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Classical Case Studies

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A. A. Deshpande, K. Das K., and V. K. Thapar Mesenteric venous thrombosis in hyperhomocysteinemia
A. A. Deshpande, K. Das K., and V. K. Thapar, 10 October 2006
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Abstract

Acute abdominal pain can be the result of many different pathophysiological processes, intestinal ischemia being one of them. Early recognition and appropriate treatment can reduce the potential for a devastating outcome. Elevated plasma total homocysteine (hyperhomocysteinemia) is a risk factor for venous thrombosis. Very high plasma homocysteine with its excretion in urine is termed as homocystinuria. It is a rare inherited disorder of metabolism of the sulfur-containing amino acid methionine. It is known to be associated with arterial vascular diseases and venous thrombosis. Mild and moderate hyperhomocysteinemia can be present without homocystinuria and reflects certain environmental and constitutional factors. Hyperhomocysteinemia is a potentially reversible cause of thrombophilia and venous thrombosis hence it should be ruled out in all cases of apparently idiopathic venous thrombosis.

Introduction

Intestinal ischemia is responsible for 1% of all cases of acute abdomen[1]. Ten per cent of these cases are due to primary or secondary mesenteric venous thrombosis (MVT)[2] MVT needs treatment with anticoagulation and sometimes, surgical intervention [3]. Many cases of primary MVT are now shown to be due to prothrombotic states [4]. Elevated level of homocysteine (hyperhomocysteinemia- Hhcy) is a reversible prothrombotic state [5]. There have been very few case reports of hyperhomocysteinemia leading to MVT so far [6].

We present a rare case of a patient with portal vein, splenic vein and superior mesenteric vein thrombosis with Hhcy.

Case report

A 26-year-old male presented with 2 days of colicky periumbilical pain and melena. Patient was a non-alcoholic, chronic tobacco chewer. There was no relevant drug history in the past. There was no history of previous abdominal surgeries or trauma. On examination, pulse rate was 110/min, systolic blood pressure was 90 mm/Hg. Respiratory rate was 32/min. He was mildly dehydrated. Abdomen was distended but non-tender. There was no organomegaly. Per rectal examination confirmed melena. The symptoms were out of proportion to the signs. The respiratory, cardiovascular and neurological systems were normal on examination. The leukocyte count was 18,000, liver function tests and renal function tests were normal. Lipid profile was normal. International Normalized Ratio on admission was 1.23. The patient was resuscitated. Esophagogastroduodenoscopy was normal. Ultrasound revealed mild ascites with portal and splenic vein thrombosis, with multiple dilated hypoperistaltic bowel loops. CT Angiography of abdomen revealed minimal hepatomegaly with portal vein, splenic vein and superior mesenteric vein thrombosis (Fig. 1) with dilated thick-walled bowel loops. Fig. 1. CT Angiography - pointer demonstrating superior mesenteric vein thrombus. A diagnosis of idiopathic mesenteric venous thrombosis with bowel ischemia was made. The patient was managed conservatively with intravenous fluids, nasogastric aspiration, antibiotics, and low molecular weight heparin. Protein C, Protein S, Antithrombin III, Factor V Leiden, and Serum Homocysteine levels were checked. Serum Homocysteine levels were >100 Ámol/l (normal 5-12 Ámol/l, mild 15-30Á mol/l, moderate 30-100Ámol/l, severe >100Ámol/l). Protein C, Protein S, Antithrombin III, and Factor V Leiden values were normal. Patient's symptoms improved on conservative management and oral sips with oral anticoagulation (warfarin) was started. Patient was also started on combination of folic acid and pyridoxine orally. An INR of 2.1 was achieved on day 5 and warfarin was continued in titrated doses.

