "A case report of gastric antral vascular ectasia (watermelon stomach) as a rare cause of gastrointestinal bleeding". The Turkish journal of gastroenterology 16 (4): 244–5. PMID 16547859. 
  4. ^ a b c d e f g Nguyen, Hien; Le, Connie; Nguyen, Hanh (2009). "Gastric antral vascular ectasia (watermelon stomach)-an enigmatic and often-overlooked cause of gastrointestinal bleeding in the elderly". The Permanente journal 13 (4): 46–9. PMC 2911825. PMID 20740102. 
  5. ^ a b c d e f g h Yildiz, Baris; Sokmensuer, Cenk; Kaynaroglu, Volkan (2010). "Chronic anemia due to watermelon stomach". Annals of Saudi Medicine 30 (2): 156–8. doi:10.4103/0256-4947.60524. PMC 2855069. PMID 20220268. 
  6. ^ Rider, JA; Klotz, AP; Kirsner, JB (1953). "Gastritis with veno-capillary ectasia as a source of massive gastric hemorrhage". Gastroenterology 24 (1): 118–23. PMID 13052170. 
  7. ^ a b c d e f g h i j k l m n o p q r s Tuveri, Massimiliano; Borsezio, Valentina; Gabbas, Antonio; Mura, Guendalina (2007). "Gastric antral vascular ectasia—an unusual cause of gastric outlet obstruction: report of a case". Surgery today 37 (6): 503–5. doi:10.1007/s00595-006-3430-3. PMID 17522771. 
  8. ^ a b c d e f g h i j k l m n o p q r Rosenfeld, G; Enns, R (2009). "Argon photocoagulation in the treatment of gastric antral vascular ectasia and radiation proctitis". Canadian Journal of Gastroenterology 23 (12): 801–4. PMC 2805515. PMID 20011731. 
  9. ^ a b c Spahr, L; Villeneuve, J-P; Dufresne, M-P; Tasse, D; Bui, B; Willems, B; Fenyves, D; Pomier-Layrargues, G (1999). "Gastric antral vascular ectasia in cirrhotic patients: absence of relation with portal hypertension". Gut 44 (5): 739. doi:10.1136/gut.44.5.739. PMC 1727493. PMID 10205216. 
  10. ^ a b c d e f g h 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. doi:10.4253/wjge.v2.i8.298. PMC 2999147. PMID 21160630. 
  11. ^ 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. doi:10.1007/BF01540274. PMID 2721320. 
  12. ^ a b c d e Ceribelli, A; Cavazzana, I; Airò, P; Franceschini, F (2010). "Anti-RNA polymerase III antibodies as a risk marker for early gastric antral vascular ectasia (GAVE) in systemic sclerosis". The Journal of rheumatology 37 (7): 1544. doi:10.3899/jrheum.100124. PMID 20595295. 
  13. ^ a b 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. 
  14. ^ Scleroderma Association website. Accessed September 29, 2010.
  15. ^ Marie, I.; Ducrotte, P.; Antonietti, M.; Herve, S.; Levesque, H. (2008). "Watermelon stomach in systemic sclerosis: its incidence and management". Alimentary Pharmacology & Therapeutics 28 (4): 412. doi:10.1111/j.1365-2036.2008.03739.x. 
  16. ^ Ingraham, KM; O'Brien, MS; Shenin, M; Derk, CT; Steen, VD (2010). "Gastric antral vascular ectasia in systemic sclerosis: demographics and disease predictors". The Journal of rheumatology 37 (3): 603–7. doi:10.3899/jrheum.090600. PMID 20080908. 
  17. ^ a b c Spahr, L; Villeneuve, JP; Dufresne, MP; Tassé, D; Bui, B; Willems, B; Fenyves, D; Pomier-Layrargues, G (1999). "Gastric antral vascular ectasia in cirrhotic patients: absence of relation with portal hypertension". Gut 44 (5): 739–42. doi:10.1136/gut.44.5.739. PMC 1727493. PMID 10205216. 
  18. ^ 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. 
