Rev Bras Otorrinolaringol

2008;74(1):16-20. ORIGINAL ARTICLE

Incidence and evolution of

nasal polyps in children and
adolescents with cystic fibrosis

Silke Anna Thereza Weber 1, Giesela Fleischer

Ferrari 2 Keywords: diagnosis, endoscopy, cystic fibrosis, polyposis,
therapy.

Summary

N asal polyps are a clinical sign of alert for investigating

Cystic Fibrosis (CF). Aims: To study the incidence of nasal
polyps in children and adolescents with cystic fibrosis, its
possible association with age, gender, clinical manifestations,
genotype and sweat chlorine level, and its evolution with
topical steroid therapy. Methods: Clinical symptoms, sweat
chlorine level and genotype were studied in 23 cystic fibrosis
patients. Nasal polyps were diagnosed by nasal endoscopy
and treated with topical steroids during 6 months, followed by
a second nasal endoscopy. Fisher test was used for statistical
analysis. Results: Nasal polyps were found in 39.1% of the
patients (five bilateral, four unilateral), all older than six years,
recurrent pneumonia in 82.6%, pancreatic insufficiency in 87%
and malnutrition in 74%. No association was seen between
nasal polyps and sweat chlorine level, genotype, clinical sings
of severity and nasal symptoms. Seven patients improved in
their nasal polyps with topical steroids, six showed complete
resolution. Conclusion: The study showed a high incidence
of nasal polyps in older children, who span the entire range
of clinical severity, even in the absence of clinical nasal
symptoms. Topical steroid therapy showed good results.
An interaction among pediatricians and otolaryngologists is
necessary for diagnosis and follow-up.

1
PhD. Professor of Otolaryngology - Medical School of Botucatu.
2
PhD. Professor of Pneumopediatrics - Medical School of Botucatu.
Medical School of Botucatu - UNESP Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço
Send correspondence to: Profa. Dra. Silke Anna Theresa Weber Faculdade de Medicina de Botucatu - UNESP Departamento de Oftalmologia, Otorrinolaringologia e
Cirurgia de Cabeça e Pescoço Distrito de Rubião Júnior s/n Botucatu SP 18618-970.
Tel/fax: (0xx14) 3811-6256 / 3811-6081 - E-mail: [email protected]
Paper submitted to the ABORL-CCF SGP (Management Publications System) on October 4th, 2006 and accepted for publication on February 10th, 2007. cod. 3434.

