title: Cystic fibrosis-associated bilateral pseudomucocele: case Report.
abstract: Cystic fibrosis, also known as mucoviscidosis, is a monogenetic disorder that is presented as a multisystemic disease. The incidence is approximately 1: 2500 live births. The pathophysiologic mechanism is a qualitative change in all exocrine secretions of the body. An increased viscosity of those secretions leads to stasis and mechanical obstruction, resulting in an impaired function of secretory and target organs. Nose and sinuses are involved due to abnormal mucociliary clearance, responsible for chronic rhinosinusitis, nasal polyps and sinus pseudomucocele.
  
  
  OBJECTIVE
  show a rare case of bilateral pseudomucocele in a child with cystic fibrosis.
  
  
  METHODS
  M.F.B.R., 2 years old, male, with nasal obstruction and recurrent pulmonary infections. Clinical findings were copious nasal secretion and posterior nasal drip. The CT scan of the paranasal sinuses showed an image that was suggestive of pseudomucocele, with opacification of maxillary and ethmoid sinuses. The sweat test presented meaningful results. We preferred surgical treatment, after patient hospitalization, to control the pulmonary manifestations. The child presented improvement of nasal obstructive symptoms.
  
  
  CONCLUSIONS
  Pseudomucocele is a disease that has been increasingly included in the routine of the differential diagnoses since CT scans became part of sinus disease semiology. Patients with pseudomucoceles have enjoyed relevant increases in their survival, thanks to current treatment modalities.
authors.affiliation: 2nd Year-Resident - Santa Marcelina Hospital.
authors.forename: Karla
authors.initials: K
authors.lastname: Palma Portes
authors.affiliation: PhD. Professor of Otolaryngology - FMUSP, Full Professor of Otolaryngology - PUC Campinas Medical School. Coordinator of the Medical Residency in Otolaryngology - Santa Marcelina Hospital.
authors.forename: Silvio Antonio
authors.initials: SA
authors.lastname: Monteiro Marone
authors.affiliation: PhD Student - Department of Otorhinolaryngology - Medical School - University of São Paulo (FMUSP), Professor of Otolaryngology - FMUSP, professor in charge of rhinology of the Residency Program in Otorhinolaryngology - Santa Marcelina Hospital.
authors.forename: Elder
authors.initials: E
authors.lastname: Yoshimitsu Goto
authors.affiliation: 3rd Year-Resident - Santa Marcelina Hospital.
authors.forename: Cleber
authors.initials: C
authors.lastname: Palma
authors.affiliation: 3rd Year-Resident in Otolaryngology.
authors.forename: Maria Renata
authors.initials: MR
authors.lastname: Macca Ferreira Jorge
authors.affiliation: MD. ENT - Hospital Santa Marcelina Hospital- SP.
authors.forename: Denilson Antonio
authors.initials: DA
authors.lastname: Cavazzani Szkudlarek
mesh_terms: D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D009078:Mucocele / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D015508:Nasal Obstruction / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D014057:Tomography, X-Ray Computed