Neuromuscular Disorders Presenting as Congenital Bilateral Vocal Cord Paralysis

Congenital bilateral vocal cord paralysis (BVCP) can be associated with an underlying neuromuscular disorder, and may present before other features of the neuromuscular disorder become apparent. All infants less than 12 months of age presenting with BVCP between July 1987 and July 1999 at the Royal Children's Hospital, Melbourne, in whom a neuromuscular disorder was subsequently diagnosed were followed. Three children in whom BVCP was diagnosed soon after birth and before recognition of an underlying neuromuscular disorder were identified. All presented with upper airway obstructive symptoms at birth, had a diagnosis of bilateral abductor vocal cord paralysis made at awake flexible laryngoscopy, and had no underlying structural laryngeal abnormality on microlaryngoscopy and bronchoscopy. Two children required a tracheostomy, and 1 child was weaned from nasopharyngeal continuous positive airway pressure after 3 weeks. Subsequent neuromuscular symptoms were recognized between 4 months and 7 years later, leading to diagnoses of facioscapulohumeral myopathy, spinal muscular atrophy, and congenital myasthenia gravis. In each case, the prognosis for recovery from symptoms related to BVCP reflected that of the underlying neuromuscular disorder. This experience suggests that congenital BVCP may be a feature of an unrecognized neuromuscular condition. This possibility should be considered particularly in the presence of associated neurodevelopmental or neuromuscular dysfunction, or in cases in which BVCP is progressive.