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 month s of age presenting with BVCP between July 1987 and July 1999 at the Royal Children's Hospital, Melbourne, in whom a neuromuscular disorder was subseq uently diagnosed were followed. Three children in whom BVCP was diagnosed s oon after birth and before recognition of an underlying neuromuscular disor der were identified. All presented with upper airway obstructive symptoms a t birth, had a diagnosis of bilateral abductor vocal cord paralysis made at awake flexible laryngoscopy, and had no underlying structural laryngeal ab normality on microlaryngoscopy and bronchoscopy. Two children required a tr acheostomy, and I child was weaned from nasopharyngeal continuous positive airway pressure after 3 weeks. Subsequent neuromuscular symptoms were recog nized between 4 months and 7 years later, leading to diagnoses of facioscap ulohumeral myopathy, spinal muscular atrophy, and congenital myasthenia gra vis. In each case. the prognosis for recovery from symptoms related to BVCP reflected that of the underlying neuromuscular disorder. This experience s uggests that congenital BVCP may be a feature of an unrecognized neuromuscu lar condition. This possibility should be considered particularly in the pr esence of associated neurodevelopmental or neuromuscular dysfunction. or in cases in which BVCP is progressive.