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Multiple macrodontic multituberculism.
Martinez R Benjamin1, Fariña S Rodrigo, Robert J Gorlin
1Oral Pathology, School of Dentistry, Universidad Mayor, Santiago, Chile.
American Journal of Medical Genetics. Part A
|July 2, 2003
Summary
Multiple macrodontia and multituberculism (E-WJ) is a rare dental anomaly. This case study highlights typical dental alterations and proposes a new name, "multiple macrodontic multituberculism," for this condition.
Area of Science:
- Dentistry
- Oral pathology
- Genetics
Background:
- Ekman-Westborg and Julin (1974) described a condition of multiple macrodontia and multituberculism affecting teeth without other anomalies.
- This condition, previously termed E-WJ, is characterized by unique dental crown alterations.
Observation:
- A case report of a 12-year-old Chilean patient presenting with typical dental alterations consistent with E-WJ.
- Histopathologic examination revealed specific findings, including the absence of a predentin layer and significantly reduced enamel epithelium.
Findings:
- The observed dental anomalies in the Chilean case align with the original description of E-WJ.
- Histopathologic findings provide further insight into the microscopic changes occurring in the tooth crowns.
Implications:
- The findings suggest that E-WJ is not a syndrome but a distinct dental anomaly.
- The proposed name "multiple macrodontic multituberculism" more accurately reflects the condition's characteristics and uncertain etiology.
- This research contributes to a better understanding of rare dental anomalies and their classification.