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 INTRODUCTION polyp incidence through endoscopy in children and ado-
lescents with cystic fibrosis being followed in the outpa-
Cystic fibrosis (CF) is the most common lethal ge- tient ward. The specific goals were:
netic disease in Caucasians, of autosomal, recessive trans- 1- to assess age, gender, clinical symptoms and the
mission, at a rate of 1:2000 live births in this population1,2, genetic mutation of these patients, and the association
in Brazil, the incidence is of 1:9500 in Paraná3, 1:8700 in between these data and nasal polyposis;
Santa Catarina4 and 1:10.0005 in Minas Gerais. 2- assess polyposis evolution with topical steroi-
The variable severity associated with the clinical ds.
manifestations (distinctive phenotypes) depends partially
on the genotype and results from the obstructive pheno- PATIENTS AND METHODS
mena, thus characterizing the cystic fibrosis:
1. Chronic suppurative obstructive pulmonary The present contemporary cross-sectional and pros-
disease; pective cohort was approved by the Ethics in Research
2. Pancreatic insufficiency with digestion problems Committee of the Botucatu Medical School - UNESP, under
and malabsorption, resulting in secondary malnutrition; protocol # 1743/2005. The parents/guardians signed an
3. Increased concentrations of chlorine and sodium, Informed Consent Form.
and age. In 2005 we assessed the 23 patients being followed
4. Adult male infertility. at the Cystic Fibrosis Reference Center Outpatient Ward,
Symptoms onset varies broadly, depending on mu- with ages ranging between 1 year and 9 months and 22
tation type, homozygote patients for the genetic mutation years and 8 months.
F508 start having symptoms in the first 2-4 months of From their charts, we obtained data related to gen-
life. The classic clinical picture starts with dry cough, ta- der, age, clinical manifestations of CF such as meconium
chypnea, mild intercostal pulling, or, it may manifest itself ileum, malnutrition, pancreatic insufficiency and repetition
as acute infection, like bronchiolitis. The clinical course pneumonia, and laboratorial exams to confirm CF, such as
evolves with recurrent pneumonia. Together with all of quantitative analysis of ion content in sweat7 and genetic
this, the patient has difficulty gaining weight, despite a vo- studies. All patients underwent otolaryngological evalua-
racious appetite, enlarged and more frequent foul-smelling tion and suffered nasal endoscopy. During the consultation
defecation, diarrhea or steatorrhea (oily feces) 1,2. we obtained information related to nasal obstruction, oral
Cystic fibrosis is diagnosed based on at least two breathing, asthma and sinusitis.
of the four clinical-laboratorial aspects: family history of Nasal endoscopy was carried out under topical
cystic fibrosis, pancreatic insufficiency, chronic suppu- anesthesia, using a flexible Storz pediatric bronchoscope
rative obstructive pulmonary disease and high levels of of 2.4mm in diameter, or a rigid 30º, 2.4mm Storz scope.
chlorine and sodium (>60mEq/l) in their sweat secretion. In the nasal exam we described whether or not
Other clinical data that suggest the diagnoses are: me- polyps were present, following the staging classification
conium ileum and/or intestinal obstruction with atresia, proposed by Johansson et al.15 (level 0 - absent, level
deficient weight-height development, heat stress, chronic I - polyp in the middle meatus, level II - polyp going
pansinusitis, nasal polyps, volvus and intusception, and through the middle turbinate with clear nasal floor, level
azoospermia6,7. III - polyp filling up the entire nasal cavity, whether or
Clinical manifestations in the upper airways (UAW) not there is secretion and its color, nasal mucosa aspect
happen to 100% of the patients, including recurrent sinu- (color, edema, degeneration).
sitis, rhinitis and/or nasal polyposis8-11. The incidence of Those patients with nasal polyposis were prospecti-
nasal polyps has been reported in 6 to 48% of the cases12,13, vely followed up and submitted to clinical treatment with
by the time cystic fibrosis is diagnosed, about 4% of the pa- topical steroids in the habitual dose (mometasone 200
tients have some symptoms associated with nasal polyps. mcg per day) for 6 months. After this period, the nasal
It is believed that about 14% of the patients with cystic endoscopic exam was repeated.
fibrosis will require surgery to treat the polyps8,10,11,14. For statistical analysis, the data obtained were des-
Based on these data from the literature, the depart- cribed in their mean and standard deviation values. Age,
ments of pediatric pneumology and otorhinolaryngology gender, clinical symptoms and genetic mutations were
of the Botucatu Medical School - UNESP, decided to assess associated with the presence of polyps. We used Fisher’s
UAW involvement in patients with cystic fibrosis in the Exact Test, at a significance level of p<0.05.
outpatient ward.
RESULTS
OBJECTIVE
The median age of the 23 patients was of six years
The general goal of our paper was to assess nasal and four months, and 20 of them were males.

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 Most of the patients presented with the classical cli-
nical manifestations, and 82.6% had repetition pneumonia,
87% had pancreatic insufficiency, 74% malnutrition and
13%, meconium ileum. CF was confirmed in all of them,
with too high chlorine levels in the sweat, the mean value
was of 92.093 ± 24.625 mEq/l. Genetic mutations were
investigated in all subjects. We found eight patients with
F 508/other genetic mutation, three F 508/F508, one
F508/G 542X, one G542X/other, one R1162X/R1162X
and in nine patients we did not observe mutations.
As to respiratory complaints, 35% of the patients
had asthma, 22% had frequent sinusitis - as diagnosed by
pediatric pneumologists and 22% were diagnosed with
oral breathing.
Nasal endoscopy revealed nasal polyps in 39.1%
(nine patients) of the patients. Of these, five patients had
bilateral polyposis and four had unilateral disease, level
Figure 2. Endoscopic image of a grade I nasal polyp in the right nasal
I in four, level II in one patient and Level III in four pa-
cavity of patient # 7. - P - polyp CM - middle turbinate
tients (Figures 1 and 2). Nasal polyposis was diagnosed
in children as of six years and three months of age, with
incidence increase as age increased (Graph 1). There was
no association between gender, age, clinical severity and
genetic mutation with nasal polyposis.

Graph 1. Distribution of cystic fibrosis patients in relation to age and

nasal polyps present.

level I polyps. One patient had his polyposis improve

from level III to level II. Two patients with bilateral level
III and another with unilateral level I did not show any
improvement. One patient had level I unilateral polyp
involution, however he later had a new level I polyp in
the contralateral side - according to the endoscopic exam
(Table I).

