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  Table of Contents    
CASE REPORT
Year : 2014  |  Volume : 32  |  Issue : 1  |  Page : 83-86
 

Segmental odontomaxillary dysplasia


1 Department of Oral Medicine and Radiology, Mahatma Gandhi PostGraduate Institute of Dental Sciences, Pondicherry, Puducherry (UT), India
2 Department of Oral Medicine and Radiology, Krishnadevaraya Dental College and Hospital, Bengaluru, Karnataka, India

Date of Web Publication15-Feb-2014

Correspondence Address:
Arti Agrawal
Department of Oral Medicine and Radiology, Mahatma Gandhi Postgraduate Institute of Dental Sciences, Gorimedu, Indira Nagar, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.127071

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   Abstract 

Segmental odontomaxillary dysplasia is a type of hemi-maxillofacial dysplasia. It is a sporadic, mesoectodermal dysplasia that presents early in life, possibly as early as in utero and exhibits male gender predominance (1.7:1.0). Its features include enlargement of the soft tissue and/or bone of one hemimaxilla that may produce mild facial asymmetry, sclerotic radiographic bone changes and dental developmental abnormalities. The dense bone, which often exhibits a radiographic vertical orientation of the trabecular bone pattern, is generally associated with delayed eruption of the teeth. Congenitally missing premolar teeth (eithe or both) is a common feature of this condition that is of significant diagnostic value. Ipsilateral cutaneous findings have been reported in 23% of cases. Immature woven bone forming irregular patterns are seen histologically. Herein, we present a case report on segmental odontomaxillary dysplasia in a
13-year-old boy.


Keywords: Facial asymmetry, hemimaxillofacial dysplasia, segmental odontomaxillary dysplasia


How to cite this article:
Agrawal A, Daniel J, Vijeev V. Segmental odontomaxillary dysplasia. J Indian Soc Pedod Prev Dent 2014;32:83-6

How to cite this URL:
Agrawal A, Daniel J, Vijeev V. Segmental odontomaxillary dysplasia. J Indian Soc Pedod Prev Dent [serial online] 2014 [cited 2019 Dec 8];32:83-6. Available from: http://www.jisppd.com/text.asp?2014/32/1/83/127071



   Introduction Top


Segmental odontomaxillary dysplasia is a rare developmental disorder that causes a unilateral disturbance of the maxilla associated with an abnormal growth and maturation of bone, teeth and adjacent gingival tissue, with or without facial cutaneous lesions. [1]

The prevalence of this condition is not well established as the available literature regarding this condition is scanty and largely consists of case reports only. [4] Until date, <40 cases have been reported in the English literature and to our knowledge this is the third case in the Indian scenario.

The age of onset ranges from 5-27 years but is commonly diagnosed in the first decade of life with slight male predominance. [1] The etiology of segmental odontomaxillary dysplasia is unclear and diagnosis is mainly based on clinical and radiographic presentation. [4]


   Case Report Top


The present case report is about a 13-year-old boy who reported to the Department of Oral Medicine and Radiology, Mahatma Gandhi PostGraduate Institute of Dental Sciences, Pondicherry, India with a chief complaint of swelling inside the mouth in relation to the left side upper jaw for the past 1 month. Swelling was gradually progressing in size and it was not associated with pain. Previous medical and dental history of the patient was non-contributory.

On examination, there was gross facial deformity and bony hard swelling on the left side middle one-third of the face; and loss of left eye from birth [Figure 1]. Deviated nasal septum was present. Skin over the swelling was normal with no evidence of hyperpigmentation.
Figure 1: Gross facial asymmetry and bony hard swelling

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Intra-orally a single approximately 4 cm × 3 cm bony hard swelling on the buccal aspect of left maxillary posterior alveolar ridge involving the left maxillary first molar and extending posteriorly until the maxillary tuberosity [Figure 2]. Erupting left maxillary second molar was present over the bony swelling. Mucosa over the swelling was normal and there was no tenderness on palpation over the swelling. Clinically retained first and second deciduous molar teeth were present. On the basis of clinical features, a provisional diagnosis of unilateral segmental odontomaxillary dysplasia was given. Differential diagnosis of monostotic fibrous dysplasia, regional odontodysplasia, gingival fibromatosis, central hemangioma and tumour of bone were considered.

