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CC BY-NC-ND 4.0 · Int Arch Otorhinolaryngol 2023; 27(03): e423-e427
DOI: 10.1055/s-0042-1746193
Original Research

Outcomes of Endoscopic Sinus Surgery for Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia

Diogo Barreto Plantier 
1   Department of Otorhinolaryngology and Ophthalmology, School of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
,
Fábio de Rezende Pinna 
1   Department of Otorhinolaryngology and Ophthalmology, School of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
,
Mary Anne Kowal Olm 
2   Department of Pathology, School of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
,
Rodrigo Athanázio 
3   Pulmonary Division, Instituto do Coração, School of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
,
Renata Ribeiro de Mendonça Pilan 
1   Department of Otorhinolaryngology and Ophthalmology, School of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
,
Richard Louis Voegels 
1   Department of Otorhinolaryngology and Ophthalmology, School of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
› Author Affiliations Funding The author(s) did not receive financial support for the research. The author Diogo Barreto Plantier was a CNPQ doctoral scholarship holder.
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Abstract

Introduction Primary ciliary dyskinesia (PCD) is a rare inherited disease associated with impairment of mucociliary transport and, consequently, with a high incidence of chronic rhinosinusitis. For patients with chronic rhinosinusitis who remain symptomatic despite medical treatment, endoscopic sinus surgery is a safe and effective therapeutic option. However, to date, no studies have been found evaluating the effect of surgery on the quality of life associated with the effect on olfaction and nasal endoscopy findings of patients with primary ciliary dyskinesia and chronic rhinosinusitis.

Objective To describe the effect of endoscopic sinus surgery on the quality of life, on olfaction, and on nasal endoscopy findings of adults with PCD and chronic rhinosinusitis.

Methods Four patients who underwent endoscopic sinus surgery were included. The Sinonasal Outcome Test-22 (SNOT-22) score, the Nasal Obstruction Symptom Evaluation (NOSE) questionnaire, and the Lund-Kennedy score were collected preoperatively and at 3 and 6 months postoperatively. The olfaction as assessed with the University of Pennsylvania Smell Identification Test (UPSIT), which was administered preoperatively and 3 months postoperatively.

Results A total of 4 patients with a mean age of 39.3 years old (3 men and 1 woman) completed the study. All patients showed clinically significant improvement in the SNOT-22, NOSE, and Lund-Kennedy scores at 3 months postoperatively, and this improvement was sustained throughout the follow-up period. However, olfaction did not improve after surgery.

Conclusion The endoscopic sinus surgery treatment of chronic rhinosinusitis in adults with PCD was associated with improvement in quality of life and endoscopic findings. However, no improvement in olfaction was demonstrated. Studies with a larger number of patients and control groups should help confirm these findings.

Keywords

chronic rhinosinusitis - endoscopic sinus surgery - hyposmia - kartagener syndrome - paranasal sinuses - primary ciliary dyskinesia


