International Journal of Health & Allied Sciences

: 2013  |  Volume : 2  |  Issue : 3  |  Page : 153--158

Regional odontodysplasia and its treatment maneuver

Prashanth S Murthy, Seema Deshmukh 
 Department of Paedodontics and Preventive Dentistry, JSS Dental College and Hospital, JSS University, Mysore, Karnataka, India

Correspondence Address:
Prashanth S Murthy
29 A, 5th main road, Aravinda Nagar, Mysore, Karnataka


Regional odontodysplasia is a rare nonhereditary developmental anomaly affecting dental tissues derived from both ectoderm and mesoderm. It has characteristic clinical, histologic, and radiographic findings. Although various factors seem to be associated with this condition, the etiology remains uncertain yet. Because of its characteristics findings, this condition is diagnosed easily but remains difficult to treat. Various schools of thought exist regarding the treatment. However, the treatment decision varies from case to case. The aim of this paper is to elaborate the condition with a review of literature and to discuss the controversies existing over the treatment options so as to provide successful treatment outcome.

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Murthy PS, Deshmukh S. Regional odontodysplasia and its treatment maneuver .Int J Health Allied Sci 2013;2:153-158

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Murthy PS, Deshmukh S. Regional odontodysplasia and its treatment maneuver . Int J Health Allied Sci [serial online] 2013 [cited 2024 Feb 25 ];2:153-158
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Regional odontodysplasia (RO) is a rare, nonhereditary developmental dental anomaly characterized by defect in the epidermal and mesenchymal tissues involved in tooth development. Hence, deficiencies are noted in the enamel and dentin formation. This condition was first described by Hitchin in his paper "Unerupted deciduous teeth in youth aged 15΍" in 1934. But according to the literature, most authors credit McCall and Wald for the first report of this condition. [1] Complete bibliography of this condition was described by Crawford and Aldred. [2] McCall and Wald described this condition as arrested tooth development. Since then, various similar cases have been reported under various terminologies like odontogenesis dysplasia, odontogenesis imperfecta (Chaudhry et al., 1961), unilateral dental malformations, non-hereditary segmental amelogenesis imperfecta, and ghost teeth. [3] In 1954, the term "shell teeth" was introduced by Rushton to describe the radiographic findings of the condition. In 1963, Zegarelli and Kutscher first coined the term "odontodysplasia" and the prefix "regional" was added by Pindborg in 1970 as the condition affects several adjacent teeth in a particular segment. Since then, the term "regional odontodysplasia" has become the accepted terminology. [4]

The prevalence of this condition is unknown since most articles in the literature on this condition are in the form of case reports. According to the literature review, females are observed to have more predilection towards this condition (1.4:1) and there is no association with race. [5] The condition can affect both primary and permanent dentition. If primary teeth are affected, the permanent teeth are affected as well. However, the permanent first molars are affected only if the adjacent primary molars exhibit RO. The condition is usually unilateral although exceptions can be found. It is usually localized in one arch, with higher incidence in the maxilla, although there have been case reports showing involvement of both the arches. [6],[7],[8],[9],[10]

 Etiology and Etiopathogenesis

The etiology for this condition still remains uncertain. Various factors have been attributed. According to one theory, there can be somatic mutation in the early development. Another theory suggests there could be vascular disturbances creating local ischemia. This theory is most accepted as there have been many cases reported with vascular nevi or hemangiomas on the affected side. [1],[3] Also, it has been reported that there is presence of vascular inclusions, hemorrhage, and calcified red blood cells on the enamel surfaces. [11] Moreover, ligation or resection of various head and neck arteries in experimental animals has also resulted in similar changes in the dental hard tissues. [12] Hence, local circulatory disturbances can create local ischemia affecting the odontogenesis. However, a neural disorder cannot be ruled out. It is suggested that there can be local neural disorder in the form of failure of the neural crest cells to migrate. Other possible causes include local trauma, irradiation, neural damage, hyperpyrexia, metabolic and nutritional disorders, vitamin deficiencies, latent viral infection in the odontogenic epithelium, pharmacotherapy during pregnancy, and syndromal involvement. [3]

The expected etiopathogenesis could be excessive expression of matrix metalloproteinases (MMPs) as suggested by Courson et al. These enzymes are essential during tooth morphogenesis. MMP-1 plays an important role in bone and tooth root resorption during orthodontic treatment and MMP-2 is capable of cleaving amelogenin (protein of enamel matrix). According to the study conducted by Crouson et al., there is increase in the levels of these MMPs and their natural inhibitors (TIMP-1 and -2) in RO. However, the parallel increase in the natural inhibitors is not enough to inhibit the enzymatic activity of MMP, resulting in tissue destruction. The mechanism involved in increasing these enzymes remains uncertain. [13]

 Criteria for Diagnosis

The diagnosis of RO is based on clinical, radiographic, and histologic findings. Generally, the affected teeth fail to erupt, but when erupted, they are small, hypoplastic, or hypocalcified with deep pits and clefts which could be connecting the pulp [Figure 1]. There can be abscess or fistulae even in the absence of caries. Radiographically, the enamel and dentin of the affected teeth are thin compared to their normal counterparts, giving the teeth ghost-like appearance [Figure 2]. The pulp chamber can be enlarged with open apices. [14] The affected teeth are usually associated with pulp stones. Histologically, the enamel prisms are irregular, lack prismatic structure, and are hypoplastic containing degenerated globular calcifications. The dentin is thin with reduced number of tubules which are tortuous in shape. Histologic features of pericoronal dental follicle have been described by Gardner and Sapp. Two types of calcifications have been noted. Type A calcifications are multiple and irregularly set in a cellular fibrous connective tissue, and often form whorls in concentric pattern. Type B calcifications are larger, consisting of mineralized fine fibrillar matrix. [15] Schmid and Meier reported that even the root cementum was hypoplastic. [16]{Figure 1}{Figure 2}

