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[Unilateral nasal obstruction in children: Pai syndrome]

Adrián Zanetta1, Giselle Cuestas, Maricruz Oviedo

  • 1Servicio de Endoscopia Respiratoria, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. adrianzanetta67@hotmail.com

Archivos Argentinos De Pediatria
|November 2, 2011
PubMed

Insights

Unilateral nasal obstruction in children, often from foreign bodies, can also stem from rare congenital masses. This case highlights a congenital nasal lipoma causing respiratory failure, successfully treated with surgery.

Area of Science:

  • Otolaryngology
  • Pediatric Surgery
  • Medical Genetics

Background:

  • Unilateral nasal obstruction in children typically results from foreign bodies, presenting with rhinorrhea and fetid odor.
  • Less common causes include trauma, neoplasms, congenital malformations, or iatrogenic factors.
  • Congenital intranasal masses can manifest at birth or be incidentally discovered during pediatric examinations.

Observation:

  • A rare case of unilateral nasal respiratory failure due to a congenital nasal lipoma was observed.
  • The patient also presented with craniofacial anomalies consistent with Pai syndrome, a genetic disorder.
  • Imaging studies were crucial for diagnosis and to exclude intracranial extension.

Findings:

  • Congenital nasal lipoma was identified as the cause of unilateral nasal obstruction and respiratory failure.
  • The patient's craniofacial anomalies were associated with Pai syndrome, characterized by congenital nasal polyps as a primary marker.
  • Surgical intervention successfully resolved the nasal obstruction.

Implications:

  • This case underscores the importance of considering congenital masses in pediatric unilateral nasal obstruction.
  • Early diagnosis and surgical management are crucial for resolving respiratory compromise caused by such lesions.
  • Recognition of associated craniofacial anomalies can aid in diagnosing rare genetic syndromes like Pai syndrome.

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