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CASE REPORT
Year : 2019  |  Volume : 16  |  Issue : 1  |  Page : 70-74

A case report: A rare presentation of mandibular condylar fibrous dysplasia


1 Department of Surgery, University of Ilorin, Ilorin, Nigeria
2 Department of Surgery, University of Ilorin Teaching Hospital, Ilorin, Nigeria
3 Department of Microbiology, University of Ilorin, Ilorin, Nigeria

Date of Web Publication5-Mar-2019

Correspondence Address:
Dr. Moshood Folorunsho Adeyemi
Department of Surgery, University of Ilorin, Ilorin
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njbcs.njbcs_4_18

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  Abstract 


Fibrous Dysplasia is a fibro-osseous lesion characterized by the progressive replacement of normal bone by fibrous connective tissue resulting from arrest of bone maturation from woven bone to lamellar bone by a missense mutation. We report a 33-year old patient who presented with inability to open her of a month duration. On examination, patient had facial assymetry, anterior and posterior crossbite but scissors bite on the contralateral side, mouth deviation to the left on mouth opening associated with limited mouth opening, and there was no apparent swelling at the right preauricular region. The patient subsequently underwent surgery under general anesthesia following computed tomography (CT) scan. Fibrous dysplasia is a rare occurrence of the mandibular condyle, and its possibility in this location should be considered, pending a histopathologic report and the clinical importance of CT scan in the evaluation of temporomandibular joint lesions should be emphasized.

Keywords: Condylectomy, cross-bite, fibro-osseous, missense mutation, occlusal splint, scissors bite


How to cite this article:
Adeyemi MF, Ernest MA, Igben E, Adeyemi HA. A case report: A rare presentation of mandibular condylar fibrous dysplasia. Niger J Basic Clin Sci 2019;16:70-4

How to cite this URL:
Adeyemi MF, Ernest MA, Igben E, Adeyemi HA. A case report: A rare presentation of mandibular condylar fibrous dysplasia. Niger J Basic Clin Sci [serial online] 2019 [cited 2019 Mar 21];16:70-4. Available from: http://www.njbcs.net/text.asp?2019/16/1/70/253410




  Introduction Top


Fibrous dysplasia (FD) is a benign intramedullary, fibro-osseous lesion characterized by the progressive replacement of normal bone by fibrous tissue.[1] Fibro-osseous lesions constitute 13% of oral tumors in Nigeria[2] and 9.4% in Ghana.[3] FD represents 2.5% of bone disorders and approximately 7% of benign bone lesion.[4] Fibro-osseous lesions (e.g., FD) affect the maxilla more than the mandible.[5] The presence of fibro-osseous lesion or FD in the mandibular condyle is rare.[6]

It is a slow-growing and self-limiting lesion, but a rapid growth rate around puberty is usually seen. Present understanding is that FD develops from a defect (genetic mutation or gene deletion) during bone formation at the embryonic stage, resulting in failure of bone to mature.[7] The cellular and molecular basis of this lesion has been reported to be caused by the mutation of stimulatory alpha subunit of G protein in the dysplastic bone lesion, which has caused alteration in the differentiation of osteoblastic cells and abnormal elevation of cAMP, thus affecting the structure of several target cells. The transcription and expression of downstream genes is affected, resulting in the alterations of osteoblast recruitment and function in dysplastic bone lesions.[8]

FD of the mandibular condyle may result in preauricular swelling, facial asymmetry, limited mouth opening, and jaw deviation on mouth opening and/or pain at the temporomandibular joint (TMJ). Radiographically, FD usually presents as a relatively well-defined mixed radio-opaque and radiolucent expansive lesion, with an imperceptible border. Computed tomography (CT) scan is the best imaging modality to depict bony changes in FD.[9] It is, however, important to note that magnetic resonance imaging (MRI) appearance may not be as specific as the CT scan.[10]

The treatment of FD can be conservative or surgical depending on a host of factors, some of which include the size of the lesion, the location of the lesion, the patient's age, and the extent of functional and cosmetic derangement. We, therefore, report the surgical treatment of FD of mandibular condyle without apparent preauricular swelling.