Discussion

Mesenteric Venous Thrombosis (MVT) is the least common cause of mesenteric ischemia, representing upto 10% of all patients with acute mesenteric ischemia [2]. Primary (idiopathic) or secondary thrombosis of the superior mesenteric vein accounts for 95% of cases of mesenteric venous thrombosis. The most common causes for secondary MVT are hereditary or acquired prothrombotic states, malignancy, intraabdominal inflammatory conditions, the postoperative state, cirrhosis and portal hypertension and oral contraceptive use in young women [3]. In our patient none of the local intraabdominal factors were present. MVT can present in an acute, sub-acute or chronic form. In the acute form, pain is the most prominent symptom and the initial abdominal examination may be normal. In our case also, the symptoms were far more significant than the signs. High index of suspicion with immediate radiologic evaluation in the form of multislice CT scan with CT angiography is useful in achieving early diagnosis [3]. The treatment of MVT is anticoagulation alone or with thrombectomy in selected patients. Surgery is also required in case of bowel infarction and peritonitis. In spite of early diagnosis and aggressive treatment, mortality rate in MVT is high [3]. The common hypercoagulable states are protein C deficiency, Antiphospholipid antibody syndrome, factor V Leiden, malignancy, protein S deficiency, hyperhomocysteinaemia, antithrombin III deficiency, prothrombin G20210A, increased factor VIII and sticky platelet syndrome [7]. Homocysteine is an intermediate of methionine metabolism. Hyperhomocysteinemia can be present with or without classical homocystinuria. Classical Homocystinuria is an inherited autosomal recessive defect in methionine metabolism [5]. This defect leads to a multisystemic disorder of the connective tissue, muscles, CNS, and cardiovascular system and is associated with many phenotypical changes. In the absence of classical homocystinuria, mild to moderate increases in homocysteine levels occur in 5-10% of the population [7]. These are observed in various settings such as increasing age; postmenopausal women; patients with renal failure, hypothyroidism, leukemia, or psoriasis; drug therapy with methotrexate, nitrous oxide, isoniazid, and some antiepileptics-these states are characterized by milder elevations of serum homocysteine levels in contrast to homocystinuria [5]. Elevated levels of homocysteine are known to be a risk factor for arterial and venous thrombosis [7]. Our patient was found to have extremely high levels of homocysteine in blood. His other investigations for a hypercoagulable state yielded negative result. Hyperhomocysteinemia is a potentially reversible cause of thrombophilia and serious complications can be prevented by early intervention [5]. Treatment of hyperhomocysteinemia consists of a special diet restricted in protein and methionine and supplemented with cysteine, pyridoxine, folic acid, and vitamin B12 [8].

With improved diagnostic techniques, more cases of Mesenteric Venous Thrombosis have been shown to be related to primary clotting disorders, with only 10% of cases now being classified as idiopathic [7]. All patients with diagnosed mesenteric venous ischemia should undergo evaluation for a hypercoagulable state. The work-up should include antithrombin III, protein C and protein S levels, serum homocysteine levels, as well as routine coagulation profiles. If a prothrombotic state is detected, long-term Warfarin therapy (at least 6 months, and possibly lifelong) is recommended.

Conclusion

In a young patient with a mesenteric venous thrombosis a coagulation screen is mandatory to achieve a proper diagnosis and to formulate the correct line of management. Hyperhomocystinemia with or without the classical homocystinuria should be looked for and treated to avert life-threatening complications.

This article was first published on GastroHep.com in 2007.

References

  1. Horton KM, Fisherman EK. Multi-detector row CT of mesenteric ischemia: Can it be done? Radiographics 2001; 21: 1463.
  2. Rhee RY, Gloviczki P. Mesenteric venous thrombosis. Surg Clin North Am 1997; 77(2): 327-338.
  3. Kumar S, Sarr MG, Kamath PS, Current Concepts: Mesenteric Venous Thrombosis, N Engl J Med 2001; 345:1683-8.
  4. Bradburg AW, Brittenden J, McBride K, Ruckley CV. Mesenteric ischemia: a multidisciplinary approach. Br J Surg 1995; 82:1446-59.
  5. Quere I, Gris JC, Dauzat M. Homocysteine and venous thrombosis. Semin Vasc Med. 2005; 5(2):183-9.
  6. Berk DR, Ahmed A. J Portal, splenic, and superior mesenteric vein thrombosis in a patient with latent essential thrombocythemia and hyperhomocysteinemia. J Clin Gastroenterol. 2006 ;40(3): 227-8.
  7. Thomas RH. Hypercoagulability syndromes. Arch Int Med. 2001;161: 2433-9.
  8. Yap S. Classical Homocystinuria: vascular risk and its prevention. J Inherit Metab Dis. 2003; 26(2-3): 259-65.

This article was first published on GastroHep in October 2006.

 
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