|Year : 2014 | Volume
| Issue : 1 | Page : 57-59
Condylar aplasia with contralateral temporomandibular joint ankylosis
Akoijam Ibohal Singh, Pebam Manisana Singh, Nilottpal Dey, Nepram Sanjib Singh
Department of Plastic Surgery, Regional Institute of Medical Sciences, Lamphelpat, Imphal, Manipur, India
|Date of Web Publication||24-Jun-2014|
Dr. Akoijam Ibohal Singh
Department of Plastic Surgery, Regional Institute of Medical Sciences, Lamphelpat, Imphal - 795 004, Manipur
Source of Support: None, Conflict of Interest: None
A rare case of unilateral aplasia of the mandibular condyle along with contralateral ankylosis of the temporomandibular joint in a 15 year old boy is presented here. It was not associated with any soft tissue aberrations suggestive of any syndromic etiology. Aplasia is a rare anomaly and refers to the insufficient development of the mandibular condyle. True agenesis of the mandibular condyle is extremely rare. The simultaneous existence of condylar aplasia on one side and temporomandibular joint ankylosis on the other side makes this case an even rarer one.
Keywords: Aplasia, Condyle, Mandible, Temporomandibular joint
|How to cite this article:|
Singh AI, Singh PM, Dey N, Singh NS. Condylar aplasia with contralateral temporomandibular joint ankylosis. J Med Soc 2014;28:57-9
| Introduction|| |
Aplasia of the mandibular condyle is one of the several facial manifestations of syndromes such as hemifacial microsomia, Goldenhaar's syndrome, and Treacher Collin's syndrome, especially the first two. ,, The incidence is estimated to be one in 5600 births, with Treacher Collin's syndrome even more rarely seen (Gorlin et al., 1990). Aplasia of the mandibular condyle without any other facial malformations is extremely rare but has been reported previously (Prowler and Glassman, 1954). In the present report, a patient with a unilateral missing mandibular condyle along with contralateral ankylosis of the temporomandibular joint is presented and the treatment outlined and discussed.
| Case Report|| |
A 15-year-old boy presented to the Department of Chest Medicine with chief complaints of sleep apnea and snoring. The parents of the boy gave a history of fall from a running tractor when he was only 4-years old. In the accident, he had sustained trauma to his abdomen for which an abdominal exploration was done. The parents could not remember any trauma to the face. On physical examination, there was reduced mouth opening [Figure 1]a and a receded chin [Figure 1]b. The patient was subsequently referred to plastic surgery services.
|Figure 1 : (a) Preoperative inadequate mouth opening; (b) Preoperative picture showing chin receding|
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At maximum mouth opening, i.e. an interincisional distance of hardly 10 mm, the chin deviated toward the right side. A panoramic radiograph orthopantomogram (OPG) revealed that the left mandibular condyle was totally absent and the ramus abruptly terminated below the level of the sigmoid notch. On the right side, there was ankylosis of the temporomandibular joint [Figure 2]a. In three-dimensional (3D) computerized tomography (CT) reconstruction, flattening of the temporal articular fossa and aplasia of the mandibular condyle [Figure 2]b were detected on the left side of the mandible. The right condyle was completely fused to the temporal bone.
|Figure 2 : (a) 3D CT face showing ankylosis of right Temporomandibular joint; (b) 3D CT scan face showing complete absence of left condyle. 3D = Three-dimensional, CT = Computed tomography, 3D = Three-dimensional. (c) CT = Computerized tomography view, CT = Computerized tomography|
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Our plan was to carry out a two-staged operation for the purpose of achieving a satisfactory mouth opening and correction of the receded chin. For the left side, as there was neither functional nor aesthetic deformity, no active intervention was planned. For the right side, in the first stage, gap arthroplasty along with interposition of temporalis muscle graft was performed. After a year, a second stage sliding genioplasty was done to correct the chin deformity. Following the operations, mouth opening of 3 cm was achieved without any attendant chin deviation [Figure 3]a. With the restoration of chin contour [Figure 3]b, there was improvement in sleep apnea and the symptoms of snoring also decreased.
