1/5. Congenital insensitivity to pain--review and report of a case with dental implications.Pain is a protective mechanism for the body. Absence of pain is a symptom in several disorders, both congenital and acquired. The congenital types are present at birth and affect the number and distribution of types of nerve fibers. At present, 5 types of hereditary sensory and autonomic neuropathies have been identified. The various disorders within this group are classified according to the different patterns of sensory and autonomic dysfunction and peripheral neuropathy and the presence of additional clinical features such as learning disability. However, the field is currently moving away from classification based on clinical presentation toward classification based on underlying genetic abnormality. In the absence of pain, patients are at risk of late presentation with illnesses or injuries, and have an increased incidence of traumatic injury. Self-mutilation is an almost invariable feature of these disorders. We report the case of a patient with congenital insensitivity to pain that presented with self-mutilation injuries to his hands and oral tissues caused by biting. The severe nature of these injuries necessitated serial extraction of his primary teeth soon after eruption, which led to a cessation of the problem. The mutilation has not returned following the eruption of the first of his permanent teeth, suggesting that he has learned not to bite himself, even though to do so causes him no discomfort.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
2/5. Congenital insensitivity-to-pain with anhidrosis (CIPA): a case report with 4-year follow-up.Congenital Insensitivity-to-pain with anhidrosis (CIPA) is a rare disorder in which pain perception is absent from birth, despite the fact that all other sensory modalities remain intact or minimally impaired and tendon reflexes are present. The challenge in dentistry is to manage the self-mutilation behavior avoiding serious damages especially to oral structures, hands and fingers. A Brazilian case of CIPA is presented and discussed with clinical documentation of the oral-related problems over a 4-year follow-up. A conservative treatment (mouthguard-like appliance) was proposed with the objective to avoid full mouth extraction.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
3/5. Congenital insensitivity to pain with anhidrosis in a 2-month-old boy.In a 2-month-old boy with congenital insensitivity to pain and anhidrosis, the initial symptom was recurrent fever of unknown origin. After eruption of the first teeth, self-mutilation of the tongue was limited by extraction of the teeth. Unmyelinated fibers were essentially lacking, and the number of small myelinated fibers was decreased in sural nerve. This case is the youngest to be diagnosed and the first one in which nerve biopsy clearly showed the characteristic findings.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
4/5. Congenital autonomic dysfunction with universal pain loss.Three patients who appear to have a previously undescribed congenital neuropathy are described. None is of Ashkenazi Jewish extraction, but each seems to fulfill the clinical diagnostic criteria for familial dysautonomia. All lack overflow tears, fungiform papillae, and deep-tendon reflexes; intradermal administration of histamine did not produce an axon flare. Intraocular instillation of dilute mecholyl produced miosis in the one patient tested. In contrast to patients with familial dysautonomia, the three patients had universal loss of pain sensation, profound hypotonia, and unusual facies. Pathologic examination of the sural nerve in one patient was not consistent with the usual findings in familial dysautonomia. These patients are believed to have a previously undescribed congenital neuropathy.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
5/5. Consequences of serious oral injury associated with the congenital analgia syndrome.Three sisters at the ages of seven months, twelve years, and thirteen years presented with the initial damages to the oral tissues and the distinctive long-term effects in conjunction with the congenital analgia syndrome. The severity of this syndrome justifies the consideration of a prophylactic extraction of the primary dentition. A controlled mastication will be more likely with increasing age and eruption of the permanent teeth.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |