Anesthetic management of an infant with Joubert syndrome for cardiac surgery

• Published in: Journal of clinical anesthesia Vol. 25; no. 6; pp. 488 - 490
• Main Authors: Bhaskar, Pradeep, MD, John, Jiju, MD, Sivamurthy, Suresh K., MD, Lone, Reyaz A., MS, Mch, Tysarowski, Pawel A., MD, MBA, Riyas, Mohammed K., MD, Syed, Syed A., M. MED, Bhat, Akhlaque N., MS, FRCS, FPCS, Sallehuddin, Ahmed, FRCS, MBA
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.09.2013; Elsevier; Elsevier Limited
• Subjects: Abnormalities, Multiple; Airway management; Anesthesia; Anesthesia & Perioperative Care; Anesthesia, Inhalation - methods; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Anesthetics, Inhalation - administration & dosage; Apnea; Ataxia; Biological and medical sciences; Cardiac surgery; Cardiopulmonary Bypass; Cerebellar Diseases - complications; Cerebellar Diseases - diagnosis; Cerebellar Diseases - surgery; Cerebellum - abnormalities; Congenital anomalies; Dexmedetomidine; Eye Abnormalities - complications; Eye Abnormalities - diagnosis; Eye Abnormalities - surgery; Female; Humans; Infant; Intensive care; Joubert syndrome; Kidney Diseases, Cystic - complications; Kidney Diseases, Cystic - diagnosis; Kidney Diseases, Cystic - surgery; Magnetic Resonance Imaging; Malformations of the nervous system; Medical sciences; Methyl Ethers - administration & dosage; Narcotics; Neonatology; Neurology; NMR; Nuclear magnetic resonance; Opioids; Pain Medicine; Postoperative period; Pulmonary Valve Stenosis - surgery; Retina - abnormalities; Retina - surgery; Sevoflurane; Sleep Apnea, Central - etiology; Ventilation; Apnea; Congenital anomalies; Joubert syndrome; Dexmedetomidine; Opioids; Neonatology; Anesthesia; Cardiac surgery; Human; α2-Adrenergic receptor; Heart; Agonist; Nervous system diseases; Infant; Opiates; Congenital anomaly; Genetic disease; Cerebral disorder; Clinical management; Anesthetic; Malformation; Surgery; Central nervous system disease; α-Adrenergic receptor agonist; AnesthesiaApnea
• ISSN: 0952-8180; 1873-4529
• DOI: 10.1016/j.jclinane.2013.02.013

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.