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.

show a rare case of bilateral pseudomucocele in a child with cystic fibrosis.

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.

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.