Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 701-703 |
| Number of pages | 3 |
| Journal | British Journal of Anaesthesia |
| Volume | 97 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - Nov 2006 |
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Keywords
- Anaesthesia, paediatric
- Complications, intubation tracheal
- Equipment, bronchoscope
- Fibrodysplasia ossificans progressiva
ASJC Scopus subject areas
- Anesthesiology and Pain Medicine
Cite this
Anaesthetic management of a child with fibrodysplasia ossificans progressiva. / Tumolo, Miriam; Moscatelli, A.; Silvestri, G.
In: British Journal of Anaesthesia, Vol. 97, No. 5, 11.2006, p. 701-703.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Anaesthetic management of a child with fibrodysplasia ossificans progressiva
AU - Tumolo, Miriam
AU - Moscatelli, A.
AU - Silvestri, G.
PY - 2006/11
Y1 - 2006/11
N2 - 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.
AB - 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.
KW - Anaesthesia, paediatric
KW - Complications, intubation tracheal
KW - Equipment, bronchoscope
KW - Fibrodysplasia ossificans progressiva
UR - http://www.scopus.com/inward/record.url?scp=33750138930&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33750138930&partnerID=8YFLogxK
U2 - 10.1093/bja/ael251
DO - 10.1093/bja/ael251
M3 - Article
VL - 97
SP - 701
EP - 703
JO - British Journal of Anaesthesia
JF - British Journal of Anaesthesia
SN - 0007-0912
IS - 5
ER -