Prevalence of portal vein thrombosis in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension.

INTRODUCTION

Portal vein thrombosis (PVT) has been increasingly diagnosed in pediatric patients owing to the widespread use of non-invasive radiological techniques. Although the prevalence of PVT in adults with cirrhosis and intrahepatic non-cirrhotic portal hypertension ranges from 0.6 to 26 % and 13 to 46 %, respectively, no available data exist in the pediatric population. The prevalence of PVT in children with cirrhotic and intrahepatic non-cirrhotic portal hypertension was evaluated in this study.

METHODS

This retrospective study included children with cirrhosis and intrahepatic non-cirrhotic portal hypertension, which consisted of congenital hepatic fibrosis (CHF) and idiopathic noncirrhotic portal hypertension (INCPH). Patients with extrahepatic portal venous obstruction were excluded from the study. The presence of PVT was evaluated using abdominal Doppler ultrasonography and/or CT. Etiological, clinical, and laboratory findings were compared between the groups.

RESULTS

One hundred and forty-two patients with cirrhosis (mean admission age: 64.6 months ± 66.4, mean follow-up duration: 46.8 months ± 45.6) and 41 patients with non-cirrhotic patients (CHF=16, INCPH = 25, mean admission age: 126 months ± 64.2) were enrolled in this study. The prevalence of PVT was not significantly different between cirrhotic (8.5 %) and non-cirrhotic (9.7 %) patients. The incidence of PVT was significantly higher in patients with biliary atresia than in those with other etiologies in the cirrhotic group (p = 0.022). The frequency of PVT was higher in patients who had Child-Pugh score ≥7 in the cirrhotic group, but the difference was not statistically significant (p = 0066). The PVT group required more liver transplantations than the non-PVT group (p = 0.038).

CONCLUSION

The prevalence of PVT was similar in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension in our cohort, which is compatible with adult studies. Biliary atresia is found to be an important risk factor for PVT in our pediatric population. It might be associated with rapid progression of the disease, ascending cholangitis, and embryological abnormalities. These patients should be routinely evaluated to identify portal vein complications and early warning signs during follow-up.