Cystic fibrosis-associated bilateral pseudomucocele: case Report.
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.
Palma Portes
Karla
K
2nd Year-Resident - Santa Marcelina Hospital.
Monteiro Marone
Silvio Antonio
SA
PhD. Professor of Otolaryngology - FMUSP, Full Professor of Otolaryngology - PUC Campinas Medical School. Coordinator of the Medical Residency in Otolaryngology - Santa Marcelina Hospital.
Yoshimitsu Goto
Elder
E
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.
Palma
Cleber
C
3rd Year-Resident - Santa Marcelina Hospital.
Macca Ferreira Jorge
Maria Renata
MR
3rd Year-Resident in Otolaryngology.
Cavazzani Szkudlarek
Denilson Antonio
DA
MD. ENT - Hospital Santa Marcelina Hospital- SP.
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