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Cavernous Transformation and Portal Vein Obstruction

Cavernous Transformation and Portal Vein Obstruction is a topic covered in the Select 5-Minute Pediatrics Topics.

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Basics

Description

  • Cavernous transformation: the collection of collaterals that develop around an obstructed vessel
  • Portal vein obstruction
    • Can occur anywhere along the course of the main portal vein or splenic vein, between the hilum of the spleen and the porta hepatis
    • In pediatrics, obstruction is most typically of the portal vein.
  • Major cause of prehepatic portal hypertension

Epidemiology

  • Most children with portal vein thrombosis present between birth and 15 years of age.
  • Acute presentation is rare.
  • Chronic cases present with complications of portal hypertension.
  • Gastrointestinal (GI) bleeding is more typical in patients presenting <7 years of age.
  • Splenomegaly in the absence of symptoms is more typical for patients aged 5–15 years.

Risk Factors

Genetics

A genetic basis of this problem has not been identified, although congenital abnormalities of the heart, major blood vessels, biliary tree, and renal system are often found.

Pathophysiology

  • In cirrhosis and hepatic malignancies, the thrombus usually begins intrahepatically and spreads to the extrahepatic portal vein.
  • In most other etiologies, the thrombus usually starts at the site of origin of the portal vein.
  • Occasionally, thrombosis of the splenic vein propagates to the portal vein, most often resulting from an adjacent inflammatory process (e.g., severe pancreatitis).
  • Asymptomatic splenomegaly or upper GI hemorrhage results from extrahepatic portal hypertension.
  • Less commonly, ascites or failure to thrive, and portopulmonary hypertension

Etiology

50% of portal vein obstructions are idiopathic. Identified causes include the following:

  • Congenital vascular anomaly
    • Portal vein malformation
    • Webs or diaphragms within the portal vein
  • Clot resulting from a hypercoagulable state
  • Clot from other causes:
    • Omphalitis
    • Umbilical vein catheterization
    • Portal pyelophlebitis
    • Intra-abdominal sepsis
    • Surgery near the porta hepatis
    • Sepsis
    • Cholangitis
    • Dehydration
    • Trauma
  • Other causes for portal vein obstruction in older children:
    • Ascending pyelophlebitis from perforated appendicitis
    • Primary peritonitis, cholangitis, and pancreatitis causing a splenic vein thrombosis
    • Inflammatory bowel disease

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Citation

Cabana, Michael D., editor. "Cavernous Transformation and Portal Vein Obstruction." Select 5-Minute Pediatrics Topics, 7th ed., Wolters Kluwer Health, 2015. Medicine Central, im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14148/all/Cavernous_Transformation_and_Portal_Vein_Obstruction.
Cavernous Transformation and Portal Vein Obstruction. In: Cabana MD, ed. Select 5-Minute Pediatrics Topics. 7th ed. Wolters Kluwer Health; 2015. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14148/all/Cavernous_Transformation_and_Portal_Vein_Obstruction. Accessed April 21, 2019.
Cavernous Transformation and Portal Vein Obstruction. (2015). In Cabana, M. D. (Ed.), Select 5-Minute Pediatrics Topics. Available from https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14148/all/Cavernous_Transformation_and_Portal_Vein_Obstruction
Cavernous Transformation and Portal Vein Obstruction [Internet]. In: Cabana MD, editors. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. [cited 2019 April 21]. Available from: https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14148/all/Cavernous_Transformation_and_Portal_Vein_Obstruction.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Cavernous Transformation and Portal Vein Obstruction ID - 14148 ED - Cabana,Michael D, BT - Select 5-Minute Pediatrics Topics UR - https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14148/all/Cavernous_Transformation_and_Portal_Vein_Obstruction PB - Wolters Kluwer Health ET - 7 DB - Medicine Central DP - Unbound Medicine ER -