Vol 43 # 3 September 2011 - Kma.org.kw

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230 KUWAIT MEDICAL JOURNAL September 2011 Case Report Splenic Vein Thrombosis: A Rare Complication of Celiac Disease Anwar QKHS Alenezi 1 , Jamal Monzer Hilal 2 , Ali Mahmoud Behbehani 2 , 1 Department of Gastroenterology and Hepatology, Al-Jahra Hospital, Kuwait 2 Department of Internal Medicine, Al-Jahra Hospital, Kuwait Kuwait Medical Journal 2011; 43 (3): 230-233 ABSTRACT The principal cause of splenic vein thrombosis (SVT) is pancreatic disease. Isolated splenic vein thrombosis (ISVT) is a very rare complication of celiac disease. Only few cases are reported worldwide. Affected patients develop left-sided portal hypertension often complicated by splenomegaly and isolated gastric varices. The condition is usually asymptomatic, but patients may complain of non-specific abdominal pain. Gastric variceal bleeding in this context is uncommon but should it occur, it could be life threatening and splenectomy would be the treatment of choice. The role of anticoagulation is controversial and the risk of further thrombotic events must be balanced against that of variceal bleed. We report a case of a patient with celiac disease (CD) who presented with a new onset non-specific abdominal pain, who was found to have ISVT complicated by portal hypertension, splenomegaly and non-bleeding isolated gastric varices. He was successfully managed with anticoagulation. KEY WORDS: anticoagulation, celiac disease, gastric varices, splenic vein thrombosis INTRODUCTION The most common cause of splenic vein thrombosis (SVT) is pancreatic disease. Other causes include umbilical vein catheterization, inherited and acquired hypercoagulable states, abdominal surgery, retroperitoneal fibrosis, benign gastric ulcers and renal cysts [1,2] . Affected patients develop splenomegaly and gastric varices secondary to left-sided portal hypertension in the absence of liver cirrhosis. SVT is a rare presentation of celiac disease (CD). Although the association has long been reported, the exact pathophysiology is not well understood. Patients can have life-threatening variceal bleeding. Management, which is not well-definedintheliterature, is not without risks. CASE REPORT A 38-year-old Pakistani gentleman presented in January 2005 to his local hospital in Pakistan complaining of chronic diarrhea and weight loss. An upper gastrointestinal (GI) endoscopy showed proximal small bowel villous atrophy. Duodenal biopsies revealed subtotal villous atrophy with mild lymphocytic infiltration of the intraepithelial cells. He had positive antigliadin antibodies (AGA), and was therefore diagnosed with CD. He was advised to adhere to a gluten free diet. Initially the patient experienced marked symptomatic improvement, but few months later he moved to Kuwait where he failed to adhere to a Gluten free diet and eventually relapsed. He presented to our hospital in Kuwait in March 2006 complaining of fatigue and chronic cramping abdominal pain associated with loose bowel motions. He also reported abdominal distension with swelling of both lower limbs. He gave no history of weight loss or GI bleeding. On examination he had splenomegaly with shifting dullness and bilateral pitting lower limbs edema. He had no lymphadenopathy or signs of chronic liver disease. His laboratory investigations (Table 1) revealed pancytopenia with hypocalcemia, hypoalbuminemia and low serum ferritin and red cell folate. He had a normal coagulation profile. Stool analysis for infection and occult blood (OB) was negative. His celiac serology was strongly positive. A repeat upper GI endoscopy showed isolated ectopic gastric varices without endoscopic features of high variceal bleed risk. This finding raised the suspicion of thrombosis in the splanchnic circulation. An ultrasound scan of the abdomen confirmed the presence of portal hypertension with splenomegaly and ascites in the absence of liver cirrhosis. Doppler studies demonstrated splenic vein occlusion with multiple collaterals. A CT angiography of the abdomen (Fig. 1) confirmed the above findings beyond doubt. Address correspondence to: Ali Behbehani, MRCP (UK), Department of Internal Medicine, Al-Jahra Hospital, Kuwait. Tel: 00965-9751 7551, E-mail: allbah@hotmail.com
