Progression of noncirrhotic portal hypertension in a pediatric population

J Clin Exp Hepatol. 2011 Dec;1(3):169-76. doi: 10.1016/S0973-6883(11)60234-X. Epub 2012 Jan 2.


Background/objectives: The optimal management of children with noncirrhotic portal hypertension is controversial. Some groups suggest early and aggressive surgical intervention, while others report long-term success with conservative management.

Methods: We conducted a retrospective study of 26 patients with noncirrhotic portal hypertension treated at our institution. We compared platelet counts, white blood cell (WBC) counts, spleen size, hospital admissions, gastrointestinal bleeds, and longitudinal trends of specific clinical parameters using standard univariate and time-trend analytic techniques.

Results: Mean age at the time of diagnosis was 5.2 years. Portal vein thrombosis was found in 84.6% of patients (n=22). There was one mortality related to malignancy. There was not a progression of hypersplenism in patients that did not receive a shunt and conversely, we did not notice a significant decrease in spleen size following shunt surgery (P=0.2). Platelet and WBC counts trended downward among patients managed medically, while platelets increased and WBC counts remained stable in surgical patients. There was a significant decrease in hospital admissions for gastrointestinal bleeding following surgical intervention in the shunt group compared with nonshunt (P=0.0009).

Conclusion: While our analysis was limited given small sample sizes and selection bias, it suggests that the majority of pediatric patients with noncirrhotic portal hypertension will do well long-term without surgical intervention.

Keywords: CT, computed tomography; EHPVO, extrahepatic portal vein obstruction; GI, gastrointestinal; Hypersplenism; IVC, inferior vena cava K=1000; MRI, magnetic resanance imaging; PVT, portal vein thrombosis; TIPS, transjugular intrahepatic portosystemic shunt; WBC, white blood cell; leucopenia; pediatrics; portal hypertension; portal system; portal vein; portosystemic shunt; surgery; thrombocytopenia; thrombosis.