Emergency tracheostomy in a patient with Melnick-Needles Syndrome and sleep apnoea

Abstract

A six-year-old girl with a rare bone dysplasia (Melnick-Needles Syndrome) presented with a five month history of severe sleep apnoea, weight loss and failure of thrive. The syndrome is associated with craniofacial abnormalities, including micrognathia. Following a multi-disciplinary assessment an elective tracheostomy was considered the most appropriate treatment. The patient developed severe respiratory distress 10 days prior to the arranged date of surgery and required an emergency tracheostomy. This resulted in a dramatic return to health. The recognition of severe sleep apnoea in patients with craniofacial abnormalities and the role of initial tracheostomy are discussed.