A 41-year-old woman with no past history of interest presented with diffuse abdominal pain, more intense in the epigastrium, of 1 week's duration, together with nausea, bilious vomiting and poor general condition. The pain increases with coughing and inspiration. No fever or dysthermic sensation. No choluria or acholia.
On physical examination the patient is conscious and oriented, affected by pain and appears serious. Mucocutaneous pallor. The patient is afebrile and normotensive. Cardiopulmonary auscultation with no findings. Abdomen without masses or megaliths, with generalised abdominal pain on palpation, more intense in the epigastrium, with no signs of peritoneal irritation (Murphy negative). No signs of collateral circulation. No oedema in the lower limbs. No signs of deep vein thrombosis (DVT).
Laboratory tests showed normal biochemistry with increased transaminases (GOT: 146 U/L, GPT: 171 U/L). Haemoglobin: 11.4 g/dL, haematocrit: 34%, leucocytes: 12700 without neutrophilia and platelets: 291000. Haemostasis with INR: 1.3 and cephalin time 53.3 sec.
A CT scan was performed showing a thrombus with fatty density extending from the left kidney to the left renal vein (LRV), inferior vena cava (IVC) and right atrial ostium. Free suprahepatic veins. No complete occlusion of the IVC lumen. Non-specific abnormality of the pancreatic head.

Due to this last data from the CT scan together with the intense epigastralgia, it was decided to perform an abdominal-pelvic ultrasound, finding: liver and intra- and extrahepatic biliary tract normal. Lipoma in IVC. Ultrasound signs of cholecystitis, probably perforated and possible pancreatitis. Free fluid in the pelvis, perihepatic, perisplenic and right perirenal spaces (more than in the previous CT scan).
A differential diagnosis of bilio-pancreatic pathology vs. Budd-Chiari Syndrome was suggested, so it was decided to perform a second CT scan showing bilateral pleural effusion, thickening of gastric folds with increased contrast uptake and angiomyolipoma (AML) vs. renal lipoma extending towards the IVC and ostium of the right atrium, with passage of contrast throughout the venous structures, with portal vein and suprahepatic veins permeable. There are images suggestive of hepatic congestion: hepatomegaly, increased calibre of the portal vein and suprahepatic veins with delayed arrival of contrast to them, periportal oedema, spleen and pancreas with blue colour and abundant free fluid.

With a presumptive diagnosis of Budd-Chiari syndrome secondary to thrombus in the cava, paracentesis was performed under ultrasound control, in order to exclude with greater certainty an infectious/tumoral peritoneal process as the cause of the symptom. An analytical result of transudate was obtained, ruling out a peritoneal process. Transesophageal echography showed good ventricular function with no intracavitary thrombus, the tricuspid valve was free and there was pericardial effusion.
Finally, with a diagnosis of Budd-Chiari Syndrome secondary to cava thrombus (LMA vs. Lipoma), and in view of the patient's worsening clinical condition with poor general condition, urgent surgery was decided 48 hours after her arrival at the Emergency Department.
SURGICAL INTERVENTION. It was a thrombus with surgical level IV as it presented a supradiaphragmatic situation reaching the ostium of the right atrium. A chevron incision was made and access to the retroperitoneum by opening from the fixed loop to Winslow's hiatus to allow complete ascending release of the intestinal package, including ligation of the inferior mesenteric vein. Hepatic mobilisation is achieved by sectioning the round, coronary, right and left triangular and falciform ligaments. The left kidney and its corresponding hilum are then released, starting with the release of the vena cava, beginning at the infrarenal level and ascending to the suprahepatic level with control of the right renal vein (RKV) and section of the remaining branches except for the suprahepatic veins, which are found to be free of tumour thrombus and are the only ones that attach the vena cava to the liver. The diaphragmatic hiatus is widened and the thrombus is squeezed out up to the infradiaphragmatic level. Clamping of the hepatic pedicle (Pringle manoeuvre), proximal and distal IVC and RVD with subsequent cavotomy and opening of the IVR, complete removal of the thrombus and left nephrectomy.

PATHOLOGICAL ANATOMY: predominantly lipomatous angiomyolipoma of the renal sinus with vascular infiltration.