  19. ^ "Watermelon Stomach"Genetic and Rare Diseases Information Center (GARD), National Institution of Health.
  20. ^ "Watermelon Stomach and Radiiation Proctopathy CCS Publishing, August 1, 2011
  21. ^ a b c 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. ^ a b UT Southwestern Medical Center. "Digestive Specialists Freeze Out Esophagus Cancer With New Therapy." ScienceDaily 31 Jul. 2008. Retrieved from Science Daily on April 9, 2011.
  23. ^ Greenwald, BD (May 21 2007). Esophagus - Cryotherapy Ablation of Early Esophageal Cancer. The DAVE Project. Retrieved April 9, 2011 from The DAVE Project website.
  24. ^ "Cryoablation," 2010-2011. Found at Boston Medical Center website; retrieved April 9, 2011.
  25. ^ Kerry Dunbar, "Cryotherapy," Johns Hopkins Blogs, January 29, 2009, citing (1) Dumot JA, et al. Results of Cryospray Ablation for Esophageal High Grade Dysplasia (HGD) and Intramucosal Cancer (Imca) in High Risk Non-Surgical Patients. Gastrointestinal Endoscopy, Volume 67, Issue 5, April 2008, Page AB176; and (2) Canto MI, et al. Low Flow CO2-Cryotherapy for High Risk Barrett’s Esophagus (BE) Patients with High Grade Dysplasia and Early Adenocarcinoma: A Pilot Trial of Feasibility and Safety a Pilot Trial of Feasibility and Safety. Gastrointestinal Endoscopy, Volume 67, Issue 5, April 2008, Pages AB179-AB180. Found at Johns Hopkins Blogs, retrieved April 9, 2011.
  26. ^ Greenwald, Bruce D., John A. Dumot, J. David Horwhat, Charles J. Lightdale, & Julian A. Abrams, "Safety, tolerability, and efficacy of endoscopic low-pressure liquid nitrogen spray cryotherapy in the esophagus," Diseases of the Esophagus, Volume 23, Issue 1, pages 13–19, January 2010 (first published online: June 9, 2009). doi:10.1111/j.1442-2050.2009.00991.x. Found online at Wiley online; retrieved April 9, 2011.
  27. ^ a b c Khan S, Tudur Smith C, Williamson P, Sutton R (2006). "Portosystemic shunts versus endoscopic therapy for variceal rebleeding in patients with cirrhosis". In Khan, Saboor A. "Cochrane Database of Systematic Reviews". Cochrane Database Syst Rev (4): CD000553. doi:10.1002/14651858.CD000553.pub2. PMID 17054131. 
  28. ^ a b c Saab S, Nieto JM, Lewis SK, Runyon BA (2006). "TIPS versus paracentesis for cirrhotic patients with refractory ascites". In Saab, Sammy. "TIPSS versus paracentesis for cirrhotic patients with refractory ascites". Cochrane Database Syst Rev (4): CD004889. doi:10.1002/14651858.CD004889.pub2. PMID 17054221. 
  29. ^ a b c d 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. 
  30. ^ "paracentesis" at Dorland's Medical Dictionary
  31. ^ 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. doi:10.1016/S1072-7515(03)00600-8. PMID 14585429. 
  32. ^ 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. doi:10.1016/j.gie.2008.02.021. PMID 18533150. 
  33. ^ a b c Katsinelos, P; Chatzimavroudis, G; Katsinelos, T; Panagiotopoulou, K; Kotakidou, R; Tsolkas, G; Triantafillidis, I; Papaziogas, B (2008). "Endoscopic mucosal resection for recurrent gastric antral vascular ectasia". VASA. Zeitschrift fur Gefasskrankheiten. Journal for vascular diseases 37 (3): 289–92. doi:10.1024/0301-1526.37.3.289. PMID 18690599. 

External links[edit]

  • Thonhofer, R; Siegel, C; Trummer, M; Gugl, A (2010). "Clinical images: Gastric antral vascular ectasia in systemic sclerosis". Arthritis and rheumatism 62 (1): 290. doi:10.1002/art.27185. PMID 20039398.