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Thoracic CT findings in Birt-Hogg-Dube syndrome

Prachi P Agarwal1, Barry H Gross, Ben J Holloway

  • 1Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan Health System, 1500 E Medical Center Dr., Ann Arbor, MI 48109, USA. prachia@med.umich.edu

AJR. American Journal of Roentgenology
|January 25, 2011
PubMed

Insights

Birt-Hogg-Dubé syndrome causes lung cysts that are typically numerous, larger, and lower lung predominant. These thin-walled cysts vary in shape and size, often being multiseptated, aiding in diagnosis.

Area of Science:

  • Pulmonology
  • Radiology
  • Genetics

Background:

  • Birt-Hogg-Dubé syndrome is a rare genetic disorder.
  • Thoracic manifestations, particularly lung cysts, are a key feature.
  • Accurate CT imaging characteristics are crucial for diagnosis.

Purpose of the Study:

  • To detail the computed tomography (CT) features of lung cysts in Birt-Hogg-Dubé syndrome.
  • To identify other associated thoracic findings in affected individuals.

Main Methods:

  • Retrospective review of thoracic CT scans from 17 patients with Birt-Hogg-Dubé syndrome.
  • Analysis of cyst presence, distribution, size, number, and morphology.
  • Documentation of any additional thoracic abnormalities.

Main Results:

  • Cystic lung disease was present in 15 of 17 patients.
  • Cysts were predominantly bilateral and located in the lower lung zones.
  • Cysts ranged from 0.2 to 7.8 cm, frequently multiseptated, and varied in shape.

Conclusions:

  • Birt-Hogg-Dubé syndrome is characterized by lower lobe predominant, thin-walled lung cysts of varying size and shape.
  • The frequent presence of multiseptated cysts is a notable finding.
  • These CT findings can help distinguish Birt-Hogg-Dubé syndrome from other cystic lung diseases.
Abstract

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