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| CASE SERIES |
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| Year : 2021 | Volume
: 2
| Issue : 3 | Page : 88-91 |
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Regional odontodysplasia: A rare case series and review
Rajib Sikdar, Avik Narayan Chatterjee, Khooshbu Gayen, Supreet Shirolkar, Anisha Bag, Subir Sarkar
Department of Pedodontics and Preventive Dentistry, Dr. R Ahmed Dental College and Hospital, Kolkata, West Bengal, India
| Date of Submission | 25-May-2021 |
| Date of Decision | 16-Jun-2021 |
| Date of Acceptance | 26-Jun-2021 |
| Date of Web Publication | 25-Aug-2021 |
Correspondence Address:
Rajib Sikdar
Department of Pedodontics 2C, Dr. R Ahmed Dental College and Hospital, Kolkata - 700 014, West Bengal
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jpcdoh.jpcdoh_21_21
Odontodysplasia is a rare nonhereditary developmental anomaly of dental hard tissue with unknown etiology, arising from both ectodermal and mesodermal components. Hitchin in 1934 first described it as a localized arrest of tooth development generally affecting one quadrant of the jaw. When it just affects one quadrant, it is referred to as “regional odontodysplasia,” but when it crosses the midline and affects more than one quadrant, it is referred to as “generalised odontodysplasia.” In this two case series, various types of odontodysplasia have been described, one of which is confined to a single quadrant and the other crossing the midline. The cases of odontodysplasia can only be treated by multidisciplinary approach and restorative and prosthetic rehabilitation and regular follow-up require for improving the quality of life of patients.
Keywords: Generalized odontodysplasia, ghost teeth, odontodysplasia, odontogenesis imperfecta, odontogenic dysplasia, regional odontodysplasia
How to cite this article:
Sikdar R, Chatterjee AN, Gayen K, Shirolkar S, Bag A, Sarkar S. Regional odontodysplasia: A rare case series and review. J Prim Care Dent Oral Health 2021;2:88-91 |
How to cite this URL:
Sikdar R, Chatterjee AN, Gayen K, Shirolkar S, Bag A, Sarkar S. Regional odontodysplasia: A rare case series and review. J Prim Care Dent Oral Health [serial online] 2021 [cited 2022 Jun 29];2:88-91. Available from: http://www.jpcdoh.org/text.asp?2021/2/3/88/324539 |
| Introduction | |  |
Odontodysplasia is a rare developmental anomaly which is both mesodermal and ectodermal in origin.[1] Hitchin in 1934 first described regional odontodysplasia,[2] and later, in 1947, Mc Call and Wald first described its radiological features.[3] The term “odontodysplasia” was coined by Zegarelli et al. in 1963.[4] Pindborg added the term “regional” with odontodysplasia to describe its nature of affecting one quadrant of the jaw.[5] If more than one quadrant is affected, then it is called “generalized odontodysplasia.” Rushton also called it “shell teeth,” to describe the radiological characteristics such as hypoplastic and hypocalcified enamel and dentin of this condition.[6] Odontogenic dysplasia, localized arrested tooth development, ghost teeth, odontogenesis imperfecta, unilateral dental malformation, and familial amelodentinal dysplasia are some of the other names used to describe regional odontodysplasia.[7],[8]
Regional odontodysplasia frequently affects both primary and permanent dentition.[9] According to Lustmann et al., the maxilla is two times more affected than the mandible[9],[10] and central and lateral incisors are most commonly affected than posterior teeth.[11] The left quadrant of the jaw is more frequently affected than the right side and it rarely crossed the midline.[9],[12]
The rate of incidence is <1 cases per 1,000,000 live births[13],[14] and girls are far more affected than boys (female predilection: 1.37:1).[9],[15] However, there is no ethnic prevalence.[16]
Regional odontodysplasia is not considered a hereditary disorder,[9],[16] and some noninherited conditions could be associated with it.[8] These are,
- Vascular nevi
- Hemangioma
- Epidermal nevus syndrome
- Orbital coloboma
- Hypoplasia of affected side of the face
- Hypophosphatemia
- Ectodermal dysplasia.
