Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management

Abstract –  Congenital insensitivity to pain with anhidrosis is a rare autosomal-recessive disorder characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self-mutilating behavior, and mental retardation. The lack of sensitivity to pain results in traumatic lesions, such as ulcers, fractures, burns, bites, scars, and digital amputations. Several methods have been suggested to treat these patients; however, appropriate management is difficult, especially when the mutilation is particularly severe. This report describes the case of a 2-year-old female patient who had severe self-mutilating injuries to her tongue, hands, lips, and oral mucosa caused by biting. The patient presented digital amputation and also a premature loss of a permanent tooth germ during the treatment. The dental management is described and discussed. It is important to include the dentist on the multidisciplinary team to reduce the frequency and severity of the self-inflicted lesions in these patients, also to prevent complications.     

Orthodontic treatment and management of limited mouth opening and oral lesions in a patient with congenital insensitivity to pain: case report

Summary  Congenital insensitivity to pain is a rare clinical syndrome characterized by dramatic impairment of pain perception since birth and is generally caused by a hereditary sensory and autonomic neuropathy with loss of the small-calibre, nociceptive nerve fibres. We report a 9-year-old case, with a generalized congenital insensitivity to pain. The patient was referred to our Department by a private orthodontist for severe limited mouth opening and multiple oral ulcers which greatly worsened after starting the orthodontic treatment. The management of his oral lesions of the limited mouth opening and of the orthodontic treatment are described. The management approach aimed to improve mandibular range of motion and associated stretching and a self-modeling mouthguard to avoid cheek self-biting. This protocol allowed continuing the orthodontic treatment to restore the occlusion. Finally, good occlusion, normal function and better quality of patient's life were achieved.     

The dental management of a partially edentulous patient suffering from hepatolenticular degeneration (Wilson's disease)

A bstract — A case of Wilson's sickness and its sequelae is described. A treatment procedure is suggested using a combination of skeleton designed cobalt chromium dentures and roach ball precision attachments and its advantages for the patient outlined.     

Dental management of oral self-mutilation in neurological patients: a case of congenital insensitivity to pain with anhidrosis

Hereditary sensory and autonomic neuropathy type IV is a rare disease characterized by fever episodes, mental retardation of different intensity, recurrent episodes of fever secondary to anhidrosis, little or no perspiration and congenital insensitivity to pain. Oral self-mutilation is also a characteristic sign. In this article, we present the case of an infant, aged 22 months, who showed these clinical characteristics and was treated with a dental device to prevent the patient from injuring her tongue. This device consisted of two acrylic splints joined at the back in the posterior sector, it provided an anterior open bite and allowed the infant to breathe through her mouth. The lesions of the patient had improved after using the device but the patient died due to the medical problem. Neuropathies treatment is a great challenge for medical teams. Dentists should form part ot these teams because of the bucal implications that may appear. Different appliances can be designed in order to solve the special problems each case may present.     

Pathological fracture of the mandible in a paediatric patient with congenital insensitivity to pain with anhidrosis (CIPA)

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.     

The dental patient with a congenital bleeding disorder

Congenital bleeding disorders account for approximately one in 10,000 births. Dentists are often anxious about delivering treatment to this special group of patients. In the Irish Republic, patients with inherited bleeding disorders have their dental care co-ordinated centrally at the National Centre for Hereditary Coagulation Disorders (NCHCD), St James's Hospital, Dublin. Dental care is normally integrated with routine outpatient haematological appointments. This ensures regular monitoring of oral health and the early treatment of any hard/soft tissue pathology. This article describes, in simple diagrammatic form, the normal coagulation mechanism (Figures 1 and 2), explains common coagulation terms (Appendix 1), and examines the three most common congenital bleeding disorders: haemophilia A, haemophilia B, and von Willebrand disease. General recommendations based on the current literature are provided with respect to procedures that are appropriate to perform in a general dental practice setting. Although not discussed in this article, it is important to note that non-coagulation bleeding disorders also exist. These include: hereditary haemorrhagic telangiectasia; blood vessel wall defects resulting from connective tissue disorders such as Marfan syndrome and Ehlers-Danlos syndrome; and, platelet disorders such as Bernard-Soulier syndrome, resulting in defective platelet adhesion.     

