Korean J Anesthesiol.  2009 Aug;57(2):259-263. 10.4097/kjae.2009.57.2.259.

Anesthetic management in a pediatric patient with infantile phase Prader-Willi Syndrome: A case report

• Kim JY
• Lee JH
• Kim EJ
• Lee SK
• Ban JS
• Min BW

• KMID: 2238103
• DOI: http://doi.org/10.4097/kjae.2009.57.2.259

Abstract

Prader-Willi syndrome is a genetic disorder characterized by infantile hypotonia, childhood obesity, characteristic facial appearance, mental retardation, hypogonadism and short stature. It is described as a 2-stage disorder with an infantile hypotonic phase, followed by a childhood obese phase. The first phase, during the newborn and infancy period, is characterized by marked hypotonia, poor sucking, swallowing, coughing, crying, and episodes of asphyxia. Since these signs of poor strength cause poor reversal in the postoperative period, muscle relaxants should be used cautiously, especially in patients in the first phase of the syndrome. We experienced the anesthetic management of a 5-month-old female patient with Prader-Willi syndrome under general anesthesia, without muscle relaxant for excision of BCG lymphadenitis and a preauricular mass.

Keyword

Hypotonia; Muscle relaxant; Prader-Willi syndrome

MeSH Terms

Anesthesia, General
Asphyxia
Cough
Crying
Deglutition
Female
Humans
Hypogonadism
Infant
Infant, Newborn
Intellectual Disability
Lymphadenitis
Muscle Hypotonia
Muscles
Mycobacterium bovis
Obesity
Postoperative Period
Prader-Willi Syndrome