Orofacial features and pediatric dentistry in the long-term management of Infantile Pompe Disease children.

Background: Glycogen storage disease type II (GSDII) or Pompe disease is a rare autosomal recessive metabolic disorder that leads to intracellular glycogen storage in many tissues, mainly in skeletal muscle, heart and liver. Facial muscle weakness and altered craniofacial growth are very common in Pompe disease children. In this paper we describe the orofacial features in two children affected by GSDII and illustrate a multidisciplinary approach that involved enzyme replace therapy, non-invasive ventilation (NIV) and pediatric dentistry with 5-year follow-up.

Pain Experience and Behavior Management in Pediatric Dentistry: A Comparison between Traditional Local Anesthesia and the Wand Computerized Delivery System.

To evaluate the pain experience and behavior during dental injection, using the Wand computerized delivery system versus conventional local anesthesia in children and adolescents. An observational crossover split mouth study was performed on 67 patients (aged 7 to 15 years), requiring local anesthesia for dental treatments in both sides of the dental arch. Patients received both types of injections in two separate appointments, one with the use of a Computer Delivery System (the Wand STA system) and one with the traditional syringe.