ABSTRACT
Objectives: Chronic rhinosinusitis (CRS) is prevalent in the
Cystic Fibrosis (CF) population. CRS exacerbations in CF are thought to
contribute to pulmonary exacerbations. Literature regarding the impact
of endoscopic sinus surgery (ESS) is inconclusive. This study examines
rates of lung function decline and pulmonary exacerbation in CF patients
who have undergone ESS.
Design : Retrospective review of medical records.
Setting: Academic Hospital.
Participants: 40 adult CF patients.
Main outcome measures: Rate of lung function decline (Forced
Expiratory Volume1(FEV1) % predicted), pulmonary exacerbations (IV/oral
antibiotic therapy +/- hospital admission) and total days hospitalized 2
years post-operatively was collected. CRS patients undergoing ESS were
matched to those without ESS by gender, age, and F508del genotype.
Results: Forty patients (mean age 37.4, 60% male) were
reviewed. No significant difference was found between the surgical group
and controls in baseline FEV1(72.5% vs. 72.7%, p=0.98), 2-year
pre-operative pulmonary exacerbations (3.05 vs. 1.65, p=0.10), or
Lund-Mackay scores (12.25 vs. 11.55, p=0.71). No significant difference
was found in 1-year (70.5% vs. 72.8%, p=0.84) or 2-year (70.4% vs.
72.6% p=0.80) post-operative FEV1 and 2-year post-operative pulmonary
exacerbations (1.7 vs. 1.45, p=0.87). A significant increase was
identified in total number days hospitalized post-operatively (4.85,
p=0.02). In the surgical group, no significant difference was identified
between preoperative and postoperative FEV1, 1 -year (-2.51%, p=0.32)
and 2-years after ESS (-3.10%, p=0.51), postoperative pulmonary
exacerbations (-1.28, p=0.11), or in days hospitalized (3.74, p=0.14).
Conclusions: In this study, ESS does not appear to
significantly improve FEV1 or decrease the number of pulmonary
exacerbations post-operatively.
Keywords: Cystic Fibrosis, Sinus Surgery, Outcomes, Lung
Function, Sinusitis, Forced Expiratory Volume, Nasal polyposis
INTRODUCTION
Cystic fibrosis (CF) is a genetic disorder involving the upper and lower
airways and digestive system, affecting 30,000 children and adults in
the United States, 4,000 in Canada, and 70,000 worldwide [1,2]. CF
is an autosomal recessive disease based on a mutation in the CF
transmembrane conductance regulator (CFTR) gene. The CFTR gene is
encoded on the q31 region of the long arm of chromosome 7 [3]. The
CFTR protein regulates the components of sweat, digestive fluid and
mucus by controlling the transport of chloride ions across epithelial
membranes. In CF, lack of functional CFTR impairs chloride and
bicarbonate transport across the apical epithelium producing thickened
secretions, dysfunctional mucociliary clearance, and chronic airway
bacterial infection [4]. Imbalance of electrolyte transport from
CFTR dysfunction reduces airway surface liquid depth and increases the
viscosity of mucins in the airway 30–60 times higher than seen in
patients without CF [5]. The mucostasis results in progressive
cycles of infection and inflammation in the respiratory tract,
culminating in respiratory failure. Although advances in the clinical
care of the CF patient have increased the average lifespan, the most
common cause of mortality continues to be airway disease in the form of
progressive bronchiectasis and ultimately respiratory failure [6].
Sinonasal disease in the form of chronic rhinosinusitis, with or without
nasal polyposis, is prevalent in the CF population. Radiologic findings
of paranasal sinus opacification on computed tomography can be found in
almost 100% of patients with CF, with a reported incidence of nasal
polyposis between 33% and 57% [7-9]. Exacerbations of
rhinosinusitis in CF are thought to contribute to pulmonary
exacerbations that may suggest transmission of bacterial infection from
the upper to the lower airway. Support for this association includes
studies establishing the presence of similar bacteria in pulmonary and
sinonasal cultures [10-12]. Colonization of both the upper and lower
airways with Pseudomonas aeruginosa is a well-known phenomenon in
patients with cystic fibrosis (CF) [13]. These findings indicate the
sinuses act as a bacterial reservoir for transmitting disease to the
lower airways, making aggressive treatment and ongoing management of
sinonasal disease a priority for improving pulmonary outcomes [14].
Current literature regarding the effect of sinus surgery and management
of chronic rhinosinusitis on pulmonary and clinical outcomes in the
cystic fibrosis population is conflicting. This study will review a
10-year experience at ’[removed for blind peer review]’.comparing
outcomes in cystic fibrosis patients. Specifically, this study examines
the rates of lung function decline (i.e. FEV 1 % predicted) and
pulmonary exacerbation rates (i.e. need for IV
antibiotics/hospitalization) in CF patients who have undergone
endoscopic sinus surgery as compared to a group of CF patients with CT
or endoscopic findings of chronic rhinosinusitis but without endoscopic
sinus surgery.
MATERIALS AND METHODS