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Int J Clin Exp Med 2013;6(5):393-403
Case Report
Perioperative management of a patient with Rett syndrome
Hiromi Kako, David P Martin, Richard Cartabuke, Allan Beebe, Jan Klamar, Joseph D Tobias
Departments of Anesthesiology & Pain Medicine, Nationwide Children’s Hospital, Columbus, Ohio; Department of Anesthesiology, The
Ohio State University, Columbus, Ohio; Department of Orthopedic Surgery, Nationwide Children’s Hospital, Columbus, Ohio
Received March 26, 2013; Accepted April 10, 2013; Epub May 22, 2013; Published June 1, 2013
Abstract: Rett syndrome is a neurodevelopmental disorder that results from mutations in the genes encoding methyl-cytosine-
guanosine binding protein 2 located on the X chromosome. Clinical features of central nervous system involvement include regression
of developmental milestones in the late infant and early toddler stages, mental retardation, seizures and other electroencephalographic
abnormalities. Given the invariable association of this degenerative disorder with orthopedic deformities including scoliosis, patients
with Rett syndrome may present for anesthetic care during various surgical procedures. The complexity of the end-organ involvement,
specifically the progressive nature of respiratory and cardiac involvement, makes the anesthetic care of such patients challenging.
Specific perioperative concerns include potential difficulties with airway management, an underlying seizure disorder, an increased
sensitivity to anesthetic agents, prolonged QT syndrome, and diabetes mellitus. We present an 11-year-old girl with Rett syndrome who
required anesthetic care for posterior spinal fusion. Previous reports of anesthetic care for these patients are reviewed, the end-organ
involvement discussed, and options for anesthetic care presented. (IJCEM1303023).
Keywords: Rett syndrome, long QT syndrome, diabetes mellitus
Address correspondence to: Dr. Hiromi Kako, Department of Anesthesiology & Pain Medicine, Nationwide Children’s Hospital, 700
Children’s Drive, Columbus, Ohio 43205. Phone: 614-722-4200; Fax: 614-722-4203; E-mail: Hiromi.Kako@nationwidechildrens.org
Case Report
Perioperative management of a patient with Rett syndrome
Hiromi Kako, David P Martin, Richard Cartabuke, Allan Beebe, Jan Klamar, Joseph D Tobias
Departments of Anesthesiology & Pain Medicine, Nationwide Children’s Hospital, Columbus, Ohio; Department of Anesthesiology, The
Ohio State University, Columbus, Ohio; Department of Orthopedic Surgery, Nationwide Children’s Hospital, Columbus, Ohio
Received March 26, 2013; Accepted April 10, 2013; Epub May 22, 2013; Published June 1, 2013
Abstract: Rett syndrome is a neurodevelopmental disorder that results from mutations in the genes encoding methyl-cytosine-
guanosine binding protein 2 located on the X chromosome. Clinical features of central nervous system involvement include regression
of developmental milestones in the late infant and early toddler stages, mental retardation, seizures and other electroencephalographic
abnormalities. Given the invariable association of this degenerative disorder with orthopedic deformities including scoliosis, patients
with Rett syndrome may present for anesthetic care during various surgical procedures. The complexity of the end-organ involvement,
specifically the progressive nature of respiratory and cardiac involvement, makes the anesthetic care of such patients challenging.
Specific perioperative concerns include potential difficulties with airway management, an underlying seizure disorder, an increased
sensitivity to anesthetic agents, prolonged QT syndrome, and diabetes mellitus. We present an 11-year-old girl with Rett syndrome who
required anesthetic care for posterior spinal fusion. Previous reports of anesthetic care for these patients are reviewed, the end-organ
involvement discussed, and options for anesthetic care presented. (IJCEM1303023).
Keywords: Rett syndrome, long QT syndrome, diabetes mellitus
Address correspondence to: Dr. Hiromi Kako, Department of Anesthesiology & Pain Medicine, Nationwide Children’s Hospital, 700
Children’s Drive, Columbus, Ohio 43205. Phone: 614-722-4200; Fax: 614-722-4203; E-mail: Hiromi.Kako@nationwidechildrens.org
