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Clinical and prothrombotic profile of hepatic vein outflow tract obstruction

Seema Alam1, Rajeev Khanna, Amar Mukund

  • 1Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi, 110070, India, seema_alam@hotmail.com.

Indian Journal of Pediatrics
|July 26, 2013
PubMed

Insights

Hepatic venous outflow tract obstruction (HVOTO) in children often presents with ascites and edema. Prothrombotic factors, particularly MTHFR mutations, are common causes, and treatments like angioplasty or TIPSS are effective.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Vascular Medicine

Background:

  • Hepatic venous outflow tract obstruction (HVOTO) is a critical condition in pediatric liver disease.
  • Understanding its clinical presentation and prothrombotic profile is essential for timely diagnosis and management.

Purpose of the Study:

  • To investigate the clinical features, prothrombotic causes, and outcomes of pediatric hepatic venous outflow tract obstruction (HVOTO).
  • To identify common genetic mutations and risk factors associated with HVOTO in children.

Main Methods:

  • Prospective cohort study of pediatric patients with acute or chronic liver disease.
  • Screening for HVOTO using ultrasound, Doppler imaging, and contrast-enhanced multidetector computed tomography (MDCT).
  • Genetic testing for prothrombotic mutations, including MTHFR and Factor V Leiden.

Main Results:

  • 13 out of 162 (7.4%) children with chronic liver disease were diagnosed with HVOTO.
  • Ascites and pedal edema were the most common clinical signs.
  • Prothrombotic causes were identified in 10 out of 13 cases (76.9%), with MTHFR gene mutations being the most frequent.
  • Interventions such as angioplasty and transjugular intrahepatic portosystemic shunt (TIPSS) led to asymptomatic outcomes with improved growth.

Conclusions:

  • Clinical signs like ascites, pedal edema, and hepatomegaly should prompt suspicion of HVOTO in pediatric liver disease.
  • A significant majority of HVOTO cases in children have an underlying prothrombotic cause, with MTHFR mutations being prevalent.
  • Angioplasty and TIPSS are effective treatment modalities for managing pediatric HVOTO, leading to favorable patient outcomes.
Abstract

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