Dentomaxillofacial Radiology (2008) 37, 240244 2008 The British Institute of Radiology http://dmfr.birjournals.

org

CASE REPORT

Multiple enamel pearls in two siblings detected by volumetric computed tomography

A rare case of multiple enamel pearl formation is presented involving the maxillary molars in two siblings incidentally recognized during volumetric CT examination. Although the pathogenesis of ectopic enamel formation is not known, possible mechanisms to account for this phenomenon are discussed in the context of current knowledge regarding root genesis. The radiographic presentation of enamel pearls and its clinical significance is also discussed. The observation of multiple enamel pearls in two siblings raises the possibility of a hereditary association in the formation of enamel pearls. Dentomaxillofacial Radiology (2008) 37, 240244. doi: 10.1259/dmfr/86859829 Keywords: enamel pearls; volumetric tomgraphy; enameloma; tooth

Introduction The phenomenon of ectopic development of enamel on the root surface, variedly referred to as enameloma, enamel pearl, enamel drop or enamel nodule, is not well understood.1,2 The use of the term enameloma is discouraged as it connotes a neoplastic process for a lesion that is more consistent with a form of heterotopic enamel development.3 The enamel pearl is distinguished from enamel extensions, which are tongue-like linear continuations of cervical enamel over the root surface. These enamel extensions are structurally different from enamel pearls, although in some cases continuity may be identified between enamel pearls and the cementoenamel junction simulating an extension.4 Enamel pearls may consist entirely of enamel connected to cementum or root dentin, or may show incorporation of a cone of dentin with or without pulpal extension; the last two are referred to as composite enamel pearls.5 Free enamel pearls, consisting entirely of enamel, may be found in the periodontal ligament space. An intradental variety has also been described, characterized by an enamel nodule that is totally surrounded by dentin.5 Risnes1 observed enamel pearls on 2.28% molars of 8854 teeth examined grossly. Turner6 reported an
*Correspondence to: Dr Tarnjit Saini, Department of General Dentistry, Creighton University Medical Center, School of Dentistry, Omaha, NE 68178, USA; E-mail: [email protected] Received 2 February 2007; revised 18 July 2007; accepted 23 July 2007

incidence of 0.2% for maxillary molars and 0.03% for mandibular molars. The common site of location of the enamel pearl is adjacent to the furcation or furrow of the root, especially the bifurcation or trifurcation areas of maxillary and mandibular molars.2 The distal proximal surfaces of the maxillary molars and the buccal or lingual surfaces of the mandibular molars are the preferred sites of occurrence.7 Maxillary second and third molars are more commonly involved than the first molars.8 Enamel pearls rarely occur on premolars or anterior teeth.9 The size of clinically recognizable enamel pearls may vary from 0.3 mm to 4 mm, with the mean diameter reportedly 0.960.43 mm.7 The average distance of the enamel pearl from the cementoenamel junction was found to be 2.81.00 mm.7 Brabant10 did not observe enamelomas or enamel extensions on primary teeth, although Arys and Dourov11 reported microscopic evidence of ectopic enamel formation in 33% of primary molars examined. Pederson12 reported varying prevalence rates among ethnic populations, with a markedly higher occurrence in the Eskimo population (9.7%). Multiple pearls on a single tooth have been reported, but the occurrence of more than two pearls on a single tooth is a rare phenomenon.5 Involvement of multiple teeth is even less common. We report the occurrence of bilateral enamel pearls involving multiple maxillary molars at identical sites in two female siblings, raising

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the possibility of a genetic association, noted during three-dimensional volumetric CT examination of the maxillofacial structures.

Case report A 19-year-old female patient (Subject A) presented to the Maxillofacial Imaging Center at the Creighton University School of Dentistry with a history of facial trauma. Volumetric CT of the maxillofacial region was conducted with an i-CAT (Imaging Sciences, Hatfield, PA) unit. The exposure parameters used to acquire the images were 120 kVp, 23 mAs, 17613 cm cylindrical field of view and voxel size of 0.3 mm. The study incidentally revealed the presence of bilateral enamel pearls involving all maxillary molars with the exception of the left first molar. The enamel pearls were easily recognized on the root surfaces due to the presence of high-density ectopic enamel compared to radicular dentin or surrounding alveolar bone (Figure 1). All enamel pearls were located near the distal furcation between the disto-buccal and palatal roots of the maxillary molars (Table 1). A well-defined, corticated, linear radiolucent rim, consistent with the periodontal ligament space, was observed around the periphery of all enamel pearls except in those cases where the enamel pearl was close to the alveolar crest. The patient had two siblings, Subjects B and C, who also underwent volumetric tomography of the maxillofacial structures. Enamel pearls were identified in the 18-year-old sister (Subject B), who showed bilateral involvement of the first and second molars involving distal furcation between disto-buccal and palatal roots (Figure 2). The sole exception was the right maxillary

