There is literature indicating that DE is an isolated anomaly. During the bell stage of tooth formation, DE may occur as a result of an unusual growth and folding of the inner enamel epithelium and ectomesenchymal cells of dental papilla into the stellate reticulum of the enamel organ. Diagnosis
Diagnosis of DE can be difficult when there is no signs and symptoms of necrotic or infected pulp. It is a challenging task to differentiate between a true periapical lesion and a normal periapical radiolucency of a dental follicle of an immature apex. Pulp tests (test results of immature teeth can be misleading, as they are known to give unreliable results)
Check and see if there is an elevated, flat wear facet on the occlusal surface of the tooth
Test cavity which has an absence of pain sensation and has an empty pulp chamber/ canal. Radiographs (usually periapical) - a V-shaped radiopaque structure could be seen superimposing on top of the affected crown. It could detect DE before tooth eruption. However, DE presentation on the radiograph can be quite similar to a mesiodens or a compound odontoma. Classification
The anterior DE tubercles have an average width of 3.5mm and length of 6.0mm, while posterior tubercles have an average 2.0mm in width and length of up to 3.5mm. If the cusp of Carabelli is present, the tooth associated are often larger mesiodistally and it is not uncommon that a DE involved tooth has an abnormal root pattern.There are 4 different ways to classify/ categorize DE involved teeth.
Dens evaginatus is a rare odontogenic developmental anomaly that is found in teeth where the outer surface appears to form an extra bump or cusp. Premolars are more likely to be affected than any other tooth. It could occur unilaterally or bilaterally. Dens evaginatus (DE) typically occurs bilaterally and symmetrically. This may be seen more frequently in Asians (including Chinese, Malay, Thai, Japanese, Filipino and Indian populations).The prevalence of DE ranges from 0.06% to 7.7% depending on the race. It is more common in men than in women, more frequent in the mandibular teeth than the maxillary teeth. Patients with Ellis-van Creveld syndrome, incontinentia pigmenti achromians, Mohr syndrome, Rubinstein-Taybi syndrome and Sturge Weber syndrome are at a higher risk of having DE. Signs and symptoms
It is important to diagnose DE early and provide appropriate treatment to help prevent periodontal disease, caries, pulpal complications and malocclusion. It occurs on the cingulum/occlusal surface of the teeth. The extra cusp can cause occlusal interference, displace of the affected tooth and/or opposing teeth, irritates the tongue when speaking and eating and decay the developmental grooves. Temporomandibular joint pain could be experienced secondarily due to occlusal trauma caused by the tubercle.This cusp could be worn away or fractured easily. In 70% of the cases, the fine pulpal extension were exposed which can lead to infection, pulpal necrosis and periapical pathosis. Associated anomalies
Additional tubercules
Aesthetic and/or occlusion problems
Agenesis
Bifid cingula
Exaggerated cusp of Carabelli
Gemination
Impaction
Labial drifting
Labial groove
Mesiodens
Megadont
Odontoma
Peg-shaped lateral incisor
Prominent marginal ridge
Shallow groove in the lateral incisor
Shovel-shaped incisor
Supernumerary

Cause
The cause of DE is still unclear.