title: Monopharmacologic general anaesthesia with sevoflurane in paediatric patient with Prader-Willi syndrome.
abstract: Prader-Willi syndrome (PWS) is a genetic disease caused by a loss of paternal genes located in chromosome 15. Children affected by this syndrome often have preterm delivery; during childhood the hallmarks are: severe infantile hypotonia and feeding problems. Afterward, neurologic manifestations, endocrine signs and dysmetabolic abnormalities are usually seen together with craniofacial manifestations and musculoskeletal abnormalities. Obesity causes sleep abnormalities including sleep apnea. The case we present is of a 5 year old child (CA) scheduled for strabismus surgery. The child has a lot of typical (PWS) signs. A number of anaesthesiologic problems are associated with (PWS). Some of them relate to obesity, others to facial dysmorphism. Moreover, the syndrome may give a prolonged and exaggerated response to every sedative drug. P.W.S. is also characterized by thermoregulatory disorders. Sleep apnea occurs often. Considering all these problems, we planned a monopharmacologic anaesthesiologic procedure using sevoflurane.
authors.affiliation: Section of Anesthesia and Resuscitation, Department of Medical and Surgical Intensive Care, University of Florence, Florence.
authors.forename: S
authors.initials: S
authors.lastname: Rinaldi
authors.forename: L
authors.initials: L
authors.lastname: Rizzo
authors.forename: A
authors.initials: A
authors.lastname: Di Filippo
authors.forename: S
authors.initials: S
authors.lastname: Secchi
authors.forename: G
authors.initials: G
authors.lastname: Paternoster
authors.forename: M S
authors.initials: MS
authors.lastname: La Torre
authors.forename: C
authors.initials: C
authors.lastname: Pascente
authors.forename: M R
authors.initials: MR
authors.lastname: Stanzani
mesh_terms: D000769:Anesthesia, Inhalation; D018685:Anesthetics, Inhalation / Q000493:pharmacokinetics; D001833:Body Temperature Regulation; D002675:Child, Preschool; D005145:Face / Q000002:abnormalities; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology; D007431:Intraoperative Complications / Q000517:prevention & control; D008738:Methyl Ethers / Q000493:pharmacokinetics; D009123:Muscle Hypotonia / Q000209:etiology; D009765:Obesity / Q000209:etiology; D011218:Prader-Willi Syndrome / Q000150:complications / Q000523:psychology; D012120:Respiration Disorders / Q000209:etiology; D000077149:Sevoflurane; D013285:Strabismus / Q000150:complications / Q000601:surgery*
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane