Different anaesthetic problems in Prader-Willi syndrome

Abstract

Aim of this work is to discuss retrospectively the anaesthesia procedures for two children (aged three and twelve), with Prader-Willi Syndrome (PWS). The older child had some problems specific of the second stage of PWS: obesity, with difficult venous access, and sleep apnoea, while the younger child presented difficult tracheal intubation that appears to be a common characteristic of both stages of PWS, a feature that is confirmed in literature by similar difficulties found by other Authors. We didn’t find any particular challenge when using any kind of anaesthetic drugs. Despite the small number of cases reported, we suggest that difficult tracheal intubation could be of clinical relevance in patients with PWS. Moreover, we conclude that all drugs used in anaesthesia practice could be considered safe. Caution should be used with long acting sedatives, potentially depressing ventilation. An epileptogenic agent, like ketamine, should be used also with caution, and it is definitely not recommended in patients with a history of seizures, like our two children.

Keywords

• General anaesthesia
• Prader-Willi syndrome
• difficult airway