A rare case of congenital aneurysm of the portal system at l
A 20-years-old male presented to the emergency room with persistent abdominal pain. On physical examination, the pain was localized to the upper quadrants, however, the abdomen was treatable, and no other particular clinical signs were disclosed. The patients did not have prior clinical records. The patient underwent ce-CT of the chest and abdomen, which disclosed a huge aneurysmatic dilatation involving the portal trunk (axial diameters 30 × 40 mm), the distal splenic vein (axial diameters 37 × 39 mm) and the distal tract of both superior and inferior mesenteric vein (respectively SMV and IMV), complicated by a massive thrombosis extending to the main intra-hepatic portal branches. Moreover, the following findings were observed: enhancement of vasa vasorum of the portal walls, porto-systemic shunts characterized by vicariant activation of the capsular venous branches with an enlarged right hepatic vein, splenomegaly with longitudinal diameter of 15 cm, pancreatic tail structural changes characterized by poor glandular representation and large intra and extra-glandular fluid collections. As a result, a therapy with low-molecular-weight heparin (LMWH) was adopted. Nine days later, the follow up ce-CT confirmed the previous findings except for partial peripheral re-habitation of spleno-portal thrombosis and the activation of peri-portal cavernomatosis.

During the hospitalization, the physicians excluded the presence of previous causes of acquired portal venous aneurysm including acute, and chronic pancreatitis on the base of the unremarkable clinical history, as well as, of thrombophilic diseases on the base of an unremarkable panel for genetic, immunologic, and serum coagulation disorders. As a consequence, the hypothesis of congenital aneurysm complicated by superimposed thrombosis was proposed; the pancreatic findings were interpreted as secondary to portal venous chronic pathology rather than the cause.

Fifteen days later the patient was discharged recommending anticoagulant home therapy. After 3 months, the patient underwent a contrast enhanced MRI of the abdomen, which disclosed a complete recanalization of the porto-spleno-mesenteric aneurysm. Subsequently, after 6 months, the anticoagulant therapy was suspended. Unfortunately, 25 days later, the patient returned to the emergency room for the onset of intense abdominal pain. The ce-CT documented the extensive thrombotic recurrence of the porto-spleno-mesenteric aneurysm. The pancreatic findings were stable respect to the previous CT scans. Currently, the patient is asymptomatic under chronic therapy with new oral anticoagulants (NOACs), and undergoes periodic clinical follow-up.