DISCUSSION

The patients assessed during diagnosis had the

classical cystic fibrosis clinical signs and symptoms, such
as meconium ileum, pancreatic insufficiency, malnutrition
Figure 1. Endoscopic image of a grade III polyp in the right nasal cavity
and repetition pneumonia. Diagnostic confirmation was
of patient #1. P - polyp S - nasal septum
carried out based on two abnormal levels of chlorine ion
in their sweat.
After six months of clinical treatment with topical Sinusal disease, diagnosed by CT scan, has been
steroids in the habitual dose, we observed that of the reported in CF in up to 100% of the cases9,10,11,13,14, although
nine children with polyps, seven (67.7%) improved. Six the percentage of adult patients with clinical manifesta-
had complete polyp remission. Of these, in regards to the tion is much lower7,8,13. It is believed that the thick mucus
classification, two had bilateral level III polyposis, one reduces ciliary movement, causes meatal obstruction and
had bilateral level II polyposis and three had unilateral both hypercapnia and hypoxia facilitate the development

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Table 1. Clinical manifestations in patients with cystic fibrosis and nasal polyps and its evolution with clinical treatment.

Oral Genetic Polyposis and Treatment

Patient Age(m) Asthma Sinusitis
Breathing mutation Before After
R1162X/ Bilateral Bilateral
01 103 No No No
R1162X Grade III Grade III
Right
02 102 Yes No No F508/? Absent
Grade I
Left Left
03 139 Yes No No ?/?
Grade I Grade I
Bilateral
04 75 No No No G542X/? Absent
Grade III
Bilateral
05 106 Yes Yes Yes ?/? Absent
Grade III
Bilateral Bilateral
06 139 No No No F508/? Grade III Grade II
Right Right
07 272 No No No ?/?
Grade I Grade I
Bilateral
08 179 Yes No No ?/? Absent
Grade III
Left
09 164 Yes No No ?/? Absent
Grade I

of local infection/inflammation. In the population studied survival prognosis.

we observed an incidence of sinusitis and oral breathing In 1992, Ramsey et al.11 had already seen small
in 22% of the patients, and such incidence rates are similar polyps during endoscopic exam, unseen by anterior rhi-
to the ones reported by other authors9,10,13,19; however only noscopy. Similar to the study carried out by Henriksson
one of these patients reported nasal polyps. Similar data et al.13, the polyps were classified as small in 68% of the
were published in 2002 by Henriksson et al.13 in a study patients; in 23% the polyps were in the meatus, being
involving 111 patients with cystic fibrosis with median age diagnosed only by means of nasal endoscopy. In our
of 18 years. During nasal endoscopic exam they observed study, four (45%) patients had small polyps, stressing the
nasal polyps in 39% of the patients; however they found importance of routine nasal endoscopy and clinician-oto-
no correlation between nasal obstruction, nasal secretion laryngologist interaction.
and polyps in these patients. It is believed that up to 20% of the patients with
In the literature, nasal polyposis has been reported nasal polyps require surgery8,9,11, and endoscopic surgery
at an incidence rate of 6 to 48% in patients with CF10,11,17, is the best approach8,20. According to Kingdom et al.14,
and a recent national study16 showed that children of a the  F 508 genetic mutation is the most relevant in pa-
median age of 9.5 years had an incidence rate of 15.2%. tients with CF and polyposis, requiring nasal surgery. In
In the present study, the incidence of nasal polyposis our study, we did not find any association between nasal
was similar to the one reported in the literature; however, polyp presence or severity and genotype.
consider the finding of 39.1% of children and adolescents Surgical recurrences are frequent, reported in up
with high levels of polyposis, because the aforementioned to 13% of the patients who undergo functional surgery.
studies included adults in their series. When patients below Thus, there are authors who suggest treatment with topi-
10 years of age were assessed, the incidence reported was cal steroids as a routine approach to treat nasal polyps,
between 5 and 15.2%16,18. Schmitt et al.18 in 2005, assessing resorting to surgery only in cases of failures21. In the group
893 children with CF, found nasal polyps in only 5% of studied, topical steroids showed improvement in seven
the children, and in 0.2% cystic fibrosis was diagnosed (77.7%) patients, of whom 85.7% had complete polyp
due to the presence of polyps. This is a very important involution. These results are better than the ones seen in
piece of data, because although the isolated polyp does the literature, showing polyposis improvement in 56% of
not always mean cystic fibrosis, the otolaryngologist must the cases8. For the specific population of patients with
investigate it because of its severity and importance in cystic fibrosis there is no data about clinical treatment
early diagnosis and treatment in order to provide a better development in longer periods.

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 CONCLUSIONS 8. Cepero R, Smith RJH, Cathin FI, Bressler KL, Furuta GT, Sandesa KC.
Cystic Fibrosis - an otolaryngologic perspective. Otolaryngol Head
In the population studied, the incidence of nasal Neck Surg 1987;97:356-60.
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