Krampfanfall und prominente abdominelle Venen

In January, 2007, a 55-year-old man was brought to our hospital, 30 min after having a generalised tonic-clonic seizure. He had diabetes and hypertension, and was prescribed glibenclamide and amlodipine. He had no other medical history of note—in particular, there was no history of previous seizures, meningitis, encephalitis, febrile convulsions in infancy, birth trauma, head injury, a space-occupying lesion, or psychiatric illness. He was a farmer; he had a low income, but was eligible for state medical care.
We immediately noticed prominent veins on the patient's abdomen (figure), flanks, and back. Blood in the infraumbilical vein flowed towards the umbilicus, indicating obstruction of the inferior vena cava. The right common carotid, left brachial, left ulnar, and left radial pulses were feeble. Other pulses were normal. The blood pressure was 190/110 mm Hg in the right arm, but only 130/80 mm Hg in the left arm. The cardiovascular findings were otherwise normal. The liver edge was smooth—and 5 cm below the costal margin. The spleen was palpable, 4 cm below the costal margin. There was no ascites. Apart from postictal drowsiness, nothing abnormal was found on neurological examination. The apparently blocked inferior vena cava, hepatomegaly, and splenomegaly consistent with portal hypertension, led to a provisional diagnosis of Budd-Chiari syndrome (BCS). The apparent widespread arterial occlusion indicated that thrombophilia was the likely cause. Blood tests showed very high concentrations of urea and creatinine: 27·1 mmol/L and 186 μmol/L, respectively. Liver function tests gave normal results, except that the albumin concentration was only 32 g/L. The patient's serum concentration of cholesterol was slightly raised at 5·4 mmol/L; the triglyceride concentration was normal. The serum concentrations of antinuclear antibody, lupus anticoagulant, protein C, protein S, and antithrombin III were all normal, as were the INR and activated partial thromboplastin time. However, the concentrations of IgM and IgG antibodies to cardiolipin were high, at 35 M phospholipid U/mL and 27 G phospholipid U/mL, respectively (normal ranges <15>

The presence of arterial and venous thrombosis, together with increased concentrations of anticardiolipin antibodies, indicated a diagnosis of antiphospholipid syndrome. The patient was given anticoagulant drugs—first heparin, then nicoumalone. We prescribed phenytoin for 2 days, to reduce the likelihood of seizures while anticoagulant treatment was started. When the patient was last seen, in July, 2007, blood flow was satisfactory in the internal carotid and right renal arteries, but remained low in the right common carotid, left subclavian, and left renal arteries. The serum anticardiolipin antibody concentration remained high, confirming the diagnosis of antiphospholipid syndrome.1 However, the patient had had no seizures since admission.

In BCS, blockage of the hepatic vein causes liver damage. Primary BCS is caused by an anatomical abnormality.2 Secondary BCS is caused by tumour or thrombosis, which is typically caused by a hypercoagulable state.3 The patient can present with symptoms of BCS, or of its cause. Our patient reported that his abdominal veins had been prominent for 25 years; he had only presented because of his seizure—which is likely to have been caused by thrombosis of his cerebral arteries.

References

1 JS Levine, W Branch and J Rauch, The antiphospholipid syndrome, N Engl J Med 346 (2002), pp. 752–763.

2 KVN Menon, V Shah and P Kamath, The Budd-Chiari Syndrome, N Engl J Med 350 (2004), pp. 578–585.

3 WV McDermott, MD Stone, A Bothe Jr and C Troy, Budd-Chiari syndrome. Historical and clinical review with an analysis of surgical corrective procedures, Am J Surg 147 (1984), pp. 463–467

The Lancet Volume 370, Issue 9594, 6 October 2007-12 October 2007, Page 1282