Paranasal mucoceles in children with cystic fibrosis: Management of a not so rare clinical condition

Am J Otolaryngol. 2021 Nov-Dec;42(6):103107. doi: 10.1016/j.amjoto.2021.103107. Epub 2021 Jun 16.

Abstract

Purpose: Paranasal mucocele (PM) is reported as a complication in children with cystic fibrosis (CF) in up to 4% of patients. The objective of this study was to identify PMs in the personal large series of children with CF and to assess their diagnosis and treatment.

Material and methods: Medical records of children with CF and PM who were admitted and treated by means of endoscopic nasal surgery between 2004 and 2020 were retrospectively reviewed.

Results: Thirty-four patients were included in the study (mean age 7.7 years). CT scan of sinuses showed a total of 53 PMs. Nasal endoscopic findings suggestive for PM were present in almost 80% of patients. PMs were located in the maxillary, ethmoid, and sphenoid sinuses in 29/34 (85.3%, bilateral in 17 cases), 4/34 (11.8%) and 1/34 (2.9%) patients, respectively. Marsupialization of PMs was performed in all patients using an endoscopic transnasal approach. No complications were observed. Resolution of symptoms and normalization of the endoscopic nasal picture was evident in all patients. After a mean follow-up of 85 months, no recurrences were observed.

Conclusions: To the best of our knowledge, this is the largest series of CF patients with PMs. Even if not frequently reported in the literature, PMs should not be considered an uncommon finding in patients affected by CF. Routine nasal endoscopy is mandatory to favor early diagnosis. Endoscopic transnasal marsupialization represents the gold standard of care for patients with CF and PM(s).

Keywords: Cystic fibrosis; Endoscopic endonasal surgery; Paranasal disease; Paranasal mucocele; Pediatric endoscopic sinus surgery.

MeSH terms

  • Adolescent
  • Age Factors
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Endoscopy / methods*
  • Female
  • Humans
  • Infant
  • Male
  • Mucocele / diagnosis
  • Mucocele / etiology
  • Mucocele / pathology
  • Mucocele / surgery*
  • Nasal Surgical Procedures / methods*
  • Paranasal Sinus Diseases / diagnosis
  • Paranasal Sinus Diseases / etiology
  • Paranasal Sinus Diseases / pathology
  • Paranasal Sinus Diseases / surgery*
  • Paranasal Sinuses / diagnostic imaging
  • Paranasal Sinuses / surgery
  • Rare Diseases
  • Retrospective Studies
  • Tomography, X-Ray Computed
  • Treatment Outcome