A 41-year-old woman with no history of interest presented with diffuse abdominal pain of greater intensity in the epigastrium of 1 week duration, together with nausea, bilious vomiting and poor general condition.
Pain increases with cough and inspiration.
She reported no fever or dysthermic sensation.
No coluria or acholia.
On physical examination the patient is conscious and oriented, affected by pain and impressive of gravity.
Mucocutaneous Pallor.
Patient afflicted and normotensive.
Cardiopulmonary arrest with no findings.
The abdomen was free of masses or enlargements, with generalized abdominal pain due to palpation, more intense in the epigastrium, with no signs of peritoneal irritation (Murphy negative).
No collateral circulation was observed.
No lower limb edema.
No signs of deep vein thrombosis (DVT).
Analytically, the patient presented normal biochemical transaminase increase (GOT: 146 U/L, GPT: 171 U/L).
Hemogram with hemoglobin: 11.4 g/dL, hematocrit: 34%, leukocytes: 12700 without neutrophilia and platelets: 291000.
Hemostasis with INR: 1.3 and cefalin time 53.3 sec.
A CT scan showed a fat-density thrombus extending from the left kidney to the left renal vein (LRV), inferior vena cava (IVC) and right atrium ostium.
Free suprahepatic veins.
No complete occlusion of the IVC lumen.
Unspecific pancreatic head abnormality.
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Because of this last CT data together with the intense epigastralgia, it was decided to perform an abdominopelvic echo: liver and intrahepatic and extrahepatic bile duct normal.
Lipoma in IVC.
Signs and symptoms suggestive of pancreatitis.
Free fluid in the pelvis, perihepatic, perisplenic and right perirenal spaces (in greater amounts than in previous CT scans).
A differential diagnosis between biliopancreatic disease vs. Budd-Chiari syndrome was made, so it was decided to perform a second CT scan showing bilateral pleural effusion, thickening of gastric folds extending the portal vein ostomy with increased IVCA.
There are images suggestive of hepatic congestion: hepatomegaly, increased caliber of the portal and suprahepatic veins with delay and the arrival of contrast to them, periportal edema, spleen and pancreas retrograde and abundant fluid.
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With a presumptive diagnosis of Budd-Chiari syndrome secondary to thrombus in the vena cava, paracentesis under ultrasound guidance is performed in order to rule out with greater certainty the peritoneal infectious/tumor process as a cause.
A transudate analytical result was obtained discarding peritoneal process.
Transesophageal ultrasound showed good ventricular function without intracardiac thrombus, with free tricuspid and pericardial effusion.
Finally, with a diagnosis of Budd-Chiari syndrome secondary to vena cava thrombus (AML vs. Lipoma), and due to clinical worsening of the patient with poor general condition, emergency surgery was decided upon 48 hours after admission.
QUIRITY INTERVENTION.
It is a thrombus with surgical level IV presenting supradiaphragmatic situation reaching the right atrium ostium.
An incision was made and the retroperitoneum was accessed by opening from fixed loop to Winslow hiatus to allow complete ascending release of the intestinal package, including ligation of the inferior mesenteric vein Che.
Hepatic mobilization is achieved by seccioning the ligaments, coronary stenosis, right and left and sickle cell.
The left kidney and its corresponding hilium are then released, starting with the release of the vena cava, starting at the infrarenal level and ascending to the suprahepatic level with control of the right renal vena cava.
Thrombolysis was performed to the infradiaphragmatic segment.
Liver clamping (Pringle maneuver), proximal and distal IVC and RRV was performed with subsequent cavotomy and opening VRI, complete thrombus extraction and left nephrectomy.
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PATOOLOGICAL ANAATOMY: angiomyolipoma predominantly affecting the renal sinus with non vascular malformations.
