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Anaesthesia in a patient with Prader-Willi syndrome and severe burn injury: a case report


Biro, Peter (2011). Anaesthesia in a patient with Prader-Willi syndrome and severe burn injury: a case report. Jurnalul Român de Anestezie Terapie Intensivã, 18(2):149-152.

Abstract

Prader-Willi syndrome (PWS) is a congenital developmental disorder which is related to severe problems during anaesthesia. Of particular interest are the morphological and patho-physiological alterations which may lead to considerable respiratory complications, including a higher risk of regurgitation and aspiration, and the tendency towards post-surgical hypoventilation. Further difficulties may arise from mental retardation, disturbed thermoregulation, and an unstable glucose metabolism.
In our patient these features have been aggravated by burn injuries exceeding 35% of body surface. Appropriate care of circulatory and respiratory function, as well as supervision of blood glucose level and body temperature during a period of several weeks with repeated operations was necessary to avoid further complications. Additional findings we discovered in our patient were narrow airways not corresponding to the patient's age and a significant resistance to non depolarising muscle relaxants that might have been caused by the burn injuries.
The importance of PWS for anaesthesiologists can be estimated by the circumstance, that it is considered to be nearly as frequent as trisomy-21. PWS in connection with extensive burns has yet not been reported. This case and a recent review of the relevant literature on anaesthetic management of PWS are extensively discussed.

Prader-Willi syndrome (PWS) is a congenital developmental disorder which is related to severe problems during anaesthesia. Of particular interest are the morphological and patho-physiological alterations which may lead to considerable respiratory complications, including a higher risk of regurgitation and aspiration, and the tendency towards post-surgical hypoventilation. Further difficulties may arise from mental retardation, disturbed thermoregulation, and an unstable glucose metabolism.
In our patient these features have been aggravated by burn injuries exceeding 35% of body surface. Appropriate care of circulatory and respiratory function, as well as supervision of blood glucose level and body temperature during a period of several weeks with repeated operations was necessary to avoid further complications. Additional findings we discovered in our patient were narrow airways not corresponding to the patient's age and a significant resistance to non depolarising muscle relaxants that might have been caused by the burn injuries.
The importance of PWS for anaesthesiologists can be estimated by the circumstance, that it is considered to be nearly as frequent as trisomy-21. PWS in connection with extensive burns has yet not been reported. This case and a recent review of the relevant literature on anaesthetic management of PWS are extensively discussed.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Anesthesiology
Dewey Decimal Classification:610 Medicine & health
Uncontrolled Keywords:case report
Language:English
Date:2011
Deposited On:16 Aug 2012 07:19
Last Modified:05 Apr 2016 15:55
Publisher:Clinica ATI
ISSN:1582-652X
Official URL:http://www.jurnalul-anestezie.ro/2011/2/11.html
Permanent URL: https://doi.org/10.5167/uzh-64150

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