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Portal Hypertension, Pediatric

Pamela L. Valentino, MD, MSc, FRCP(C) Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Definition of portal hypertension (P-HTN): elevation of portal pressure (PP) >5 mm Hg

    • Risk of developing complications occurs with “clinically significant P-HTN”: PP >10 mm Hg

    • Si...

DIAGNOSIS

HISTORY

  • History of neonatal umbilical catheterization

  • History of chronic hepatitis; underlying medical problem such as cystic fibrosis, tyrosinemia, glycogen storage disease, Wilson disease

  • His...

TREATMENT

GENERAL MEASURES

Management of EV: 
  • Risk of bleeding from EV is increased in setting of P-HTN and the presence of splenomegaly and thrombocytopenia. Surveillance endoscopy and primary prophyla...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Patients are followed closely for hepatic decompensation.

  • Growth failure, recurrent life-threatening bleeds not controllable with prophylactic intervention, refrac...

ADDITIONAL READING

  • Abd El-Hamid N, Taylor RM, Marinello D, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King’s College Hospital experience. J Pediatr Gastroente...

CODES

ICD9

  • 572.3 Portal hypertension

  • 572.2 Hepatic encephalopathy

  • 573.5 Hepatopulmonary syndrome

  • 456.8 Varices of other sites

  • 456.1 Esophageal varices without mention of bleeding

ICD10

  • K76.6 Portal hypertens...

FAQ

  • Q: What is my child’s long-term prognosis?

  • A: Disease prognosis of P-HTN depends on the underlying cause. Variceal bleeding associated with prehepatic causes of P-HTN such as PVT typically becomes ...

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