PROLONGED DYSPHAGIA CAUSED BY CONGENITAL PHARYNGEAL DYSFUNCTION

We describe two patients with severe, isolated, congenital dysphagia c aused by paralysis of the pharyngeal muscles, who recovered at the age s of 40 months and 20 months, respectively, No other evidence of neuro logic or muscular dysfunction was present except for a transient paral ysis of the adductors of the vocal cords in one child, Radiocinematogr aphic studies showed paralysis of the pharyngeal stage of swallowing, with minimal involvement of the oral stage. One child refused oral fee ding for several months after apparent radiologic recovery, Two other patients with a similar disorder died of tracheal aspiration at the ag es of 8 months and 4 months, respectively, Autopsies showed no abnorma lity of the central nervous system, and the cranial nerves involved in swallowing were normal, Only five other well-studied cases of this sy ndrome have been reported, These observations demonstrate the existenc e of a type of severe, idiopathic, congenital dysphagia related to par alysis of the constrictor muscles of the pharynx, with a propensity to recover after several months or years if properly managed, The cause of the disorder is obscure, but it is probably related to a dysfunctio n of the central nervous system.