Anaesthetic management of a child with fibrodysplasia ossificans progressiva.
Fibrodysplasia Ossificans Progressiva (FOP) is a rare inherited disease in which progressive ossification of striated muscles leads to severe disability and respiratory impairment early in life and there are associated characteristic congenital skeletal malformations. Although this condition may prove demanding for the anaesthetist, few reports are available regarding anaesthesia in paediatric patients. We review the clinical features of the disease relevant to anaesthesia and describe the perioperative care of an 18-month-old boy with FOP who underwent endoscopic third-ventriculostomy for hydrocephalus associated with a brainstem mass. We emphasize the importance of an appropriate anaesthetic management of these patients, as inadequate care may lead to exacerbation of the disease with permanent sequelae.
Tumolo
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Department of Anaesthesia and Intensive Care, Giannina Gaslini Children's Hospital Largo Gerolamo Gaslini 5, 16147 Genova Quarto, Italy. miriamtumolo@virgilio.it
Moscatelli
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A
Silvestri
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G
D000768:Anesthesia, General / Q000379:methods*; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery; D007223:Infant; D008297:Male; D009221:Myositis Ossificans / Q000150:complications*; D019990:Perioperative Care / Q000379:methods; D014696:Ventriculostomy / Q000379:methods