Radiographic examination of the patient revealed retained deciduous and delayed eruption of the premolar teeth, altered trabecular pattern and haziness in the left maxillary sinus with reduced size of the sinus [Figure 3]. Intra-oral peri-apical radiograph revealed radio-opacity distal to left maxillary first molar suggestive of bony swelling with erupting second and third maxillary molar teeth [Figure 4]. Computed tomography revealed involvement of maxillary bone including the floor of maxillary sinus and hard palate [Figure 5]. It also showed the deviation of the nasal septum towards the right side [Figure 6]. On the basis of clinical findings; and radiographic findings, a clinical diagnosis of unilateral segmental odontomaxillary dysplasia was made.
Figure 2: Bony hard swelling and expansion of jaw with retained deciduous and missing premolar teeth

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Figure 3: Altered trabecular pattern and reduced size of the left maxillary sinus

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Figure 4: Radio-opacity distal to 26 with erupting molar teeth

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Figure 5: Coronal computed tomography image showing reduced size of the left maxillary sinus

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Figure 6: Coronal computed tomography image showing deviation of nasal septum

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   Discussion Top


Hemi-maxillofacial dysplasia was first recognized by Miles DA et al. in 1987 in a report of two cases. This disorder has similar clinical manifestations as segmental odontomaxillary dysplasia but may demonstrate some degree of variable expressivity. Danforth RA et al. in 1990 reported a series of eight cases and named the condition. Packota GV et al. in 1996 reported most common radiographic features of segmental odontomaxillary dysplasia in a study of 12 cases. [4] Segmental odontomaxillary dysplasia has only recently been delineated from other osseo-odontogenic dysplasias. [4]

Segmental odontomaxillary dysplasia is an uncommon, nonhereditary, non-progressive developmental disorder affecting the upper jaw and related dental components. [4]

The etiopathogenesis of segmental odontomaxillary dysplasia is unknown, although it is suggested that it might be due to a localized developmental abnormality in utero, a unilateral developmental field defect involving anlagen of the first and second branchial arches, or a post-zygotic mutation or a similar phenomenon affecting a progenitor cell and could have resulted in an altered clone of cells that influenced the morphogenesis of the ecto-mesodermal tissues in a segment of the maxilla and the overlying facial tissues. A viral or bacterial infection of the maxillary division branches of the trigeminal nerve have been proposed as initial causative factors in segmental odontomaxillary dysplasia. [7],[ 8]

All cases reported appear to represent sporadic occurrence with no inheritance pattern. Males were affected more than females with a male-to-female ratio of 1.8:1. The condition is commonly diagnosed in the first decade. The right and left sides of the maxilla are almost equally affected. [7]

Clinically, segmental odontomaxillary dysplasia usually presents as a non-progressive facial asymmetry, ipsilateral gingivo-dento-alveolar maxillary involvement from the canine eminence anteriorly until the maxillary tuberosity posteriorly and with or without facial cutaneous lesions. The most common complaints being missing teeth, abnormal spacing; and delayed eruption. [7],[ 9]

Radiographically, segmental odontomaxillary dysplasia shows mainly ill-defined radio-opacity with thickened and coarse irregular bone trabeculation pattern and reduction of the maxillary sinus size. [1],[ 7]

Facial cutaneous manifestations of segmental odontomaxillary dysplasia are variable and affect up to 48% of patients of whom 80% are males. The most common facial cutaneous manifestations are hypertrichosis followed by facial erythema, lip hypopigmentation; and Becker's nevus. Other cutaneous manifestations include "hairy nevus," discontinuity of the vermilion border, depression of the cheek; and facial hyperpigmentation. When hypertrichosis is the associated cutaneous lesion, it is only limited to males. [7]

Clinical features of our case (unilateral bony expansion of the maxilla, delayed eruption of premolar teeth and gross non-progressive asymmetry of mid-face) correlated well with the original description of the condition. Radiographic feature of an ill-defined radio-opacity in the affected area and an almost imperceptible maxillary sinus correlated with the findings reported by Wilfredo Alejandro et al.