  Case Report Top


We report the case of a 33-year-old trader who was referred from the gynecology clinic on account of inability to open her mouth of approximately 1 month duration. There was history of clicking at the right TMJ and spontaneous dislocation and reduction of the TMJ while eating 3 months prior to the onset of the complaint. There was no preceding history of trauma, but there was history of infertility of approximately 10 years duration. Patient had presented to a peripheral center where a provisional diagnosis of unilateral TMJ dislocation was made and manual reduction was attempted, which failed as symptoms persisted.

On examination, there was facial asymmetry with jaw deviation to the left on mouth opening. The mouth opening was 15 mm with associated anterior cross-bite, extending from the right upper lateral incisors to the left upper first premolar, and scissors bite on the right side. The overjet was reversed (−6 mm). As shown in [Figure 1] an assessment of right unilateral TMJ dislocation was made. CT scan revealed an irregular expansion of hyperdense lesion seen on the right mandibular condyle with areas of calcification and an extension to the posteromedial aspect of the lesion associated with thinning out of the cortex. There was no involvement of the mandibular fossa nor intracranial extension as shown in [Figure 2] suggestive of osteochondroma, FD, central giant lesion, and ameloblastoma of the right mandibular condyle. A right mandibular condylectomy was done through a combined preauricular and right submandibular incision following a successful intubation under general anesthesia. A preauricular incision was made as a vertical limb in a preauricular skin just in front of the pinna and continued downward passing backward below the pinna behind the angle of the mandible and curved forward to the body of the mandible in the second upper cervical skin crease as a reference point to prevent injury to marginal mandibular branch of the facial nerve. The skin flap was raised along the zygomatic arch to expose the insertion of the masseter and parotideomasseteric fascia. Blunt dissection of the masseter and parotideomasseteric fascia was continued posteriorly toward the neck and anteriorly to the sigmoid to expose the capsule; the submandibular skin flap was also raised to expose the plastyma, which was dissected to gain access to the ascending ramus. This was cleared subperiosteally. Both the facial vein and artery were identified and ligated in the course of dissection. The mandible was sectioned below the condylar neck, around the ascending ramus, and 2 cm below the lesion using osteotome and mallet. Mandibulo-maxillary fixation was subsequently placed intraoperatively to stabilize the occlusion. Intraoperative assessment of patient occlusion and facial appearance was satisfactory. A drain was placed into the surgical defect and the flaps raised were, repositioned, and sutured in layers. Drain was removed 3 days postoperatively when it was no longer active. The resected tumor was irregular in shape with multiple extensions/protrusions (head) with a broad base, and the tumor was cartilaginous in certain aspect and bony in other areas. Specimen was sent for histopathological evaluation, which confirmed FD. Initial impressions of osteochondroma and condylar hyperplasia were made based on clinical and radiographic presentation. Mandibulo-maxillary fixation was removed after 3 weeks postoperatively, and the patient was referred to the physiotherapy department to retrain the muscles of mastication to correct the deviation of the jaws and facial palsy. Patient also developed hypertrophic scar, and topical application of silicon gel was prescribed.
Figure 1: (a) Facial asymmetry with the patient in frontal view. (b) Limited mouth opening (15 mm) and deviation to the left. (c) Showing anterior and posterior cross-bite on the left and scissors bite on the right. (d) Posterior cross-bite of the upper left premolar. (e) Anterior cross-bite and scissors bite on the scissor bite on the posterior teeth on the right

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Figure 2: CT scan with the arrow pointing in the direction of the lesion in the right condyle of the mandible. Irregular expansion of hyperdense lesion seen on the right mandibular condyle with areas of calcification and an extension to the posteromedial aspect of the lesion associated with thinning out of the cortex. There is no involvement of the mandibular fossa or intracranial extension

