Dentinal Dysplasia Type I: A Case Report with a 6-Year Followup

Abstract

Introduction. Dentin dysplasia is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology that is inherited as an autosomal pulpal morphology. Case Presentation. A 7-year-old female who had problems in chewing function was referred to Oral and Maxillofacial Surgery Department at the Faculty of Dentistry in Ondokuz Mayıs University. In the radiographic examination, it was determined that some of the unerupted permanent teeth of the patient had short, blunted, and malformed roots with obliterated pulp chambers, although the bone below the teeth showed well-defined margins. This unusual case of generalized short roots presents a case demonstrating both classic and atypical features of dentinal dysplasia type I (DDI) in the mixed and permanent dentitions. Conclusion. There are still many issues in the diagnosis and management of patients with dentin dysplasia. Early diagnosis, clinical and radiographic findings, as well as treatment of this condition and the initiation of effective preventive strategies may help prevent or delay loss of dentition.

Guided endodontic treatment of multiple teeth with dentin dysplasia: a case report

“…Extraction was suggested for teeth with pulp necrosis and periapical abscess, and endodontic treatment was considered to be contraindicated in those with severe calci cation of the pulp (35). Furthermore, endodontic therapy might be limited in young children, as an early age of the patient is associated with poor compliance in such di cult and lengthy treatment.…”

“…In DD cases in need of endodontic therapy, conventional orthograde endodontic treatment was reported to be potentially substitutable with periapical curettage and retro lling, especially if the roots display normal length and development (32,33). In the case of DD-1, extraction of teeth with periapical radiolucencies might be favoured over endodontic therapy due to the above-mentioned technical challenges and anatomic limitations (34)(35)(36).…”

Dentin Dysplasia Type I: Report of Two Cases

“…The DD is a genetic defect of dentin formation, although its etiology is still considered a mystery. 1 Wesley et al 7 proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts, leading to abnormal differentiation and/or function of the ectomesenchymal-derived odontoblasts. The DD is usually an autosomal dominant condition, and there is a 50% chance that a child born to an affected parent will itself be affected.…”

“…The DD is usually an autosomal dominant condition, and there is a 50% chance that a child born to an affected parent will itself be affected. 1 A DDI is also called radicular DD, primarily affecting the root's portions of the teeth and producing teeth with shortened roots and periapical radiolucencies. 8 Type II DD also called coronal DD is characterized by large coronal pulp chamber containing denticles in permanent teeth and total pulp chamber obliteration in primary teeth.…”

“…Dentin dysplasia (DD), the autosomal dominant hereditary disturbance in dentin formation, is a rare anomaly with unknown etiology, which occurs approximately 1 in every 100,000 in the world. 1,2 The first case reported with this condition was in 1920 termed as "rootless teeth", and in 1939, Rushton termed this condition as DD. 3 Dentinal dysplasia is classified into radicular dentin dysplasia (DDI) and coronal dysplasia (DDII).…”