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Extrahepatic Portal Vein Thrombosis in Childhood: Risk Factors, Clinical Manifestations, and Management
Ivan Yankov1, Rayna Shentova-Eneva2, Hristo Mumdzhiev3
1Department of Pediatrics, University Hospital "Saint George," Medical University of Plovdiv, Plovdiv, Bulgaria.
Insights
Extrahepatic portal vein thrombosis (EHPVT) in children is often linked to inherited clotting disorders and umbilical vein catheterization. Early ultrasound screening is recommended for at-risk pediatric patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Vascular Medicine
Background:
- Extrahepatic portal vein thrombosis (EHPVT) is a significant cause of portal hypertension in pediatric populations.
- Understanding EHPVT's clinical features and risk factors is crucial for effective management in children.
Purpose of the Study:
- To identify the clinical manifestations of EHPVT in pediatric patients.
- To determine the predisposing risk factors associated with the development of EHPVT in children.
Main Methods:
- A single-center retrospective cohort study involving 12 pediatric patients diagnosed with EHPVT.
- Analysis of clinical presentations, systemic and local risk factors, endoscopic findings of esophageal varices, and treatment approaches.
Main Results:
- The most common initial symptom was upper gastrointestinal bleeding (50%), followed by splenomegaly (25%).
- Key risk factors included inherited prothrombotic disorders (83.3%) and umbilical vein catheterization (41.7%).
- All patients had esophageal varices (grade ≥2 in most cases); treatments included propranolol and surgical shunts.
Conclusions:
- Proactive ultrasound screening is indicated for pediatric patients with known risk factors for EHPVT.
- Early diagnosis and timely management are essential for improving outcomes in children with EHPVT.
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 risk factors for development of EHPVT in pediatric patients.
Subjects And Methods:
This was a single-center retrospective cohort study. A total of 12 children (6 boys and 6 girls) took part in the study. We noted the clinical presentations and the predisposing risk factors for development of EHPVT in all patients. 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:
The median age of subjects 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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