Researchers based in Romania characterized aspects of the disease course and risk factors involved with extrahepatic portal vein thrombosis (EHPVT) in pediatric patients, in a retrospective analysis recently published in the Journal of Clinical Medicine.

EHPVT, according to the study authors, is a disorder that may cause portal hypertension in children. EHPVT is rare, occurring in 1 of every 100,000 live births, the authors explained in their report.

The analysis included data for children with EHPVT who were treated at the Emergency Clinical Hospital for Children Cluj-Napoca in Transylvania, Romania, between January 2011 and December 2020. Multiple patient characteristics, treatment characteristics, and outcomes were evaluated to identify clinical characteristics and risk factors associated with EHPVT.

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The researchers identified 63 children in this analysis who had EHPVT. The mean patient age at diagnosis was 5.14 years, with the diagnostic age ranging from 6 months to 17 years and 10 months.

The majority of patients (69.84%) had thrombocytopenia, and 85.71% demonstrated splenomegaly during an initial physical examination. Most common initial symptoms included hematemesis and melena in 49.21% of patients and splenomegaly in 34.92%. Most patients (96.83%) had 1 or more known risk factors for EHPVT. The most common risk factors included umbilical vein catheterization in 73.02% and neonatal bacterial infections in 47.62% of patients.

A total of 48 pediatric patients had been evaluated for protein C, protein S, antithrombin III, and homocysteine concentrations and activity, with 91.67% showing reduced levels of protein C, protein S, and antithrombin III. A total of 42 patients were evaluated for thrombophilia-associated mutations, with 91.30% of these patients testing positive.

Most patients (88.89%) showed esophageal varices on initial endoscopy. Large varices were present in 44.44% of patients, while 30.16% had medium varices, and 14.29% had small varices. Gastric varices were present in 22.22% of patients.

Gastrointestinal bleeding was treated with octreotide infusion in all cases, with this therapy leading to cessation of bleeding in 65.90%. Slightly fewer than half of patients (41.27%) were treated with emergency endoscopic band ligation, with a small number of patients (7.94%) receiving sclerotherapy. For prophylaxis, 95.24% of patients were given b-adrenergic antagonists. Endoscopic variceal band ligation or sclerotherapy was used in 17.46% of patients. Surgical treatment was used with 23.81% of patients because of recurrent gastrointestinal bleeding or severe thrombocytopenia.

“There is a need for a management protocol and preventive approach for children with EHPVT to improve their outcomes,” the study authors concluded in their report.


Grama A, Pírvan A, Sîrbe C, et al. Extrahepatic portal vein thrombosis, an important cause of portal hypertension in children. J Clin Med. 2021;10(12):2703. doi:10.3390/jcm10122703