Step 1: General Approach to Management of Extrahepatic Portal Hypertension.
The management of EHPH focuses on addressing the underlying cause, controlling complications, and preventing variceal bleeding.
Step 2: Management of Complications.
(1) Variceal Bleeding:
- Endoscopic Therapy: Endoscopic variceal ligation (EVL) or sclerotherapy is performed to control bleeding from esophageal or gastric varices.
- Beta-blockers: Non-selective beta-blockers (e.g., propranolol) can reduce portal pressure and prevent variceal bleeding.
- Vasoactive Drugs: Drugs like octreotide may be used to control acute bleeding episodes.
(2) Splenomegaly and Thrombocytopenia:
- Splenectomy: In severe cases of splenomegaly or thrombocytopenia causing bleeding, a splenectomy may be considered.
(3) Ascites Management:
- Diuretics: Loop diuretics (e.g., furosemide) and potassium-sparing diuretics (e.g., spironolactone) are used to manage ascites.
- Paracentesis: Removal of excess fluid from the abdominal cavity if ascites becomes severe.
Step 3: Long-term Management.
(1) Shunt Surgery or Transjugular Intrahepatic Portosystemic Shunt (TIPS): In some cases, surgical procedures such as a portosystemic shunt or TIPS may be performed to bypass the portal obstruction and reduce portal hypertension.
(2) Management of Underlying Causes: If EHPH is secondary to another condition (e.g., thrombophili, treatment of the underlying cause may help prevent further complications.
(3) Liver Transplantation: In cases where there is progressive liver damage or failure associated with EHPH, liver transplantation may be considered.
Step 4: Follow-up and Monitoring.
Regular monitoring for variceal bleeding and ascites management is important. Ongoing surveillance with imaging and endoscopy may be necessary to assess the condition and manage complications.