Lesch-Nyhan syndrome: evaluation of a modified bite device to prevent bite injuries

Lesch-Nyhan syndrome (LNS) is a serious form of hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency, a hereditary purinemetabolismdisorder. The prevalence reported in the literature is 1/380,000 to 235,000 births. Males are affected and females are heterozygous carriers. LNS patients present a combination of hypotonia, spasticity, and neurological and behavioral disorders. They also show an obsessive-compulsive self-injurious behavior with bites and injuries to the lips, tongue, cheeks and fingers. The literature offers little scientific contribution related to the management of this problem. The authors describe their experience with a 4-year-old LNS patient and present a viable solution to control and avoid bite injuries, namely a specifically modified bite. The patient was treated at the Pediatric Dentistry Department of "Sapienza" University of Rome with a modified bite with the internal surface, in contact with the teeth, realized in 2-mm-thick soft silicone, and the exterior part consisting of a transparent resin shell with front and rear shields to separate lips and cheeks from the dental arches. At a 12-month follow-up visit, compliance was excellent: the child wore the device with regularity and without discomfort, even during soft-food feeding. No intraoral bite injuries were found, with a general improvement of the young patient's quality of life

External root resorption management of an avulsed and reimplanted central incisor: A case report

Background: Avulsion and reimplantation of permanent teeth represent a major challenge in terms of treatment and long-term prognosis. The present study reported clinical management of external root resorption of an avulsed and reimplanted maxillary central incisor. Case report: A 9-year-old boy reported an uncomplicated crown fracture and avulsion of tooth 11 and complicated crown fracture of tooth 21 due to trauma. Reimplantation of element 11 was obtained within 30 min post-trauma and 3 days after both elements were diagnosed with necrotic pulp. In addition, tooth 11 showed early external root resorption. Both elements underwent endodontic treatment and root closure with apical plug using calcium-silicate-based cement. At 6-month follow-up root resorption appeared to be arrested. Twenty-four months after trauma the clinical results were stable, although signs and symptoms of ankylosis were observed. Conclusions: An immediate endodontic approach and use of calcium-silicate-based cement seemed to contrast the progression of root resorption of an avulsed and reimplanted central incisor after 24 months of follow-up

Bisphosphonates therapy in children with osteogenesis imperfecta: clinical experience in oral surgery

Objectives: to define the possible complications of oral surgery in childhood in patients affected by type 1 Osteogenesis imperfecta (OI) and treated with bisphosphonates (BP). METHODS The study was conducted among 20 patients in childhood with an age range 8-14 (12 e 8 ) affected by OI. Patients were initially evaluated at the Policlinico Umberto I, University Hospital of Rome, Rare Disease Center Skeletal Dysplasia-Bone Metabolic Pathologies and after at the Policlinico Umberto I, University Hospital of Rome, Head and Neck Department, UOC Pediatric Dentistry. RESULTS From this experience, we showed that a proper patient management from the medical and dental point of view can protect these patients from the risk of post-operative problems, such as onj, soft tissue flogos, intraoral and extraoral fistulas, failure to heal the post-extractive alveolus, infections, post-operative pain and pathological fractures. The follow-up, ranging from a minimum of 2 years to a maximum of 5 years, have not demonstrated the presence of particular complications or healing defects. CONCLUSIONS The clinical experiences observed in these patients are encouraging because no postoperative complications have been observed compared to patients non-affected by OI