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  Discussion Top


FD, the most common fibro-osseous lesion is an intramedullary neoplasm originally named by Lichenstein in 1938.[1],[11] FD is characterized by the progressive replacement of normal bone by fibrous tissue resulting from failure of bone maturation. Asamao et al.[5] reported fibro-osseous lesions to be the most common benign jaw tumors in northern Nigeria; it is relatively common in Nigeria than other African countries.[5],[11] FD is the most common fibro-osseous lesion,[11] making it the most common benign jaw tumor in northern Nigeria. Ajagbe et al.[12] reported the occurrence of FD to be the highest during the first, second, and third decade of life, and rarely appears in the elderly. However, it has also been established that tumor regression may occur after becoming stationary during the fourth decade of life and at cessation of sexual maturity.[7] As the lesion has been reported to be seen more in children and young adults, its occurrence in the case under review cannot be ruled out considering the patient's age and the unpredictable natural course of FD.[12] Opinions are actually divergent regarding the sex predilection of this lesion. Reis et al.[13] reported FD to have equal predilection for both males and females. In separate studies by Halina et al.[14] and Ajagbe et al.,[12] the lesion was reported to be more frequent in females than in males. Asamao et al.[5] reported a male preponderance for fibro-osseous lesions. FD affects the maxilla more frequently than the mandible, however, FD of the TMJ is a rare occurrence as reported by Jorinde et al.[6] Other rare TMJ lesions are osteochondroma, osteoma, synovial chondromatosis, ganglion, synovial cyst, simple bone cyst, aneurysmal bone cyst, epidermal inclusion cyst, heamangioma, nonossifying fibroma, Langerhans cell histiocytosis, plasma cell myeloma, and sarcoma. The bone-forming or cartilage-forming tumors such as osteoblastoma or condylar hyperplasia are the most common condylar lesions.[15] In a study conducted by Brown et al.,[16] the skull and facial bones were involved in 10–25% cases of monostotic FD and in 50% of cases of polyostotic fibrous dysplasia. Structures commonly affected by FD are the long bones, ribs, craniofacial bones, and the pelvis.

A review of literature demonstrated a consensus regarding the unknown etiology of FD. However, the present understanding is that all types of FD result from a defect in the bone maturation process, occurring during the embryonic stage of development. At a certain time during development, there is mutation in the gene that encodes for a cytoplasmic transducer protein necessary for bone maturation. Consequently, this defective cell produces more cells that are defective in this transducer protein by mitosis, resulting in a population of cells capable of producing dysplastic fibrous bone rather than mature bone.[7]

