Etiology and long-term outcome of extrahepatic portal vein obstruction in children

Table 1 Demographic data, clinical presentation and risk factors for extrahepatic portal vein obstruction in the study group

	n (%)
Gender
Male	13 (43.3)
Female	17 (56.7)
Ethnicity
Jewish	15 (50)
Arab	14 (46.6)
Russian	1 (3.3)
Referral
Israeli	19 (63.3)
Palestinian	11 (36.7)
Gestational age
Term	23 (76.7)
Preterm	7 (23.3)
Clinical presentation
Splenomegaly	13 (43.3)
UGI bleeding	12 (40.0)
Cytopenia	5 (16.6)
Elevated liver enzymes	2 (6.6)
Risk factor for EHPVO
Perinatal events
Umbilical catheterization	6 (20)
Omphalitis	2 (6.6)
Sepsis, NEC	1 (3.3)
Hypercoagulable state
APLA syndrome	2 (6.6)
Protein S and C deficiency	1 (3.3)
MTHFR mutation homozygosity	1 (3.3)
Unknown	17 (56.6)

EHPVO: Extrahepatic portal vein obstruction; UGI: Upper gastrointestinal; MTHFR: Methyltetrahydrofolate reductase; APLA: Anti phospholipid antibodies.

Table 2 Clinical characteristics and course of Israeli and Palestinian children with extrahepatic portal vein obstruction n (%)

	Israeli	Palestinian	P
Gender (M/F)	11/8	2/9	0.06
Age at diagnosis (yr)
mean ± SD	6 ± 5.1	3.5 ± 3.8
Median (range)	7 (0.1-16)	2 (0.75-12)	NS
Etiology
Perinatal events	4 (21)	4 (36.3)	NS
Hypercoagulability	2 (10.5)	2 (18.2)
Variceal bleeding	9 (47.3)	9 (81.8)	NS
Follow-up (yr, mean ± SD)	6.5 ± 5.3	3.0 ± 2.2	NS
Surgery	7 (36.8)	4 (36.3)	NS

NS: Not significant.

Table 3 Endoscopic and surgical treatment of children with extrahepatic portal vein obstruction

Procedure	n (%)
Sclerotherapy	13 (43.3)
Variceal banding	7 (23.3)
Surgery
Splenorenal shunt	9 (30.0)
Mesocaval shunt	1 (3.3)
Meso-Rex shunt	1 (3.3)
Splenectomy	1 (3.3)