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Pulmonary arteriovenous malformations in children.

L S Fox, W L Buntain, D Brasfield

    Journal of Pediatric Surgery
    |February 1, 1979
    PubMed
    Summary

    Pulmonary arteriovenous malformations (PAVMs) are a treatable cause of persistent cyanosis in children. Surgical resection of PAVMs in pediatric patients, particularly those with Rendu-Osler-Weber syndrome, offers a curative outcome.

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    Area of Science:

    • Cardiology
    • Pediatric Surgery
    • Genetics

    Background:

    • Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular connections in the lungs.
    • PAVMs can lead to life-threatening hypoxemia, presenting as persistent cyanosis in children.
    • These malformations are often associated with hereditary hemorrhagic telangiectasia, also known as Rendu-Osler-Weber syndrome.

    Observation:

    • Three pediatric patients, all female and aged 2-4 years, presented with symptoms suggestive of PAVMs.
    • All patients had a family history of Rendu-Osler-Weber syndrome.
    • Pulmonary angiography confirmed the diagnosis of PAVMs in all three cases.

    Findings:

    • The PAVMs were located in various lobes, including the left lower lobe, right upper lobe, and right middle and lower lobes.

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  • All three patients underwent successful and uneventful surgical resection of the affected lung segments.
  • Post-operative recovery was uncomplicated for all patients.

    • Implications:

    • Surgical resection is an effective treatment for pediatric pulmonary arteriovenous malformations.
    • Early diagnosis and intervention can prevent severe complications associated with PAVMs.
    • Genetic counseling and screening for Rendu-Osler-Weber syndrome are important in affected families.