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Bifid epiglottis.

B Stroh1, F L Rimell, N Mendelson

  • 1Department of Pediatrics, University of Minnesota and Hennepin County Medical Center, Minneapolis 55455, USA.

International Journal of Pediatric Otorhinolaryngology
|April 17, 1999
PubMed
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Bifid epiglottis, a rare congenital defect, often links to limb anomalies and potentially lethal hypothalamic hamartomas. Early recognition is crucial for managing respiratory distress and associated conditions.

Area of Science:

  • Medical Genetics
  • Pediatric Surgery
  • Congenital Abnormalities

Background:

  • Bifid epiglottis is a rare congenital anomaly.
  • It is frequently associated with other congenital defects, notably limb anomalies (90%) and hypothalamic hamartomas/hypopituitarism (50%).
  • These associated anomalies, particularly hypothalamic hamartomas, can be life-threatening if not identified and treated.

Observation:

  • A 10-week-old infant presented with stridor and aspiration.
  • Laryngoscopy revealed a bifid epiglottis.
  • Further evaluation identified a hypothalamic hamartoma, indicative of Pallister-Hall syndrome.

Findings:

  • The case highlights the association between bifid epiglottis and Pallister-Hall syndrome.
  • Bifid epiglottis can cause severe respiratory distress due to laryngeal cartilage laxity and chronic aspiration.

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  • Surgical intervention may be necessary for managing respiratory complications.

    • Implications:

    • Emphasizes the importance of thorough evaluation for associated anomalies in infants with bifid epiglottis.
    • Early diagnosis of conditions like hypothalamic hamartoma and hypopituitarism is critical for timely management and improved outcomes.
    • This case contributes to the understanding of bifid epiglottis and its syndromic associations, guiding clinical practice.