Epidemiology: Rare (0.05-0.5% in autopsy studies), but leading cause of portal HTN in non-cirrhotics in West.  Often assx until variceal bleeding occurs.  WIDE variety of etiologies.


Etiologies: Virchow’s Triad

1.   Cirrhosis (24-32%): decreased portal flow, periportal lymphangitis

2.   Neoplasm (21-24%): direct invasion, extrinsic compression

a.   most common: pancreatic (11-12%), primary hepatocellular (5-6% in Western, much higher elsewhere)

b.   others associated: lung, stomach, prostate, uterus, kidney, cholangioca, carcinoid, primary liver lymphoma

1.   Infection (10-25%- most common cause in children): portal pyemia- often secondary to apendicitis, biliary tract infections, sepsis, amoebic colitis, diverticulitis

2.      Inflammatory: pancreatitis (3-5%), appendicitis, cholecystitis, EtOH hepatitis, perforated DU.

3.      Myeloproliferative disorders (3-12%): may be responsible for many “idiopathic” cases.

4.      Hypercoagulable states

a.            inherited: ProC, ProS, AT III

b.            acquired: nephrotic syndrome, DIC, IBD, malignancy, estrogen use.

1.   Other: non-cirrhotic portal fibrosis, trauma, post-surgical (esp. post-splenectomy).


Clinical features: Hematemesis/melena from ruptured varices is most common presentation.

1.   SXS/SNS: increased abd girth, pain, N/V, anorexia, weight loss, diarrhea

2.   EXAM: splenomegaly (75-100%), mild hepatomegaly, abd tenderness.  Ascites less common, mild, transient.

LABS: may be mild elevations of transaminases, alk phos, bilis.

*may be differentiated from Budd-Chiari by hepatomegaly,

  ascites, hepatocellular dysfunction seen in Budd-Chiari..


Imaging: Start with ultrasound, MR if available 

1.   Venogram: gold standard.

2.   US: highly sensitive (>90%), but operator dependent.

3.   CT: less sensitive (75%), highly specific. 

4.   MR: highly sensitive and specific.




1.   Variceal hemorrhage: average 5 episodes/pt.

2.   Hepatic encephalopathy: uncommon unless coexisting cirrhosis or shunt surgery.

3.   Small bowel infarction: extension of thrombus to SMV



1.   Acute:

a.   Control acute bleeding: correct coagulopathy, transfuse, SB balloon, vasopressin, endoscopic sclerotherapy, surgery.

b.            Catheter directed thrombolysis: some anecdotal successes. 

1.   Chronic:

a.   Treat underlying etiology.

b.            Prophylactic sclerotherapy/banding of varices.

c.   Surgical shunt: usually spenorenal, very effective for non-cirrhotics.

d.            Anticoagulation: no clear benefit unless underlying hypercoagulable state.


Cohen, et al. Am J Med. Feb 1992, 92: 73-182.