A 27-year-old woman with a debut of the disease two years ago in the form of severe pancolitis and subsequent corticodependence, on maintenance treatment with azatriopine and oral mesalazine.
He was admitted for abdominal pain, bloody diarrhoea and headache accompanied by motor deficits in the left lower limb.
Physical examination: diffuse abdominal pain on deep palpation. Rectal examination with fresh haematic debris. Discrete functional impotence in the left lower limb and paraesthesia in both upper limbs.
Laboratory tests on admission showed haemoglobin 11 g/dl, ESR 78 mg/l and minimal enzyme mobilisation (GOT 74 IU/l, GPT 96 IU/l, GGT 182 IU/l, FA 148 IU/l).
Neurological assessment indicated computed tomography (CT) and magnetic resonance imaging (MRI) of the brain, showing thrombosis of the longitudinal venous sinus. Rectosigmoidoscopy: severe mucosal involvement from the anal margin. Biopsies compatible with active ulcerative colitis, ruling out infection by cytomegalovirus. Treatment was started with intravenous corticosteroids at full doses (1 mg/kg/day) and low molecular weight heparin, with progressive normalisation of bowel movements.
After a week of hospitalisation, increased abdominal perimeter with semiology of ascites and neurological deterioration (myoclonias in lower limbs, decreased visual acuity), accompanied by increased cytolysis (GOT 600 IU/l, GPT 463 IU/l) and coagulopathy. Abdominal Doppler ultrasound was performed: hepatomegaly. Porta of 9.5 mm capturing Doppler only in its right branch. Prominent hepatic artery. Suprahepatic with minimal flow uptake, flattened. Echogenic content in inferior cava. Ascites.
Further study by CT and abdominal angio-MRI: intrahepatic cava decreased in calibre with image of intraluminal thrombus. Loss of signal intensity of the suprahepatic vessels along their entire length. Porta permeable. Hepatomegaly. Ascites.

Other causes of liver disease were ruled out and a diagnostic paracentesis was performed (leucocytes 20, glucose 86, protein 1.8, albumin 1.4, GASA 1. Cytology negative). Study of thrombophilia with relative deficit of hepatic synthesis factors (factor V 36.63%, VII 21%), without primary procoagulant disorder (antiphospholipid antibody, Ham's test and Leyden's factor V, negative. Prothrombin and methylenetetrahydrofolate reductase gene, homocysteine, antithrombin III, protein C and S were normal).
With suspicion of Budd-Chiari syndrome, treatment with full-dose sodium heparin is started. Clinical deterioration continued over the following days, with tension ascites and severe coagulopathy (prothrombin activity 38%).
After multidisciplinary assessment, the patient was transferred to another centre for attempted placement of a percutaneous intrahepatic percutaneous portosystemic shunt system, from which we were informed of the need for joint fibrinolysis due to associated portal thrombosis. The subsequent evolution was torpid with the appearance of haemorrhagic complications at different levels, including a massive cerebral haemorrhage and the patient's final death.