Overview

Dentin Dysplasia (DD), an uncommon autosomal dominant hereditary disorder characterised by a disturbance in dentin formation affecting either the primary (baby) or both (primary and permanent) dentitions. It has a prevalence rate of approximately one patient in every one lakh. In 1920, Ballschmiede first reported such a condition as ‘rootless teeth’, and in 1939, Rushton termed this condition as Dentin Dysplasia. Based on radiological findings, Witkop in 1972 classified DD into

1.  Type I: Radicular DD
2.  Type II: Coronal dentin dysplasia¹

What is dentin dysplasia type I?

Dentin dysplasia type I is an inherited disorder. It is characterised by abnormal synthesis of the dentin and is also known as radicular dentin dysplasia.

The following are the features. 

• The underdeveloped, abnormally shaped pulp tissue is mostly present in the roots of the teeth
• The teeth may lack a pulp chamber or may also be half-moon-shaped, that is placed in short or abnormally shaped roots

The condition may affect both baby (primary) and adult (permanent) teeth. As the roots are abnormally short in structure, it usually leads to the premature loss of teeth. The color of the teeth is usually normal or amber coloured.²

Early detection of dentin dysplasia type I

In dentin dysplasia, both primary and permanent dentitions display clinically normal crowns of the teeth, but defects in dentin formation and lower than normal content of pulp are present. There are four subtypes for this abnormality. Here comes the role of a pediatric dentist.³

Diagnosis

Early diagnosis of the condition is essential for the initiation of effective preventive treatment. In this regard, the pediatric dentist plays a crucial role in the early diagnosis of this disorder and in advising patients on measures to prolong the retention of affected teeth. Management of patients with dentinal dysplasia poses quite a challenge for a dentist to treat.⁴

Diagnosis is individualised and is based on family history, pedigree construction, and detailed clinical examination. Diagnosis of genetics may become useful in the future once sufficient disease-causing mutations are discovered.

Treatment

Treatment of DD-I should usually be initiated from infancy and be continued into adulthood, with several options, including crowns,over-dentures, and dental implants 

Based on the prevalent age and the condition of the dentition of the patient. This early diagnosis in life and intervention of the right kind of treatment that follows the outlined recommendations by the pediatric dentist ensures good aesthetics and function. Treatment aims to remove sources of infection or pain, restore aesthetics, and protect posterior (back) teeth from wear and tear.⁵

Crowns

In the primary dentition, stainless steel crowns on the molars protect the tooth from wear and tear and maintain the occlusal vertical dimension. Usage of composite facings or composite strip crowns promotes aesthetics. However, in delayed cases, this is not the solution. 

Over-dentures

When the teeth undergo a good amount of attrition to the level of the gingiva, the only treatment option available is to provide over-dentures. Children usually adapt well to over-dentures, but they need to be reviewed regularly, and dentures are refabricated as the child grows.

Tooth removal

If abscesses develop, pulp therapy is unsuccessful, and removal of the affected teeth is the only available option. Younger children tend to cooperate less. Hence, to perform this extensive procedure, a general anaesthetic may be required to facilitate treatment.⁵

Cast occlusal onlay

As the permanent dentition erupts, it should be closely monitored in relation to the rate of tooth wear, and intervention undertaken only if necessary. Timely intervention with cast occlusal onlays on the first permanent molars and eventually the premolars helps to minimise tooth wear and maintain the occlusal vertical dimension. Minimal tooth preparation is prioritised until the child reaches adulthood. 

If clinically indicated, a full mouth rehabilitation may be considered at this point in the treatment plan. Teeth with short, thin roots and marked cervical constrictions are often considered unfavourable for crown preparation as they will easily chip off. A good amount of loss of pulp chambers and root canals in teeth may lead to pus formation, which will make endodontic therapy a difficult task.⁵

FAQs

Who is a pediatric dentist?

Pediatric dentists are dental surgeons who deal with the dental problems of child patients. 

What is the occlusal vertical dimension?

Occlusal vertical dimension or vertical dimension of occlusion (VDO) refers to the measurement of the height of the lower face when the upper and lower teeth are in maximum intercuspation.⁶

What are over-dentures?

Over-dentures refer to a removable dental prostheses that cover and rest on one or more remaining natural teeth, the roots of natural teeth, and/or dental implants.⁷

What are cast occlusal onlays? 

An onlay is an indirect restoration covering one or more cusps of a back tooth. It is designed to provide the lost strength to a tooth that has been weakened by caries, tooth fracture, or by a previously placed large restoration. Resin, cast gold, or ceramic may be used. An onlay covers all of the cusps of the teeth and provides excellent protection against fracture. But the tooth preparation is technically very challenging.⁸

What is endodontic therapy?

Endodontic therapy treats disorders of the pulp–dentin complex and Periapical (area beneath the teeth) tissues. It helps to preserve or revive living tissues within the tooth or can maintain the natural tooth structure when vitality cannot be sustained. This includes procedures such as root canal therapy, vital pulp therapy, and regenerative endodontics.⁹

What preventive measures should be undertaken by a DD-I case patient?

• Control of the ingestion of sugar
• Maintaining oral hygiene
• Regular dental visits for a professional cleaning of teeth and the detection and restoration of decayed teeth
• In the case of young patients, early diagnosis and interventions like stainless steel crowns will protect the affected teeth and maintain function¹⁰

Summary

A multidisciplinary approach can provide an elaborate treatment plan for oral rehabilitation of patients with DD type I. The outcome of a diagnosis of DD largely depends upon the age of the patient when it was diagnosed and the quality of the treatment provided. Early diagnosis helps in undertaking vital decisions as to which age requires what interventions to prolong the retention or rehabilitation of affected teeth to maintain good esthetics and function, thereby minimizing nutritional deficits and psychosocial distress.¹