Overview
Definition:
Portal hypertensive gastropathy (PHG) is a condition characterized by mucosal and submucosal changes in the stomach lining, occurring secondary to increased portal venous pressure, typically in the setting of cirrhosis
These changes are thought to result from increased mucosal blood flow, venous congestion, and intramural edema, leading to a fragile and easily bleeding mucosa.
Epidemiology:
PHG is a common complication of portal hypertension, with its prevalence varying widely in studies, ranging from 5% to over 90% in patients with cirrhosis, depending on the diagnostic criteria and patient population
It is a significant cause of non-variceal upper gastrointestinal bleeding in patients with liver disease.
Clinical Significance:
PHG contributes significantly to morbidity and mortality in patients with portal hypertension by causing chronic or acute gastrointestinal bleeding
This bleeding can range from occult blood loss leading to iron deficiency anemia to severe, life-threatening hematemesis and melena
Understanding PHG is crucial for appropriate diagnosis and management, particularly when differentiating it from gastric varices, which have different treatment strategies.
Clinical Presentation
Symptoms:
Intermittent or chronic occult blood loss leading to iron deficiency anemia
Intermittent or recurrent upper GI bleeding presenting as hematemesis (vomiting blood) or melena (black, tarry stools)
Dyspeptic symptoms such as abdominal discomfort, nausea, or early satiety may be present but are non-specific
Bleeding may be mild or severe, sometimes life-threatening.
Signs:
Pallor and signs of anemia (e.g., fatigue, dyspnea on exertion)
Epigastric tenderness may be present in some patients
Signs of underlying liver disease such as jaundice, ascites, splenomegaly, and caput medusae are common
Vital signs may be normal or show signs of hypovolemia (tachycardia, hypotension) in cases of significant bleeding.
Diagnostic Criteria:
There are no universally agreed-upon diagnostic criteria for PHG
diagnosis is largely endoscopic
The Japanese Research Society for Portal Hypertension has proposed a classification based on endoscopic findings, grading PHG from mild (Grade 1: diffuse erythema, mosaic pattern) to severe (Grade 2: elevated edematous areas, vascular ectasia)
Biopsies are often non-specific but can help rule out other pathologies.
Diagnostic Approach
History Taking:
Detailed history of liver disease (etiology, duration, complications like ascites, encephalopathy)
History of upper GI bleeding or dyspepsia
Medications (NSAIDs, anticoagulants)
Alcohol intake
Previous endoscopic findings
Family history of GI disorders.
Physical Examination:
General examination to assess for pallor, jaundice, and signs of chronic liver disease (ascites, edema, splenomegaly, spider angiomata, palmar erythema)
Abdominal examination for tenderness, hepatomegaly, splenomegaly, and ascites
Rectal examination to check for melena or fresh blood.
Investigations:
Complete blood count (CBC) to assess for anemia and thrombocytopenia
Liver function tests (LFTs) including bilirubin, albumin, prothrombin time (PT), and INR
Viral serology for Hepatitis B and C
Ultrasound abdomen with Doppler to assess portal vein patency, diameter, and flow
Endoscopy (esophagogastroduodenoscopy - EGD) is the gold standard to visualize mucosal changes, assess for varices, and rule out other causes of bleeding
Biopsies can be taken but are often non-diagnostic for PHG itself.
Differential Diagnosis:
Gastric varices (must be differentiated, as management differs)
Peptic ulcer disease
Mallory-Weiss tears
Gastritis
Esophagitis
Gastric malignancy
Dieulafoy's lesion
Angiodysplasia.
Management
Initial Management:
Hemodynamic stabilization with intravenous fluids and blood transfusions if necessary
Correction of coagulopathy if present
Nasogastric lavage to assess for ongoing bleeding and clear gastric contents
Pharmacological management to reduce portal pressure.
Medical Management:
Beta-blockers (e.g., propranolol, nadolol) are the mainstay for reducing portal pressure and preventing variceal bleeding, and they can also help reduce PHG bleeding
Somatostatin analogues or octreotide may be used for acute bleeding episodes
Proton pump inhibitors (PPIs) are typically used to reduce gastric acid and aid mucosal healing, though their direct effect on PHG bleeding is debated.
Surgical Management:
The role of surgery in PHG is generally limited and reserved for refractory bleeding that does not respond to medical management or endoscopic therapy
Surgical options are usually directed at reducing portal pressure, such as portosystemic shunts (e.g., TIPS - transjugular intrahepatic portosystemic shunt, surgical shunts like mesocaval, splenorenal)
Direct surgical resection of PHG lesions is rarely performed due to diffuse nature and poor healing
In severe, life-threatening bleeding unresponsive to other measures, distal splenorenal shunt or even portacaval shunt might be considered, but with significant risks
Gastric devascularization procedures are generally not effective for PHG.
Supportive Care:
Nutritional support is essential for patients with liver disease
Regular monitoring for signs of bleeding and liver decompensation
Management of ascites and hepatic encephalopathy.
Complications
Early Complications:
Severe acute gastrointestinal hemorrhage leading to hypovolemic shock
Rebleeding following initial treatment
Anemia secondary to chronic blood loss.
Late Complications:
Chronic iron deficiency anemia impairing quality of life
Progressive liver dysfunction
Complications related to treatment of portal hypertension (e.g., hepatic encephalopathy, shunt dysfunction).
Prevention Strategies:
Optimal medical management of underlying liver disease
Early diagnosis and management of portal hypertension
Prophylactic beta-blocker therapy in patients with cirrhosis and portal hypertension, particularly those with increased risk of bleeding.
Prognosis
Factors Affecting Prognosis:
Severity of underlying liver disease (Child-Pugh score, MELD score)
Degree of portal hypertension
Presence and severity of bleeding
Response to medical and endoscopic therapy
Development of complications from treatment interventions.
Outcomes:
Prognosis is largely determined by the underlying liver disease
PHG itself is a chronic condition that requires ongoing management
Bleeding episodes can be managed with medical therapy and sometimes TIPS
The goal is to control bleeding and prevent recurrent episodes while managing the liver disease.
Follow Up:
Regular follow-up with a gastroenterologist or hepatologist is crucial to monitor liver disease status, portal hypertension, and for recurrent bleeding
Endoscopic surveillance may be required
Long-term medical therapy, especially beta-blockers, is typically needed.
Key Points
Exam Focus:
PHG is a common cause of GI bleeding in cirrhosis, secondary to portal hypertension, distinct from variceal bleeding
Endoscopy is key for diagnosis
Medical management focuses on reducing portal pressure (beta-blockers) and acid suppression
Surgical role is limited to refractory bleeding, typically by reducing portal pressure (e.g., TIPS).
Clinical Pearls:
Always consider PHG in a patient with cirrhosis presenting with GI bleeding, especially if varices are absent or small
Differentiating PHG from gastric varices on endoscopy is critical for management
Refractory PHG bleeding may necessitate TIPS, not surgical resection of gastric mucosa.
Common Mistakes:
Mistaking PHG for gastric varices and treating it with sclerotherapy or band ligation, which are ineffective for PHG
Overlooking the contribution of PHG to bleeding in patients with known varices
Delaying TIPS in appropriately selected patients with refractory PHG bleeding.