Portal vein thrombosis: Insight into physiopathology, diagnosis, and treatment

Table 1 Most frequent local risk factors for PVT[3,8,9,17,18,64,79]

Local risk factors for PVT (70%)
Cancer
Any abdominal organ
Focal inflammatory lesions
Neonatal omphalitis, ombilical vein catheterization
Diverticulitis, appendicitis
Pancreatitis
Duodenal ulcer
Cholecystitis
Tuberculous lymphadenitis
Crohn’s disease, ulcerative colitis
Cytomegalovirus hepatitis
Injury to the portal venous system
Splenectomy
Colectomy, gastrectomy
Cholecystectomy
Liver transplantation
Abdominal trauma
Surgical portosystemic shunting, TIPS
Iatrogenic (fine needle aspiration of abdominal masses etc.)
Cirrhosis
Preserved liver function with precipitating factors (splenectomy, surgical portosystemic shunting, TIPS dysfunction, thrombophilia)
Advanced disease in the absence of obvious precipitating factors

PVT: Portal vein thrombosis; TIPS: Transjugular intrahepatic portosystemic shunt.

Table 2 Most frequent systemic risk factors for PVT[3,8,9,17,18,64,79]

Systemic risk factors for PVT (30%)
Inherited
Factor V Leiden mutation
Factor II (prothrombin) mutation
Protein C deficiency
Protein S deficiency
Antithrombin deficiency
Acquired
Myeloproliferative disorder
Antiphospholipid syndrome
Paroxysmal nocturnal hemoglobinuria
Oral contraceptives
Pregnancy or puerperium
Hyperhomocysteinemia
Malignancy

Table 3 Prevalence of thrombotic risk factors in series of routinely investigated, consecutive adult patients with non tumorous and non cirrhotic, acute or chronic, PVT[126]

Risk factor	PVT patients (%)
Myeloproliferative disorders	30-40
Atypical	14
Classical	17
Antithrombin deficiency	0-26
Protein C deficiency	0-26
Protein S deficiency	2-30
Factor V Leiden mutation	6-32
Prothrombine mutation	14-40
TT677 methylene tetrahydrofolate reductase (MTHFR) genotype	11-50
Antiphospholipid syndrome	6-19
Hyperomocisteinemia	12-22
Recent pregnancy	6-40
Recent oral contraceptive use	12

Table 4 AASLD recommendations for diagnosis of acute and chronic PVT[126]

AASLD recommendations for diagnosis of acute PVT	AASLD recommendations for diagnosis of chronic PVT
Consider a diagnosis of acute PVT in any patient with abdominal pain of more than 24 h duration, whether or not there is also fever or ileus	Consider a diagnosis of chronic PVT in any patient with newly diagnosed portal hypertension
If acute PVT is suspected, computed tomography (CT) scan, before and after injection of vascular contrast agent, should be obtained for early confirmation of diagnosis. If CT scan is not rapidly available, obtain Doppler-sonography	Obtain Doppler-sonography, then either CT scan or MRI, before and after a vascular contrast agent, to make a diagnosis of chronic PVT
In patients with acute PVT and high fever, septic pylephlebitis should be considered, whether or not an abdominal source of infection has been identified, and blood cultures should be routinely obtained	Base the diagnosis on the absence of a visible normal portal vein and its replacement with serpiginous veins
In acute PVT, the possibility of intestinal infarction should be considered from presentation until resolution of pain. The presence of ascites, thinning of the intestinal wall, lack of mucosal enhancement of the thickened intestinal wall, or the development of multiorgan failure indicate that intestinal infarction is likely and surgical exploration should be considered

Table 5 Intraoperatory grading of PVT extension[17]

Yerdel’s grading
< 50% occlusion of the portal vein
> 50% occlusion of the portal vein (including total occlusion)
Complete thrombosis of both portal and proximal superior mesenteric vein
Complete thrombosis of portal vein and proximal and distal superior mesenteric vein