International Journal of Clinical Medicine, 2011, 2, 212-217
doi:10.4236/ijcm.2011.23035 Published Online July 2011 (http://www.SciRP.org/journal/ijcm)
Factors Associated to the Presence of
Pneumothorax in Cystic Fibrosis Patients in the
City of Madrid
Prados Concepción1*, Carpio Carlos2, Martínez María Teresa3, Máiz Luis4, Girón Rosa5, Barrio
Isabel6, Salcedo Antonio7, García Hernández Gloria8, Gómez Carrera Luis9, Álvarez-Sala Rodolfo10,
The Neumomadrid Cystic Fibrosis Work Group11
1Prados Sánchez, Concepción, Department of Pneumology, La Paz University Hospital, Madrid, Spain; 2Carpio Segura, Carlos Javier,
Department of Pneumonology, La Paz University Hospital, Madrid, Spain; 3Martínez Martínez, María Teresa, Department of Pneu-
monology, Doce de Octubre University Hospital, Madrid, Spain; 4Máiz Carro, Luis, Department of Pneumonology, Ramón y Cajal
University Hospital, Madrid, Spain; 5Girón Moreno, Rosa, Department of Pneumonology, La Princesa University Hospital, Madrid,
Spain; 6Barrio de Agüero, María Isabel, Department of Pediatric Pneumonology, La Paz University Hospital, Madrid, Spain; 7Salcedo
Posadas, Antonio, Department of Pediatric Pneumonology, Gregorio Marañón University Hospital, Madrid, Spain; 8García
Hernández, Gloria, Department of Pediatric Pneumonology, Doce de Octubre University Hospital, Madrid, Spain; 9Gómez Carrera,
Luis, Department of Pneumonology, La Paz University Hospital, Madrid, Spain; 10Álvarez-Sala Walther, Rodolfo, Department of
Pneumonology, La Paz University Hospital, Madrid, Spain; 11Carmen Antelo Landeira, Carmen Martínez Carrasco, Juan José
Cabanillas Martin, Jose Ramón Villa Asensi, Madrid, Spain.
{mmartinezm.hdoc, asalcedop.hgugm, ralvarezw.hulp}@salud.madrid.org
Received January 4th, 2011; revised March 16th, 2011; accepted April 1st, 2011.
ABSTRACT
Background: To identify risk factors associated with pneumothorax and to determine the prognosis of cystic fibrosis
patients following an episode of pneumothorax in the city of Madrid. Methods: Records of 17 patients (10 males; age
24.4 ± 17.5 years) and 32 controls, and a total of 44 pneumothorax episodes were studied. We have analyzed the char-
acteristics of the pneumothorax, the microbiology, the lung function tests (LFT) and the prognosis of patien ts. Two con-
trols with cystic fibrosis and without pneumothorax matched for sex and age were selected. Results: Eight male and
three female patients with pneumothorax were older than 18 years. The mean age of the first pneumothorax episode
was 18 .3 years (±9.6). The group with pneumothorax had a mean body mass index of 19.2(±2.42 kg/m2) and in the con-
trol group it was 26.5 (±1.98 kg/m2). Pseudomonas aeruginosa was present in fourteen patients (82%) with pneu-
mothorax and in eleven patients (34.4%) in the control group (p = 0.002). Pneumothorax predominantly occurred in
the coldest seasons. There was a significant drop in both forced vital capacity (FVC) and forced expiratory volume in
one second (FEV1) after the pneumothorax. In the same way, FEV1 and FVC were greater in the control group. Six pa-
tients (35.4%) with pneumothorax and two patients in the control group have died (p < 0.05). Conclusions: Patients
with pneumothorax are more likely to have P. aeruginosa colonization. LFT drop after an episode of pneumohorax.
Patients with pneumothorax have worse LFT than patients without pneumothorax. Mortality is greater in patients with
pneumothorax.
Keywords: Cystic Fibrosis, Pleural Disease, Pneumoth orax
1. Introduction
Cystic fibrosis (CF) is an autosomal recessive disease
caused by the presence of mutations in a single gene on
chromosome 7, which encodes the cystic fibrosis trans-
membrane conductance regulator (CFTR) protein [1].