second molar, in which the enamel pearl was located on the palatal surface of the palatal root. The roots of the involved molars in Subjects A and B had normal divergence. In the case of Subject B, the roots of the second molars were fused. Subject C, the 17-year-old brother of the proband, did not show any radiographic evidence of enamel pearl formation. The distance of the enamel pearls from the cementoenamel junction varied from 1.8 mm to 5.1 mm. The measurements were made from the reconstructed images in axial, sagittal and coronal sections employing the XoranCAT software (Xoran Technologies, Ann Arbor, MI). The level of the alveolar crest in relation to the enamel pearls varied from 0 mm to 3.9 mm. Differences in the radiographic density of enamel pearls were not related to their size or location. The presence of homogeneous radiographic density was taken as an indication that the enamel pearl was composed entirely of enamel whereas a pattern of heterogeneous density was interpreted to indicate the composite nature of the anomaly.

Discussion The formation of ectopic enamel requires the presence of differentiated ameloblasts apical to the cementoenamel junction. In humans, Hertwigs epithelial root sheath (HERS) or its residues, the epithelial rests of Malassez, have been implicated as the likely sources of ectopic ameloblasts.13 During crown formation, the differentiation of highly specialized dental cells such as ameloblasts and odontoblasts requires reciprocal spatial and temporal induction between odontogenic epithelium and ectomesenchymal

Figure 1 12 mm thick corrected sagittal sections of the maxillofacial regions displayed in maximum intensity projection (MIP) mode of (a) the right and (b) left side of Subject A, showing the presence of enamel pearls (arrows). (a) Note the presence of a tongue ring overlapping the right mandibular canine
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Table 1

Multiple enamel pearls in two siblings T Saini et al

Consensus morphometric measurement of enamel pearls Distance from Mesiodistal Buccolingual Inferio-superior Distance from alveolar crest width (mm) width (mm) height (mm) CEJ (mm) (mm) Location 2.1 3.0 2.4 1.8 3.4 1.8 3.0 1.8 1.5 2.4 2.4 2.1 2.4 2.4 2.0 3.3 2.1 1.8 3.0 2.4 2.0 2.7 2.4 2.4 2.4 2.7 1.8 2.4 1.9 5.1 2.7 4.5 3.0 2.1 1.8 2.1 0.0 2.7 3.9 2.4 1.8 0.0 0.0 2.0 0.0 Distal furcation Distal furcation Distal furcation Distal furcation Distal bfurcation Palatal to palatal root Distal furcation Distal furcation Distal furcation Radiographic density Heterogeneous Heterogeneous Homogeneous Homogeneous Heterogeneous Homogeneous Heterogeneous Homogeneous Homogeneous

Tooth Subject number* A 18 17 16 27 28 18 17 27 28

Roots Diverging Diverging Diverging Diverging Diverging Diverging Fused Fused Diverging

*FDI (Federation Dentaire Internationale) World Dental Federation ISO-3950 notation. CEJ, cementoenamel junction

cells of the dental papilla. This process is mediated by growth factors, components of the basement membrane and adhesion molecules/receptors. The exact mechanism of induction is not clear, but the role of the inner enamel epithelium (IEE) basement membrane in mediating signals for odontoblastic differentiation is well recognized.14 Different theories have been proposed to explain the ectopic presence of enamel pearls. One suggestion is that the inner cell layer of HERS fails to detach from the newly formed dentin matrix, resulting in ameloblastic differentiation and formation of ectopic enamel.3 Since HERS cells in mouse teeth remain in contact with the newly formed dentin matrix for a longer period as compared with human teeth, it might be expected that the mouse teeth would show higher incidence of enamel pearls. However, to our knowledge this has not been demonstrated Stone9 postulated that the budding of the cervical loop cells result in enamel pearl formation. It has also been proposed that the quiescent cells of the rests of Malassez may differentiate into ameloblasts and give rise to ectopic enamel formation in the PDL space.13 Some of these free enamel pearls may later form a cemental union with the root surface.15 Kalnins13 demonstrated that under experimental conditions intended to replicate occlusal trauma to the developing mouse teeth, the ameloblasts were found to detach from the enamel matrix and form ectopic enamel. In this scenario, the cells forming free enamel pearls would not require the presence of odontoblasts or dentin matrix for ameloblastic differentiation as they had previously been in contact with dentin matrix. Free enamel pearls have been demonstrated in scorbutic guinea pigs, pigs with fluorosis and in vitamin A-deficient rats.13 The intradental variety of the enamel pearl may form due to incorporation of epithelial cells into the dentin matrix of the papilla prior to the onset of mineralization. The cells may originate from the IEE or the transient enamel knot present during bell stage, the latter of which is responsible for the morphogenesis of the occlusal anatomy.16 It should be emphasised that in root genesis the dentin matrix does not commence mineralization until the deposition of a cemental matrix and enmeshing of the newly laid
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collagen fibrils of cemental and dentin origin at the site of the future cementodentinal junction.14 This may provide an opportunity for entrapment of the epithelial cells. Garant17 described the presence of enamel and ameloblasts on the external surface of the pulp chamber near the bifurcation. He proposed that ameloblastic differentiation occurs during fusion of thepithelial process to form multirooted teeth. These epithelial processes are formed by unequal extensions of the apical rim of the enamel organ. It can be speculated that they may possess potential for amelogenesis due to their origin as a continuity of the IEE, as compared to HERS cells which are formed later by the cervical loop proliferation. Enamel pearls should be distinguished from enamel projections, which are tongue-like radicular extensions from the cementoenamel junction commonly seen near the bifurcation of the second mandibular molars.4 Pindborg3 pointed out that the orientation of the cementoenamel border varies with race. Eskimos, Lapps and American Indians show more frequent extensions in relation to root bifurcations and furrows. The enamel rods do not reach the surface of the enamel extension. The superficial layer of the extensions consists of thick aprismatic enamel similar to the cervical enamel.18 Their presence on the root surface increases susceptibility to periodontal pocket formation.4 Enamel pearls occur more apically than enamel extensions. The architecture of the enamel in the pearl is similar to the occlusal enamel, with some variations in the direction of enamel rods, interprismatic substance, and the presence of HunterShreger bands.19 Enamel pearls may show a superficial covering of the enamel by acellular cementum20 that may facilitate attachment of the periodontal ligament fibres and formation of a regular periodontal ligament space around the pearl. The quality of the enamel in the enamel pearls has been studied, demonstrating areas of hypomineralization and the presence of superficial concavities filled with organic matter on the enamel surface.21 The microhardness of enamel in the larger diameter pearls was similar to that of occlusal enamel.22 The dentin core in composite enamel pearls is

