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Dentin dysplasia type I: a case report and review of the literature.

Toomarian L, Mashhadiabbas F, Mirkarimi M, Mehrdad L - J Med Case Rep (2010)

Bottom Line: There are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia.The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described.Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.

View Article: PubMed Central - HTML - PubMed

Affiliation: Pediatric Department, Zahedan University of Medical Sciences, Zahedan, Iran. mirkarimi200@yahoo.com.

ABSTRACT

Introduction: Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and peri-apical radiolucencies of non carious teeth.

Case presentation: We present a case of dentin dysplasia type I in a 12-year-old Iranian boy, and the clinical, radiographic and histopathologic findings of this condition and treatment are described.

Conclusions: There are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia. The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described. Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.

No MeSH data available.


Related in: MedlinePlus

In histopathologic examination, a variable thickness of non-keratinized stratified squamous epithelium with arch-shaped appearance is evident (×40).
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Figure 5: In histopathologic examination, a variable thickness of non-keratinized stratified squamous epithelium with arch-shaped appearance is evident (×40).

Mentions: The cystic lesion was enucleated and sent for histopathological examination. Based on the pedodontist's and surgeon's view, it was decided to extract the remaining root of the lower left first permanent molar during surgery because of extensive caries. The histopathological features were consistent with the clinical diagnosis of a radicular cyst. The cystic cavity was lined with a variable thickness of non keratinized stratified squamous epithelium with arch-shaped appearance and exocytosis in the underlying connective tissue was severely infiltrated by chronic inflammatory cells. Extravasated red blood cells and hemosiderin pigments were also seen (Figure 5). The extracted primary teeth were sent for histological examination. The ground section was examined with a stereomicroscope: the superficial dentin of the crown appeared normal, but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin, and deeper layers of dentin had an atypical tubular pattern (Figure 6). These features are consistent with those of DD type I, confirming the diagnosis based on the clinical and radiographic features. It is anticipated that more permanent teeth may be lost due to severe mobility and may undergo spontaneous pulpal necrosis. The possibility of endosseous implants is being explored for when the patient reaches his late teens and growth is complete.


Dentin dysplasia type I: a case report and review of the literature.

Toomarian L, Mashhadiabbas F, Mirkarimi M, Mehrdad L - J Med Case Rep (2010)

In histopathologic examination, a variable thickness of non-keratinized stratified squamous epithelium with arch-shaped appearance is evident (×40).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2823758&req=5

Figure 5: In histopathologic examination, a variable thickness of non-keratinized stratified squamous epithelium with arch-shaped appearance is evident (×40).
Mentions: The cystic lesion was enucleated and sent for histopathological examination. Based on the pedodontist's and surgeon's view, it was decided to extract the remaining root of the lower left first permanent molar during surgery because of extensive caries. The histopathological features were consistent with the clinical diagnosis of a radicular cyst. The cystic cavity was lined with a variable thickness of non keratinized stratified squamous epithelium with arch-shaped appearance and exocytosis in the underlying connective tissue was severely infiltrated by chronic inflammatory cells. Extravasated red blood cells and hemosiderin pigments were also seen (Figure 5). The extracted primary teeth were sent for histological examination. The ground section was examined with a stereomicroscope: the superficial dentin of the crown appeared normal, but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin, and deeper layers of dentin had an atypical tubular pattern (Figure 6). These features are consistent with those of DD type I, confirming the diagnosis based on the clinical and radiographic features. It is anticipated that more permanent teeth may be lost due to severe mobility and may undergo spontaneous pulpal necrosis. The possibility of endosseous implants is being explored for when the patient reaches his late teens and growth is complete.

Bottom Line: There are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia.The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described.Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.

View Article: PubMed Central - HTML - PubMed

Affiliation: Pediatric Department, Zahedan University of Medical Sciences, Zahedan, Iran. mirkarimi200@yahoo.com.

ABSTRACT

Introduction: Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and peri-apical radiolucencies of non carious teeth.

Case presentation: We present a case of dentin dysplasia type I in a 12-year-old Iranian boy, and the clinical, radiographic and histopathologic findings of this condition and treatment are described.

Conclusions: There are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia. The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described. Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.

No MeSH data available.


Related in: MedlinePlus