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Rendu-Osler-Weber syndrome presenting with pulmonary arteriovenous fistula

A M Halefoglu1

  • 1Department of Radiology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey. halefoglu@hotmail.com

Australasian Radiology
|June 4, 2005
PubMed

Insights

Pulmonary arteriovenous fistulas are abnormal connections in the lungs. This case highlights Rendu-Osler-Weber syndrome as a potential cause, especially with a family history.

Area of Science:

  • Vascular Medicine
  • Genetics
  • Pulmonology

Background:

  • Pulmonary arteriovenous fistulas (PAVF) are abnormal vascular connections between pulmonary arteries and veins.
  • Rendu-Osler-Weber syndrome (Hereditary Hemorrhagic Telangiectasia) is an autosomal dominant genetic disorder associated with vascular malformations, including PAVF.

Observation:

  • A 49-year-old male presented with dyspnea, cough, hemoptysis, and epistaxis.
  • Physical examination revealed nasal telangiectasias, cyanosis, and a left-sided systolic bruit.
  • Imaging confirmed a 5-cm pulmonary arteriovenous fistula in the left lower lobe.

Findings:

  • The patient's symptoms and physical findings were consistent with a PAVF.
  • A family history of a niece with a suspected PAVF suggested a possible genetic link.
  • The diagnosis of Rendu-Osler-Weber syndrome was considered due to the presence of PAVF and suggestive family history.

Implications:

  • This case underscores the importance of considering Rendu-Osler-Weber syndrome in patients with PAVF, particularly those with a family history.
  • Early diagnosis and management of PAVF in Rendu-Osler-Weber syndrome are crucial to prevent complications.
  • Further research into the genetic basis and clinical manifestations of PAVF in Rendu-Osler-Weber syndrome is warranted.

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