Publication History

Received: 04 November 2021

Accepted: 01 March 2022

Article published online:
04 August 2023

© 2023. Fundação Otorrinolaringologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Horani A, Ferkol TW, Dutcher SK, Brody SL. Genetics and biology of primary ciliary dyskinesia. Paediatr Respir Rev 2016; 18: 18-24
    PubMedGoogle Scholar
  • 2 Goutaki M, Meier AB, Halbeisen FS. et al. Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. Eur Respir J 2016; 48 (04) 1081-1095
    CrossrefPubMedGoogle Scholar
  • 3 Behan L, Rubbo B, Lucas JS, Dunn Galvin A. The patient's experience of primary ciliary dyskinesia: a systematic review. Qual Life Res 2017; 26 (09) 2265-2285
    CrossrefPubMedGoogle Scholar
  • 4 Behan L, Leigh MW, Dell SD, Dunn Galvin A, Quittner AL, Lucas JS. Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD). Thorax 2017; 72 (09) 832-839
    CrossrefPubMedGoogle Scholar
  • 5 Goutaki M, Maurer E, Halbeisen FS. et al; PCD Italian Consortium, Swiss PCD Group, French Reference Centre for Rare Lung Diseases, Genetic Disorders of Mucociliary Clearance Consortium. The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results. Eur Respir J 2017; 49 (01) 1601181
    CrossrefPubMedGoogle Scholar
  • 6 Frija-Masson J, Bassinet L, Honoré I. et al. Clinical characteristics, functional respiratory decline and follow-up in adult patients with primary ciliary dyskinesia. Thorax 2017; 72 (02) 154-160
    CrossrefPubMedGoogle Scholar
  • 7 Bhatt JM, Muhonen EG, Meier M, Sagel SD, Chan KH. Rhinosinusitis in Pediatric Primary Ciliary Dyskinesia: Impact of Disease. Otolaryngol Head Neck Surg 2019; 161 (05) 877-880
    CrossrefPubMedGoogle Scholar
  • 8 Gliklich RE, Metson R. The health impact of chronic sinusitis in patients seeking otolaryngologic care. Otolaryngol Head Neck Surg 1995; 113 (01) 104-109
    CrossrefPubMedGoogle Scholar
  • 9 Bezerra TFP, Piccirillo JF, Fornazieri MA. et al. Assessment of quality of life after endoscopic sinus surgery for chronic rhinosinusitis. Braz J Otorhinolaryngol (Engl Ed) 2012; 78 (02) 96-102
    PubMedGoogle Scholar
  • 10 Shapiro AJ, Davis SD, Polineni D. et al; Diagnosis of Primary Ciliary Dyskinesia. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med 2018; 197 (12) e24-e39
    CrossrefPubMedGoogle Scholar
  • 11 Lucas JS, Barbato A, Collins SA. et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J 2017; 49 (01) 1-25
    CrossrefPubMedGoogle Scholar
  • 12 Fokkens WJ, Lund VJ, Mullol J. et al. EPOS 2012: European position paper on rhinosinusitis and nasal polyps 2012. A summary for otorhinolaryngologists. Rhinology 2012; 50 (01) 1-12
    CrossrefPubMedGoogle Scholar
  • 13 Orlandi RR, Kingdom TT, Hwang PH, Smith TL, Alt JA, Baroody FM. et al. International Consensus Statement on Allergy and Rhinology: Rhinosinusitis. Int Forum Allergy Rhinol 2016; Feb; 6 (Suppl. 01) S22-S209
    CrossrefPubMedGoogle Scholar
  • 14 Kosugi EM, Chen VG, Fonseca VM, Cursino MM, Mendes Neto JA, Gregório LC. Translation, cross-cultural adaptation and validation of SinoNasal Outcome Test (SNOT): 22 to Brazilian Portuguese. Braz J Otorhinolaryngol Sep-Oct 2011; 77 (05) 663-669
    CrossrefPubMedGoogle Scholar
  • 15 Bezerra TFP, Padua FGM, Pilan RRDM, Stewart MG, Voegels RL. Cross-cultural adaptation and validation of a quality of life questionnaire: the Nasal Obstruction Symptom Evaluation questionnaire. Rhinology 2011; 49 (02) 227-231
    CrossrefPubMedGoogle Scholar
  • 16 Fornazieri MA, dos Santos CA, Bezerra TFP, Pinna FdeR, Voegels RL, Doty RL. Development of normative data for the Brazilian adaptation of the University of Pennsylvania Smell Identification Test. Chem Senses 2015; 40 (02) 141-149
    CrossrefPubMedGoogle Scholar
  • 17 Parsons DS, Greene BA. A treatment for primary ciliary dyskinesia: efficacy of functional endoscopic sinus surgery. Laryngoscope 1993; 103 (11 Pt 1): 1269-1272
    CrossrefPubMedGoogle Scholar
  • 18 Tang X, Zou J, Liu S. Endoscopic sinus surgery for treatment of kartagener syndrome: a case report. Balkan Med J 2013; 30 (02) 244-247
    CrossrefPubMedGoogle Scholar
  • 19 Alanin MC, Aanaes K, Høiby N. et al. Sinus surgery can improve quality of life, lung infections, and lung function in patients with primary ciliary dyskinesia. Int Forum Allergy Rhinol 2017; 7 (03) 240-247
    CrossrefPubMedGoogle Scholar
  • 20 Pifferi M, Bush A, Rizzo M. et al. Olfactory dysfunction is worse in primary ciliary dyskinesia compared with other causes of chronic sinusitis in children. Thorax 2018; 73 (10) 980-982
    CrossrefPubMedGoogle Scholar
  • 21 Queiroz APL, Athanazio RA, Olm MAK. et al. Translation of the quality-of-life measure for adults with primary ciliary dyskinesia and its application in patients in Brazil. J Bras Pneumol 2019; 45 (03) e20170358
    CrossrefPubMedGoogle Scholar