Indian Journal of Dental Research

CASE REPORT
Year
: 2014  |  Volume : 25  |  Issue : 1  |  Page : 102--106

Unilateral segmental odontomaxillary dysplasia: A rare entity of 3 cases and review


Shalu Rai, Rohit Malik, Sapna Panjwani, Deepankar Misra, Sankalp Verma 
 Department of Oral Medicine and Radiology, Institute of Dental Studies and Technology, Modinagar, Uttar Pradesh, India

Correspondence Address:
Shalu Rai
Department of Oral Medicine and Radiology, Institute of Dental Studies and Technology, Modinagar, Uttar Pradesh
India

Abstract

Segmental odontomaxillary dysplasia (SOD) is an uncommon, nonhereditary, rare developmental disorder primarily involving the posterior part of the maxilla and related dental components. It is a rare condition of uncertain etiology that results in painless unilateral expansion of the posterior dentoalveolar complex, gingival hyperplasia, lack of one or both premolars in the affected area, delayed eruption of adjacent teeth and malformations of the primary molars. Radiographically, the affected bone is thickened and irregular in outline, with a coarse trabecular pattern that is vertically oriented resulting in a relatively radioopaque granular appearance. The treatment is focused on extraction of the involved teeth, while in some milder cases the teeth may be retained for a long period. The treatment plan should be based on the degree of involvement as well as the functional and esthetic needs in each case. Considering the rarity of the condition, we report three cases of SOD with variable presentations in a 45-year-old, 24-year-old and 23-year-old individual.



How to cite this article:
Rai S, Malik R, Panjwani S, Misra D, Verma S. Unilateral segmental odontomaxillary dysplasia: A rare entity of 3 cases and review.Indian J Dent Res 2014;25:102-106


How to cite this URL:
Rai S, Malik R, Panjwani S, Misra D, Verma S. Unilateral segmental odontomaxillary dysplasia: A rare entity of 3 cases and review. Indian J Dent Res [serial online] 2014 [cited 2020 Nov 27 ];25:102-106
Available from: https://www.ijdr.in/text.asp?2014/25/1/102/131154


Full Text

Hemimaxillofacial dysplasia (HD) was first recognized by Miles, Lovas and Cohen in 1987. This disorder has similar clinical manifestations as segmental odontomaxillary dysplasia (SOD), but may demonstrate some degree of variable expressivity, for example, facial asymmetry, facial hypertrichosis, unilateral maxillary dysplasia and hypoplastic teeth. [1] In 1990, similar features were described by Danforth et al. with absence of facial hypertrichosis, and this was termed as "segmental odontomaxillary dysplasia (SOD)." [1] SOD is an uncommon, nonhereditary developmental anomaly involving the maxilla, gingiva and dentition of the same arch. [2] The etiology of SOD is unclear. Reports have suggested a viral or bacterial infection or a local developmental abnormality that originates in utero. The diagnosis of SOD is mainly based on clinical and radiographic presentation, but may be augmented by histological findings. [2] To the best of our knowledge, 44 cases have been reported in the literature, including the three cases in our report.

 Case Report



Two male patients aged 45 years and 24 years reported to the outpatient department for consultation of painless growths in the right maxilla and a 23-year-old male patient reported with a painless growth on the left side. All the three patients have the same complaint since birth, which progressed with advancing age. All the patients were otherwise asymptomatic, with an unremarkable medical and family history. The clinical, radiological and histological features of all the patients are discussed in this case report.

