PMC3586467_01
(a) Case 1. Oral condition of GOD at time of presentation. (b) Panoramic view showing ghost-shaped teeth, enlarged pulp chambers and root canals, and short roots and open apices. (c) Lateral cephalometric view depicting dolichocephaly. (d) Bilateral clinodactyly of the little fingers. (e) Multiple nevi on the neck.
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Microdontia was also evident (Figure 1(a)). The roots had enlarged pulp chambers and root canals, short roots, and open apices (Figure 1(b)). The patient was dolichocephalic (Figure 1(c)). He had clinodactyly and multiple nevi on both his neck and back (Figures 1(d) and 1(e)).
(a) Histological examination showing dentin having torturous dentinal tubules and globular masses of dentine interrupting them (original magnification 20x). (b) Gingival biopsy examination showing odontogenic tissue in the epithelium and intramesenchymal calcifications (original magnification 20x). (c) Panoramic view showing apexification trial. (d) RCT without crowning was done for incisors and upper-right second molar. RCT with crowning was performed for upper-right second premolar and first molar and upper-left first premolar. Crowning without RCT was done for the vital upper-left premolars, left second molar, lower left second premolar, lower left second molar, lower canines, right first premolar, and upper-right first molar. (e) Final oral condition at 18 years of age.
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Radiolucency of the upper-right incisors and the lower-right lateral incisor persisted, although the radiolucent area was markedly smaller in the lower-right lateral incisor (Figures 2(d) and 2(e)).
PMC3586467_02
(a) Histological examination showing dentin having torturous dentinal tubules and globular masses of dentine interrupting them (original magnification 20x). (b) Gingival biopsy examination showing odontogenic tissue in the epithelium and intramesenchymal calcifications (original magnification 20x). (c) Panoramic view showing apexification trial. (d) RCT without crowning was done for incisors and upper-right second molar. RCT with crowning was performed for upper-right second premolar and first molar and upper-left first premolar. Crowning without RCT was done for the vital upper-left premolars, left second molar, lower left second premolar, lower left second molar, lower canines, right first premolar, and upper-right first molar. (e) Final oral condition at 18 years of age.
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Radiolucency of the upper-right incisors and the lower-right lateral incisor persisted, although the radiolucent area was markedly smaller in the lower-right lateral incisor (Figures 2(d) and 2(e)).
PMC3586467_03
(a) Case 2. Oral condition of ROD of the upper-left quadrant at time of presentation. (b) Panoramic view of Case 2 showing multiple impacted teeth in the upper left quadrant and a missing lower right first premolar. (c) Lateral cephalometric view depicting dolichocephaly. (d) Panoramic view of Case 2 twin brother showing a missing lower left first premolar.
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At the affected area, the gingiva was enlarged, fibrous, and tense (Figure 3(a)). Dolichocephaly was noted during the extraoral examination (Figure 3(c)), and there was no evidence of multiple nevi on the skin. A panoramic radiograph revealed multiple impacted teeth in the upper-left quadrant and a missing lower-right first molar (Figure 3(b)). The patient had a twin brother whose teeth had erupted normally, except for the lower-first molar on the left side, which was congenitally absent (Figure 3(d)).
(a) Vacuum formed tray for the lower jaw to prevent overeruption of lower teeth. (b) Surgical exposure of the impacted teeth. (c) Panoramic view for the case after surgical exposure and placement of the porcelain fused to metal bridge. (d) Final oral condition at 17 years of age.
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Surgical exposure of the impacted teeth was performed, followed by porcelain-fused-to-metal fixed restoration as a temporary aesthetic solution (Figure 4(b)). Three years later, the gingiva at the restoration (as in Case 1) showed signs of inflammation, and a panoramic radiograph showed asymptomatic periapical radiolucency in the upper-left first premolar (Figures 4(c) and 4(d)).
PMC3586467_04
(a) Vacuum formed tray for the lower jaw to prevent overeruption of lower teeth. (b) Surgical exposure of the impacted teeth. (c) Panoramic view for the case after surgical exposure and placement of the porcelain fused to metal bridge. (d) Final oral condition at 17 years of age.
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fig4
Surgical exposure of the impacted teeth was performed, followed by porcelain-fused-to-metal fixed restoration as a temporary aesthetic solution (Figure 4(b)). Three years later, the gingiva at the restoration (as in Case 1) showed signs of inflammation, and a panoramic radiograph showed asymptomatic periapical radiolucency in the upper-left first premolar (Figures 4(c) and 4(d)).