J Korean Acad Pediatr Dent.  2021 Feb;48(1):50-63. 10.5933/JKAPD.2021.48.1.50.

Dental Characteristics of Microcephalic Osteodysplastic Primordial Dwarfism Type II

• Park H
• Song JS
• Shin TJ
• Hyun HK
• Kim YJ
• Lee SH
• Kim JW

• KMID: 2513910
• DOI: http://doi.org/10.5933/JKAPD.2021.48.1.50

Abstract

Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is an autosomal recessive inherited disorder form of primordial dwarfism, caused by mutations in the pericentrin gene. The purpose of the study was to examine the clinical and radiological features, physicochemical properties and microstructures of the tooth affected with MOPD II.
The mandibular 2nd molar was collected from the MOPD II patient. Micro-computerized tomography, scanning electron microscopy, energy dispersive spectrometry and Vickers microhardness analysis were performed on the MOPD II and the normal control.
The morphology of the MOPD II tooth appeared to have malformed pulp and root and showed a small size. The mineral density measurement showed that the MOPD II tooth had similar scores in the enamel, but lower scores in the root 1/2 and apical dentin compared to the normal control. The microhardness values were smaller in the cusp enamel, root 1/2 dentin and apical dentin of the MOPD II compared to the normal control.
In this study, the dental characteristics and the physicochemical properties of a tooth affected with MOPD II were analyzed to improve understanding of the oral manifestations of the disease and to assist in proper dental treatment by identifying precautions.