On clinical examination, the 45-year-old patient revealed a lesion involving the right maxilla and producing no significant facial asymmetry beyond a mild fullness of the right cheek. There was marked increase in the buccopalatal dimension of the maxillary right alveolus along with gingival hyperplasia extending from the distal portion of 15 to the distal of 18, which was more pronounced on the palatal side. Teeth 11, 21-28 were uneventfully extracted 2 years back. The rest of the teeth appeared to be normal with the absence of any hypoplasia and displacement. Other cutaneous findings revealed the presence of ipsilateral hypertrichosis. Clinical examination of the 24-year-old male patient also revealed slight facial asymmetry along with fullness of the right cheek. Intraoral examination revealed a marked buccopalatal expansion of the maxillary right alveolus with a gingival overgrowth extending from the distal of 14 to the distal portion of 18, and was more pronounced on the buccal side. Tooth 16 was found to be missing, with a history of extraction 3 years back. No abnormality was detected pertaining to the morphology of the teeth, except for a marked palatal displacement of tooth 15 and buccal displacement of teeth 17 and 18. There was absence of any cutaneous manifestation. Clinical examination of the 23-year-old individual also revealed a marked buccopalatal expansion of the left maxillary alveolus along with gingival hyperplasia extending from the 17 to 18 region. Fracture with respect to tooth 14 was evident and teeth 15-17 were found to be missing, with a history of extraction as the patient met with an accident 6 months back. There was no abnormality pertaining to the morphology of the teeth and there were no cutaneous findings [Figure 1].{Figure 1}

Radiographical manifestations

Case 1

The intraoral periapical radiograph with respect to teeth 16-18 revealed an ill-defined granular bone pattern along with horizontal bone loss [Figure 2]a. A maxillary cross-sectional occlusal radiograph did not reveal any significant bone changes except for missing teeth with respect to teeth 21-28 and 11 and bone loss with respect to teeth 12-15 and an ill-defined radiolucency on the periapical region with respect to tooth 15 [Figure 2]d. The panoramic radiograph revealed missing teeth 21-28, 11, 31-33, 37 and 41-44 and generalized bone loss with sparse trabecular pattern in the mandibular body. An ill-defined homogenous radio opacity evident 3-4 mm apical to the distal root of tooth 38, suggestive of idiopathic osteosclerosis, and also external root resorption with respect to the distal root of tooth 38 were evident. No other abnormality of the sinus, teeth, jaws and associated structures was found [Figure 3]a.{Figure 2}{Figure 3}

Case 2

The intraoral periapical radiograph with respect to teeth 15, 17 and 18 revealed missing tooth 16 with pneumatization of the maxillary sinus with respect to the tooth 16 region [Figure 2]b. The maxillary cross-sectional occlusal radiograph revealed palatally displaced tooth 15 and buccally displaced teeth 17 and 18 along with missing tooth 16. No other pathology or distinct changes were evident [Figure 2]e. The panoramic radiograph revealed missing tooth 16, displacement with respect to tooth 15 and fractured crown with respect to tooth 11. No other abnormality of the teeth, jaws and associated structures was found [Figure 3]b.

Case 3

The intraoral periapical radiograph with respect to teeth 14-17 revealed missing teeth 15-17 [Figure 2]c. The maxillary cross-sectional occlusal radiograph revealed root canal filling material with respect to tooth 11, missing teeth 15-17 and fractured tooth 14. No other pathology or distinct changes were evident [Figure 2]f The panoramic radiograph taken before the extraction of teeth revealed the presence of two miniplates in the symphyseal region, missing teeth 34, 35, 36 and 44 and periapical pathology with respect to tooth 34. No other abnormality of the teeth, jaws and associated structures was found [Figure 3]c.

Histological sections revealed regular short, broad, bony trabeculae with irregular basophilic cemental lines. Some areas in the trabeculae showed crowding of cementocytes. Some areas showed immature bony trabeculae with minimal calcification and osteoblastic rimming. Marrow spaces showed laying down of loose fibrous tissue[Figure 4].{Figure 4}

 Discussion



The present cases are a combination of the features of HD and SOD because of the absence of dental abnormalities and presence of hypertricosis. The acronym HATS (Hemimaxillary enlargement, Asymmetry of the face, Teeth abnormalities, Skin findings) was introduced in 2004 by Welsch and Stein. [3] They reported two cases with skin lesions. One patient reported having bakers nevus. Our cases had all the features of HATS, with additional cutaneous findings in the form of facial hypertricosis. [3] According to a literature review published between 1987 and 2011, [2],[3],[4],[5],[6],[7],[8] 41 cases of SOD/HD have been published, although the exact number still varies as all the cases are not well documented. We present the clinical features including the cutaneous findings along with radiographic features [Table 1] for a total of 44 cases including the present cases. The age of the patient at presentation is variable but the condition is usually discovered during childhood, with most common complaints being missing teeth, abnormal spacing and delayed eruption. [9] This condition is slightly more common in males than in females. No tendency for its occurrence in any specific ethnic group has been reported. [2] Radiographically, there is presence of thick and coarse, irregularly oriented bony trabeculae producing a sclerotic appearance. Also, there can be a presence of vertical orientation of trabeculae in some patients. The above features were not obvious in our patients. The following conditions were considered for differential diagnosis:{Table 1}

