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A Journal on Anesthesiology, Resuscitation, Analgesia and Intensive Care
Minerva Anestesiologica 2002 October;68(10):783-90
language: English, Italian
Manopharmacologic general anaesthesia with sevoflurane in paediatric patient with Prader-Willi syndrome
Rinaldi S., Rizzo L., Di Filippo A., Secchi S., Paterno-ster G., La Torre M. S., Pascente C., Stanzani M. R.
Section of Anesthesia and Resuscitation Department of Medical and Surgical Intensive Care University of Florence, Florence
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 pro- blems, we planned a monopharmacologic anaesthesiologic procedure using sevoflurane.