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Thoracic CT findings in Birt-Hogg-Dube syndrome.
AJR Am J Roentgenol. 2011 Feb; 196(2):349-52.AA

Abstract

OBJECTIVE

Birt-Hogg-Dubé syndrome manifests in the thorax as lung cysts. The purpose of this article is to describe the CT characteristics of cysts in patients with Birt-Hogg-Dubé syndrome and to note other thoracic findings.

MATERIALS AND METHODS

The thoracic CT examinations of 17 patients with Birt-Hogg-Dubé syndrome were reviewed retrospectively for the presence, anatomic distribution (upper lung predominant, lower lung predominant, or diffuse), extent (size, number), and morphology (shape, wall thickness) of cysts. Any additional thoracic findings were also noted.

RESULTS

The study population consisted of 13 women (76%) and four men (24%) with a mean age of 50.2 ±15.2 years. Two patients (12%) had normal findings on CT. Fifteen patients had cystic lung disease, all of whom had more than one cyst. Most patients had bilateral (13/15, 87%) and lower lung-predominant cysts (13/15, 87%). The cysts varied in size from 0.2 to 7.8 cm. The largest cysts were located in the lower lobes of 14 of 15 patients (93%). Of the nine patients with large cysts, most had at least one multiseptated cyst (7/9, 78%). Five of 15 patients (33%) had more than 20 cysts. Cyst shape varied among the 15 patients and also within individual patients (10/15, 67%) ranging from round to oval, lentiform, and multiseptated. Cysts showed no central or peripheral predominance.

CONCLUSION

Discrete thin-walled cysts in patients with Birt-Hogg-Dubé syndrome are more numerous and larger in the lower lobes and vary in size and shape. Large lung cysts are frequently multiseptated. These features may aid in differentiating Birt-Hogg-Dubé syndrome from other more common cystic lung diseases.

Authors+Show Affiliations

Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan Health System, 1500 E Medical Center Dr., Ann Arbor, MI 48109, USA. prachia@med.umich.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

21257886

Citation

Agarwal, Prachi P., et al. "Thoracic CT Findings in Birt-Hogg-Dube Syndrome." AJR. American Journal of Roentgenology, vol. 196, no. 2, 2011, pp. 349-52.
Agarwal PP, Gross BH, Holloway BJ, et al. Thoracic CT findings in Birt-Hogg-Dube syndrome. AJR Am J Roentgenol. 2011;196(2):349-52.
Agarwal, P. P., Gross, B. H., Holloway, B. J., Seely, J., Stark, P., & Kazerooni, E. A. (2011). Thoracic CT findings in Birt-Hogg-Dube syndrome. AJR. American Journal of Roentgenology, 196(2), 349-52. https://doi.org/10.2214/AJR.10.4757
Agarwal PP, et al. Thoracic CT Findings in Birt-Hogg-Dube Syndrome. AJR Am J Roentgenol. 2011;196(2):349-52. PubMed PMID: 21257886.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Thoracic CT findings in Birt-Hogg-Dube syndrome. AU - Agarwal,Prachi P, AU - Gross,Barry H, AU - Holloway,Ben J, AU - Seely,Jean, AU - Stark,Paul, AU - Kazerooni,Ella A, PY - 2011/1/25/entrez PY - 2011/1/25/pubmed PY - 2011/3/4/medline SP - 349 EP - 52 JF - AJR. American journal of roentgenology JO - AJR Am J Roentgenol VL - 196 IS - 2 N2 - OBJECTIVE: Birt-Hogg-Dubé syndrome manifests in the thorax as lung cysts. The purpose of this article is to describe the CT characteristics of cysts in patients with Birt-Hogg-Dubé syndrome and to note other thoracic findings. MATERIALS AND METHODS: The thoracic CT examinations of 17 patients with Birt-Hogg-Dubé syndrome were reviewed retrospectively for the presence, anatomic distribution (upper lung predominant, lower lung predominant, or diffuse), extent (size, number), and morphology (shape, wall thickness) of cysts. Any additional thoracic findings were also noted. RESULTS: The study population consisted of 13 women (76%) and four men (24%) with a mean age of 50.2 ±15.2 years. Two patients (12%) had normal findings on CT. Fifteen patients had cystic lung disease, all of whom had more than one cyst. Most patients had bilateral (13/15, 87%) and lower lung-predominant cysts (13/15, 87%). The cysts varied in size from 0.2 to 7.8 cm. The largest cysts were located in the lower lobes of 14 of 15 patients (93%). Of the nine patients with large cysts, most had at least one multiseptated cyst (7/9, 78%). Five of 15 patients (33%) had more than 20 cysts. Cyst shape varied among the 15 patients and also within individual patients (10/15, 67%) ranging from round to oval, lentiform, and multiseptated. Cysts showed no central or peripheral predominance. CONCLUSION: Discrete thin-walled cysts in patients with Birt-Hogg-Dubé syndrome are more numerous and larger in the lower lobes and vary in size and shape. Large lung cysts are frequently multiseptated. These features may aid in differentiating Birt-Hogg-Dubé syndrome from other more common cystic lung diseases. SN - 1546-3141 UR - https://www.unboundmedicine.com/medline/citation/21257886/Thoracic_CT_findings_in_Birt_Hogg_Dube_syndrome_ L2 - https://www.ajronline.org/doi/10.2214/AJR.10.4757 DB - PRIME DP - Unbound Medicine ER -