Full Mouth Rehabilitation in Patient with Dentinogenesis Imperfecta in Primary Dentition: A Case Report

INTRODUCTION

Dentinogenesis imperfecta or hereditary opalescent dentin, was first described in the late 19th century.¹ It is a localized mesodermal dysplasia affecting both the primary and permanent dentition. It is defined as “A hereditary developmental disturbance of the dentin originating during the histodifferentiation stage of tooth development.”² It was found to be the rarest dental anomaly with a prevalence of 0.09% in the city of Indore, India.³

It may be seen alone or in conjunction with the systemic hereditary disorder of the bone, Osteogenesis Imperfecta. The other two types are autosomal dominant conditions that have been linked to allelic mutations of the DSPP gene encoding dentin phosphoprotein and dentin sialoprotein.^4,5

The treatment goals for treating such patients with primary dentition are:

1. Maintain Dental Health by preserving vitality, form, and size of the dentition.
2. Enhance Esthetic to Prevent psychological issues.
3. Provide Functional Dentition which helps in chewing.
4. Prevents loss of vertical dimension.
5. Maintains the arch length.
6. Avoid interfering with the eruption of the remaining permanent teeth.⁸

The two most accepted classifications are given by Shield and Witkop.^6,7

CASE REPORT

5-year-old/ male patient reported to the department of Paediatric and Preventive Dentistry of Sri Aurobindo College of Dentistry with the presenting complaint of inability to eat food due to difficulty in chewing, brown discoloration of all his teeth since birth and excessive wearing of teeth. Patient gave a medical history of viral hepatitis, nutritional anemia, developmental delay, and speech delay. No history of bone fracture was given. Intra-oral examination showed generalized brownish discoloration with primary dentition, severe attrition with 53,52,61,63,64,72,73,74,75,82,83,84,85;dehiscence with 54,55,64,65,75; Greyish discoloration with 16,26,36,31,41,46; Root Stumps were present with 51,62 ; 31,41 were seen erupting. (FIGURE 1a, 1b) Extraoral examination revealed developing class III malocclusion.

Radiographic examination (Figure 2) revealed short & conical roots with peri-apical radiolucency. The coronal structure had wide pulp chambers and cervical constriction. Generalised severe boneless was noted. The Final diagnosis of Dentinogenesis Imperfecta type 2 was given on the basis of clinical and radiographic assessment. A comprehensive, tailor-made treatment plan was finalised according to the needs of the patient. Initial study models were prepared.

Treatment was divided into 4 stages:

Stage 1: This was the preventive stage in which stainless steel crowns were given to the teeth which had severe attrition but no dehiscence (84,85,74). (Figure 3)

Stage 2: Teeth with hopeless prognosis were extracted 54,55,64,65. 75 was not planned to be extracted at this stage.

Stage 3: Prosthetic rehabilitation of the upper arch with overlay denture was done. Secondary impressions were taken from special tray prepared from primary cast (Figure 4a,b). On these impressions cast was poured and trial denture was fabricated (Figure 4c). Post fabrication try in was done and adjustments according to patient’s comfort were made (Figure 4d). After making adjustments in the try in denture, final over lay denture was delivered (Figure 4e). C clasps were added bilaterally to provide added retention. Mono-plane occlusion was kept to minimize masticatory forces acting on lower teeth.

Stage 4: Oral Hygiene instructions were enforced and patient counselling was done regarding the importance of having follow-up visits in a short period of time (for early detection of any new fracture or abscess and also to check the need for changing the prosthesis).

DISCUSSION

Treatment was done according to AAPD guidelines.2In initial treatment molars which had severe attrition and no dehiscence were restored with stainless steel crowns (85,84,74).Wide pulp chambers and thin dentin caused difficulty in tooth preparation. Hence, stainless steel crowns were given by Hall’s technique.⁸ In a case report byTorija ET, stainless steel crowns were given in a 18 month old child suffering from dentinogenesis imperfecta to prevent further damage to the coronal structure.⁹ Other authors like Sapir S, Shapira J have also advocated early diagnosis and prevention with preformed crowns for patients suffering from dentinogenesis imperfecta.¹⁰

75 had hopeless prognosis, so stainless steel crown was not planned. Also, extraction was not planned to prevent the loss of vertical height, allow normal growth of the facial bones and temporomandibular joint and avoid interference with the eruption of the remaining permanent teeth.

Overlay dentures were planned for maxillary arch, it enhanced mastication and esthetics, maintains alveolar bone by the retention of teeth and increased proprioception. Syriac G et. al. Schneidman E et.al. also advocated rehabilitation with overlay dentures to prevent further damage.^11,12 The denture along with stainless steel crowns has helped in restabilising the vertical height of the face and in turn enhance the aesthetics.

After 3 months follow up improvement in the health of the child was noted (Figure 5 a,b). This has helped him to improve his aesthetics, rebuild his self-confidence and improve the quality to his life.

CONCLUSION

Full mouth rehabilitation in patients with Dentinogenesis Imperfecta can help in improving esthetics, enhances mastication thus give a positive psychological support and better quality of life.

REFERENCES