Disorders of the Pleura |

Birt-Hogg-Dubé Syndrome and the Prevalence of Pneumothorax in Relation to Air Travel and Diving FREE TO VIEW

Paul Johannesma; Tijmen van der Wel; Marinus Paul, MD; Arjan Houweling, MD; Marianne Jonker; JanHein van Waesberghe, MD; Jeroen Van Moorselaar, MD; Fred Menko, MD; Pieter Postmus, MD
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VU University Medical Center, Amsterdam, Netherlands

Chest. 2014;146(4_MeetingAbstracts):448A. doi:10.1378/chest.1994673
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SESSION TITLE: Pleural Disease Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: The aim of this study was to evaluate whether air travel and diving in patients with BHD is associated with an increased prevalence of spontaneous pneumothorax (SP) and thoracic symptoms < 1 month.

METHODS: ​A survey was sent to a cohort of BHD patients with a proven pathogenic FLCN mutation (N=190).

RESULTS: In total 158 (83.2%) patients completed and returned the questionnaire. Of these 9.5% was an active smoker, 42.5% was a former smoker (mean: 19 pack years) and 7.6% had a history of inhaling drugs. Sixty-seven of 158 BHD patients (38%) had a history of SP, with a mean of 2.48 episodes (range 1-10), 35.8% (n=24) had a history of bilateral episodes. In total 145 BHD patients had “ever” traveled by airplane for a total of 1582 flights in Europe (mean 10.91 flights) and 946 intercontinental flights (mean 6.5 flights). Thirteen of 67 BHD patients (19.4%) developed SP <1 month after air traveling; 38.5% traveled intercontinental, one patient developed two episodes of SP <1 month after flying to China. For 5 patients this was the first episode of SP. Complaints reported in this group of 145 included shortness of breath (4.1%), chest pain (6.2%), palpitations (2.8%), anxiety (9.7%), abnormal fatigue (3.4%), nausea (4.1%), dizziness (0.7%), abnormal headache (3.4%), abnormal chills (1.4%) and lightheadedness (4.8%). These occurred in total in 30 patients (20.7%). In total 54 patients had “ever” dived, all for recreational purposes. Depth was categorized in 0-3 meters (87%), 3-10 meters (48.1%), >10 meters (14.8%). Two of 54 BHD patients (3.7%) developed a SP<1 month after diving, both at a depth between 3-10 meters. Complaints occurred in 10 patients (18.5%): shortness of breath (11.1%), anxiety (3.7%), dizziness (1.9%), abnormal fatigue (1.9%), abnormal chills (1.9%), and hemoptysis (1.9%).

CONCLUSIONS: As most BHD patients traveled without the occurrence of SP or complaints, travel or diving warning should not be given in advance to all of them, however, if ever diagnosed with SP it is dependent on the way this was treated whether there is a sufficient reduction of risk of recurrence. We state that this treatment should be pleurodesis of the complete thoracic cavity.(see other abstract Johannesma et al.)

CLINICAL IMPLICATIONS: Although the percentages of SP and complaints after air travel or diving are relatively high, it remains unclear whether there is a direct causal link. If thoracic complaints occur within one month after diving or air traveling, should undergo thoracic imaging to confirm or exclude a SP.

DISCLOSURE: The following authors have nothing to disclose: Paul Johannesma, Tijmen van der Wel, Marinus Paul, Arjan Houweling, Marianne Jonker, JanHein van Waesberghe, Jeroen Van Moorselaar, Fred Menko, Pieter Postmus

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