Monostotic fibrous dysplasia

Fibrous dysplasia or a related fibro-osseous lesion was considered early because of the unilateral maxillary expansion. Biopsy, however, did not reveal the typical histologic "Chinese character" pattern encountered in fibrous dysplasia. [5]

Regional odontodysplasia

Clinical and radiographic features were suggestive of regional odontodysplasia. In that condition, the affected teeth may fail to erupt and exhibit a radiographic pattern described as pale, wispy tooth images with a lack of contrast between dentin and enamel. Soft tissue swelling has also been reported. [5]

Gingival fibromatosis

Because of the maxillary enlargement and fibrous gingivae in the canine area, this condition was briefly considered. [5]

Central hemangioma

Radiographic imaging may show a honeycomb or soap-bubble appearance and, sometimes, resorption of tooth roots in the area. Before biopsying the lesion, aspiration was attempted but no excessive blood flow occurred. [5]

Treatment of SOD remains unknown as the management protocol in most case reports has not been discussed to date. Once diagnosed, SOD seems to remain stable and may or may not require surgical intervention. The primary goal of the treatment is to retain the deciduous teeth thus facilitating eruption of the permanent teeth so that occlusion can be restored. Usually, definitive treatment is delayed until after the pubertal growth spurt. The lack of reports in adults suggests the possibility of spontaneous regression with age. [2] But, all our cases reported are adults; therefore, the standard of care is close observation and limited recontouring in cosmetically objectionable cases. Even though SOD is a rare entity, the radiologist must consider SOD in the differential diagnosis of entities such as monostotic fibrous dysplasia, regional odontodysplasia, hemifacial hyperplasia and gingival fibromatosis. The clinical and radiological features of SOD are well described but the condition is probably underreported due to misdiagnosis. Thus, it is important to recognize this unusual unilateral developmental anomaly. [2]

This report with a review of the literature attempts to increase the knowledge about SOD among general dentists and, furthermore, to encourage long-term treatment and follow-up reports that are lacking in the literature.

References

1Becktor KB, Reibel J, Vedel B, Kjnr I. Segmental odontomaxillary dysplasia: Clinical, radiological and histological aspects of four cases. Oral Dis 2002;8:106-10.
2Kuklani RM, Nair MK. Segmental odontomaxillary dysplasia: Review of the literature and case report. Int J Dent 2010;2010:837283.
3Welsch MJ, Stein SL. A syndrome of hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings (HATS). Pediatr Dermatol 2004;21:448-51.
4Packota GV, Pharoah MJ, Petrikowski CG. Radiographic features of segmental odontomaxillary dysplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;82:577-84.
5DeSalvo MS. Segmentai odontomaxillary dysplasia (hemimaxillofacial dysplasia): Case report. Pediatr Dent 1996;18:154-6.
6Paticoff K, Marion RW, Shprintzen RJ, Shanske AL, Eisig SB. Hemimaxillofacial dysplasia: A report of two new cases and further delineation of the disorder. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83:484-8.
7Prusack N, Pringle G, Scotti V, Chen SY. Segmental odontomaxillary dysplasia: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:483-8.
8Velez I, Vedrenne D, Cralle P, Yap S. Segmental odontomaxillary dysplasia. Report of two cases and review of the literature. Today's FDA 2002;14:20-1.
9Buyse ML. Birth defects encyclopedia. The Center for Birth Defects Information Services, Inc., Blackwell Scientific Publications: Cambridge, MA: Blackwell Scientific Publications; 1990. p. 1646-7.