According to literatures till now, three cases of regional odontodysplasia along with vascular nevi, seven cases of generalized odontodysplasia, nine cases of regional odontodysplasia with hemangioma, and six cases of regional odontodysplasia associated with facial hemiatrophy have been reported.[17]
| Case Reports | | |
Case 1
An 11-year-old female child patient reported to the Department of Paediatric and Preventive Dentistry of Dr. R. Ahmed Dental College and Hospital, Kolkata, West Bengal, India, with a complaint of noneruption of teeth on the left quadrant of the upper jaw. The child was born after a normal pregnancy of nonconsanguineous marriage of a healthy couple. There was no medical history of any complication or regular drug use during pregnancy, and the healthy child was born without any significant medical event.
On clinical examination, the child was in mixed dentition stage with normal eruption and shedding pattern except in the left maxillary quadrant. Upon history taking, it was found that 61 and 62 became carious with abscess formation and extracted early in childhood. 63, 64, 65, and 26 are found in the left maxillary quadrant, with grossly carious 63 and 64 and presence of generalised crowding. 13, 14, 15, and 16 erupted in the opposite (right maxillary) quadrant in normal order without any complication .
On radiographic examination (orthopantomogram [OPG] and cone-beam computed tomography [CBCT]), it was found that there was localized developmental malformation of both primary and developing permanent dentition. Crown and root structures of teeth 21, 22, 23, and 24 were badly malformed, and there was low contrast between enamel and dentin in developing teeth, indicating hypomineralization [Figure 2]. However, enamel and dentin formation of 25 was normal. Hematological investigation showed a normal blood profile and sodium, calcium, phosphate, alkaline phosphatase (ALP), and acid phosphatase within normal range. Hence, all the clinical and radiological investigations were pointing toward the diagnosis of regional odontodysplasia. | Figure 2: Radiographic view showing ghost teeth in the left upper quadrant
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Case 2
A 12-year-old female child patient reported to the Department of Paediatric and Preventive Dentistry of Dr. R. Ahmed Dental College and Hospital, Kolkata, West Bengal, India, with a complaint of noneruption of teeth on the left quadrant of the upper jaw. The child was born from consanguineous marriage (maternal cousin) of a healthy but elderly couple. There was no medical history of any complication or regular drug use during pregnancy, but the child was born prematurely with a history of blood loss during cesarean section.
On clinical examination, the child was in mixed dentition stage with normal eruption and shedding pattern except in the left maxillary quadrant. During previous dental history taking, it was found that primary teeth in the left maxillary quadrant (61, 62, 63, 64, and 65) became readily carious upon eruption and resulted in spontaneous pain and frequent abscess formation. Parents also noticed that teeth became very brittle and spontaneously exfoliated in small fragments. Due to the same reason, Root Stamp of 64 and 65 was extracted by a local dentist at 6 years of age. The right primary maxillary central incisor (51) was retained in the oral cavity. A left maxillary permanent molar is present in the mouth, however it is severely decayed [Figure 3]. This early exfoliation and extraction results in abnormal vertical and transverse growth of the alveolus. All of this information pointed to a hypoplastic tooth structure.
For further investigation, OPG and CBCT with three-dimensional reconstruction were done. On radiographic examination, it was found that developing permanent teeth in the left maxillary quadrant (21, 22, 23, 24, and 25), right maxillary permanent central incisor (11), and erupted left maxillary permanent molar (26) were severely malformed. Teeth showed a lack of contrast and no demarcation between enamel and dentin and wide pulp chamber. 11, 21, 22, 23, 24, and 25 showed a lack of root formation. All of these radiological findings were indicative of hypoplastic and hypomineralization type of tooth development [Figure 4]. Hematological investigation showed a normal blood profile and sodium, calcium, phosphate, ALP, and acid phosphatase within normal range. This case was one of the rarest cases of regional odontodysplasia crossing midline. Clinical Features of the cases is given in [Table 1].
| Discussion | | |
Regional odontodysplasia is a rare developmental anomaly of dental hard tissue. According to PubMed database, only 168 cases are reported until 2019.[15] It is not considered a hereditary disorder because etiology and pathogenesis are not completely known.[9],[16] Many theories of development of regional odontodysplasia are suggested by the researchers, among them vascular disorder most common. According to these theories, any vascular disorder in the region of tooth development leads to deficient blood circulation in that region leads to ischemia and abnormal development of dental hard tissue.[18] Other than vascular disorder, several other etiological factors can be responsible for the development of regional odontodysplasia.[8],[14],[15],[17],[19] These are,
- Local trauma/infection
- Teratogenic drugs
- Rh incompatibility
- Irradiation
- Neural damage
- Hyperpyrexia
- Metabolic and nutritional disorders
- Vitamin deficiency
- Activation of latent virus residing in odontogenic epithelium
- Somatic mutations of the PAX9 gene and disorder of must cell migration.