Management of a patient suffering with Cherubism with dental implants

Cherubism is a rare non-neoplastic, fibro-osseous hereditary disorder characterized by bilateral expansion of the maxilla and mandible producing a characteristic facial appearance. It can affect the facial and dental growth of the individual and often results in gross aesthetic and functional deficiencies. The teeth may also be displaced or submerged and these problems can often compromise successful restorative rehabilitation. This paper describes the restorative management of an adult patient with Cherubism involving a fixed implant retained mandibular restoration. The care utilized 3D planning software and implant insertion guides to facilitate an early loading protocol and the use of optimum bone quality/volume areas.     

The asthma patient and dental management

Asthma is increasing worldwide at a dramatic rate. The way the medical community defines asthma is changing, from considering it solely a respiratory disease to recognizing it as a more complex inflammatory disease process. This means that the treatment of asthma is becoming multifocal, depending upon the varied symptoms displayed. Reflecting this change is a shift to the use of corticosteroids in addition to the more traditional pulmonary medications. Dentists should be aware of the newer classifications and treatment modalities. There needs to be an understanding of the role these pharmaceutical agents play in the needs and treatment of our dental patients. Recent trends in the diagnosis and management of asthma are examined and recommendations for managing the asthmatic patient are provided.     

The management of the Muslim dental patient

There are aspects of the practice of the religion of Islam that have some relevance to receiving dental treatment. This article aims to provide dentists with background knowledge of normal practices which may affect the treatment offered. The author does not attempt to inform the reader about Islam, but to assist the dentist in the management of a Muslim patient. Much of the content of this article describes how to manage a patient who is fasting during the Islamic month of Ramadan. Ramadan takes place this year in early October, lasting for 29 or 30 days. During Ramadan patients may present to dentists with the signs and symptoms described in this article.     

Adult patient visits to physicians for dental problems

BACKGROUND AND OBJECTIVES: Most physicians lack substantive training in dentistry and are usually not capable of providing definitive dental care. Therefore, physician offices are generally not the most appropriate site for the management of most dental problems. This study was conducted to examine the rate with which patients visit physician offices for the treatment of dental problems and their satisfaction with the treatment received. METHODS: Data on dental related problems were collected through a random telephone survey of English-speaking Maryland residents over the age of 20. A random digit dial methodology was used to generate the sampling frame. A total of 811 interviews were conducted. The overall survey has a margin of error of +/- 3.44% at the 95% confidence level. RESULTS: 5.6% of respondents reported seeing a physician for a dental problem during the prior year. Almost 80% reported being satisfied with the treatment received, while 36.4% reported needing follow-up care with a dentist for treatment of the same problem. Respondents expressing greater satisfaction with their visit to the physician were less likely to report needing to see a dentist for follow-up care (p < .05). CONCLUSIONS: Additional studies are needed to assess the quality and appropriateness of physician management of dental problems.     

Psychosocial concomitants to dental fear and behaviour management problems

BACKGROUND: Children with dental behavioural management problems (DBMP) form a heterogeneous group, where personal characteristics play significant roles. Attention to everyday life and family situation as additional background facets may help to better understand and treat these patients. AIM: This study describes everyday life and family situation in child/adolescent patients referred because of DBMP, as compared to patients in ordinary dental care. DESIGN: A study group of 230 referred patients (8-19 years old; 118 girls) was compared to a reference group of 248 same-aged patients (142 girls) without DBMP. Patients and parents were interviewed according to a semistructured protocol. RESULTS: Patients referred because of DBMP more often lived in low socioeconomic status families, had parents not living together, fewer leisure-time activities, and were assessed as doing worse in social interactions compared to the reference group. Half of the study group had personal professional support, and some had experienced interventions by the social authorities. Whether these findings apply also to children/adolescents with DBMP who are not referred to specialist care remains to study. CONCLUSIONS: Many children and adolescents referred because of DBMP have a burdensome life and family situation. This should be paid attention to in research and in clinical care.     

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.