Budd-Chiari syndrome in children: Challenges and outcome

Table 2 Clinical presentation, site of block, radiological intervention, and outcomes of major studies in children with Budd-Chiari syndrome

	Nagral et al[12], %	Kathuria et al[13], %	Sharma et al[14], %	Singh et al[15], %	Shukla et al[32], %
No. of patients	16	45	32	113	43
Age at presentation, median (range)	22 (4-132) mo	10.5 (2-16) yr	9 (4.5-214) mo	10 (1.5-17) yr	16.7 (10-19) yr
Symptom duration, median (range)	3 (0.5-48) mo	8.9 mo (5 d–8 yr)	-	-	12.7 (0.5-150) mo
Diagnosis by USG	63	95.6	60	-	-
Symptoms
Ascites	81	82	96	84	91
GI bleed	25	35	8	22	23
Jaundice	12.5	20	24	12	14
Hepatomegaly	N/A	85	96	N/A	67
Splenomegaly	N/A	70	N/A	N/A	37
Abdominal vein dilation	N/A	70	70	N/A	N/A
Type of block
Only HV	94	71	76	74	67
Only IVC	6	4	0	2	9
Both HV + IVC	0	25	24	24	24
RI, n	13	25	24	53	24
Angioplasty alone	4	2	7	7	-
Angioplasty + stenting	3	20	3	40	10
DIPS	-	-	-	5	-
TIPSS	6	3	14	1	14
Follow-up duration, median (IQR)	31 (12-54) mo	6.5 (0.5-86) mo	44 (5-132) mo	13.5 (1-155) mo	41 (12-168) mo
Successful RI
Angioplasty	25	100	43	100	-
Angioplasty + stenting	100	87	67	90	90
DIPS	-	-	-	80%	-
TIPSS	80	67	72	100	80
Procedure related complications	TIPSS encephalopathy = 1; Neck hematoma = 1	Anaesthesia related death = 1; Neck Hematoma-1; Hemoperitoneum = 1	No major	TIPSS; Encephalopathy = 1	N/A
Stent patency rate	-	Overall 75%	-	87% at 1 yr, 82% at 5 yr, 62% at 10 yr	Overall 75%
Mortality	2: GI bleeding 1, liver failure = 1	3: Intracranial bleeding = 1, anaesthesia related = 1, liver failure = 1	5: Intracranial bleeding = 2, GI bleeding = 1, HCC = 1, liver failure = 1	3: Procedure related = 2, head trauma = 1	4: Intracranial bleeding = 1, HCC = 1, liver failure = 2
HPS	N/A	N/A	4 (12, 5%)		-
HCC	N/A	N/A	1 (3%)		1 (2.3%)

N/A: Not available; HV: Hepatic vein; IVC: Inferior vena cava; USG: Ultrasonography; GI: Gastro-intestinal; HV: Hepatic vein; IVC: Inferior vena cava; RI: Radiological intervention; DIPS: Direct intrahepatic porto-systemic shunt; TIPSS: Transjugular intrahepatic porto-systemic shunt; HPS: Hepato-pulmonary syndrome; HCC: Hepatocellular carcinoma.