The Role of Dentists in Diagnosing Osteogenesis Imperfecta in Patients with Dentinogenesis Imperfecta

Resident: Adam J. Bottrill
Date: 09FEB11
Region: Providence
Article title: The Role of Dentists in Diagnosing Osteogenesis Imperfecta in Patients with Dentinogenesis Imperfecta
Author(s): Teixeira, Cleonice DDS, MSc et al.
Journal: JADA
Page #s: 906-914
Vol:No Date: Vol 139, July 2008
Major topic: Osteogenesis Imperfecta, Dentinogenesis Imperfecta
Minor topic(s): Scanning Electron Microscopy
Type of Article: Case report, Analysis
Main Purpose: The purpose of this article is to describe a clinical case of diagnosis of dintinogenesis imperfecta (DI), in which a literature review combined with an analysis of dental alterations led to indications of osteogenesis imperfecta (OI).

Key points in the article discussion:

I. DI: hereditary disorder resulting in defective dentin formation in both primary and permanent teeth.

A. Types:
1. Type I: Associated with OI... dental manifestation of the underlying collagen defect
2. Type II: Most common, opalescent dentin, no associations
3. Type III: Brandywine MD association

B. Clinical: opalescent discoloration (brown, gray or yellow), bulbous crowns, roots narrower than usual, root canals can become obliterated over time. Possible early loss of enamel due to weak dentin.

II. OI: "brittle bone disease" is a genetic disorder that affects the connective tissue and is characterized by bone fragility and fractures, blue sclera, long bone deformity, lowered hearing capacity, can be related to OI.

A. 50% of OI cases reported to have some form of DI.

B. Because DI can go unnoticed, if DI is confirmed, the patient should be closely examined and OI should be considered.

III. Case Report

A. 11 mo, white female refered with CC of yellow discoloration of teeth. Normal growth and no other evidence of FH.

B. Primary dentition: yellow/brown discoloration with enamel fractures, dentin exposure began at 3yo. Severe attrition, pulpa exposures on posterior teeth.

C. Following primary teeth exfoliation, esthetics improved. Sealants placed on posterior occlusal surfaces.

D. Follow-up at age 15yo: opalescent gray discoloration. Multiple restorations needed to repair fractured enamel. Corncob shaped roots and bulbous crowns. MHx revealed multiple bone fractures (11) through arms, hands, feet, trunk during childhood. Lastly, deficient occlusion with bilateral crossbite.

E. Scanning electron microscopy (SEM) performed on an extracted 3rd molar revealed abnormal dentinal matrix.

F. Despite multiple visits with orthopedic clinics, OI had not been Dx. Dentist Dx DI associated with OI type I... verified by endocrine/orthopedic specialist.

G. By age 18, stable clinical presentation, pulpal obliteration, more distinct dental discoloration, new bone fractures.

IV. Discussion:

A. At first, the clinical and radiographic evidence supported DI type II due to lack of acssociated symptoms.

B. It was observed that the permanent teeth were less affected than primary and that she did not seem more susceptable to carious lesions than normal teeth. (possibly due to impeded progression with the irregular dentinal tubules and structure)

C. For the future, pt must be instructed on ways of avoiding bone fracture and osteoporosis.

D. Dental manifestations, though only occuring in 50% of OI, can be the most clinically evident signs of the disease.

E. Note: Obliteration of pukp chambers should alert dental professionals to the possibility of OI. Consider early endodontic intervention.

V. Conclusions:

A. Early and appropriate dental care can lead to improved control of oral disease, function and esthetics.

B. We should always consider the possibility of OI with any case of DI.

C. Detailed MH is important.

Assessment of article: I found it very valuable as a clinician. OI and DI are both diseases we should be intimately familiar with. The authors may have dug into the SEM material a little more than I liked, but the entire article was useful and relevant. It is also always nice to hear a case that was "solved" by a dentist. Goes against the stereotype that we aren't "REAL" doctors. Likey.