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Hemangioma - A pointer to Abernethy syndrome?

Shyam S Kothari1

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Summary

Cutaneous hemangiomas may indicate congenital portosystemic shunts, such as Abernethy syndrome, even without apparent liver disease. This finding is crucial for diagnosing conditions like pulmonary arterial hypertension and arteriovenous malformations.

Keywords:
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Area of Science:

  • Vascular Medicine
  • Pediatric Gastroenterology
  • Dermatology

Background:

  • Congenital portosystemic shunts can lead to serious conditions like pulmonary arterial hypertension (PAH) and pulmonary arteriovenous malformations.
  • Abernethy syndrome is a specific type of congenital portosystemic shunt.
  • Cutaneous hemangiomas have not previously been recognized as a sign of these shunts.

Purpose of the Study:

  • To describe the association between cutaneous hemangioma and Abernethy syndrome.
  • To highlight hemangioma as a potential clinical indicator for congenital portosystemic shunts.

Main Methods:

  • Case report of two children with Abernethy syndrome.
  • Clinical presentation of severe cyanosis (pulmonary arteriovenous malformations) and severe PAH.

Main Results:

  • Both children presented with cutaneous hemangiomas.
  • One child had severe cyanosis due to pulmonary arteriovenous malformations; the other had severe PAH.
  • These findings suggest a link between hemangioma and congenital portosystemic shunts.

Conclusions:

  • Cutaneous hemangioma may serve as a clinical sign pointing towards congenital portosystemic shunts.
  • This association can be present even when overt liver disease is absent.
  • Early recognition of hemangioma could aid in diagnosing Abernethy syndrome and associated cardiopulmonary complications.