The anaesthetic management of an infant with frontometaphyseal dysplasia (Gorlin–Cohen syndrome)

Abstract

We describe an infant with frontometaphyseal dysplasia, who presented to us twice for anaesthesia for orthopaedic surgery. These patients have facial asymmetry, mandibular hypoplasia, bradycardia, restrictive lung disease, primary pulmonary hypertension, skeletal abnormalities and difficult endotracheal intubation. The patient also showed laryngeal stridor because of laryngomalacia, vocal cord paralysis and subglottic stenosis. Light premedication along with atropine, ECG and blood pressure monitoring, gradual inhalational induction and intubation of the spontaneously breathing patient, careful positioning and postoperative CPAP are recommended.