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Figure 2 12 mm thick corrected sagittal sections of the maxillofacial regions displayed in MIP mode of the (a) right and (b) left side of Subject B showing the presence of enamel pearls (arrows)

continuous with dentin of the carrier root and contains large areas of interglobular dentin.21 The classical morphologic appearance of the enamel pearl is characterized by a sessile fusion with the root dentin. In one study, a root-like connecting pedicle was demonstrated between the pearls and the dentin of carrier roots.22 Interestingly, the root pedicle of the pearls exhibited a normal distribution of cementum and Sharpeys fibres similar to the root of carrier teeth. This finding supports the postulation that HERS forms a new enamel organ, resulting in the formation of an extra tooth-like structure fused to the root of carrier tooth.22 Worth2 had previously suggested that composite enamel pearls containing pulpal tissue should be considered a form of geminated tooth. Enamel pearls are incidentally recognized during routine radiography as hemispherical dense opacities projecting from the boundaries of the root surface.2,8,9 They may result in deep pocket formation and may therefore be mistaken as dental calculus. The enamel pearl should be differentiated from pulp stones or composite resin-based cervical restorations. The overlap of the images of the furcation boundaries in multirooted teeth commonly simulates a circular radiopacity near the pulpal floor and may be mistaken for enamel pearls.2 A follow-up radiograph taken with a corrected angle projection will usually allow for an accurate diagnosis.8 We found that the density, architecture and location of composite enamel pearls can be easily recognized by high-resolution volumetric computed tomography. This modality also helps in assessing the distance between the enamel pearl and the alveolar crest or the furcation areas for prognostic evaluation of the future risk of periodontal bone loss. Gaspersic22 denied the role of heredity and stressed that local mechanical factors play the prime role in the genesis of enamel pearls. He further stated that the

folding of HERS near root furrows, particularly in third molars, may occur due to lack of arch space, initiating amelogenesis and ectopic enamel formation. Subjects A and B in this series showed both adequate arch space and skeletal base. The roots of the involved molars were flared and normally divergent, with the exception of the fused roots of the maxillary second molars in Subject B. The adjacent roots were not in contact with each other. Therefore, lack of arch space was not considered as a possible local aetiological factor for enamel pearl formation in this series. Pederson12 stated that the occurrence of multiple enamel pearls may be due to as yet undefined constitutional factors, implicating some unknown genetic factors. The identification of enamel pearls is an incidental finding and requires no intervention. Goldstein7 suggested that the enamel pearls have a weaker attachment to the periodontal ligament, therefore rendering these areas more prone to periodontal breakdown and pocket formation. In selective cases, one could perform odontoplasty to remove or recontour the enamel pearls. In summary, the pathogenesis of ectopic enamel pearl is not known. The inner cells of HERS are proposed to be the cause of enamel pearl formation, presumably due to a failure of the epithelial cells to detach from the newly laid dentin matrix. This process may be mediated by persistence of the basement membrane of the inner cell layer of HERS and failure of the genetic regulatory system, which is supposed to normally arrest ameloblastic differentiation in these cells. We report two cases of composite enamel pearls occurring bilaterally at identical sites, involving multiple molars, in two female siblings, suggesting the possibility of a hereditary association. Interestingly, these anomalies were first noted incidentally during three-dimensional volumetric CT examination of the maxillofacial structures.
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References
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