Anesthetic Challenges And Management In Patient With Ataxia-Telangiectasia

Abstract

Louis-Bar Syndrome is a synonym for a very rare complex neurodegenerative disorder ataxia-telangiectasia (A-T). This is an autosomal recessive inherited disease that encompasses abnormalities in the cerebellum, multisystem degeneration, immunodeficiency, increased risk for malignancy and consecutive respiratory insufficiency. Most of the patients are radiosensitive and any exposing to ionization may lead to progression of the disease. Potential risks from anesthesia, mechanical ventilation, and postoperative complications in these patients have been insufficiently discussed in the literature. We present a successful anesthetic and respiratory management with one-lung ventilation in a patient with Louis-Bar Syndrome who underwent video assisted thoracoscopy (VATS) for recurrent pleural effusion.