ABSTRACT

Rubinstein-Taybi syndrome (RTS) is a rare, autosomal dominant syndrome presenting with mental retardation and physical abnormalities, including broad thumbs, big and broad toes, short stature and craniofacial anomalies. Special attention was paid to the possibilities of difficult airway, aspiration pneumonia and cardiovascular dysfunction during anaesthesia. Micrognathia, retrognathia, broad nasal bridge, abnormally large or ‘beak-shaped’ nose, hypoplastic maxilla and small mouth-typical dysmorphic facial features are one of the biggest causes of the difficult airway in this syndrome. Approximately one-third of the affected individuals have a variety of congenital heart diseases. Recurrent respiratory infections are likely to be the result of microaspiration or gastro-oesophageal reflux in this syndrome. In this case report, we discussed the anaesthesia management of a child with RTS who underwent right endoscopic dacryocystorhinostomy.

Keywords: Rubinstein-Taybi syndrome, airway management, general anaesthesia