Treatment can only be limited to restoring the functional dental occlusion in the affected side by orthodontic tooth movement and/or prosthetic replacement of missing teeth and should be delayed until the pubertal growth spurt has ended. [7],[15]


   Conclusion Top


Segmental odontomaxillary dysplasia is a rare condition and characterized by variability of its clinical and radiological features and may mimic other fibro-osseous lesions. Therefore, the definitive diagnosis of the lesion remains dependent on histological evaluation. As gingiva-dento-alveolar involvement is constant finding in all cases of segmental odontomaxillary dysplasia, the role of the dentist is crucial in early diagnosis. [10]

 
   References Top

1.González-Arriagada WA, Vargas PA, Fuentes-Cortés R, Nasi-Toso MA, Lopes MA. Segmental odontomaxillary dysplasia: Report of 3 cases and literature review. Head Neck Pathol 2012;6:171-7  Back to cited text no. 1
    
2.Gorlin RJ,Cohen MM,Hennekam RC. Syndromes with unusual facies: Other syndromes. In: Motalsky AG,Bobrow M, Harper PS, Scriver C, editors. Text Book of Syndromes of the Head and Neck. 4 th ed. McGraw Hill. Inc and Oxford university press, U.S.A.; 2001, p. 1048.  Back to cited text no. 2
    
3.Bhatia SK, Drage N, Cronin AJ, Hunter ML. Case report: Segmental odontomaxillary dysplasia - A rare disorder. Eur Arch Paediatr Dent 2008;9:245-8.  Back to cited text no. 3
    
4.Kuklani RM, Nair MK. Segmental odontomaxillary dysplasia: Review of the literature and case report. Int J Dent 2010;2010:837283.  Back to cited text no. 4
    
5.Pandey S, Pai KM, Nayak AG, Vineetha R. Unilateral segmental odontomaxillary hypoplasia: An unusual case report. Imaging Sci Dent 2011;41:39-42.  Back to cited text no. 5
    
6.Drake DL. Segmental odontomaxillary dysplasia: An unusual orthodontic challenge. Am J Orthod Dentofacial Orthop 2003;123:84-6.  Back to cited text no. 6
    
7.Shah A, Latoo S, Ahmed I, Malik AH, Hassan S, Bhat A, et al. Midline segmental odontomaxillary dysplasia. Ann Maxillofac Surg 2012;2:185-9.  Back to cited text no. 7
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8.Pandey S, Pai KM, Nayak AG, Vineetha R. Unilateral segmental odontomaxillary hypoplasia: An unusual case report. Imaging Sci Dent 2011;41:39-42.  Back to cited text no. 8
    
9.Drake DL. Segmental odontomaxillary dysplasia: An unusual orthodontic challenge. Am J Orthod Dentofacial Orthop 2003;123:84-6.  Back to cited text no. 9
    
10.Whitt JC, Rokos JW, Dunlap CL, Barker BF. Segmental odontomaxillary dysplasia: Report of a series of 5 cases with long-term follow-up. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:e29-47.  Back to cited text no. 10
    
11.Prusack 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.  Back to cited text no. 11
    
12.Yassin OM, Rihani FB. Combined cutaneous findings with segmental odontomaxillary dysplasia: Review of the literature and proposal of a new clinical classification. Int Med Case Rep J 2008;1:7-11.  Back to cited text no. 12
    
13.Miles DA, Lovas JL, Cohen MM. Hemimaxillofacial dysplasia a newly recognized disorder of facial asymmetry, hypertrichosis of the facial skin, Oral Surg Oral Med Oral Pathol 1987 Oct;64:445-8.  Back to cited text no. 13
    
14.Danforth RA, Melrose RJ, Abrams AM, Handlers JP. Segmental odontomaxillary dysplasia. Oral Surg Oral Med Oral Pathol 1990 Jul;70:81-5  Back to cited text no. 14
    
15.Packota GV, Pharoah MJ, Petrikowski CG. Radiographic features of segmental odontomaxillary dysplasia. A study of 12 cases. Oral Surg Oral Med Oral Pathol Oral Radiolendod 1996 Nov;82:577-84.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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    Abstract
   Introduction
   Case Report
   Discussion
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    References
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