Use of intraoperative laryngeal electromyography to evaluate stridor in children with arthrogryposis

Arthrogryposis is a rare, congenital condition characterized by joint contractures of the extremities with muscle weakness and fibrosis. The otolaryngological manifestations of this disorder may include stridor, chronic aspiration, and Pierre Robin sequence, among others. Prior reports of vocal fold immobility associated with arthrogryposis have attributed it to recurrent laryngeal nerve paralysis, rather than to cricoarytenoid joint restriction. The objective of this study was to determine whether children with arthrogryposis and vocal fold immobility demonstrated laryngeal electromyography (L-EMG) findings consistent with recurrent laryngeal nerve paralysis or with cricoarytenoid joint restriction.

A retrospective, institutional chart review of children with otolaryngological manifestations of arthrogryposis was performed; 6 children were identified. Three patients had vocal fold immobility documented by flexible laryngoscopy. These 3 children were prospectively evaluated with direct laryngoscopy and intraoperative L-EMG.

The 3 children with arthrogryposis and vocal fold dysfunction had laryngoscopy-confirmed vocal fold immobility or significant restriction of motion. The intraoperative L-EMG tracings obtained from all 3 patients demonstrated motor unit action potentials without evidence of denervation.

This series, albeit small, suggests that the vocal fold dysfunction related to arthrogryposis may be attributable to cricoarytenoid joint restriction or poor laryngeal coordination, rather than to nerve paralysis, as originally postulated.