CF originates different respiratory complications like
pneumothorax, hemoptysis and allergic bronchopulmo-
nary aspergillosis (ABPA) [2]. Pneumothorax is a fre-
quent and life-threatening complication in patients with
CF with an average annual incidence of 0.64% to 1%
[3,4]. Pneumothorax has been related to the presence of
increased transpulmonary air pressure difference and
lung hyperinflation due to chronic inflammation of the
airways [5]. This respiratory complication worsens the

Factors Associated to the Presence of Pneumothorax in Cystic Fibrosis Patients in the City of Madrid 213
prognosis of patients, since it increases their morbidity
and mortality [6].
The aim of the study was to analyze the risk factors
associated with the presence of pneumothorax in pa-
tients with CF in the city of Madrid, and to describe the
prognosis of patients following an episode of pneu-
mothorax.
2. Patients and Methods
2.1. Patients
The patients were recruited from the CF specialized
units from years 1989 to 2009. There were a total of 44
episodes of pneumothorax that experienced 17 of the
419 patients (4.1%) that are included in the CF patient
database in the Community of Madrid. Ten patients
were men (58.8%) and seven were women (41.2%), with
a mean age of 23.82 ± 13.5 years-old (range 8 - 72
years-old). The diagnosis of CF was established accord-
ing to the clinical findings, the sweat chloride test and
the genetic study [7].
Also, a control group of 32 CF patients without pneu-
mothorax was elected from the CF patient database. This
group was integrated by 16 men (50%) and 16 women
(50%) with a mean age of 20.43 ± 18.7 years-old (range
8 - 28 years-old).
2.2. Methods
The variables that were retrieved for the analyses in-
cluded: 1) demographic data: sex, age, date of birth and
date of CF diagnosis; 2) data related to the pneumotho-
rax: date of the first episode, number of recurrences,
characteristics of each episode (side, size, symptoma-
tology associated, manage); 3) microbiology: airways
culture before the pneumothorax; 4) lung function tests
(LFT) before and one year after the pneumothorax:
forced expiratory volume in one second (FEV1), forced
vital capacity (FVC); 5) body mass index (BMI); 6)
other respiratory complications (ABPA, hemoptysis);
and 7) outcomes (mortality, lung transplant). In the con-
trol group, the same variables were retrieved with the
exception of data related to pneumothorax; in these
cases, we have chosen the first ant the last spirometry
collected in the history of each patient. Confidentiality
was kept for the data retrieved according to the Law of
Data Protection.
2.3. Statistics
Statistical analyses were performed using SPSS version
(SPSS v 17, Inc. Chicago, IL, USA). The demographic
and clinical characteristics were expressed as mean ± SD
or as percentages. Comparisons between quantitative va-
riables were made using t-student or U-Mann Whitney
test according to their normal distribution. Chi-square
test was used to compare qualitative variables.
3. Results
Of the 419 patients registered in the CF patient database
in the city of Madrid, 17 (4.06%) had experienced, at
least, one episode of pneumothorax, collecting a total of
44 episodes, with an annual incidence of 0.5%. The
mean age in the group with pneumothorax was 23.82 ±
13.51 years-old and in the control group was 20.46 ±
4.85 years-old. Both groups did not have significant
differences comparing their mean age and proportion of
sexes. In the group with pneumothorax, the mean age
specified by sex was 23 ± 6.58 years-old (range 8 - 33
years-old) for men and 26.57 ± 20.39 years-old (range
16 - 72 years-old) for women (p > 0.05). The group with
pneumothorax was divided according to the age of the
first episode in two groups: <18 years and ≥18 years.
There were four episodes of pneumothorax (24%) in the
first group and 13 episodes (76%) in the second group.
The difference of both groups was significant (p = 0.04).
In the second group most patients were males (nine pa-
tients).
Nine patients had suffered only a single episode of
pneumothorax (52.9%). The mean age of the first epi-
sode was 18.29 ± 9.6 years-old (range 5 - 72 years-old).
