Indian Journal of Dental Research

CASE REPORT
Year
: 2015  |  Volume : 26  |  Issue : 2  |  Page : 210--213

Non-syndromic bilateral condylar aplasia: A rare case


Peeyush Shivhare, S Lata, P Balaji, Ashish Gupta 
 Department of Oral Medicine and Radiology, Raja Rajeswari Dental College and Hospital, Ramholli Cross, Kumbalgodu, Bengaluru, Karnataka, India

Correspondence Address:
Dr. Peeyush Shivhare
Department of Oral Medicine and Radiology, Raja Rajeswari Dental College and Hospital, Ramholli Cross, Kumbalgodu, Bengaluru, Karnataka
India

Abstract

The temporomandibular joint is one of the most complex joints of the human body. It consists of the mandibular condyle, glenoid fossa and the articular eminence of the temporal bone. Aplasia of the condyle is usually seen as part of a syndrome otherwise it is rare. We report a case of bilateral condylar aplasia in a 20-year-old male not associated with any syndrome. The patient reported to the department with a chief complaint of the underdeveloped lower jaw. Clinical examination, conventional radiographs, and 3D computer tomography images revealed a complete absence of condyle on the right and left sides.



How to cite this article:
Shivhare P, Lata S, Balaji P, Gupta A. Non-syndromic bilateral condylar aplasia: A rare case.Indian J Dent Res 2015;26:210-213


How to cite this URL:
Shivhare P, Lata S, Balaji P, Gupta A. Non-syndromic bilateral condylar aplasia: A rare case. Indian J Dent Res [serial online] 2015 [cited 2020 Oct 29 ];26:210-213
Available from: https://www.ijdr.in/text.asp?2015/26/2/210/159173


Full Text

Temporomandibular joint (TMJ) is considered a ginglymous diarthrodial joint capable of both rotational and translatory movements. It consists of the mandibular condyle, glenoid fossa and the articular eminence of the temporal bone. The congenital deformities and developmental abnormalities of the mandibular condyle can be classified as hypoplasia or aplasia, hyperplasia, and bifidity. Hypoplasia or aplasia of the mandibular condyle indicates underdevelopment or nondevelopment associated mainly with various craniofacial abnormalities. These may be either congenital or acquired. [1]

Growth disturbances in the development of mandibular condyle may occur in utero late in the first trimester and may result in disorders such as aplasia or hypoplasia of the mandibular condyle. As compared to hypoplasia, hyperplasia of the mandibular condyle is not visible at birth and seems to be gradually acquired during growth. [1]

 Case Report



A 20-year-old male reported to the oral medicine and radiology department with a chief complaint of the underdeveloped lower jaw, which was first noticed during childhood and gradually progressed. There was no history of any trauma or any systemic diseases during birth. Patient's parents gave a history of consanguineous marriage. There was no family history of the present problem.

The general physical examination did not reveal any abnormalities. The vital signs were within normal limits. Extraoral examination revealed facial asymmetry with severe retruded mandible [Figure 1] and [Figure 2]. Mouth opening was restricted (25 mm) with no deviation or deflection [Figure 3]. Condyles were not palpable on both sides. Intraorally there was crowding in upper and lower anterior teeth with increased overjet and overbite. The molar relation was angles class II bilaterally. Deep palate was also seen. No other important clinical extraoral or intraoral findings were observed. Based on clinical findings, a provisional diagnosis of bilateral ankylosis and differential diagnosis of bilateral condylar hypoplasia or aplasia were given.{Figure 1}{Figure 2}{Figure 3}

After clinical examination, radiographic examinations were performed. Panoramic radiograph showed complete absence of condyle on the right side and left a side. Glenoid fossa was not developed on both sides. Ramus and body of the left side were comparatively smaller than the right side. Antigonial notch was prominent bilaterally [Figure 4]. Posterioanterior view showed facial asymmetry [Figure 5]. Lateral skull views showed severe retruded and micrognathic mandible [Figure 6]. Condylar aplasia on right and left sides was confirmed by reverse town and transpharngeal views. Computer tomography (CT) was advised for additional information. 3D CT showed complete condylar aplasia on the left side and condylar aplasia with two bony projections on right side [Figure 7], [Figure 8], [Figure 9], [Figure 10]. Magnetic resonance imaging showed complete absence of articular disc bilaterally [Figure 11]. After radiographic confirmation, patient was advised complete systemic evaluation and referred to General medicine, cardiology, ophthalmology, ear, nose, and throat and orthopedics to rule out any syndromes. The medical evaluation revealed no abnormalities. Based on the clinical and radiographic findings, a final diagnosis of nonsyndromic agenesis of right and left condyle was given. Patient was referred to oral surgeon and orthodontist for the best possible treatment.{Figure 4}{Figure 5}{Figure 6}{Figure 7}{Figure 8}{Figure 9}{Figure 10}{Figure 11}

 DISCUSSION



The TMJ initially develops as two separate areas of mesenchymal blastemas that are, condylar blastema and temporal blastema near the eventual location of the mandibular condyle and glenoid fossa in the 8 th week of gestation. [2],[3] The mandibular condyle and temporal blastemas move towards each other as the joint develops by 12 th week. At birth, the articular surfaces of both the mandibular condyle and temporal bones are covered with fibrous connective tissue. Later, this tissue is slowly converted to fibrocartilage as the fossa deepens, and the mandibular condyle develops under functional influences. [4],[5]

Congenital (primary) condylar hypoplasia is characterized by unilateral or bilateral underdevelopment of the mandibular condyle and usually occurs as part of some systemic condition originating in the first and second branchial arches [Table 1]. [1],[6],[7],[8]{Table 1}

Acquired (secondary) condylar hypoplasia takes place if the condyle is injured during active growth because of which development may be arrested. The most common causes are mechanical injury, such as trauma (before the age of 2), infection of the joint itself or the middle ear, childhood rheumatoid arthritis, radiotherapy and Parathyroid hormone-related protein deficiency, which affect bone formation and chondrocyte differentiation. [1],[9],[10]

In a newborn child, the TMJ is mobile. After the eruption of the first permanent dentition at the age of 7 years, the articular eminence begins to be prominent, and its development continues at an accelerated pace until 12 years of age. If a condyle is present, an adaptive well-defined fossa and articular eminence should be present. Hence, in the case of the absence of a condyle, those coherent structures should also be absent. [11],[12] In our case, the complete absence of the right and left condyle and glenoid fossa were observed.

Aplasia of the mandibular condyle without any other facial malformations is an extremely rare condition. [8] There are very less cases of nonsyndromic mandibular condyle aplasia reported. [9],[13],[14],[15],[16],[17],[18] Our case also presented condylar aplasia without any other features suggestive of any syndrome.

Various treatment approaches have been proposed for treating condylar aplasia and possibilities for influencing mandibular growth. Most of the time, it is treated by multimode with the help of an oral surgeon, general surgeon, plastic surgeon, and orthodontics. [9],[17],[19]

The treatment could then be a costochondral graft transplant, preferably before the growth spurt, orthognathic surgery at the end of the growth period, or both. [19] Surgery is often required, but the timing and regimen of this choice are still an issue to be resolved. [17]

 CONCLUSION



Nonsyndromic condylar hypoplasia and aplasia are exceedingly rare conditions. We report a rare case of bilateral total condylar aplasia, not related to any pathological disorder. This condition should be separated from different associated syndromes based on different clinical features and investigations. A very few case reports are published in the literature, to date. Hence, our case is very special in this aspect.

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