title: Laryngomalacia and intra-neural striated muscle in an infant with the Freeman-Sheldon syndrome.
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.
authors.affiliation: Division of Pediatric Pathology, James Whitcomb Riley Hospital for Children, Indianapolis, IN 46202-5200.
authors.forename: C A
authors.initials: CA
authors.lastname: Galliani
authors.forename: B H
authors.initials: BH
authors.lastname: Matt
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology*; D001049:Apnea / Q000209:etiology; D002828:Choristoma / Q000150:complications / Q000473:pathology*; D006801:Humans; D007223:Infant; D007818:Laryngeal Diseases / Q000473:pathology*; D008297:Male; D008865:Microstomia / Q000150:complications*; D009132:Muscles; D010524:Peripheral Nervous System Neoplasms / Q000150:complications / Q000473:pathology*; D013577:Syndrome