Ethmoidal mucocele causing proptosis in a pediatric patient with cystic fibrosis: A case report

Area of Science:

• Otolaryngology
• Ophthalmology
• Pediatrics
• Genetics

Background:

• Ear Nose Throat (ENT) pathology is frequently undiagnosed in cystic fibrosis (CF) patients.
• Undiagnosed ENT conditions in CF can lead to severe, life-threatening complications.
• Early detection and management of ENT issues are crucial for CF patient outcomes.

Observation:

• A 2-year-old cystic fibrosis patient presented with progressive left eye proptosis, limited adduction, and exotropia.
• Magnetic resonance imaging (MRI) revealed an ethmoidal mucocele compressing the medial rectus muscle and optic nerve.
• Ophthalmoscopy showed disc edema and diffuse vascular congestion, indicating increased intracranial pressure or optic nerve compromise.

Findings:

• Surgical drainage and removal of the ethmoidal mucocele were performed.
• Microbiology assessment identified Escherichia coli (E. coli) as the causative agent.
• Treatment with cefotaxime and dexamethasone resulted in complete resolution of ocular symptoms.

Implications:

• This case underscores the importance of considering ENT evaluations in pediatric CF patients, even without prior complications.
• Prompt surgical and medical intervention can effectively manage sight-threatening complications arising from sinus pathology in CF.
• Integrated care pathways for CF should include routine ENT and ophthalmological screening to prevent severe outcomes.