Anaesthetic management of a child with stone man syndrome: Look before you leap!

摘要

Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP. Videolaryngoscopes (VLs) provide a non-line-of-sight view and require less anterior force to visualise the glottis, may provide an alternative to fibreoptic intubation for airway management in such cases. Use of VL has only been reported once in an adult with FOP for nasotracheal intubation. We describe the successful anaesthetic management of an 11-year-old child with FOP and anticipated difficult airway.