Aj. Curran et al., EMERGENCY TRACHEOSTOMY IN A PATIENT WITH MELNICK-NEEDLES SYNDROME ANDSLEEP-APNEA, Journal of Laryngology and Otology, 107(7), 1993, pp. 647-648
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.