September 2011 KUWAIT MEDICAL JOURNAL 231 Fig. 1: Abdominal CT angiography showing splenomegaly, splenic vein thrombosis near the hilum (white arrow), collaterals (arrow head), and ascites (black dashed arrow) Thrombophilia screen and hepatitis B and C serology were all negative. Ascitic fluid analysis revealed a transudate with a serum to ascitic albumin gradient (SAAG) > 11 g/l, consistent with portal hypertension. The patient was diagnosed with CD complicated by ISVT and left-sided portal hypertension. The pros and cons of anticoagulation were explained to the patient, and with his consent we started anticoagulation. Subcutaneous low molecular weight heparin was given first followed by warfarin, aiming for an international normalized ratio (INR) of 2 - 3. Stool OB was checked several times after anticoagulation and remained persistently negative. Hemoglobin levels were also stable and hence splenectomy was deferred. DISCUSSION CD is an immune-mediated enteropathy caused by sensitivity to gluten in genetically susceptible individuals [1] . The clinical presentation is highly variable, ranging from the classical malabsorption syndrome to an atypical disease; where extra-intestinal manifestations predominate. The disease can also run subclinical and latent courses [2] . CD is usually complicated by splenic atrophy with secondary hyposplenism and the appearance of Howell-Jolly bodies in the blood film, a complication that may lead to fatal infections by encapsulated bacteria [3-4] . Our patient had paradoxical splenomegaly with hypersplenism and secondary pancytopenia. His low serum ferritin and red cell folate may have also contributed to the cytopenia. Isolated gastric varices denote a left-sided portal hypertension. This picture should always alert the caring physician to the possibility of isolated splenic vein thrombosis (ISVT), a complication rarely seen in CD [5] . Other rare thrombotic phenomena reported in CD include: portal vein thrombosis [6] , cerebral venous thrombosis [7] , central retinal vein occlusion [8] , deep venous thrombosis (DVT) of the legs [9] , and Budd- Chiari syndrome [10-12] . The mechanism of thrombosis in CD is not clear. Case reports have occasionally stated a clear etiology such as antiphospholipid antibodies, anticardiolipin antibodies, lupus anticoagulant [13] , protein S deficiency and hyperhomocysteinemia. However, except for hyperhomocysteinemia secondary to nutritional deficiencies (folate, B12, B6) and malabsorption [14-15] , it is not clear whether these etiological factors are coincidental findings Table 1: The patient’s laboratory results Test Result Normal range Test Result Normal range Hb MCV Total WCC Platelets ESR INR Total protein Albumin Corrected calcium Phosphorus ALP Glucose Serum B12 Red cell folate Ferritin Plasma homocysteine Urine homocysteine 11.4 g/dl 72.3 3.1 x 10 9 /l 146 x 10 9 /l 21 mm/hr 1.119 55.9 g/l 23.9 g/l 1.7 mmol/l 1.01 mmol/l 142 IU/l 4.71 mmol/l 210 pmol/l 240 nmol/l 16 ug/l 9.1 Mm/l Negative 13.6 - 17.5 80 - 100 4.8 - 10.8 150 - 450 < 10 60 - 82 35 - 55 2.1 - 2.6 0.8 - 1.6 26 - 121 3.9 - 6.1 133 - 675 >372 20 - 400 4 - 11.2 Mm/l Negative AGA (IgA) (IgG) AEA Anti tTG ANA Anti phospholipid Anti cardiolipin Lupus anticoagulant Factor V Leiden Protein C Protein S Antithrombin Ascitic fluid Total wcc Protein Albumin Glucose 56 U/ML 14 U/ML positive 23 U/mL Negative Negative Negative 29.0 S 2.919 R 84.4% 79.6% 109% 110 cells/mm3 12.9 g/L < 10 g/L 7.15 mmol/L < 30 < 30 Negative 0 - 15 U/mL 26.3 - 46.3 2.2 - 3.5 70 - 140 60 - 140 71 - 115 < 500 Hb: hemoglobin, MCV: mean corpuscular volume, WCC: white cell count, ESR: erythrocyte sedimentation ratio, INR: international normalized ratio, ALP: alkaline phosphatase, AGA: anti-gliadin antibody, AEA: anti-endomyseal antibody, tTG: tissue transglutaminase, ANA: antinuclear antibody
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