Airway management of a child with frontometaphyseal dysplasia (Gorlin Cohen syndrome).

[cohen syndrome]

Frontometaphyseal dysplasia (FMD ), also called Gorlin-Cohen syndrome , is a rare hereditary X-linked dominant craniotubular bone disorder . The presentation describes the airway management of a 2 -year -old child suffering from FMD with significant retrognathia , posted for major long bone corrective osteotomy . Induction with a combination of dexmedetomidine and ketamine preceded a successful endotracheal intubation under spontaneous ventilation .