A Rare Case of Extrahepatic Portal Venous Obstruction in a Nine-Year-Old Female and Its Management: A Case Report

Extrahepatic portal vein obstruction (EHPVO) is a kind of liver vascular disease that causes structural abnormalities in the portal veins, including cavernomatous metamorphosis and obstruction. It is the most common cause of esophageal varices-related hematemesis in youngsters. Significant risk fact...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2022-12, Vol.14 (12), p.e32150
Main Authors: Udo, Uduak A, Garg, Tulika, Omar, Zainab Talal O, Mungu, Etaluka Blanche, Aiyadurai, Sanathan, Una, Idoroeyin S, Sunday, Goodness C, Ajekigbe, Omolola, Chaudhry, Hassan A, Khan, Aadil
Format: Article
Language:English
Subjects:
Abdomen
Anticoagulants
Asymptomatic
Beta blockers
Biopsy
Case reports
Children & youth
Disease prevention
Endoscopy
Esophagus
Gastroenterology
General Surgery
Heart rate
Hematemesis
Hypertension
Liver cirrhosis
Liver diseases
Lymphatic system
Patients
Pediatrics
Surgery
Surgical outcomes
Ultrasonic imaging
Veins & arteries
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Summary:Extrahepatic portal vein obstruction (EHPVO) is a kind of liver vascular disease that causes structural abnormalities in the portal veins, including cavernomatous metamorphosis and obstruction. It is the most common cause of esophageal varices-related hematemesis in youngsters. Significant risk factors include congenital abnormalities, dehydration, sepsis, trauma, hypercoagulable conditions, and multiple transfusions. Acute extrahepatic portal vein blockage is often ignored because patients are usually asymptomatic. Subacute and chronic stages can cause symptoms including splenomegaly and hematemesis without hepatic decompensation. Imaging studies aid in the diagnosis; Doppler imaging is added to ultrasonography to visualize portal vein blood flow. MRI and CT scans are used to visualize portal vein blockage. Prevention of acute bleeding is the cornerstone in the management. Studies have shown that transhepatic thrombolysis is the preferred choice to avoid systemic side effects. Transjugular intrahepatic portosystemic shunt (TIPS) treats extrahepatic portal venous thrombosis and is typically followed by conservative variceal hemorrhage treatment. Liver transplantation is performed when other management measures fail. Here, we present a rare case of EHPVO in a nine-year-old female who was lost to follow-up for a long time and later showed signs of portal biliopathy and non-visualization of a surgically created splenorenal shunt. Re-shunting was performed after detailed conservative management, and the patient responded well to the treatment given.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.32150