Variants of Möbius' Syndrome and Central Neurologic Impairment

Abstract

Möbius' syndrome is a complex neurologic disorder; its etiology and characterization have eluded clinicians for over a century. The pure Möbius' syndrome is rare, and the involvement of the sixth and seventh nerves has been found to have a host of associated defects. These include 1) limb and shoulder abnormalities; 2) multiple cranial nerve defects; 3) occasional mental retardation; 4) external, middle, and inner ear abnormalities; 5) laryngeal neurologic defects that cause stridor, obstruction, and aspiration; and 6) severe dysphagia and associated aspiration with life-threatening pneumonias. The children with severe neurologic dysfunction from central nuclear deficiencies were in many respects so similar in their clinical course that they are included in this paper. All of the patients required a tracheotomy to support the airway and to permit tracheobronchial toilet. A feeding gastrostomy was necessary in all but one patient. To counter life-threatening aspiration in the patients with multiple CNS defects, the Lindeman laryngeal diversion was considered the procedure of choice and proved very effective. The details and problems of this procedure are discussed. The aural defects and laryngeal findings in this series of patients are described in detail.