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Paranasal sinus mucocele in cystic fibrosis
1Department of Otolaryngology and Human Communication, Children's Hospital of Philadelphia, Pennsylvania 19104.
Insights
Pediatric paranasal sinus mucoceles are rare but may indicate cystic fibrosis. Early diagnosis through mucoceles and sweat testing is crucial for young patients with this condition.
Area of Science:
- Otolaryngology
- Pediatrics
- Genetics
Background:
- Chronic sinusitis is linked to nasal polyposis and cystic fibrosis.
- Paranasal sinus mucoceles are known complications in adults but rare in children.
Observation:
- A 3-year-old presented with epiphora and medial canthus fullness.
- CT scan revealed a right ethmoid mucocele, surgically drained with symptom resolution.
Findings:
- The case highlights the rarity of pediatric mucoceles, with only ~12 reported.
- Many pediatric mucocele cases, including this one, were linked to cystic fibrosis, some previously undiagnosed.
- Sweat testing confirmed cystic fibrosis in patients presenting with mucoceles; no cases with normal sweat tests were found.
Implications:
- Pediatric paranasal sinus mucoceles can be an early diagnostic sign of cystic fibrosis.
- This presentation underscores the importance of considering cystic fibrosis in children with mucoceles.
- Prompt diagnosis and management of mucoceles may lead to earlier identification of underlying cystic fibrosis.
Abstract:
The relationship between cystic fibrosis, nasal polyposis and chronic sinusitis is clearly defined. Mucoceles of the paranasal sinuses, however, are a well-described complication of chronic sinusitis in adults; but they rarely occur in the pediatric age group. We report a three-year-old who presented with epiphora and fullness at the right medial canthus. Computed tomography confirmed a right ethmoid mucocele which was surgically drained, with resolution of symptoms. Approximately 12 other cases of mucoceles have been reported in the pediatric age group. Although many of these patients had recognized cystic fibrosis, some were previously undiagnosed. Only after presentation with a mucocele, did sweat testing reveal their underlying pathology. No pediatric patients in the literature were found with mucoceles and normal sweat tests. Paranasal sinus mucoceles may be diagnostic of cystic fibrosis and may be the presenting sign in some patients.