Budd-Chiari syndrome: Prognostic scores, special populations, and management challenges

Table 1 Variables, formulas, and interpretation for each score

Score	Type	Parameters used	Formula/cutoffs	Clinical interpretation
New Clichy Score	Clinical/Laboratory-based	Bilirubin, INR, creatinine, ascites, encephalopathy	Weighted scoring system; score > 2 indicates poor prognosis	Stratifies diseases severity; guides timing of intervention
BCS-TIPS Index	Clinical/Laboratory-based	Bilirubin, INR, creatinine, age	Score = (1.03 × creatinine) + (0.8 × bilirubin) + (0.8 × INR) + (0.3 × age); > 7 Leads to poor outcome	Predicts survival after TIPS
Zeitoun Score	Clinical/Laboratory-based	INR, bilirubin, ascites	Categorical system: Class I (mild) to Class III (severe)	Assesses prognosis pre-TIPS
PELD Score	Clinical/Laboratory-based (pediatrics)	Albumin, bilirubin, INR, growth failure, age	Standard UNOS formula; higher scores correlate with worse outcomes	Used for pediatric LT prioritization
Imaging parameters	Imaging-based (proposed/research)	HV patency, caudate lobe hypertrophy, hepatic congestion signs	Standard UNOS formula; higher scores correlate with worse outcomes	Not part of current scores; proposed for future integration

BCS: Budd-Chiari syndrome; TIPS: Transjugular intrahepatic portosystemic shunt; PELD Score: Pediatric End-stage Liver Disease Score; INR: International normalized ratio; HV: Hepatic vein; LT: Liver transplantation; UNOS: United Network. for Organ Sharing.

Table 2 Comparison and limitations of prognostic scoring systems in Budd-Chiari syndrome

Feature	New Clichy Score	BCS-TIPS Index	Zeitoun Score	PELD Score
Population	Primarily validated in adult patients with BCS	Specifically designed for adult patients with BCS who have undergone a TIPS procedure	Developed for adult patients with BCS	Designed for pediatric patients (< 12 years old) with end-stage liver disease awaiting LT
Predictors	Ascites score, Child-Pugh score, age, serum creatinine, clinicopathological form III (acute on chronic)	Age, serum total bilirubin, INR	Age, response of ascites to diuretics, Child-Pugh score, serum creatinine	Age (< 1 year), albumin, bilirubin (total), INR, growth failure
Cutoff	≥ 3.75 for predicting 1-year outcomes	< 7 indicates excellent 1-year survival (96%); ≥ 7 indicates high-risk	≤ 5.4 indicates good prognosis; > 5.4 indicates poor prognosis	≥ 15 indicates high risk of death without LT in chronic liver disease
Limitations	Primarily validated in a specific Egyptian cohort; generalizability needs confirmation. Overall survival prediction	Predicts survival specifically post-TIPS but not overall BCS survival. Generalizability may vary; did not predict 1-year shunt patency in one study	Focused on overall survival; impact of specific interventions like shunts uncertain in the original study	Primarily predicts pre transplant mortality for children < 12 years. Accuracy influenced by unmeasured factors. Not typically used for BCS prognosis

Comparison and limitations of prognostic scoring systems in Budd-Chiari syndrome adapted from[12-17]. BCS: Budd-Chiari syndrome; TIPS: Transjugular intrahepatic portosystemic shunt; PELD Score: Pediatric End-stage Liver Disease Score; INR: International normalized ratio; LT: Liver transplantation.