Regional odontodysplasia is established by eliminating other similar disorders such as amelogenesis imperfecta (AI), dentionogenesis imperfecta (DI), hypophosphatasia, and dentin dysplasia. AI and DI are inherited disorders whereas regional odontodysplasia is not considered an inherited disorder.Hypophosphatasia affects both bone and teeth, and there is a decrease in ALP enzyme activity as well as alveolar bone loss.[20] As name suggests, dentin dysplasia mainly affects dentin formation and shows radiological features of short or no root, called “rootless tooth.”[21] Clinical and Diagnostic features of Regional odontodysplasia is given in [Table 2].[8],[14],[17],[20],[21] | Table 2: Represents general features and diagnostic criteria of Regional Odontodysplasia
Click here to view |
Treatment of regional odontodysplasia should be done by a multidisciplinary approach.[13] The major goal of therapy should be to maintain space, restore masticatory function and aesthetics, reduce the psychological impact of missing teeth, maintain vertical dimension, and monitor and maintain normal jaw growth to prevent facial asymmetry.[11]
Hence, a team of plastic surgeon, oral and maxillofacial surgeon, pedodontist, orthodontist, and esthetic dentist is required to successfully treat a patient suffering from regional odontodysplasia. However, approaching toward treatment multidisciplinary team should consider several factors such as age of the child, medical history, previous dental experience and child's parental attitude regarding treatment, and number of teeth affected. There are primarily two treatment strategies suggested in the literatures: (1) in severe abscess cases, immediate extraction followed by prosthetic rehabilitation such as removable partial dentures in places of missing teeth, and (2) conservative approach such as restoration of carious teeth and long-term follow-up until the child's skeletal growth is completed, then rehabilitation such as implant.[21] Cahuana et al. also suggested autotransplantation as a treatment option, but it is limited to donor availability. Regular follow-up is essential for improving the quality of life of patients.[13]
| Conclusion | | |
Regional odontodysplasia is a rare nonhereditary developmental anomaly of dental hard tissue with unknown etiological origin affecting both primary and permanent dentition. It is generally limited to a single quadrant of the jaw and rarely crosses the midline. It should be treated with a multidisciplinary approach to improve the esthetic, psychological, and functional quality of life. This article wishes to add valuable clinical and radiological information about this rare disorder and improve the diagnostic knowledge of dentists.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 2. | Hitchin AD. Unerupted deciduos teeth in a youth aged 15 ½. Br Dent J 1934;56:631-3. |
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| 15. | Riolobos González MF, García-Navas Fernández de la Puebla L, Costa Ferrer F, Zubizarreta Macho A, Chico Hernández L. Clinical, radiographic and histologic evaluation of regional odontodysplasia: An unusual case report with 6-year follow-up. Int J Dent Oral Health 2020;7. [doi.org/10.16966/2378-7090.341]. |
| 16. | Gerlach RF, Jorge J Jr., de Almeida OP, Coletta RD, Zaia A. Regional odontodysplasia. Report of two cases. Oral Surg Oral Med Oral Pathol 1998;85:308-13. |
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| 18. | Schmid-Meier E. Unilateral odontodysplasia with ipsilateral hypoplasia of the mid-face. A case report. J Maxillofac Surg 1982;10:119-22. |
| 19. | Koskinen S, Keski-Filppula R, Alapulli H, Nieminen P, Anttonen V. Familial oligodontia and regional odontodysplasia associated with a PAX9 initiation codon mutation. Clin Oral Investig 2019;23:4107-11. |
| 20. | Ide M, Oshima Y, Chiba T, Adaniya A, Kuroki T, Asada Y. Regional odontodysplasia in maxillary right anterior regione first report: Clinical management in relation to 2 case reports. Pediatric Dent J 2020;30:124-8. |
| 21. | Silva Cunha JL, Barboza Santana AV, Alves da Mota Santana L, Meneses Santos D, de Souza Amorim K, Maciel de Almeida Souza L, et al. Regional odontodysplasia affecting the maxilla. Head Neck Pathol 2020;14:224-9. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]
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