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.

Resumen

El síndrome de Rubinstein-Taybi es una enfermedad de baja incidencia (1:125.000–700.000 RN vivos) asociada a mutaciones o microdeleciones del cromosoma 16. Los pacientes afectos presentan frecuentemente anomalías craneofaciales y torácicas que condicionan una vía aérea y ventilación dificultosas. Asimismo, asocian retraso mental y comorbilidades, entre las que cabe destacar cardiopatías congénitas, infecciones respiratorias de repetición y enfermedad por reflujo gastroesofágico, que aumenta el riesgo de broncoaspiración. En estos pacientes se ha descrito una mayor incidencia de arritmias tras la administración de fármacos, como la succinilcolina y atropina, así como una mayor incidencia de apnea e hipopnea en el postoperatorio.

Presentamos el manejo anestésico de un paciente afecto de síndrome de Rubinstein–Taybi sometido a cirugía torácica urgente por perforación esofágica y mediastinitis, con exclusión pulmonar llevada a cabo con bloqueador bronquial.