Massive gangrene of the stomach due to primary antiphospholipid syndrome: Report of two cases
Tóm tắt
Antiphospholipid syndrome (APS) is a systemic autoimmune disease, which may be primary or secondary to other autoimmune diseases. It produces thrombosis of arteries and veins of any caliber, and no organ is immune to its insult. This report describes two cases of massive gastric gangrene due to primary APS, which presented in a span of 2 years. In the first case a multiparous, 40-year-old woman presented with acute abdominal pain, hematemesis, and progressive abdominal distension, and was in azotemia and shock. A laparotomy revealed gangrene of the stomach without any other organ involvement. She was managed with a total gastrectomy and esophagojejunal anastomosis. Postoperative serology revealed a persistent elevation of anticardiolipin antibody with no other apparent predisposing cause. The histopathological examination of the specimen revealed characteristic extensive intramural vascular thrombosis without inflammatory changes in the vessel wall, confirming antiphospholipid syndrome. The second patient was a primiparous, 26-year-old woman who had severe abdominal pain in the first trimester followed by shock. An exploratory laparotomy revealed massive gangrene of the stomach with complete loss of the posterior wall and hemoperitoneum. She also underwent a total gastrectomy with esophagogastric anastomosis and was later managed in the intensive care unit, where she succumbed within 8 days. Her serology showed a highly elevated anticardiolipin antibody titer, and histopathological examination of the stomach revealed characteristic intramural vascular thrombosis without inflammatory cellular infiltrate in the vessel wall. Patients undergoing a total gastrectomy following acute gastric necrosis have very high mortality (50%–80%). Its association with APS is rare and it has not been previously reported. The combination is a formidable challenge to the physician and a dangerous disease for the patient. The rarity of the condition and its grave prognosis is highlighted.
Tài liệu tham khảo
Reyhan Diz-Ku C, Kkaya U. Hypercoagulable states: antiphospholipid antibody syndrome. Hematology 2005;10(suppl 1):33–38.
Kondo Y, Koizumi J, Nishibe M, Muto A, Dardik A, Nishibe T. Deep venous thrombosis caused by congenital absence of inferior vena cava: report of a case. Surg Today 2009;39:231–234.
Elias M, Eldor A. Thromboembolism in patients with the “lupus” type circulating anticoagulant. Arch Intern Med 1984;144:501–505.
Harris EN, Gharavi AE, Hughes GRV. Anti-phospholipid antibodies. Clin Rheum Dis 1985;11:591–609.
Al Salem AH. Intrathoracic gastric volvulus in infancy. Pediatr Radiol 2000;30:842–845.
Haas O, Rat P, Christophe M, Friedman S, Favre JP. Surgical results of intrathoracic gastric volvulus complicating hiatal hernia. Br J Surg 1990;77:1379–1381.
Le Scanff J, Mohammedi I, Thiebaut A, Martin O, Argaud L, Robert D. Necrotizing gastritis due to Bacillus cereus in an immunocompromised patient. Infection 2006;34:98–99.
Dapri G, Himpens J, Mouchart A, Ntounda R, Claus M, Dechamps P, et al. Laparoscopic transhiatal esophagogastrectomy after corrosive injury. Surg Endosc 2007;21:2322–2325.
Kumaran C, Chung AYF, Ooi LLPJ, Chow PKH, Wong WK. Coeliac artery trunk thrombosis in acute pancreatitis causing total gastric necrosis. Aust NZ J Surg 2006;76:273–274.
Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med 2002;346:752–763.
Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 1999;42(7):1309–1311.
Uthaman I, Khamasata M. The abdominal manifestation of antiphospholipid syndrome. Rheumatology (Oxford) 2007;46:1641–1647.
Cappell MS, Mikhail N, Cujral N. Gastrointestinal hemorrhage and intestinal ischemia associated with anticardiolipin antibodies. Dig Dis Sci 1994;39:1359–1364.
Mihas AA. Gastrointestinal bleeding and intestinal ischemia with anticardiolipin antibody. Dig Dis Sci 1995;40:1039.
Gappel MS. Esophageal necrosis and perforation associated with anticardiolipin syndrome. Am J Gastroenterol 1994;89:1241–1245.
Sanchez-Guerrero J, Reyes E, Alaorcon-Segovia D. Primary antiphospholipid syndrome as a cause of intestinal infarction. J Rheumatol 1993;20:2169–2170.
Schein M, Saadia R. Postoperative gastric ischemia. Br J Surg 1989;76:844–848.
Asherson RA, Servera R. Catastrophic antiphospholipid syndrome. Curr Opin Hematol 2000;7:325–329.
Servera R, Piette JC, Shoenfeld Y, Font J, Bucciarelli S, Asherson RA. Registry of the European forum on antiphospholipid antibodies for patients with the “catastrophic antiphospholipid syndrome”. European Forum on Antiphospholipid Antibodies. Accessible at: http://www.med.ub.es/mimmun/forum/caps.htm
Kalman DR, Khan A, Romain PL, Nompleggi DJ. Giant gastric ulceration associated with antiphospholipid antibody syndrome. Am J Gastroenetrol 1996;91:1244–1247.
Casey KM, Quigley TM, Kozarek RA, Raker EJ. Lethal nature of ischemic gastropathy. Am J Surg 1993;165:646–649.