There were recurrences in eight patients (47.1%) (Table
1).
All patients had pulmonary and gastrointestinal in-
volvement, with exception of one patient who was di-
agnosed of CF at the age of 72 years and who did not
have gastrointestinal involvement.
In 20 episodes of pneumothorax (45.5%) it was local-
ized in the right side and in 22 episodes (50%) it was
left-sided. Two episodes (4.5%) were bilateral, one of
which debuted with a cardiorespiratory arrest.
Regarding to the presentation, 43 (97.7%) episodes
were symptomatic (Table 2). Chest pain (79.4%) and
dyspnea (75%) were the most frequent symptoms for the
clinical debut of pneumothorax. There were three epi-
sodes of tension pneumothorax (two recorded in the
same patient), resulting one of them in a cardiopulmon-
ary arrest.
With respect to the season in which pneumothorax
occurred, eleven cases (78.57%) occurred in winter,
seven (50%) in spring, seven (50%) in autumn and five
Table 1. Distribution of pneumothorax episodes in patients
with cystic fibrosis.
Number of episodes Number of patients (%)
1 9 (52.9%)
2 3 (17.6%)
3 2 (11.8%)
7 2 (11.8%)
9 1 (5.8%)

Factors Associated to the Presence of Pneumothorax in Cystic Fibrosis Patients in the City of Madrid
214
Table 2. Symptoms of patients with pneumothorax.
Symptom Number of episodes (%)
Thoracic pain 35 (79.5%)
Dyspnea 33 (75%)
Hemoptysis 1 (2.3%)
Cyanosis 1 (2.3%)
Cardiorrespiratory arrest 1 (2.3%)
Asymptomatic 1 (2.3%)
Unknown 6 (13.8%)
(35.71%) in summer. The number of episodes occurred
in summer was less than in the other seasons (p < 0.05).
In the same way, it was observed a non-significant dif-
ference between the number of episodes in spring or
autumn comparing to them in winter, (p = 0.098). Also,
the total number of pneumothorax episodes occurred in
the most cold seasons or with greater climate variability
(winter, spring and autumn), was higher than the number
of episodes in summertime (p < 0.05).
Concerning microbiological cultures in the group with
pneumothorax, Pseudomonas aeruginosa colonized the
airways of 14 patients (82.4%). Other pathogens isolated
were Staphylococcus aureus in three cases (17.6%),
Haemophilus influenzae in two cases (11.8%), and both
Klebsiella ozonae and Burkholderia cepacea in one case
(5.8%) each one (Table 3). We found a significant dif-
ference comparing colonization by P. aeruginosa in the
group with pneumothorax with respect to the control
group (p = 0.002).
The nutritional status was established based upon the
BMI. The group with pneumothorax had a mean BMI of
19.2 ± 2.42 kg/m2; and in the control group it was 26.5 ±
1.98 kg/m2. Significant differences were found between
both groups (p = 0.01).
With regard to the LFT, FVC and FEV1 prior to the
episode of pneumothorax were 62.5% ± 17.8% (range
45% - 119%) and 43.4% ± 13.5% (range 20% - 83%),
respectively; and one year after the event, they were
45.2% ± 18.8% (range 22% - 88%) and 32.5% ± 15.4%
(range 18% - 71%), respectively. The difference in the
LFT before and after pneumothorax was significant in
both parameters (p = 0.008) (Figure 1). For the control
group, we analyzed the first and the last LFT we had in
the CF patient database. The initial FVC and FEV1 were
Table 3. Culture of the airway of CF patients with and
without pneumothorax.
Sputum culture Pneumothorax (%)
n = 17
Control (%)
n = 32
P. aeruginosa* 14 (82.4%) 11 (34.2%)
S. aureus 3 (17.6%) 13 (44.0%)
H. influenzae 2 (11.8%) 5 (15.6%)
K. ozonae 1 (5.9%) 1 (3.1%)
B. cepacea 1 (5.9%) 1 (3.1%)
*p < 0.01
Figure 1. Lung functional tests in pneumothorax and con-
trol group be fore and o ne year after pneumothor ax espisode.