Microanalysis studies have estimated that Mg/Ca and Na/Ca ratios are higher in RO enamel crystallite. Mg is known to inhibit Ca and P precipitation and crystal growth. Hence, increased levels of Mg could be responsible for the hypoplastic enamel in RO. Also, there is higher concentration of carbonate in RO crystallites. Carbonate substitutes phosphates in the crystallites, resulting in an unstable crystal lattice. These factors probably explain the reasons for poor crystalline structure of RO enamel. [11]

 Controversies Over The Treatment of RO

Controversies exist regarding the treatment of RO, as to whether to retain the affected teeth or to extract them. Various factors have to be taken into consideration before deciding the treatment plan, such as age of the patient, any relevant medical history, previous dental experience, child's and parental attitude regarding dental treatment, and the number of affected teeth. While a number of clinicians suggest immediate extraction followed by denture fabrication, some others suggest retaining and restoring the affected teeth. Immediate extraction is suggested as bacterial invasion cannot be prevented due to defective enamel and dentin, which can lead to pulpal necrosis and facial cellulitis. [17] Early extraction of the teeth is avoided as this would lead to substantial reduction in the alveolar ridge height and mass, and subsequent defect would pose immense problems for future restorations. [18] In addition, loss of vertical dimension on the affected side might lead to defective jaw development and subsequent facial asymmetry. Ideally, as many affected teeth as possible should be preserved to retain normal jaw development, especially in very young children. This would retain the normal jaw development and lessen the psychological effects. It is preferable to extract only the abscessed teeth, while retaining the others. [3] As the general quality of bone is not affected in RO, implants can be used in these cases. However, care has to be taken as bone density can be less around the affected teeth. The treatment maneuver can be divided into the following methods.

Conservative approach

Literature review [Table 1] suggests various attempts of treating the affected teeth. The different treatment options can be protective and restorative of the affected teeth. Although pit and fissure sealants can be used to seal the deep clefts, their use is controversial due the requirement to use the etchant prior to sealant application. In severe cases, however, full coverage crowns are recommended. Pulpal procedures like pulpotomy can be performed. Crown lengthening procedures can be performed in the affected area to achieve favorable restorations. Literature review suggests adopting conservative and multidisciplinary treatment approach. The various treatments performed by Melamed et al. included extraction of abscessed tooth, crown lengthening of the other affected teeth, and orthodontic treatment in the form of expansion and intrusion of extruded mandibular incisors by using utility arch. Successful achievement of apexification of the affected teeth is also suggested. [19] Hence, it is suggested to adopt a multidisciplinary treatment approach to meet the challenges offered by RO.{Table 1}

Radicular approach

Extraction of the affected teeth

This includes immediate extraction of the affected teeth. This is indicated in the conditions that present with acute abscess and pain. This is done to prevent development of facial cellulitis. This should be followed by use of removable partial denture (RPD) till the implant can be placed. RPD maintains the space, provides function and esthetics, maintains the normal vertical dimensions, and has psychological benefit.


Autotransplantation can be also be considered as one of the treatment options, particularly in cases that present with dentoalveolar discrepancy in the unaffected side. In such conditions, extraction of the affected teeth can be immediately followed by autotransplantation of the teeth from unaffected side with dentoalveolar discrepancy. [5]


RO is rather a rare developmental entity affecting both enamel and dentin of a group of teeth. In some form of odontodysplasia, skipping of teeth can be seen (an intact tooth between the affected ones). It is also found to be associated with taurodontism and hypodontia. [29] Other conditions such as dentin dysplasia, shell teeth, hypophosphatasia, dentinogenesis imperfecta, and amelogenesia imperfecta can mimic some features of RO. However, these disorders tend to affect the entire dentition. [30] According to the literature, this condition is mainly seen in females; however, according to the observation of data collected, both males and females seem to have similar prevalence.

One interesting finding in these children is the frequency of blood aspiration during routine injection procedures. Guzman et al. reported the case with hematoma formation subsequent to infiltration anesthesia administration. This again supports strongly the association with RO and presence of some vascular abnormality. [8]

There is an increased recognition of this condition by the clinicians, which helps them to achieve success in both short- and long-term management. However, controversies exist over treatment planning in RO. The goals of the treatment should be to maintain esthetics and masticatory function, avoid overeruption of opposing teeth and thereby maintain the normal vertical dimensions, maintain space, prevent development of deleterious habits, and allow for growth of alveolar ridge so as to allow for favorable future prosthetic rehabilitations. Different schools of thought exist over the treatment of this condition. According to the review of literature, most authors recommend extraction of the affected teeth as soon as it is diagnosed to prevent development of dental abscess and gingival swelling. However, extraction of the affected teeth should not be the only treatment option. Cases presenting with acute infection of the affected teeth will inevitably require extraction.


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