A 13-year-old male visited a dentist for evaluation of discolored and malformed teeth. Radiographic examination revealed teeth with extremely thin amounts of enamel and dentin.
Joen Iannucci Haring, DDS
The patient had not previously been evaluated by a dentist. He denied any history of symptoms associated with the affected teeth. When questioned about the teeth of other family members, the patient stated that no other family member had similar discolored or malformed teeth.
The patient appeared to be in a general good state of health, with no significant medical history. At the time of the dental appointment, the patient was not taking medications of any kind.
The patient`s vital signs were all found to be within normal limits. Examination of the head and neck region revealed no enlarged or palpable lymph nodes. Examination of the oral soft tissues revealed no unusual finds. No bony abnormalities were noted.
Intraoral examination revealed erupted teeth with small irregular crowns. The affected teeth were limited to the maxillary arch and exhibited a yellowish-brown discoloration and rough surface texture.
After a thorough clinical examination, periapical radiographs of all the teeth were ordered. Examination of the periapical radiographs revealed teeth with extremely thin amounts of enamel and dentin surrounding enlarged pulps (see radiograph).
Based on the clinical and radiographic examination available, which one of the following is the most likely diagnosis?
* amelogenesis imperfecta
* dentinogenesis imperfecta
* regional odontodysplasia
* dentinal dysplasia type I
* dentinal dysplasia type II
Regional odontodysplasia, also known as ghost teeth, is an uncommon developmental abnormality of teeth. This condition is nonhereditary and characterized by defective enamel and dentin, resulting in teeth that appear malformed and discolored. Most cases of ghost teeth are idiopathic, although some cases have been linked to various syndromes, vascular malformations, neural disorders, and growth abnormalities.
Although regional odontodysplasia may affect both the deciduous and permanent dentitions, the permanent dentition is most often involved. In cases of primary teeth involvement, the permanent teeth are often affected as well. There is no sex or race predilection. As the term regional suggests, this condition tends to be localized and affect a focal area of the dentition. The maxilla is involved more frequently than the mandible and there is a predilection for the anterior region of the jaws. Two or fewer quadrants are typically involved. Several adjacent teeth are often affected. Occasionally, a normal tooth may be interspersed in a quadrant of defective teeth.
The teeth affected by regional odontodysplasia may erupt or fail to erupt. The erupted defective teeth exhibit small and irregular shaped crowns. A yellowish-brown discoloration is apparent along with an irregular surface texture. Decay and associated pulpal problems (radicular cysts, periapical abscess, periapical granuloma, for example) are not uncommon.
Radiographically, a tooth affected by regional odontodysplasia exhibits extremely thin areas of enamel and dentin surrounding an enlarged pulp chamber. The radiographic appearance is said to resemble a OwispyO image of a tooth; and, in fact, the term ghost teeth refers to this indistinct, fuzzy image that lacks radiodensity. In addition, short roots, open apices, and pulp stones may be noted.
A diagnosis of regional odontodysplasia is established based on the characteristic radiographic and clinical presentation. If there is any question, an affected tooth may be removed and examined histologically.
The treatment for regional odontodysplasia is directed toward retention of existing teeth in order to maintain the alveolar ridge. The erupted teeth may be treated endodontically and then restored for a favorable esthetic appearance.
Joen Iannucci Haring, DDS, MS, is an associate professor of clinical dentistry, Section of Primary Care, The Ohio State University College of Dentistry.