Korean J Anesthesiol.  2009 Apr;56(4):466-469. 10.4097/kjae.2009.56.4.466.

Anesthetic experience for strabismus surgery in a patient with Angelman syndrome: A case report

• Lee DH
• Lee HM

• KMID: 2236539
• DOI: http://doi.org/10.4097/kjae.2009.56.4.466

Abstract

Angelman syndrome (AS) is a rare, neurodevelopmental disorder caused by a variety of genetic abnormalities involving the chromosome 15q11-13 region. Clinical manifestations include severe psychomotor retardation, epilepsy, lack of speech, cerebellar ataxia, frequent bouts of laughing, morphological defect of the facial bones and sleep disturbance. The main anesthetic considerations in AS patients are significant dominance of vagal tone, profound disorders of GABA system with defects in its receptor function, epilepsy and peripheral muscular atrophy. We report the experience of anesthetic management for strabismus surgery in a patient with AS.

Keyword

Anesthesia; Angelman syndrome; Strabismus

MeSH Terms

Anesthesia
Angelman Syndrome
Cerebellar Ataxia
Epilepsy
Facial Bones
gamma-Aminobutyric Acid
Humans
Muscular Atrophy
Strabismus
gamma-Aminobutyric Acid