Laryngomalacia and intra-neural striated muscle in an infant with the Freeman-Sheldon syndrome

Carlos A. Galliani, Bruce H. Matt

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

Striated muscle cells within peripheral nerve trunks, a very rare dishistogenetic lesion, was found in the arytenoid submucosa of a ten and a half week old afroamerican male with the Freeman-Sheldon syndrome (craniocarpotarsal dysplasia) who presented with obstructive apnea. Laryngomalacia was confirmed endoscopically. Supraglottoplasty offered temporary relief of the obstruction. Our histopathological observation is puzzling and perhaps adds a new dimension to the appropriateness of 'dysplasia'. It remains to be seen whether the neuromuscular lesion is a coincidental finding, a mere curiosity or bears any clinical significance.

Original languageEnglish (US)
Pages (from-to)243-248
Number of pages6
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume25
Issue number1-3
DOIs
StatePublished - Jan 1993

Keywords

  • Freeman-Sheldon syndrome
  • Heterotopic striated muscle
  • Laryngomalacia
  • Peripheral nerve

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine
  • Surgery

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