PMC2842968_01
Embryotoxon posterius ( ) and hypoplasia of the iris in patient 1.
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fig2
The patient presented with embryotoxon posterius, iridocorneal adhesions, and hypoplasia of the iris with the sphincter muscle visible in both eyes (Figure 2).
PMC2842968_02
OTP (11 years 3 months) showing agenesis of the upper lateral incisors, taurodontism of the molars, and microdontia with crown hypoplasia of the upper central incisors and congenital missing 18 and 28 in patient 2.
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fig3
Agenesis of the upper lateral incisors, taurodontism, microdontia as well a crown hypoplasia of the upper central incisors was present (Figure 3).
PMC2842968_03
Corectopia ( ) and hypoplasia (  ) of the iris in patient 3.
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fig4
An ophthalmologic examination identified embryotoxon posterius, iridocorneal adhesions, corectopia, and hypoplasia of the iris visible in both eyes (Figure 4).
(a) OTP (15 years) showing agenesis of 18 and 15,12, 22, 23, 25, 35, 45; hypoplasia of the crowns of the upper central incisors in patient 3. (b) Severe root resorptions of the lower incisors and the lower first molars in patient 3 after correction of the skeletal Class III relationship by means of combined orthodontic-orthognathic therapy.
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fig5
Moreover, a very rare agenesis of the upper left canine was evident (Figure 5(a)).
Lateral cephalogram of patient 3 showing a skeletal Class III relationship.
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fig6
In contrast, the maxilla showed a significant retrognathia resulting in a severe skeletal Class III relationship (Figure 6).
PMC2842968_04
Protuberant umbilicus in patient 4.
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fig7
The patient has a protuberant umbilicus (Figure 7).
(a) OPT (6 years and 4 months) showing agenesis of upper molars in patient 4. (b) OPT (18 years) showing taurodontism of upper and lower molars and paraplasia on the distal aspect of the right upper third molar in patient 4.
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fig8
Besides agenesis of the lower right second premolar, the orthopantomogram showed a very rare instance of two missing upper molars (Figure 8(a)). Moreover, taurodontism of the upper and lower molars with variable degree was evident, as well as a paraplasia on the distal aspect of the upper third molar root (Figure 8(b)).
Frequency of agenesis of permanent upper teeth in 15 patients with ARS and hypodontia.
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fig9
Teeth of the upper jaw tended to be missing more frequently (Figures 9 and 10). 
Frequency of agenesis of permanent lower teeth in 15 patients with ARS and hypodontia.
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fig10
Teeth of the upper jaw tended to be missing more frequently (Figures 9 and 10). 