Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children

Alina Grama; Alexandru Pîrvan; Claudia Sîrbe; Lucia Burac; Horia Ştefănescu; Otilia Fufezan; Mădălina Adriana Bordea; Tudor Lucian Pop

doi:10.3390/jcm10122703

Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children

J Clin Med. 2021 Jun 18;10(12):2703. doi: 10.3390/jcm10122703.

Authors

Alina Grama^{1

2}, Alexandru Pîrvan^{1

2}, Claudia Sîrbe¹, Lucia Burac², Horia Ştefănescu^{3

4}, Otilia Fufezan⁵, Mădălina Adriana Bordea⁶, Tudor Lucian Pop^{1

2}

Affiliations

¹ 2nd Pediatric Discipline, Department of Mother and Child, Iuliu Hațieganu University of Medicine and Pharmacy, 400112 Cluj-Napoca, Romania.
² Centre for Expertise in Pediatric Liver Rare Diseases, 2nd Pediatric Clinic, Emergency Clinical Hospital for Children, 400177 Cluj-Napoca, Romania.
³ Hepatology Department, Regional Institute of Gastroenterology and Hepatology, 400162 Cluj-Napoca, Romania.
⁴ Liver Research Club, 400162 Cluj-Napoca, Romania.
⁵ Department of Imaging, Emergency Clinical Hospital for Children, 400078 Cluj-Napoca, Romania.
⁶ Department of Microbiology, Iuliu Hatieganu University of Medicine and Pharmacy, 400151 Cluj-Napoca, Romania.

Abstract

One of the most important causes of portal hypertension among children is extrahepatic portal vein thrombosis (EHPVT). The most common risk factors for EHPVT are neonatal umbilical vein catheterization, transfusions, bacterial infections, dehydration, and thrombophilia. Our study aimed to describe the clinical manifestations, treatment, evolution, and risk factors of children with EHPVT.

Methods: We analyzed retrospectively all children admitted and followed in our hospital with EHPVT between January 2011-December 2020. The diagnosis was made by ultrasound or contrast magnetic resonance imaging. We evaluated the onset symptoms, complications, therapeutic methods, and risk factors.

Results: A total of 63 children, mean age 5.14 ± 4.90 (33 boys, 52.38%), were evaluated for EHPVT during the study period. The first symptoms were upper gastrointestinal bleeding (31 children, 49.21%) and splenomegaly (22 children, 34.92%). Thrombocytopenia was present in 44 children (69.84%). The most frequent risk factors were umbilical vein catheterization (46 children, 73.02%) and bacterial infections during the neonatal period (30 children, 47.62%). Protein C, protein S, antithrombin III levels were decreased in 44 of the 48 patients tested. In 42 of these cases, mutations for thrombophilia were tested, and 37 were positive. Upper digestive endoscopy was performed in all cases, revealing esophageal varices in 56 children (88.89%). All children with gastrointestinal bleeding received an octreotide infusion. In 26 children (41.27%), variceal ligation was performed, and in 5 children (7.94%), sclerotherapy. Porto-systemic shunt was performed in 11 children (17.46%), and Meso-Rex shunt was done in 4 children (6.35%). The evolution was favorable in 62 cases (98.41%). Only one child died secondary to severe sepsis.

Conclusions: EHPVT is frequently diagnosed in the last period in our region due to the increased use of umbilical vein catheterization. Furthermore, genetic predisposition, neonatal bacterial infections, and prematurity certainly play an important role in this condition. A proactive ultrasound assessment of children with risk factors for EHPVT should be encouraged for early diagnosis and treatment.

Keywords: children; portal vein thrombosis; splenomegaly; thrombophilia; umbilical vein catheter; upper gastrointestinal bleeding.