Nasal and sinus disease in cystic fibrosis

John M Robertson1, Ellen M Friedman, Bruce K Rubin

  • 1Department of Pediatrics, Texas Children's Hospital, Houston, Texas 77030, USA.

Insights

Cystic fibrosis (CF) patients frequently have paranasal sinus disease, often without symptoms. Early diagnosis and management of CF sinus disease may improve overall health and lung transplant outcomes.

Area of Science:

  • Otolaryngology
  • Pulmonology
  • Genetics

Background:

  • Paranasal and sinus disease is nearly universal in cystic fibrosis (CF) patients.
  • Symptoms are often absent, delaying diagnosis and treatment.
  • Disease characteristics like polyposis and microbial flora evolve with age.

Purpose of the Study:

  • To review the current understanding of paranasal sinus disease in CF.
  • To highlight diagnostic advancements and the spectrum of sinonasal abnormalities.
  • To discuss treatment options and their impact on overall health.

Main Methods:

  • Review of current literature on CF-related sinonasal disease.
  • Analysis of findings from endoscopic and CT imaging.
  • Evaluation of existing treatment data, including surgical outcomes.

Main Results:

  • Endoscopy and CT scans reveal a high prevalence of polyposis and specific anatomical abnormalities.
  • Age-related changes in sinonasal disease are observed.
  • Limited evidence exists for medical and surgical treatments, with no clear selection criteria for surgery.

Conclusions:

  • CF sinus disease is common and complex, with significant anatomical changes.
  • Sinus surgery may offer benefits, but further research is needed.
  • Effective management of sinonasal disease may positively impact lung function and survival post-transplant.

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