Head Injury in an Adult with Previously Undiagnosed Dandy-Walker Syndrome: A Review of the Condition and Discussion of its Anaesthetic Implications

The Dandy-Walker syndrome is a rare condition comprising dilatation of the fourth ventricle, large posterior fossa cyst and agenesis of the cerebellar vermis.The majority of cases present in the first few months of life, usually with obstructive hydrocephalus, and most require neurosurgical intervention in the form of a shunting procedure.This report describes an adult in whom the syndrome was diagnosed incidentally following severe head injury.Dandy-Walker syndrome and its anaesthetic implications are reviewed. CASE HISTORYA 39-year-old pedestrian was brought to the casualty department after being struck by a car.On arrival he had a Glasgow coma score (GCS) of 6, and a boggy swelling was noted over the left side of his scalp.Pupils were equal and reactive.A rigid neck collar was already in place and was not removed.He was breathing spontaneously, though irregularly.His other injuries included bilateral compound fractures of the tibia and fibula, a closed forearm fracture and four left-sided rib fractures.In view of his decreased level of consciousness it was decided to undertake intubation and controlled ventilation urgently, in order to maintain and protect the airway, ensure adequate oxygenation and prevent hypercapnia.It was noted that he had an unusually large head and a small jaw.Mouth-opening was restricted by the hard collar to two fingers' breadth, and inspection of the oropharynx revealed no softtissue trauma, swelling, blood or secretions.In the absence of appropriate X-rays, cervical spine fracture was assumed, and the collar was left in situ.Following preoxygenation, thiopentone 150 mg, fentanyl 100 Ilg and suxamethonium 100 mg were administered intravenously with cricoid pressure and in-line manual traction applied.Limited mouth-opening and the 'M.R.C.P., ER.