Dentinogenesis imperfecta: An early treatment strategy

Department of Pediatric Dentistry, the Hebrew University-Hadassah Faculty of Dental Medicine, Jerusalem, Israel.
Pediatric dentistry (Impact Factor: 0.56). 01/2001; 23(3):232-7.
Source: PubMed


Dentinogenesis imperfecta (DI) type 2 is a disease inherited in a simple autosomal dominant mode. As soon as the teeth erupt the parents may notice the problem and look for a pediatric dentist's advice and treatment. Early diagnosis and treatment of DI is recommended, as it may prevent or intercept deterioration of the teeth and occlusion and improve esthetics. The purpose of this article is to present the objectives, treatment options, and problems encountered in the treatment of DI in the early primary dentition. A two-stage treatment of a toddler under general anesthesia is described and discussed. This paper recommends for severe cases of DI two treatment stages performed under general anesthesia. Stage 1 is early (around age 18-20 months) and is directed to covering the incisors with composite restorations and the first primary molars with preformed crowns. Stage 2 (around age 28-30 months) seeks to protect the second primary molars with preformed crowns and cover the canines with composite restorations.

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    • "The early treatment of teeth with DI should aim to ensure good occlusion and esthetics, favorable growth of the facial bones and tempromandibular joints, and a favorable condition for the eruption of the permanent successors. Treatment includes caries prevention, attrition observation, monitoring the development of the craniofacial skeleton, and placement of artificial crowns to prevent excessive loss of the tooth structure when the deciduous teeth begin to wear (Levin, 1981; Ranta et al., 1993; Sapir and Shapira, 2001). In the present case study, the deciduous teeth manifested multiple carious lesions, attrition , and rapid wear. "
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    ABSTRACT: Dentinogenesis imperfecta (DI) associated with osteogenesis imperfecta (OI) is a genetic disorder that affects the connective tissues and results in dentine dysplasia. This case report discusses the systemic and dental manifestations of OI and DI in a 4-year-old child, with moderate presentation of both disorders, who was treated at King Fahd Military Medical Complex in Dhahran. Dental treatment included the use of strip and stainless-steel crowns under local anesthesia, as well as behavior modification techniques. Rigorous home care instructions, including reinforcement of the oral hygiene practice and avoidance of any episode that may lead to bone fracture, were discussed with the parents. The case was reevaluated at 3-month follow-up visits, wherein the medical and dental histories were updated, the child’s growth was monitored, periodic clinical and radiographic examinations were performed, and the oral hygiene was evaluated via the debris index score and caries risk assessment. Further treatment of the permanent dentition may be needed in the future.
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    • "Therefore, these diseases do not seem to be separate entities, but rather allelic ones with varying phenotypes according to the nature of the mutation. DGI type III is no longer considered as a phenotype specific to the Brandywine isolate because similar clinical features have been reported in families of different origins (Heimler et al, 1985; Sapir and Shapira, 2001). Furthermore, a genetic study revealed that the disease causing mutation in the Brandywine isolate is identical to DSPP gene mutation (c.49C>T, p.P17S) observed in a Chinese family having DGI type II (Hart and Hart, 2007; Zhang et al, 2007). "
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    ABSTRACT: Hereditary dentin defects can be grouped into three types of dentinogenesis imperfecta (DGI) and two types of dentin dysplasia. Tooth enamel is considered normal in patients with hereditary dentin defects, but is easily worn down and fractured due to DSPP mutation-induced altered dentin properties. The purposes of this study were to identify genetic cause of a family with type II DGI and enamel defects. We identified a family with type II DGI and a unique form of hypoplastic enamel defect affecting occlusal third of the crown. Family members were recruited for the genetic analysis and DNA was obtained from peripheral whole blood. Mutational analysis revealed a T to A transversion in exon 3 of the DSPP (c.53T>A, p.V18D). Haplotype analysis showed that the same mutation arose separately in two different families having DGI with similar enamel defects, indicating that this phenotype is associated with this specific DSPP mutation. Clinical features suggest that enamel formation was affected in the affected individuals during early amelogenesis, in addition to the dentin defect. We observed that a DSPP gene mutation not only influences dentinogenesis but also affects early stage amelogenesis.
    Full-text · Article · Oct 2010 · Oral Diseases
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    • "In some cases, permanent teeth seem to be less susceptible to an excessive attrition and the dental caries index is low [Sapir and Shapira, 2001; Bouvier et al., 2008]. During a clinical examination, in both dentitions, it is important to consider tooth colour (variable from normal to blue grey or yellow brown), tooth wear, abscess formation, tooth mobility and early loss of teeth [Barron et al., 2008]. "
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    ABSTRACT: Dentinogenesis imperfecta is one of the most common hereditary disorders of dentine formation. Opalescent teeth composed of irregularly formed and undemineralised dentine that obliterates pulp chambers and root canals characterize it. Complete-coverage crowns are usually the preferred restoration for patients with this condition. A 9 year-old girl presented with dentinogenesis imperfecta, congenitally missing maxillary lateral incisors and maxillary right permanent second molar retention. The treatment comprised an initial approach to allow the correct eruption of the retained second molar. The use of low-fusion metal ceramic restorations comprised a second stage to improve the aesthetic appearance and decrease the risk of overload on teeth with limited value. The patient has been recalled regularly and at the last visit, 10 years after initial prosthetic treatment, no problems or signs of complications have occurred. The patient is now aged 25 years and is still satisfied with the prosthetic rehabilitation. This case illustrates the need for appropriate and timely restorative treatment to prevent deterioration of the dentition. This case will also demonstrate that low-fusion metal ceramic restoration is a viable esthetic treatment option for today's patients.
    Full-text · Article · Oct 2010 · European Archives of Paediatric Dentistry. Official Journal of the European Academy of Paediatric Dentistry.
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