EMERGENCY TRACHEOSTOMY IN A PATIENT WITH MELNICK-NEEDLES SYNDROME ANDSLEEP-APNEA

A six-year-old girl with a rare bone dysplasia (Melnick-Needles Syndro me) presented with a five month history of severe sleep apnoea, weight loss and failure of thrive. Tle syndrome is associated with craniofac ial abnormalities, including micrognathia. Following a multi-disciplin ary assessment an elective tracheostomy was considered the most approp riate treatment. The patient developed severe respiratory distress 10 days prior to the arranged date of surgery and required an emergency t racheostomy. This resulted in a dramatic return to health. The recogni tion of severe sleep apnoea in patients with craniofacial abnormalitie s and the role of initial tracheostomy are discussed.