|Figure 3 : (a) Postoperative picture with normal chin contour; (b) Postoperative picture with adequate mouth opening|
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| Discussion|| |
Developmental anomalies of the mandibular condyle are generally classified in terms of aplasia, hypoplasia, hyperplasia, and bifidity. Aplasia means the insufficient development of the mandibular condyle. ,
Aplasia of the mandibular condyle is one of several manifestations of varying expression in syndromes including hemifacial microsomia, Goldenhaar's syndrome, and Treacher Collin's syndrome. , The literature also suggests Proteus syndrome, Morquio syndrome, and auriculocondylar syndrome, which can demonstrate condylar malformations , and condyle agenesis. , In each of these conditions, in addition to condylar aplasia, these are other facial or skeletal malformations.
Mechanical trauma during active growth, inflammatory conditions like juvenile rheumatoid arthritis and radiotherapy may be counted among the most common causes of condyle anomalies. In the case of trauma, it has to have happened before the age of 2 years. 
Parathyroid hormone-related protein deficiency also affects bone formation and chondrocyte differentiation, leading to condyle malformation. 
Some authors further affirm that mandibular deficiency can occur without any defined etiology. 
Although our patient had a history of trauma, it was not sure whether it involved the mandible. Even if it was unknowingly involved, it happened after the age of 2 years, i.e. at 4 years of age.
Furthermore, our patient did not have any associated facial malformations suggestive of any syndrome. Aplasia of the mandibular condyle without any other facial malformation is an extremely rare condition. 
Aplasia of mandibular condyle is a rare entity producing functional and cosmetic disability, which requires early intervention to prevent facial asymmetries as well as other functional disorders.
| References|| |
|1.||Krogstad O. Aplasia of the mandibular condyle. Eur J Orthodont 1997;19:483-9. |
|2.||Priolo M, Lerone M, Rosaia L, Calcagno EP, Sadeghi AK, Ghezzi F, et al. Question mark ears, temporo-mandibular joint malformation and hypotonia: Auriculo-condylar syndrome or a distinct entity? Clin Dysmorphol 2000;9:277-80. |
|3.||Wan J, Meara JG, Kovanlikaya A, Nelson MD, Don D. Clinical, radiological, and audiological relationships in hemifacial microsomia. Ann Plast Surg 2003;51:161-6. |
|4.||Kaneyama K, Segami N, Hatta T. Congenital deformities and developmental deformities of the mandibular condyle in the temporomandibular joint. Congenit Anom (Kyoto) 2008;48:118-25. |
|5.||McNeill C. Management of temporomandibular disorders: Concepts and controversies. J Prosthet Dent 1997;77:510-22. |
|6.||De Lone DR, Brown WD, Gentry LR. Proteus syndrome: Craniofacial and cerebral MRI. Neuroradiology 1999;41:840-3. |
|7.||Morgan KA, Rehman MA, Schwartz RE. Morquio's syndrome and its anaesthetic considerations. Paediatr Anaesth 2002;12:641-4. |
|8.||Ozturk S, Sengezer M, Isik S, Gul D, Zor F. The correction of auricular and mandibular deformities in auriculo-condylar syndrome. J Craniofac Surg 2005;16:489-92. |
|9.||Storm AL, Johnson JM, Lammer E, Green GE, Cunniff C. Auriculo-condylar syndrome is associated with highly variable ear and mandibular defects in multiple kindreds. Am J Med Genet A 2005;138:141-5. |
|10.||Santosh KC, Dutra ME, Costa C, Lascala CA, Lascala CE, de Oliveira JX. Aplasia of mandibular condyle. Dentomaxillofac Radiol 2007;36:420-2. |
|11.||Bowden CM Jr, Kohn MW. Mandibular deformity associated with unilateral absence of the condyle. J Oral Surg 1973;31:469-72. |
|12.||de Senna RB, Silva VK, Franca JP, Marques LS, Pereira LJ. Imaging diagnosis of the temporomandibular joint: Critical review of indications and new perspective. Oral Radiol 2009;25:86-98. |
[Figure 1], [Figure 2], [Figure 3]