ABSTRACT
Mucopolysaccharidosis is a rare inherited lysosomal storage disease characterized by increasing deposition of unmetabolized glucosaminoglycans. The surgical procedures under general anesthesia in these patients is associated with high mortality due to difficulty in airway management .The regional anesthesia is useful alternative; however, previous case reports describing epidural/spinal anesthesia have reported failure of this technique. We describe a case of mucopolysaccharidosis type I [Hurler syndrome] for repair of umbilical and inguinal hernia repair conducted under epidural anesthesia with sedation, obviating the need of intubation
ABSTRACT
A 17 year old boy, post tracheoplasty, presented to emergency in respiratory distress with presence of stridor at rest. Flexible bronchoscopy revealed granulations at tracheal graft site causing airway stenosis. The presence of graft perculded use of invasive surgical approaches due to risk of damage.The anesthetic management options in this case carried inherent risks of causing total airway obstruction and impairing surgical access. We decided to perform the electrocauterization of tracheal granulations using subglottic jet ventilation while placing the catheter above the stenotic trachea