title: Pathological fracture of the mandible in a paediatric patient with congenital insensitivity to pain with anhidrosis (CIPA).
abstract: Congenital insensitivity to pain with anhidrosis (CIPA) is a rare hereditary sensory and autonomic neuropathy (HSAN) characterized by pain, self-mutilating behaviour, anhidrosis and recurrent hyperthermia. CIPA has a multisystem involvement, including fractures of the extremities with slow healing, immunologic abnormalities, and a chronic inflammatory state. The mandible is reported to have a higher incidence of osteomyelitis, though mandibular fracture among CIPA patients, is very rare, with to our knowledge no reports in children. A case of pathological fracture of the mandible in a 6-year-old child with CIPA treated by ORIF is reported. In contrast to the slow healing reported in long bones, the mandible healed very quickly, possibly indicating that the osteoporotic mandible in this group of patients is different from that seen in the elderly. Furthermore, the standard ORIF technique can be safely used in this rare group.
authors.affiliation: Institute of Human Genetics, Soroka Medical Center, Faculty of Health Sciences, Ben Gurion University of Negev, Beer Sheva, Israel.
authors.forename: Esther
authors.initials: E
authors.lastname: Manor
authors.forename: Ben Zion
authors.initials: BZ
authors.lastname: Joshua
authors.forename: Jacov
authors.initials: J
authors.lastname: Levy
authors.forename: Peter A
authors.initials: PA
authors.lastname: Brennan
authors.forename: Lipa
authors.initials: L
authors.lastname: Bodner
mesh_terms: D001860:Bone Plates; D001861:Bone Regeneration / Q000502:physiology; D002648:Child; D005500:Follow-Up Studies; D005593:Fracture Fixation, Internal / Q000295:instrumentation / Q000379:methods; D017102:Fracture Healing / Q000502:physiology; D005598:Fractures, Spontaneous / Q000175:diagnosis* / Q000601:surgery; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008337:Mandibular Fractures / Q000175:diagnosis* / Q000601:surgery; D010012:Osteogenesis / Q000502:physiology