Extrahepatic Portal Vein Obstruction in the Pediatric Age: A Medical Challenge

Khodayar-Pardo, Parisá

doi:10.23937/2469-584x/1510035

Cited by 6 publications

(8 citation statements)

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“…Administration of hypertonic substances or highly irritating drugs on these catheters can cause endothelial damage, inflammation, fibrosis, and thrombus formation, with blood flow obstruction and neovessels formation (cavernoma). In addition, its use for extended periods as well as the association of other general risk factors (procoagulant status) or local factors (dehydration, abdominal infections, or abdominal surgery) will further increase the risk of EHPVT especially in premature neonates [ 11 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

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

Grama

Pîrvan

Sîrbe

et al. 2021

JCM

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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.

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Section: Discussionmentioning

confidence: 99%

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

Grama

Pîrvan

Sîrbe

et al. 2021

JCM

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“…More than half of our patients (58.3%) received propranolol as primary or secondary prophylaxis, and a shunt operation was performed in 41.7% of the patients, which is not in conflict with the available treatment recommendations in the pediatric population [1, 3-5, 7, 8]. Although the use of beta-blockers for primary prophylaxis in children is controversial and the evidence regarding its administration for secondary prophylaxis is sparce, it is the first-choice medical treatment for EHPVT in the everyday practice [3,5,7,17]. We use it in a significant number of patients, and the response is favorable in most of the cases.…”

Section: Discussionmentioning

confidence: 99%

“…In part of the cases, the thrombosis is spontaneously completely reversible [ 1 , 15 , 16 ]. In the rest of them, it persists and despite the formation of a collateral network some of the patients develop a PH of varying grades within the time [ 1 , 3 , 7 ]. Most of the cases of PH associated with EHPVT in childhood are diagnosed several years after the initial thrombotic event, when the PH becomes symptomatic [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

“…Based on the literature, upper gastrointestinal bleeding (UGIB) is a common manifestation of PH associated with EHPVT [ 5 ]. Other identified clinical presentations are growth retardation, splenomegaly, hypersplenism, cholangiopathy, ascites, hepatopulmonary syndrome, and portopulmonary hypertension [ 3 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

“…The main identified local cause for EHPVT in children is neonatal umbilical vein catheterization (UVC), ranging from 20% in low-income countries to 60% in developing countries [ 5 ]. Other important local triggers are transfusion through the UVC, infections (omphalitis or pylephlebitis), abdominal trauma, abdominal surgery, and congenital malformations of the vascular system [ 1 , 3 , 4 , 5 , 7 ]. Among the systemic prothrombotic factors that predispose to venous thrombosis are thrombophilia, sepsis, and dehydration [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Extrahepatic Portal Vein Thrombosis in Childhood: Risk Factors, Clinical Manifestations, and Management

et al. 2022

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Objective: Extrahepatic portal vein thrombosis (EHPVT) is a common cause of portal hypertension in children. The aim of the present study was to identify the clinical manifestations and the existing risk factors for development of EHPVT in pediatric patients. Subjects and Methods: This was a single center retrospective cohort study. In all study participants we described the clinical presentations and the predisposing risk factors for development of EHPVT. In addition, as all of them had undergone an esophagogastroduodenoscopy for detection and grading of esophageal varices as part of the treatment algorithm, we analyzed the endoscopic findings and the therapeutic approach. Results: A total of 12 children (6 boys and 6 girls) took part in the study. The median age at diagnosis was 3.5 years (range: 1-17 years). The most frequent initial clinical manifestation was upper gastrointestinal bleeding (6 cases, 50.0%) followed by splenomegaly (3 cases, 25.0%). The most frequent systemic risk factor for EHPVT was presence of inherited prothrombotic disorder (10 cases, 83.3%) and the most common local risk factor for EHPVT was umbilical vein catheterization (5 cases, 41.7%). Esophageal varices were revealed in all the study participants and in the most cases they were grade ≥ 2. Propranolol was used as primary or secondary prophylaxis in 7 children (58.3%) and in 5 children (41.7%) a shunt was performed (Meso-Rex bypass in 3 children and splenorenal shunt in 2 children). Conclusion: Patients with known systemic or local risk factors for EHPVT are indicated for proactive ultrasound screening for early diagnosis and timely management.

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Pediatric living donor liver transplantation with non-anatomical portal vein reconstruction in idiopathic extrahepatic portal vein thrombosis

Rahayatri

Gunardi

Amin

et al. 2020

Journal of Pediatric Surgery Case Reports

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Extrahepatic Portal Vein Obstruction in the Pediatric Age: A Medical Challenge

Cited by 6 publications

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

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

Extrahepatic Portal Vein Thrombosis in Childhood: Risk Factors, Clinical Manifestations, and Management

Pediatric living donor liver transplantation with non-anatomical portal vein reconstruction in idiopathic extrahepatic portal vein thrombosis

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