A 27-year-old woman with onset of the disease two years ago in the form of severe pancolitis and subsequent corticodependency, under maintenance treatment with oral azatriopine and table.
She was admitted for abdominal pain, bleeding diarrhea and headache accompanied by motor deficit in the left lower limb.
Physical manifestations: diffuse abdominal pain to deep sedation.
Rectal examination with fresh frozen remains.
Discrete functional impotence in left lower limb and paresthesias in both upper limbs.
Blood tests at admission showed hemoglobin of 11 g/dl, ESR 78 mg/l and minimal enzyme mobilization (GOT 74 IU/l, GPT 96 IU/l, GGT 182 IU/l).
Neurology assessment showed cerebral computed tomography (CT) and magnetic resonance imaging (MRI), demonstrating thrombosis of the longitudinal venous sinus.
In rectosigmoidoscopy: severe involvement of the mucosa from the anal margin.
Biopsies compatible with active ulcerative colitis were ruled out cytomegalovirus infection.
We started treatment with intravenous corticosteroids at full doses (1 mg/kg/day) and low molecular weight heparin, with progressive normalization of stools.
After one week of hospitalization, increased abdominal circumference with semiology of ascites and neurological impairment (myoclonus in lower limbs, decreased visual acuity), accompanied by increased cytolysis (GPT 600 IU/63 IU/ IU/L).
Abdominal Doppler ultrasound showed hepatomegaly.
It has a 9.5 mm port, capturing doppler only in its right branch.
Jaundice prominent.
Suprahepatic with minimal flow uptake, flat.
Echogenic content in the inferior vena cava.
Ascites.
The study was extended by abdominal CT and angio-MRI : intrahepatic vena cava with decreased intraluminal thrombus image.
Location of suprahepatic signal intensity throughout its path.
Patent door.
Hepatomegaly.
Ascites.
1.
Other causes of liver disease were ruled out and diagnostic paracentesis was performed (white blood cells 20, glucose 86, proteins 1.8, albumin 1.4, GASA 1.
Negative culture.
Negative cytology).
Thrombophilia study with relative deficit of hepatic synthesis factors (factor V 36.63%, VII 21%), without primary disorder (antiphospholipid antibodies, Ham test and factor V Leiden).
Gene of prothrombin and methylentetrahydrofolate reductase, homocysteine, antithrombin III, protein C and S, normal.
With the suspicion of Budd-Chiari syndrome, treatment with heparin sodium at full doses is initiated.
Clinical deterioration continues on consecutive days, with tension ascites and severe coagulopathy (prothrombin activity 38%).
After a multidisciplinary assessment, the patient is referred to thrombosis by another center for an attempt to place an intrahepatic percutaneous portosystemic shunt system, since they inform us of the need for a combined portal fibrinolysis.
The subsequent evolution is torpid with appearance of hemorrhagic complications at different levels, highlighting a massive cerebral hemorrhage and final death of the patient.
