LARYNGOMALACIA AND INTRA-NEURAL STRIATED-MUSCLE IN AN INFANT WITH THE FREEMAN-SHELDON SYNDROME

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.