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Dental rehabilitation of a patient with dentinogensis imperfecta: a clinical report.


Dentinogenesis imperfecta (DGI) is defined as an autosomal dominant disorder in dentin mineralization in both the primary and permanent dentitions. (1) DGI is the most common of all dental genetic diseases affecting some 1:8000 births. (2) Dentino genesis Imperfecta can be grouped into 3 clinical entities, Shields Type I, II, and III. There are several known subtypes and all share a common clinical appearance. (3)

Common characteristics include uniform yellow to blue crowns with enamel that easily fractures due to the poor dentin support. The crowns are usually bulbous or bell shaped with short roots and pulp chambers, which presents narrowed by an excessive production of the defective mineralized dentin matrix. (4) The defective dentin, shown in Figure 1, has a yellow translucent hue and takes on a characteristic gray to brownish-blue discoloration that has prompted the term hereditary opalescent dentin. (5)

In Dentinogenesis Imperfecta Type I, the dentin mineralization defects are coupled with Osteogenic Imperfecta related bone diseases characterized by a systemic condition involving bone fragility, blue sclera of the eyes, joint laxity, and hearing impairment. It is cause by a defect in collage formation. DGI-II (hereditary opalescent dentin) has the same dentin defects as DGI-I but has not been found to be related to any collagen defect and has no osteogenic component. DGI-III, also known as Brandywine type, is a complex heterogeneous disorder, which occurs in an isolated racial group. Both collagen and mineralization are affected and the teeth appear opalescent as in DGI types I and II but have a shell-like appearance. It was initially described in a triracial, isolated population in Brandywine, Maryland. (6,7) Diagnosis is based on a thorough family history, pedigree construction, and detailed clinical examination. Differential diagnosis may include hypocalcified form of amelogenesis imperfecta, congenital erythropoietic porphyria, Kostmann's condition (conditions leading to early tooth loss), cyclic neutropenia, Chediak-Hegashi syndrome, histiocytosis X, Papillon-Lefevre syndrome, tetracycline discoloration and staining of the teeth, vitamin D-dependent and vitamin D-rickets. (8)


A 19-year-old service member presented for routine dental treatment with a chief complaint/concern of pain, sensitivity, difficulty chewing, and overall displeasure with the appearance of her teeth. A review of the patient's health questionnaire revealed a medical history that was significant for a smoking habit and multiple family members with the same dental presentation. Extraoral examination revealed slight tenderness when palpating the masseter and temporalis muscles. Tenderness was thought to be related to poor occlusion. Intraoral examination revealed yellowish to blue-gray appearance of the anterior dentition and extensive dental caries associated with most maxillary and mandibular posterior teeth. The patient stated that many teeth would break easily with normal function (Figure 2). The prosthodontic evaluation revealed a dental midline discrepancy, short clinical crowns complicated with extensive dental caries, malalignment of the anterior teeth, and occlusal and aesthetic disharmonies. Photographs, radiographs, and dental casts were completed. A comprehensive periodontal review confirmed gingival tissues as firm and fibrotic, generalized 2 mm to 3 mm periodontal probe depths, moderate to heavy plaque with slight calculus accumulation, extensive caries, and bilateral mandibular lingual tori (Figure 3). The patient was referred to the oral pathologist and the diagnosis of dentinogenesis imperfecta was confirmed.





Complete rehabilitation of the dentition was based on the patient's desires, clinical findings, radiographic evaluation, and periodontal health. The treatment plan involved extraction of all hopeless teeth with extensive dental caries (numbers 3, 4, 5, 12, 13, 14, 18, and 19) and fabricating an interim removable partial denture. The fabrication and placement of the interim removable partial denture and provisional crowns appeared to resolve the facial muscle tenderness and reestablished occlusal support. Following the healing process and the reevaluation phase, the patient was treatment planned to have dental implants placed for teeth numbers 3, 4, 5, 12, 13, 14, and 19 (4.0 x 11.5 and 5.0 x 11.5; 3i-Implant Innovations Inc, Palm Beach Garden, FL). After adequate healing and implant integration, implants supported metal ceramic crowns were fabricated. Metal ceramic crowns were also fabricated for the remaining maxillary and mandibular teeth (numbers 6, 7, 8, 9, 10, 11, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29) (Figures 4 and 5).

The combined treatment plan with the prosthodontist and periodontist provided for the complete rehabilitation with full function and aesthetics. All aspects of the patient's concerns were addressed with complete resolution of the patient's chief concern. The prosthodontic phases of treatment included preparation and provisionalization of all remaining teeth. All restorations were characterized, glazed, polished, and then luted with permanent cement (Figures 6, 7, 8). Home care instructions were provided and the patient was scheduled for periodic maintenance care.






(1.) Witkop C. Hereditary defects of dentin. Dent Clin North Am. 1975;19:25-45.

(2.) Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. Philadelphia, Pennsylvania: W.B. Saunders Company; 1995:84-87.

(3.) Shields ED, Bixler D, El-Kafrawy AM. A proposed classification for heritable human dentine defects with a description of a new entity. Arch Oral Biol. 1973;18:543-553.

(4.) Regezi JA, Sciubba J. Oral Pathology, Clinical Pathologic Correlations. 2nd ed. Philadelphia, Pennsylvania: W.B. Saunders Company; 1993:509-510.

(5.) Regezi JA, Sciubba JJ. Pogrel MA. Atlas of Oral and Maxillofacial Pathology. Philadelphia, Pennsylvania: W.B. Saunders Company; 2000:152.

(6.) Langlais, RP, Miller CS. Color Atlas of Common Oral Diseases. 2nd ed. Baltimore, Maryland: Williams & Wilkins; 1998:32.

(7.) Hursey RJ Jr, Witkop CJ, Miklashek D, Sackett LM. Dentinogenesis imperfecta in a racial isolate with multiple hereditary defects. Oral Surg Oral Med Oral Pathol. 1956;9:641-658.

(9.) Barron MJ, McDonnell ST, Mackie I, Dixon MJ. Hereditary dentine disorders: dentinogenesis imperfect and dentine dysplasia. Orphanet Journal of Rare Diseases [serial online]. November 20, 2008;doc 3:31.

COL David A. Mott, DC, USA

LTC (P) Minaxi I. Patel, DC, USA

LTC Dong Soo Park, DC, USA

COL Mott is Commander, US Army Dental Activity Japan, Camp Zama.

LTC (P) Patel is Chief, Prosthodontics Services, US Army Dental Activity, Fort Stewart, Georgia.

LTC Park is Officer-in-Charge and Chief, Periodontics Services, US Army Carius Dental Clinic, Seoul, South Korea.
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Author:Mott, David A.; Patel, Minaxi I.; Park, Dong Soo
Publication:U.S. Army Medical Department Journal
Article Type:Clinical report
Geographic Code:1USA
Date:Jan 1, 2011
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