Well-tolerated portal hypertension and favorable prognosis in adult patients with extrahepatic portal vein obstruction in Japan.
OBJECTIVE
To evaluate the clinical features and prognoses in adult patients with extrahepatic portal vein obstruction (EHO) from the aspect of portal hypertension during the last 20 years in Japan.


METHODS
There were 40 EHO patients (aged 21-77 years; mean ± standard deviation [SD], 54.6 ± 15.0). Clinical findings and prognoses were examined retrospectively during the median observation period of 71.6 months.


RESULTS
Twenty-two patients (55%) showed positive signs of portal hypertension; 18 with esophageal varices (F0, one; F1, eight; F2, nine), two with gastric varices (F1, one; F2, one) and seven with mild ascites. Multivariate analysis showed that platelet count and spleen size were significant factors for the presence of gastroesophageal varices, with odds ratios of 0.989 (95% confidence interval [CI], 0.980-0.997; P = 0.011) for platelet count and 1.003 (95% CI, 1.001-1.005; P = 0.003) for spleen size. Ten of 20 patients with gastroesophageal varices received primary prophylaxis and only one patient (10%) showed variceal recurrence. The cumulative overall survival rate was 100% at 1 year, 94.2% at 3-7 years and 68.7% at 10 years. The cumulative survival rates did not differ between the patients with and without gastroesophageal varices, with and without ascites, and patterns of portal cavernoma at baseline.


CONCLUSIONS
Forty-five percent of adult EHO patients in Japan were free from signs of portal hypertension, and platelet count and spleen size are predictive for identifying patients with gastroesophageal varices. EHO patients with gastroesophageal varices show favorable prognoses comparable to those without, if primary/secondary prophylaxis was performed appropriately.
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Tadashi
T
Sekimoto
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Hitoshi
H
Maruyama
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Kazufumi
K
Kobayashi
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Soichiro
S
Kiyono
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Takayuki
T
Kondo
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Taro
T
Shimada
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Masanori
M
Takahashi
Department of Gastroenterology and Nephrology, Chiba University Graduate School of Medicine, Chiba, Japan.
Osamu
O
Yokosuka
cumulative survival rate; extrahepatic portal vein obstruction; gastroesophageal varices; portal hypertension; spleen