PMC6942748_01
(a) Panoramic radiograph showing the multiple odontogenic keratocysts in the maxilla and mandible. (b) Computed tomography of the jaw.
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fig1
On the panoramic radiograph, multiple radiolucent areas, causing dental dislocation, were observed (Figure 1(a)). With the objective of obtaining the most precise limits of the multiple lesions, computed tomography was done (Figure 1(b)). After this clinical evaluation, three major criteria were found: odontogenic keratocysts (Figure 1), bifid ribs (Figure 2(a)), and palmoplantar pits (Figures 2(b) and 2(c)), along with 10 minor criteria: hypertelorism (Figure 3(a)), falx cerebri calcification (Figure 3(b)), macrocephaly (Figure 3(c)), bifid uvula (Figure 3(d)), frontal bossing (Figure 4(a)), malocclusion (Figure 4(b)), shortened fourth metacarpal (Figure 4(c)), cardiac fibroma (Figure 4(d)), ectopic dental position, and precocious development of genitals.
(a) Bifid rib; (b, c) palmoplantar pits.
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fig2
After this clinical evaluation, three major criteria were found: odontogenic keratocysts (Figure 1), bifid ribs (Figure 2(a)), and palmoplantar pits (Figures 2(b) and 2(c)), along with 10 minor criteria: hypertelorism (Figure 3(a)), falx cerebri calcification (Figure 3(b)), macrocephaly (Figure 3(c)), bifid uvula (Figure 3(d)), frontal bossing (Figure 4(a)), malocclusion (Figure 4(b)), shortened fourth metacarpal (Figure 4(c)), cardiac fibroma (Figure 4(d)), ectopic dental position, and precocious development of genitals.
(a) Hypertelorism; (b) falx cerebri calcification; (c) macrocephaly; (d) bifid uvula.
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fig3
After this clinical evaluation, three major criteria were found: odontogenic keratocysts (Figure 1), bifid ribs (Figure 2(a)), and palmoplantar pits (Figures 2(b) and 2(c)), along with 10 minor criteria: hypertelorism (Figure 3(a)), falx cerebri calcification (Figure 3(b)), macrocephaly (Figure 3(c)), bifid uvula (Figure 3(d)), frontal bossing (Figure 4(a)), malocclusion (Figure 4(b)), shortened fourth metacarpal (Figure 4(c)), cardiac fibroma (Figure 4(d)), ectopic dental position, and precocious development of genitals.
(a) Frontal bossing; (b) malocclusion; (c) shortened fourth metacarpal; (d) cardiac fibroma.
CRID2019-1608783.004.jpg
PMC6942748_01_CRID2019-1608783.004.jpg
fig4
After this clinical evaluation, three major criteria were found: odontogenic keratocysts (Figure 1), bifid ribs (Figure 2(a)), and palmoplantar pits (Figures 2(b) and 2(c)), along with 10 minor criteria: hypertelorism (Figure 3(a)), falx cerebri calcification (Figure 3(b)), macrocephaly (Figure 3(c)), bifid uvula (Figure 3(d)), frontal bossing (Figure 4(a)), malocclusion (Figure 4(b)), shortened fourth metacarpal (Figure 4(c)), cardiac fibroma (Figure 4(d)), ectopic dental position, and precocious development of genitals.