What are the signs and symptoms of portal hypertension?

Signs and Symptoms of Portal Hypertension

Portal hypertension presents with a constellation of clinical manifestations including splenomegaly, gastroesophageal varices, ascites, and portosystemic collaterals, with gastrointestinal bleeding being the most serious complication. 1

Definition and Pathophysiology

• Portal hypertension is defined as a pathological increase in portal pressure with hepatic venous pressure gradient (HVPG) >5 mmHg, with clinically significant portal hypertension (CSPH) occurring at HVPG ≥10 mmHg 2
• The condition develops through increased intrahepatic resistance and increased portal blood flow, following the hydraulic equivalent of Ohm's law where "Pressure = Resistance × Flow" 2

Major Clinical Manifestations

Gastrointestinal Manifestations

• Gastroesophageal varices - dilated submucosal veins in the lower esophagus and stomach that can rupture and cause life-threatening hemorrhage 1
• Portal hypertensive gastropathy - characterized by a mosaic-like pattern in the gastric mucosa 1
• Gastrointestinal bleeding - the most frequent and serious complication of portal hypertension 3
• Post-prandial abdominal pain or features of incomplete bowel obstruction may occur, particularly in extrahepatic portal vein obstruction 3

Splenomegaly and Hematologic Abnormalities

• Enlarged spleen (splenomegaly) - often a prominent physical finding 3, 1
• Hypersplenism leading to reduced blood cell counts (thrombocytopenia, leukopenia) 1
• These findings may be discovered incidentally on physical examination or imaging 3

Fluid Accumulation

• Ascites - abnormal accumulation of fluid in the peritoneal cavity, the most common complication of portal hypertension 1
• Hepatic hydrothorax - pleural effusion occurring in 0%-8% of cases 1
• Ascites and bacterial infections are uncommon in extrahepatic portal vein obstruction except following episodes of gastrointestinal bleeding 3

Vascular Manifestations

• Portosystemic collaterals - abnormal vascular channels that develop to decompress the portal system, often visible on abdominal imaging 1
• Cavernous transformation of the portal vein - development of porto-portal collaterals following acute portal vein thrombosis 3
• Portal cholangiopathy - compression and deformation of bile ducts by collateral veins constituting the cavernoma 3

Neurological Manifestations

• Overt hepatic encephalopathy - uncommon in extrahepatic portal vein obstruction except following gastrointestinal bleeding 3
• Subclinical encephalopathy - more common than previously suspected 3

Biliary Manifestations

• Biliary symptoms (biliary pain, pancreatitis, cholecystitis) related to portal cholangiopathy 3
• Progressive cholestatic disease or recurrent bacterial cholangitis are rare in patients with portal cholangiopathy 3

Diagnostic Features

• Contrast between severe portal hypertension and mild or absent liver dysfunction with normal levels of transaminases, alkaline phosphatase, and gamma-glutamyl transferase in extrahepatic portal vein obstruction 3
• Doppler ultrasound is the first-line investigation for diagnosing extrahepatic portal vein obstruction 3
• CT or MRI with vascular contrast agents provide confirmation and assessment of extension 3
• MR cholangiography should be performed in patients with persisting cholestasis or biliary tract abnormalities suggesting portal biliopathy 3

Specific Presentations in Non-Cirrhotic Portal Hypertension

• In idiopathic non-cirrhotic portal hypertension (INCPH), diagnostic criteria include clinical signs of portal hypertension, exclusion of cirrhosis on liver biopsy, exclusion of chronic liver disease causing cirrhosis or non-cirrhotic portal hypertension, exclusion of conditions causing non-cirrhotic portal hypertension, and patent portal and hepatic veins 3
• Clinical signs of INCPH include splenomegaly/hypersplenism, esophageal varices, ascites, minimally increased hepatic venous pressure gradient, and portovenous collaterals 3

Prognostic Factors

• Previous gastrointestinal bleeding and size of esophageal varices are independent predictors for future gastrointestinal bleeding 3
• Presence of an underlying prothrombotic condition predicts recurrent thrombosis 3
• Age, ascites, extension to the superior mesenteric vein, and severity of underlying conditions predict mortality 3

Portal hypertension should be suspected in any patient with unexplained splenomegaly, reduced blood cell counts, or features of liver disease, and appropriate diagnostic workup should be initiated promptly to prevent life-threatening complications.