Budd-Chiari syndrome in children: Challenges and outcome

Table 3 Budd-Chiari syndrome-specific prognostic indices

Score	Equation	Cut-off	Predicted survival rate
CTP	Serum bilirubin, serum albumin, INR, ascites, HE	-	-
MELD	9.57 × log (creatinine) + 3.78 × log (total bilirubin) + 11.2 × log (INR) + 6.43	-	-
Zeitoun index	Ascites scorea × 0.75 + Child-Pugh score × 0.28 + age × 0.037 + creatinine × 0.0036	5.4 (range from 3.4 to 9.1)	At 5 yr; ≤ 5.4: 95%; > 5.4: 65%
New Clichy score	0.95 × ascites score + 0.35 × CTP score + 0.047 × age + 0.0045 × serum creatinine + 2.2 × type IIIbb—2.6	5.1 (range from 2.0 to 9.7)	At 5 yr; < 5.1: 100%; ≥ 5.1: 65%
Rotterdam BCS index	1.27 × encephalopathy + 1.04 × ascites + 0.72 × prothrombin time + 0.004 × bilirubin	Bilirubin; class I: 0–1.1; class II: 1.1–1.5; class III: ≥ 1.5 (range from 0.02 to 4.03)	At 5 yr; class I: 89%; class II: 74%; class III: 42%
TIPS-BCS index	Age (yr) × 0.08 + bilirubin (mg/dL) × 0.16 + INR × 0.63	7	1-yr OLT-free survival, ≤ 7: 95%; > 7: 12%
BCS-intervention-free survival prognostic score	Ascites (yes = 1, no = 0) × 1.675 + ln creatinine (μmol/L) × 0.613 + ln bilirubin (μmol/L) × 0.440	Interval 1: ≤ 5; interval 2: 5-6; interval 3: ≥ 6	Intervention-free survival interval 1: 78.3%, interval 2: 27.8%; interval 3: 6.8%
AIIMS-HVOTO score	1.2 × response to therapy + 0.8 × child class)	Score: < 3; 3-4; > 4	% yr survival score ≤ 3: 92%; score 3-4: 79%; score > 4: 39%

^aAscites score: 1, absent with free sodium intake and no diuretic agents; 2, easy to control with sodium restriction or diuretic agents; and 3, resistant to this treatment because of hyponatremia or functional renal failure.

^bType III’ is a binary variable coded as 1 for patients with clinicopathological findings of acute injury superimposed on chronic lesions, and 0 for the other patients.

CTP: Child-Turcotte-Pugh, MELD: Model for end stage liver disease; BCS: Budd-Chiari syndrome; AIIMS: All India Institute of Medical Sciences; HVOTO: Hepatic vein outflow tract obstruction; OLT: Orthotopic liver transplantation.