Enamel thickness and three-dimensional reconstruction in Enamel Hypoplasia related to Enamel Renal Syndrome.

Objectives: Enamel Renal Syndrome (ERS) is a rare autosomal recessive disorder presenting with hypoplastic amelogenesis imperfecta (AI), failed tooth eruption, intra-pulpal calcifications, gingival enlargement and nephrocalcinosis. The hypoplastic enamel in ERS is caused by the reduced secretion of the extracellular matrix. The study aimed to investigate enamel thickness and create a three-dimensional reconstruction in teeth that failed to erupt in ERS patients as part of a comprehensive management plan. The study was part of a project aimed at developing management guidelines for ERS in Low- and Middle-Income Countries and was approved and granted ethics reference number BM21/7/16 by the Biomedical Science Research Ethics Committee of the University of the Western Cape.
Methods: The teeth were rinsed with normal saline and stored in a 10% formalin solution. Stereomicroscopy was utilized in selection of teeth with minimal procedural damage for nano CT scanning. Three teeth were selected for further scanning with a nano CT.
The samples were scanned with a Phoenix nanotom S (General Electric Sensing and Inspection Technologies/Phoenix X-ray,Wunstorff Germany ,and the image and videography processed using the image processing software VGSTUDIO MAX 3.5 software (Volume Graphics GmbH, Heidelberg, Germany).
Results: The investigation were conducted for the appearance of enamel, volume of enamel cap and average enamel thickness. A spectrum of enamel appearance was demonstrated, ranging from rough, pitted, and globular. All samples displayed a reduced enamel thickness.
Conclusions: The unerupted teeth presented with an enamel cap of significantly reduces thickness, and volume. This may lead to difficulties and limitations during planning of restorative interventions.