title: Anesthetic management for thoracic surgery in Rubinstein-Taybi syndrome.
abstract: Rubinstein-Taybi syndrome (RTS) is a chromosomopathy associated to molecular mutations or microdeletions of chromosome 16. It has an incidence of 1:125,000-700,000 live births. RTS patients present craniofacial and thoracic anomalies that lead to a probable difficult-to-manage airway and ventilation. They also present mental retardation and comorbidity, such as congenital cardiac defects, pulmonary structural anomalies and recurrent respiratory infections, which increase the risk of aspiration pneumonia. Cardiac arrhythmias have been reported after the use of certain drugs such as succinylcholine and atropine, in a higher incidence than in general population. There is an increased risk of postoperative apnea-hypopnea in these patients. We report the anesthetic management in a RTS patient undergoing emergent thoracic surgery due to oesophageal perforation and mediastinitis. Lung isolation was achieved with a bronchial blocker guided with a fiberoptic bronchoscope and one-lung ventilation was performed successfully.
authors.affiliation: Anesthesiology, Critical Care and Pain Management Department, Hospital Universitario Virgen Macarena, University of Seville, Spain. Electronic address: eva_bg@msn.com.
authors.forename: E
authors.initials: E
authors.lastname: Blazquez
authors.affiliation: Anesthesiology, Critical Care and Pain Management Department, Hospital Universitario Virgen Macarena, University of Seville, Spain.
authors.forename: D
authors.initials: D
authors.lastname: Narváez
authors.affiliation: Anesthesiology, Critical Care and Pain Management Department, Hospital Universitario Virgen Macarena, University of Seville, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Fernandez-Lopez
authors.affiliation: Hospital Sant Joan de Déu, University of Barcelona, Spain.
authors.forename: L
authors.initials: L
authors.lastname: Garcia-Aparicio
mesh_terms: D000777:Anesthetics; D006801:Humans; D008607:Intellectual Disability; D061810:One-Lung Ventilation; D012415:Rubinstein-Taybi Syndrome; D013903:Thoracic Surgery
chemical_list: D000777:Anesthetics
keywords: Airway management; Chromosome disorders; Cromosomopatías; Esophageal perforation; Manejo de la vía aérea; One-lung ventilation; Perforación esofágica; Rubinstein–Taybi syndrome; Síndrome de Rubistein–Taybi; Ventilación unipulmonar