Anaesthetic implications of LEOPARD syndrome.
LEOPARD syndrome is a neuroectodermal disorder presumed to result from an abnormality in neural crest cells. The acronym 'LEOPARD' is derived from the clinical features which include multiple lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retarded growth, and deafness. Given the multisystem nature of the disease process, several issues may affect the perioperative care of these patients. Of primary importance are associated conditions of the cardiovascular system including congenital heart disease, conduction disturbances, and progressive hypertrophic obstructive cardiomyopathy. The authors present a 4-year old boy who presented for anaesthetic care for repair of a ventricular septal defect and pulmonary valvotomy for congenital pulmonary stenosis. The potential perioperative implications of LEOPARD syndrome are discussed.
Torres
Javier
J
The Department of Child Health, The Division of Pediatric Critical Care/Pediatric Anesthesiology, The University of Missouri, Columbia, MO 65212, USA.
Russo
Pierantonio
P
Tobias
Joseph D
JD
D000768:Anesthesia, General / Q000379:methods*; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D018686:Anesthetics, Intravenous / Q000008:administration & dosage; D001279:Atracurium / Q000008:administration & dosage / Q000031:analogs & derivatives*; D002315:Cardiopulmonary Bypass; D002675:Child, Preschool; D005283:Fentanyl / Q000008:administration & dosage; D006345:Heart Septal Defects, Ventricular / Q000601:surgery; D006801:Humans; D044542:LEOPARD Syndrome / Q000503:physiopathology*; D008297:Male; D008738:Methyl Ethers / Q000008:administration & dosage; D009466:Neuromuscular Blocking Agents / Q000008:administration & dosage; D019990:Perioperative Care; D011664:Pulmonary Valve / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000151:congenital / Q000601:surgery; D000077149:Sevoflurane
D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D008738:Methyl Ethers; D009466:Neuromuscular Blocking Agents; D001279:Atracurium; D000077149:Sevoflurane; C101584:cisatracurium; D005283:Fentanyl