What is the primary management approach for a patient with Dyskeratosis Congenita with Lichenoid Dermatosis (DCld) and portal hypertension?

Management of Decompensated Cirrhotic Liver Disease (DCLD) with Portal Hypertension

For patients with decompensated cirrhotic liver disease and portal hypertension, the primary management approach involves non-selective beta-blockers for variceal prophylaxis, endoscopic band ligation for high-risk varices, TIPS for refractory complications, and early liver transplant evaluation given the poor prognosis. 1, 2

Initial Assessment and Risk Stratification

Screening for Portal Hypertension Complications

• All patients with decompensated cirrhosis require screening endoscopy to identify esophageal varices, as variceal bleeding represents a life-threatening complication with significant mortality risk 1, 2
• Assess for clinically significant portal hypertension (CSPH) using liver stiffness measurement and platelet count, as CSPH (hepatic venous pressure gradient ≥10 mmHg) predicts risk of first decompensation 3
• Evaluate for ascites, hepatic encephalopathy, and hepatorenal syndrome, which indicate advanced disease requiring aggressive management 1, 2

Liver Function Assessment

• Patients with decompensated disease (Child-Pugh C, elevated bilirubin, low albumin, INR >1.5, or encephalopathy) should be referred immediately for liver transplant evaluation, as this represents the only definitive treatment 3
• Calculate MELD score to guide prognosis and transplant timing, as MELD ≥19 indicates high mortality risk 1, 2

Medical Management

Variceal Bleeding Prevention

• Non-selective beta-blockers (NSBBs) are first-line for primary prophylaxis of variceal hemorrhage, as they reduce portal pressure and prevent multiple complications of portal hypertension 1, 2
• For patients with medium to large varices, NSBBs are preferred over endoscopic band ligation (EBL) alone for primary prophylaxis 1
• For secondary prophylaxis (prevention of rebleeding), combined therapy with NSBBs plus EBL is mandatory, as this significantly decreases rebleeding compared to monotherapy 1, 2

Acute Variceal Bleeding Management

• Immediate administration of vasoactive agents (octreotide) followed by urgent endoscopic therapy is required, as combination therapy improves 5-day hemostasis rates (77% vs 58% with endoscopy alone) 1, 2
• Antibiotic prophylaxis must be given to all cirrhotic patients with acute upper gastrointestinal bleeding, as this reduces mortality, bacterial infections, and rebleeding 2

Ascites Management

• Medical management with diuretics (spironolactone plus furosemide) is first-line for ascites 2
• Large-volume paracentesis with albumin replacement for tense ascites causing respiratory compromise 2

Advanced Interventional Management: TIPS

TIPS Indications in Decompensated Disease

• TIPS is strongly indicated for variceal bleeding refractory to endoscopic and drug therapy (failure to control bleeding or early rebleeding despite combined medical and endoscopic treatment) 3, 1, 2
• Early or pre-emptive TIPS should be performed within 72 hours of variceal bleeding in high-risk patients (Child-Pugh C or MELD ≥19), as this improves survival 3, 1, 2
• TIPS is recommended for refractory or recurrent ascites in selected patients without contraindications 3, 1, 2
• TIPS may be considered for refractory hepatic hydrothorax in selected cases 3, 1

TIPS Contraindications in Decompensated Disease

Critical contraindications that preclude TIPS include: 3, 1, 2

• Bilirubin >50 μmol/L with platelets <75×10⁹/L
• Pre-existing hepatic encephalopathy
• Active infection
• Severe cardiac failure or severe pulmonary hypertension
• Advanced liver failure (though TIPS may serve as bridge to transplant in highly selected cases)

TIPS Procedure Considerations

• Patients should be referred to TIPS expert centers performing ≥10 cases annually, as procedural volume correlates with outcomes 3
• Covered stents are preferred over bare metal stents due to superior patency rates 2
• All TIPS procedures in decompensated patients must be discussed with the liver transplant team, as these patients require close coordination between interventional radiology and transplant hepatology 3

Post-TIPS Monitoring and Complications

• Doppler ultrasound at one week post-procedure, then at 6-12 month intervals 2
• Hepatic encephalopathy develops in approximately one-third of patients after TIPS and usually responds to lactulose and rifaximin, though severe cases may require TIPS reduction or occlusion 1, 2
• Monitor for TIPS dysfunction with regular imaging surveillance 2

Liver Transplantation

Transplant Evaluation Timing

• All patients with decompensated cirrhosis and portal hypertension should undergo liver transplant evaluation, as transplantation is the only curative therapy for end-stage liver disease 3
• Indications include: recurrent/refractory ascites, refractory variceal bleeding, hepatorenal syndrome, recurrent hepatic encephalopathy, and progressive liver failure 3
• MELD ≥15 or presence of any decompensation event (ascites, variceal bleeding, encephalopathy, jaundice) warrants transplant referral 3

TIPS as Bridge to Transplant

• In patients with severe hepatic insufficiency (INR >1.5, abnormal bilirubin, low albumin, refractory ascites or encephalopathy), TIPS may serve as a bridge to transplantation for management of intractable variceal bleeding, but transplantation remains the definitive treatment 3

Critical Pitfalls to Avoid

• Do not delay transplant evaluation in decompensated patients, as mortality increases rapidly with each decompensation event 3
• Do not perform TIPS in patients with severe liver insufficiency (Child-Pugh C with MELD >18) unless as a bridge to urgent transplantation, as post-TIPS liver failure carries high mortality 3
• Do not use TIPS for hepatorenal syndrome outside of clinical trials, as evidence remains insufficient and outcomes are poor 3
• Ensure multidisciplinary coordination between hepatology, interventional radiology, intensive care, and transplant surgery for optimal outcomes 3