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Int J Pediatr Otorhinolaryngol. 1993 Jan;25(1-3):243-8.

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

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  • 1Division of Pediatric Pathology, James Whitcomb Riley Hospital for Children, Indianapolis, IN 46202-5200.

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.

PMID:
8436471
[PubMed - indexed for MEDLINE]
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