FEV1 pre = forced expiratory volume in one second before
pneumothorax; FEV1 post = forced expiratory volume in one
second after pneumothorax; FVC pre= forced vital capacity
before pneumothorax; FVC post = forced vital capacity
after pneumothorax. *p < 0.05.
84.8% ± 18.5% (range 47% - 119%) and 82.53% ±
22.8% (range 25% - 119%) respectively; and the last
record for both parameters were 87.5% ± 21.51% (range
40% - 146%) and 78.4 ± 25.6 (range 26% - 136%) re-
spectively. The difference between the first and the last
LFT was not significant in both parameters. FVC and
FEV1 in the group with pneumothorax were lower than
in the control group, both before and after pneumothorax
(p < 0.05) (Figure 1).
ABPA was presented in six patients (53.4%) in the
group with pneumothorax and in four patients (12.5%)
in the control group. The difference was not significant.
Regarding treatment, eight (18.2%) episodes were re-
solved with conservative treatment, thirty-three (75%)
with chest tube drainage-aspiration, nine (20.5%) with
surgical pleurodesis, two (4.5%) with chemical pleuro-
desis, and one (2.3%) with suturing of bullaes (Table 4 ).
There were recurrences in eight patients (47.1%), and
one patient had nine recurrences.
At present, in the group with pneumothorax, three pa-
tients have needed lung transplantation (17.6%). Six
patients (35.4%) have died, but only two (4.5%) in direct
relation to the episode of pneumothorax. In the control
group, two patients have died (6.3%). The difference of
mortality between both group was statistically signify-
Table 4. Management of pneumothorax e pisode s.
Method of treatment Episodes number (%)
Observation 8 (18.2%)
Chest tube drainage 33 (75.0%)
Surgical pleurodesis 9 (20.5%)
Chemical pleurodesis 2 (4.5%)
Suturing of bullaes 1 (2.3%)

Factors Associated to the Presence of Pneumothorax in Cystic Fibrosis Patients in the City of Madrid 215
cant, presenting the group with pneumothorax a higher
mortality (p < 0.05). cant, presenting the group with
pneumothorax a higher mortality (p < 0.05).
4. Discussion
Survival in CF patients has increased in the recent years
due to the early diagnosis and the creation of multidisci-
plinary treatment units [8-10]. This increased survival
has been accompanied with the presence of a greater
number of age-related complications such as pneumo-
thorax. It is observed in our patients since most of them
showed the first episode of pneumothorax beyond 18
years of age, indicating that this respiratory complica-
tion is more prevalent in those who have a longer sur-
vival, as is shown in the literature [3,4,11,12]. Further-
more, in our study we have observed that patients who
had their first episode after the 18 years were predomi-
nantly men. This is explained because, as already men-
tioned in the literature, women have a tendency to lower
survival [13].
Similarly, almost 50% of patients with pneumothorax
experienced recurrences. It might had been because
treatments performed were less invasive and less aggre-
ssive than those used in pneumothorax caused by other
diseases, given that many patients may specify in the
future a lung transplant [14,15].
The most frequent symptoms of pneumothorax in our
patients were dyspnea and chest pain, which coincides
with that described in other series [3,4,16]. The number
of episodes in each hemithorax was almost the same.
Two patients had a bilateral pneumothorax, one of which
debuted with a cardiorespiratory arrest, representing
2.3% of all cases. This form of presentation and the rate
of occurrence are somewhat higher than that described
by Graf-Deuel et al. [15].
It has been discussed about the seasonal predomi-
nance in the appearance of pneumothorax, however in-
formation in patients with CF is scarce in this topic
[18,19]. We have found that this complication occurs
predominantly in colder seasons or in seasons showing
more climate variation as are fall, winter and spring time.
One possible explanation would be that there are more
atmospheric and barometric changes in these times of
the year, which may increase the frequency of respira-
tory exacerbations and the frequency of treatments with
physiotherapy or aerosols, which implies a change in
intra- and extra- thoracic pressures.
