title: Anesthetic management of an infant with Joubert syndrome for cardiac surgery.
abstract: The anesthetic implications of Joubert syndrome in an infant who required cardiac surgery using cardiopulmonary bypass (CPB) is presented. Children with Joubert syndrome present with central apnea due to malformations in the midbrain and cerebellum. These patients have a marked sensitivity to opioids. The use of dexmedetomidine along with remifentanil was effective in this case.
authors: Division of Pediatric Cardiac Surgery, Department of Cardiology and Cardiothoracic Surgery, Hamad Medical Corporation, Doha, Qatar.
authors: Pradeep
authors: P
authors: Bhaskar
authors: Jiju
authors: J
authors: John
authors: Suresh K
authors: SK
authors: Sivamurthy
authors: Reyaz A
authors: RA
authors: Lone
authors: Pawel A
authors: PA
authors: Tysarowski
authors: Mohammed K
authors: MK
authors: Riyas
authors: Syed A
authors: SA
authors: Syed
authors: Akhlaque N
authors: AN
authors: Bhat
authors: Ahmed
authors: A
authors: Sallehuddin
mesh_terms: D000015:Abnormalities, Multiple; D000769:Anesthesia, Inhalation / Q000379:methods*; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D002315:Cardiopulmonary Bypass; D002526:Cerebellar Diseases / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D002531:Cerebellum / Q000002:abnormalities; D005124:Eye Abnormalities / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D052177:Kidney Diseases, Cystic / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008738:Methyl Ethers / Q000008:administration & dosage; D011666:Pulmonary Valve Stenosis / Q000601:surgery*; D012160:Retina / Q000002:abnormalities* / Q000601:surgery; D000077149:Sevoflurane; D020182:Sleep Apnea, Central / Q000209:etiology
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane
keywords: Anesthesia; Apnea; Cardiac surgery; Congenital anomalies; Dexmedetomidine; Joubert syndrome; Neonatology; Opioids