Department of Pediatric Dentistry, Division of Oral Structure, Function and Development, Asahi University School of Dentistry, 1851 Hozumi, Mizuho, Gifu 501-0296, Japan (Director: Prof. Yasuo Tamura)
Department of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University School of Dentistry, Hozumi 1851, Mizuho, Gifu 501-0296, Japan (Director: Prof. Michio Shikimori)
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Here we present a case of a 7-year-old Japanese girl with regional odontodysplasia affecting tooth numbers 24, 25, 26, and 27. No other teeth were affected. Partially erupted tooth number 26 had a rough enamel surface and was yellow-brown in color. Tooth numbers 24, 25, and 27 had not erupted. Radiographically, numbers 24, 25, 26, and 27 seemed to be undeveloped with large pulp chambers, short roots, and open apices, and exhibited the so-called ghostlike appearance. Histopathologically, number 26 had uneven thin enamel and dentin, revealing hypoplasia and hypocalcification, and irregular dentinal tubes. The patient was followed up for 6 years and had similarity normal occlusion.
Keywords: Odontodysplasia; Ghost teeth; Hypoplastic permanent teeth
Odontodysplasia is a rare developmental anomaly in tooth formation generally observed in young patients. This disease is characterized by the malformation and hypocalcification of both the enamel and dentin, asymmetrically and locally. Since the initial description by McCall and Wald,1 approximately 125 cases have been reported in the dental literature worldwide. The disease is variously described as odontodysplasia,2 localized arrested tooth 3,4 5 6 7 development, odontogenesis imperfecta, and unilateral dental malformation. Rushton termed the disease “ghost teeth” due to the ghost-like appearance of the teeth radiographically. Here we report a case of regional odontodysplasia in a 7-year-old Japanese girl. The patient was followed up for 6 years and had normal occlusion.
This 7-year-old girl was referred to Asahi University Hospital in Gifu, Japan, for diagnosis and treatment of an unusual enlargement in the right maxilla. Her chief complaint was pain and swelling in the upper right molar region.
According to the patient’s mother, tooth numbers 64 and 65 erupted at 6 months after
were quickly destroyed by caries, which was often accompanied by abscess formation.
At 6-years of age, numbers 64 and 65 were extracted due to pain and swelling at a private dental clinic. Two weeks after the extraction of 64 and 65, tooth number 26 began to erupt. The external appearance of 26 was similar to that of 64 and 65, and associated with some swelling and redness. No undisputed cases of regional odontodysplasia were observed in other family members.
Her general condition was satisfactory. Comprehensive extraoral surface extraction and head-and-neck examinations were non-contributory, except for a slight depression of the right cheek (Figure 1).
the enamel and dentin, and were undeveloped with large pulp chambers, short roots, and open apices, and appeared ghost-like radiographically. Tooth numbers 24, 25, and 27 were still inside DISCUSSION the bone. The number and morphology of the remaining dentition were normal (Figure 3).
According to the literature, regional odontogenesis is slightly more prevalent in females and slightly more often involves the maxillary arch. In this case, the patient was female and the maxillary arch was affected. Both the dentition (primary and permanent dentition) were affected, and the age of diagnosis was during primary tooth eruption as well as during mixed dentition.9
Local infection, trauma, oral ischemia, somatic mutation, irradiation, metabolic or vitamin deficiency have been discussed as possible etiologies of this developmental 6,10,11-15 disturbance.
Possible causes of the present case could not be determined. Rushton8 considered that regional odontodysplasia could be caused by either a single act of damage with resulting subsequent abnormalities or continuing insult. It has been argued, however, that a single act of damage would have to occur in utero and it is difficult to envisage such damage extending from the deciduous to the permanent dentition, with the occasional tooth in a quadrant remaining unaffected.16 Activation of a latent virus in the odontogenic epithelium has also been 7,17,18 suggested as a cause for the disorder, but no virus particles
Local infection has also been cited,12 but the casual
Similarly, local trauma has been implicated. If trauma was definitely related to a specific tooth during development, however, then such a case probably should not be classified as regional odontodysplasia.15
Metabolic or vitamin deficiencies have also been advanced as causative factors, but it is unlikely that only specific teeth would be affected and not others at similar stages of development.14 Irradiation can cause damage to developing 7,14 teeth,7,14 but in such cases a history would be available, and all the developing teeth in the treated area would be affected. The involvement of selected teeth in regional dysplasia leaving adjacent teeth unaffected makes radiation an unlikely cause.
The somatic mutation theory,7,17 does not account for cases in which unaffected teeth have developed from the distal extension of a mesially affected dental lamina, and in addition, when teeth in both jaws are affected the chances of multiple mutations in the same patient are highly unlikely.19
A hereditary factor can be discounted because of the lack of evidence of such a history in cases reported to date.
The possibility of maternal illness, drug therapy, or both as etiologic factors was suggested by cases reported in children 14 16 whose mothers had pneumonia14 or toxemia16 during pregnancy or who took diuretics,20 tranquilizers, or hormones during the first trimester.9
Although a relationship between drugs and regional odontodysplasia has not been established, increased awareness of the possible effects of such harmful agents is required. It is well established that a great variety of drugs taken during pregnancy can cause congenital malformations, such as warfarin taken during the first trimester,21 ethanol causing fetal alcohol syndrome,22 cocaine producing congenital malformations, aromatic retinoids used for severe skin disease, and angiotensin-converting enzyme inhibitors for hypertension. Children of woman with epilepsy also appear to have increased evidence of congenital malformations; it is not certain whether this is due to drug therapy, but it seems likely.
This disease can be distinguished from other disturbances of odontogenesis because all of the histologic elements of the dental organ are abnormal in the affected teeth, while others in the same individual are normal.
The treatment plan for this patient was removal of tooth numbers 24, 25, and 27 after eruption if the hypoplasia and hypocalcification could not remedied. In many cases of odontodysplasia, the teeth cannot be preserved.25
The histopathologic features and clinical and radiographic aspects were analyzed and the diagnosis of regional odontodysplasia was established.
14,18
We reported a case of regional odontodysplasia in a 7-year-old Japanese girl. The patient was followed up for 6 years and had similarity normal occlusion.
The patient has provided written permission for publication of the case details.
We have no conflicts of interest to declare.
7,17