Marfan's syndrome was first described by Marfan in 1896, under the name dolichostenomelia. In 1902 Achard called it "arachnodactyly". This syndrome is characterized by disproportionate skeletal growth with dolichostenomelia and arachnodactyly, ectopia lentis, and dissecting aneurysm of the aorta. The oral manifestation of this syndrome, a high palatal vault, is very prevalent and may be a constant finding. Bifid uvula is also reported as well as malocclusion.
This report concerns a clinical observation of a case of Marfan's syndrome. A boy aged 5 years was examined from the dental point of view.
The findings were as follows:
1. The patient had tall and thin habitus, arachnodactyly, scoliosis producing a marked asymmetry, pectus excavatum, and a wizened facial appearance.
2. The skull and face were long and corresponding to the anthropologic concept of dolichocephaly.
3. A high palatal vault and malocclusion with deep overbite and a unilateral crossbite on the left side were found.
4. Severe dental caries were noted in all deciduous teeth.
5. The roentogenographic findings revealed that there was no demonstrable abnormality concerning bone age, and that the form, number, eruption, and development of the permanent teeth were normal.
6. As a result of cephalometric diagnosis, the mandible appeared to be prognathic and a protruded chin was noted.
7. In the findings of the maxillary and mandibular dental arches, the mesiodistal distance of each tooth crown and the length of the dental arches were within normal limits but their width was remarkably narrow.
8. The histological findings of the extracted lower left second deciduous molar were such that:
(1) there was no structural abnormality in the enamel.
(2) interglobular spaces scattered in the dentin of the pulp horn region, and the dental tubles of the innermost layer of the dentin of the roots decreased in number and ran in a disordered manner.
(3) a few, free false denticles were found in the coronal pulp chamber.
(4) remarkably thick deposition of the secondary cementum with vascular inclusions was on the bifurcation.
9. Electron probe microanalysis and microradiogram revealed normal calcification of the extracted teeth.
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