IADR Abstract Archives
Dental treatment in patients with Pompe disease under General Anesthesia
Objectives: Pompe disease (Acid maltase deficiency, Glycogen storage disease type II), is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme acid-α-glucosidase that hydrolyzes lysosomal glycogen. As a result, Lysosomal glycogen accumulates in multiple tissues and organs with muscle tissue most prominently involved. Glycogen accumulation in muscle tissue leads to progressive debilitation, organ failure and death. The aim of this report is to present the preoperative and dental management of two patients with Pompe disease.
Methods: A 24 and 27-year old female sisters diagnosed with the late-onset form of Pompe disease, presented to our department seeking dental treatment. Both patients are wheelchair users with home care ventilation support. Clinical examination revealed highly limited mouth opening and very poor oral hygiene. Maintenance of proper oral hygiene and dental treatment may be very challenging, due to the progressive nature of the disease and muscles debilitation.
Results: Special considerations with multidisciplinary team approach were taken to ensure safe dental treatment under general anesthesia. We collaborated with the departments of Neurology, Anesthesiology and the Respiratory Intensive Care Unit. Several dental treatments were performed successfully under general anesthesia without any complication.
Conclusions: The clinical spectrum of Pompe disease varies with respect to the age of onset, residual enzyme activity and organ involvement. Most individuals with late-onset Pompe disease experience progressive muscle weakness. Dental management in these patients can be challenging and special preparation and considerations must be taken. Even though, dental treatment under general anesthesia considered a high risk treatment, for these patients, it is often mandatory. Collaboration of multidisciplinary specialist in the preoperative preparations and management is the key for successful and safe dental treatment.
Methods: A 24 and 27-year old female sisters diagnosed with the late-onset form of Pompe disease, presented to our department seeking dental treatment. Both patients are wheelchair users with home care ventilation support. Clinical examination revealed highly limited mouth opening and very poor oral hygiene. Maintenance of proper oral hygiene and dental treatment may be very challenging, due to the progressive nature of the disease and muscles debilitation.
Results: Special considerations with multidisciplinary team approach were taken to ensure safe dental treatment under general anesthesia. We collaborated with the departments of Neurology, Anesthesiology and the Respiratory Intensive Care Unit. Several dental treatments were performed successfully under general anesthesia without any complication.
Conclusions: The clinical spectrum of Pompe disease varies with respect to the age of onset, residual enzyme activity and organ involvement. Most individuals with late-onset Pompe disease experience progressive muscle weakness. Dental management in these patients can be challenging and special preparation and considerations must be taken. Even though, dental treatment under general anesthesia considered a high risk treatment, for these patients, it is often mandatory. Collaboration of multidisciplinary specialist in the preoperative preparations and management is the key for successful and safe dental treatment.