How is Banti syndrome managed?

Management of Banti Syndrome

Critical Clarification: Banti Syndrome vs. Budd-Chiari Syndrome

The term "Banti syndrome" is historically outdated and refers to idiopathic non-cirrhotic portal hypertension with splenomegaly, which is a fundamentally different condition from Budd-Chiari syndrome (BCS), despite both causing portal hypertension. 1, 2 The provided evidence predominantly addresses Budd-Chiari syndrome management, so I will address both conditions separately.

---

Management of True Banti Syndrome (Idiopathic Non-Cirrhotic Portal Hypertension)

Primary Treatment Approach

Splenectomy combined with endoscopic management of varices is the definitive treatment for Banti syndrome, addressing both the underlying splenomegaly and preventing variceal bleeding complications. 2

Surgical Management

• Splenectomy serves both diagnostic and therapeutic purposes, normalizing clinical, radiological, and biological abnormalities while preventing recurrence of esophageal varices 2
• Splenectomy combined with ligation of esophageal varices may be sufficient without requiring splenorenal bypass procedures 2
• This approach addresses the primary lesion in small splenic arterioles that drives the pathophysiology 1

Pre-Surgical Requirements

• Exclude intrahepatic obstruction, extrahepatic portal vein obstruction, and cirrhosis before proceeding to splenectomy 1, 2
• Liver biopsy must confirm absence of cirrhosis or other hepatic disorders 2
• Imaging with ultrasound and splenoportography establishes the diagnosis 2

Management of Complications

• Upper gastrointestinal hemorrhage from varices requires endoscopic variceal ligation prior to or concurrent with splenectomy 1, 2
• Portal hemodynamics show increased splenic venous flow as the main contributor to portal hypertension elevation 3

---

Management of Budd-Chiari Syndrome (If This Was the Intended Question)

Immediate Initial Management

All patients with Budd-Chiari syndrome must receive lifelong anticoagulation therapy initiated immediately upon diagnosis, targeting an INR of 2-3, unless major contraindications exist. 4, 5

Anticoagulation Protocol

• Start low molecular weight heparin (LMWH) for at least 5-7 days 5
• Transition to vitamin K antagonists (VKA) targeting INR 2-3 4, 5
• Portal hypertension complications (varices, ascites) when adequately treated are NOT contraindications to anticoagulation 4
• Briefly interrupt anticoagulation only for invasive procedures including paracentesis 4
• Continue anticoagulation indefinitely, even after liver transplantation in most cases 4

Concurrent Treatment

• Treat underlying prothrombotic causes, particularly myeloproliferative disorders, immediately upon diagnosis 4, 5
• Manage portal hypertension complications following cirrhosis guidelines 4, 5

Stepwise Interventional Approach

First-Line Intervention: Angioplasty/Stenting

For patients with short hepatic vein stenosis (present in 25-30% of cases) or IVC stenosis (60% of cases), angioplasty with stenting is the first-line decompressive procedure. 4, 5

• Stenting reduces re-stenosis rates compared to angioplasty alone 4
• Most effective for focal, short-length stenoses where physiological drainage can be restored 5
• Caution: Stent misplacement may compromise subsequent TIPS or liver transplantation 4

Second-Line: TIPS (Transjugular Intrahepatic Portosystemic Shunt)

TIPS using PTFE-covered stents is the derivative treatment of choice for patients who fail medical therapy or when angioplasty/stenting is ineffective or technically impossible. 4, 5

• Indicated for patients not meeting complete or ongoing response criteria on medical therapy 4
• Symptom resolution exceeds 70% with 5-year survival rates exceeding 70% 5
• Use PTFE-covered stents to improve primary patency 5
• Hepatic encephalopathy risk is approximately 15% 5
• Perform Doppler ultrasound early post-procedure and every 6 months to detect thrombosis or dysfunction 5

Third-Line: Surgical Shunts

Discuss surgical shunting (mesocaval shunt with PTFE or autologous jugular vein) when TIPS is not feasible or fails. 4

• Mesocaval shunt is preferred over portocaval side-to-side when caudate lobe hypertrophy is present 4
• Ineffective if IVC thrombosis or severe IVC compression exists 4

Last Resort: Liver Transplantation

Liver transplantation is reserved as salvage treatment for patients in whom all derivative techniques have failed. 4, 5

• Best outcomes when thrombosis is limited to hepatic veins and underlying cause can be corrected 5
• Patients with BCS-TIPS score >7 consistently predict poor outcomes and should be considered for transplant assessment 5
• Immediate referral for fulminant hepatic failure with consideration of urgent TIPS as bridge 5

Specialized Management Considerations

Referral Requirements

All BCS patients must be managed in expert centers with hepatology, interventional radiology expertise, and formal links to liver transplant centers. 4, 6

Monitoring and Surveillance

• Screen for hepatocellular carcinoma as hypervascular liver nodules may develop 5
• Regular Doppler ultrasound for TIPS patency assessment 5
• Monitor anticoagulation closely to maintain target INR 5

Common Pitfalls

• Bleeding complications on anticoagulation have decreased from 50% to 17% with better procedural management and portal hypertension prophylaxis 4
• Avoid unfractionated heparin due to high rates of heparin-induced thrombocytopenia 4
• Rotterdam Class III patients should be considered for early TIPS 5

---

The key distinction is that true Banti syndrome requires splenectomy, while Budd-Chiari syndrome requires anticoagulation with stepwise escalation to interventional procedures based on treatment response.