Those patients with CF and pneumothorax had more
frequent the presence of P. aeruginosa than other mi-
croorganisms in their airways. It is similar to results of
other studies that concluded that colonization by P.
aeruginosa doubles the likelihood of having a pneu-
mothorax and that is associated with a greater and more
rapid functional decline [3,4,15,20].
Regarding to the nutritional status, we have seen that
the mean BMI of patients with pneumothorax was below
the normal values. Some studies had postulated that the
nutritional status in these patients is directly related to
the respiratory prognosis [21,22]. However, medical li-
terature does not indicate any association between this
index and the occurrence of pneumothorax in patients
with CF. We believe that monitoring the nutritional
status of CF patients is important to avoid further com-
plications
The LFT were affected in the pneumothorax group.
The low mean FEV1 before the first episode of pneu-
mothorax and its decrease suggest that these patients
have a severe pulmonary involvement and that it wors-
ens after a pneumothorax episode. As others authors
have mentioned, a poor respiratory functional status
favors the presence of certain pulmonary complications
[12]. We have also found that this complication worsens
the respiratory disorder of these patients. We therefore
consider that pneumothorax clearly affects the morbidity
of CF patients, as the literature postulate [3,4,11,23].
Thirteen patients with pneumothorax (76.4%) had
gastrointestinal involvement. Six patients (35.2%) were
receiving treatment or had been treated for ABPA, and it
was not different from the control group. However, in
the work of Flume et al. it was concluded that ABPA
increases 1.5 the risk of having a pneumothorax [3].
Regarding treatment, most authors agree that observa-
tion alone is only effective for small pneumothorax,
where the risk of progression and of recurrence is low
[3,23-26]. In our series, in agreement with other series,
the most frequent method of therapy was chest tube
drainage, although this method seems to be subject of
recurrences [23,24,27,28]. This conservative tendency to
manage pneumothorax in CF patients is due to the diffi-
culty to perform a future lung transplant. For this reason,
the less intrusive methods are often used, even when
bullectomy or surgical pleural abrasion have demon-
strated that are the most effective methods and with less
recurrences [4,29]. At present, large pneumothorax
(>20% of the hemithorax) are treated initially with chest
tube drainage, and only those that are not resolved
within seven to 15 days or that recourse, shall be treated
with a more aggressive method such as the apical ble-
bectomy with thoracoscopy, the ablation of blebs with
laser and the apical thoracoscopic talc poudrage
[25,30,31]. Chemical pleurodesis is reserved for patients
with high surgical risk [23].
Regarding mortality, six patients (35.3%) died in the
group with pneumothorax, two directly related to pneu-
mothorax and the remaining four within the five years of
follow-up. In the control group, two patients (6.3%) died.

Factors Associated to the Presence of Pneumothorax in Cystic Fibrosis Patients in the City of Madrid
216
In relation to mortality, the differences were significant
between both groups, probably due to the worsening of
the respiratory function.
According to the Cystic Fibrosis Foundation Consen-
sus Conference report, patients with CF should avoid
maneuvers or situations which will create marked fluc-
tuations in intrapleural pressure to prevent pneumotho-
rax. These include intense isometric exercises. In the
same way, no air travel or LFT should be undertaken for
at least two weeks following resolution of a pneu-
mothorax [32-34].
In conclusion, the percentage of episodes of pneu-
mothorax observed in our patients is similar to that de-
scribed in the literature. Most of these episodes are
symptomatic. Low nutritional status, respiratory in-
volvement, P. aeruginosa positive-culture and the pres-
ence of cold seasons or with more variation in the cli-
mate are associated with the apparition of pneumothorax
in CF patients. The occurrence of pneumothorax in CF is
associated with worsening morbidity and mortality of
patients.
5. Acknowledgements
Role of sponsors: No commercial sponsor had any in-
volvement in the design and conduct of this study,
namely collection, management, analysis, and interpre-
tation of the data and preparation, decision to submit,
review, or approval of the manuscript.
Financial/nonfinancial disclosures: Non conflict of
interests indicated by the authors.
Other contributions: We want to thank to Mr.
Ramiro Lopetegui due to his careful lecture and correc-
tions of the manuscript.
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