Opitz (G) Syndrome

Opitz syndrome, or G syndrome, is a composite of congenital malformations including hypertelorism, hypospadias, and recurrent aspiration secondary to a disordered swallowing mechanism. The mode of inheritance is probably autosomal dominant with male predominance. This paper describes an infant with this syndrome who developed progressive airway obstruction requiring several endotracheal intubations. After extubation a laryngeal granuloma developed and required tracheostomy and excision. A hallmark of the patient's life has been persistent aspiration. No vocal cord paralysis or anatomic laryngeal anomaly was present. The aspiration was apparently due to neural incoordination of laryngeal and pharyngoesophageal motor activity. Because of the threat to life posed by aspiration, early recognition of this syndrome is imperative.