PMC10067985_01
PMC10067985_02
(A to C) Facial and profile photos, left, and right; (D) Hypertelorism, convergent strabismus, broad nasal bridge, low implantation of malformed ears, extra tubercle at the top of the left ear, and straight profile; (E) Bilateral ectrodactyly in the hands; (F) Complete absence of fingers two, three, and four of both feet
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F1
At the extraoral examination, it was observed that the frontal facial analysis (Fig. 1B) showed a correct proportion of the upper, middle, and lower facial thirds; slightly deficient zygomatic projection; right and left ear shells lateralization; prominent ears; the presence of an extra tubercle at the top of the left ear; left converging right eye strabismus; bilateral epicantal folds; and broad nasal bridge. Lateral analysis (Figs 1A and C) showed a straight profile, both suitable chin-neckline, and angle of chin-neckline. In the right hand (Fig. 1E), there is a total absence of finger three. The left hand (Fig. 1D) presents all the fingers but shows the malformation of the distal phalanges of fingers three and four. Figure 1F shows the complete absence of fingers two, three, and four of both feet.
CBCT with the absence of tooth germs 1, 4, 5, 8 or 9, 12, 13, 16, 17, 18, 20, 29, 31, and 32: (A) Mandibular front view; (B) Mandibular axial view; (C) Upper panoramic view; (D) Lower panoramic view
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F3
According to cone beam computed tomography (CBCT), 13 dental ageneses were identified (Figs 3C and D):upper central incisor (eight or nine), upper first premolars (5 and 12), upper and lower second premolars (4, 13, 20, and 29), lower second molars (31 and 18), and upper and lower third molars (1, 16, 17, and 32), characterizing oligodontia. Figures 3A and B show tomography images with the position of the permanent lower canines in buccally displaced.
SMMCI in relation to the midpalatal raphe: (A to E) The images show the position of the upper canines in lingual displace
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F4
Figures 4B,D, and E show the position of this tooth practically in the midpalatal raphe, equidistant from the right and left upper lateral incisors. Regarding its shape (Figs 4A and C), we find it difficult to determine whether this incisor is tooth eight or nine, with very similar mesiobuccal and distovestibular angles and a malformation in the middle of the incisal (invagination of the incisal enamel). Figure 4D shows tomography images with the position of the upper canines in lingual displace.