g., portal vein thrombosis, chemotherapy, and radiotherapy), this website and the study failed to indicate this disorder as the underlying
condition of hepatocellular carcinoma. As a result, hepatocellular carcinoma surveillance in patients with INCPH is not recommended. Hemodynamics and, consequently, the management and prophylaxis of variceal bleeding in patients with INCPH are not entirely comparable to those in cirrhotic patients. Currently, scientific data on management and prophylaxis (i.e., primary and secondary) of variceal bleeding in INCPH patients are scarce. Nevertheless, we recommend to follow the guidelines of prophylaxis and management of cirrhotic variceal bleeding in patients with INCPH.89 Endoscopic sclerotherapy has been proven to be effective in controlling acute variceal bleeding of esophageal hemorrhage in 95% of INCPH patients.90 No scientific data have been published regarding endoscopic band ligation in these patients. However, considering the proven inferiority of sclerotherapy,
compared with endoscopic variceal ligation in cirrhotic patients, the latter treatment is currently also regarded as the most appropriate endoscopic treatment in patients with INCPH. Despite the fact that data regarding combination of endoscopic treatment with vasoactive drugs and antibiotic Atezolizumab in vivo prophylaxis are lacking, we recommend applying these treatments in INCPH patients, considering the effectiveness in cirrhotics. On the basis of Indian studies, emergency shunt surgery because of unmanageable bleeding was only required in 5% of cases with acute variceal bleeding.91 Despite the alleged safety and efficacy of shunt surgery, INCPH patients with uncontrollable hemorrhage
are currently preferentially treated with transjugular intrahepatic portosystemic shunt (TIPS) because of its lower invasiveness.24 PtdIns(3,4)P2 Taking into account the preserved liver function in these patients, the complications of this procedure observed in patients with cirrhosis (e.g., hepatic encephalopathy) are expected to be rare. However, no data are available. Concerning secondary prophylaxis of variceal bleeding in patients with INCPH, smaller studies have demonstrated a reduction of bleeding rate by endoscopic therapy.90, 92 Gastric varices are seen in nearly 25% of Indian INCPH patients.93 Portal hypertensive gastropathy is uncommon at initial presentation and is a rare cause of upper gastrointestinal bleeding.94 In patients with liver cirrhosis, nonselective β-blockers have been shown to reduce gastric mucosal blood flow and decrease recurrent bleeding in a randomized, controlled trial.95 In keeping with this, comparable treatment is applied in INCPH-induced portal hypertensive gastropathy. Patients with INCPH have a massive splenomegaly, leading to increased portal venous flow and, subsequently, portal hypertension.
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