Meanwhile, three different impressions were made for this case prior to surgery before a histological diagnosis of FD was made; (1) unilateral TMJ dislocation secondary to condylar hyperplasia, (2) fibrous dysplasia, and (3) osteochondroma. Psychological stress was implicated as an aetiological factor for TMJ dislocation in a patient presenting with facial asymmetry, posterior cross-bite, and mandibular deviation on mouth opening in a report by Singhal et al.[17] This was corroborated with the patient under review who was facing with infertility for 10 years. An initial impression of unilateral TMJ dislocation was made in this case after a review of the patient's signs and symptoms as there was no swelling at the right TMJ to suggest any pathological tumor. However, it is important to note that unilateral TMJ dislocation is known to occur mostly in females in the second and third decades of life,[18],[19],[20],[21] as seen in this case. This provisional impression was, however, ruled out after CT scan evaluation of the TMJ. Meanwhile, it is noteworthy that More et al.[15] reported difficulty in diagnosing intraosseous condylar lesion due to the similarity of symptoms with other temporomandibular disorders. Based on the radiographic evaluation of the TMJ using CT scan and clinical features, impressions such as of osteochondroma, central giant cell, fibrous dysplasia, ameloblastoma, and ossifying fibroma of the right mandibular condyle were made. This buttresses the point that CT scan evaluation is an indispensable tool in the assessment of maxillofacial lesions and conditions. Patients with osteochondroma of the mandibular condyle present with facial asymmetry, limited mouth opening, jaw deviation on mouth opening, posterior cross-bite, and scissors bite. Radiographically, osteochondroma is known to be nodular or mushroom-like in shape with a pedunculated stalk or sessile base. It also assumes other shapes depending on the surrounding structures, and appears on all sides of the condyle but mostly the anteromedial side of the condyle.[22],[23] The aforementioned findings could be seen in the case reported. However, histological evaluation of osteochondroma demonstrates proliferating chondrocytes in disorganization with ossification toward the base and an overlying periosteal capsule and medullary cavity merging with that of the underlying bone with a normal bone trabeculae; this was contrary to the histopathological findings in the case under review. Though FD is usually asymptomatic, it commonly presents as a swelling, which was absent in this present case contrary to the findings of Halina et al.[14] who reported a case of unilateral FD of the mandibular condyle in which the patient presented with limited mandibular movement, facial asymmetry, right side deviation with pain, and swelling on the right side of the face. However, in the study reported by Halina et al.,[14] the emotional lifestyle of the patient was assessed, and findings revealed difficulty in the patient raising her disabled son for more than 10 years. In the case under review, patient was emotionally and psychologically tasked as patient was infertile and has not been able to conceive despite being married for more than 10 years. The radiological appearance of FD has been reported to be extremely variable. Three radiographic patterns have been reported;[9] Type I is unilocular or multilocular radiolucency with a rather well-circumscribed border containing a network of fine trabeculae. Type II is similar to type I except there is increased trabeculations, which renders the lesion more radio-opaque and typically more mottled in appearance, and type III is quite opaque with many delicate trabeculae giving a “ground glass” appearance or “Peau d orange” appearance. The case under review presented with irregular expansion of the right mandibular condyle with areas of calcification, which can also be given away for FD. Definitive diagnosis of FD depends on patient's history, blood findings, clinical examination, radiological examination, and biopsy. Considering the history of the patient under review, the onset of symptoms was sudden which is in contrast to the gradual, progressive development of symptoms in FD. However, in the study reported by Halina et al.,[14] the emotional lifestyle of the patient was assessed, and findings revealed difficulty in the patient raising her disabled son for more than 10 years which is similar to our finding in the case under review with infertility for more than 10 years. It has been reported that some patients with FD have elevated serum level of acid phosphatase, though this has not been accepted as a diagnostic marker.[14] However, definitive diagnosis of FD is usually by DNA sequencing of cells from the lesion, histopathological evaluation cannot differentiate FD from other fibro-ooseous lesions. Histological section consists of loose cellular fibrous tissue containing slender trabeculae of woven bone of variable shape, with osteoblast scattered throughout the substance of the trabeculae. However, incisional biopsy was not done for the case under review taking into consideration the location of the tumor (medial side of the condyle) and the related vital structures.

There are numerous factors that determine if the management of FD should be by conservative surgery or radical surgery. Samman et al.[24] reported that the indication for surgery for FD depends upon the degree of deformity and functional disturbance, and that a radical approach is unnecessary considering the fact that the lesion is developmental and benign, and that the lesion merges imperceptibly with the normal bone and margins cannot be demarcated at surgery. Zenn et al.[25] also suggest a radical treatment considering the numerous relapses associated with conservative treatment. In another study by Valentini et al.,[26] the authors reported few cases of recurrence following conservative surgery, necessitating further surgery, and there was no recurrent case observed with complete resection. Ma et al.[27] found 31.8% recurrence rate in patients who received conservative bone contouring. Other conservative treatments being used include the use of occlusal splint and pamidronate administration.[14],[28] Pamidronate infusion (1 mg/kg) for 3 days every 4–6 months was found to provide pain relief and decrease in the size of the swelling in a study conducted by Eldeep et al.[28] Atalar et al.[10] reported that the management of FD is not surgical unless it causes unacceptable or progressive deformity, cranial nerve compression, pain, or development of a malignancy. There is a possibility of sarcomatous transformation in cases of FD treated by multiple surgeries, stressing the fact that radical surgery is the only guaranteed treatment choice. Radical surgical treatment in the form of condylectomy has been suggested for complex condylar tumor cases requiring surgery. Radical surgery in the form of condylectomy was carried out for the case under review because of the complex nature of the case. The posteromedial location of the lesion on the condyle and the related vital structures made it difficult to access the medial aspect of the tumor. In a study by Ashok-kumar et al.[29] the patient was placed in mandibulo-maxillary fixation for 2 weeks after condylectomy to stabilize the occlusion. Physiotherapy was also recommended to retrain the masticatory muscles, as reported in his study. In the case under review, mandibulo-maxillary fixation was provided for 3 weeks followed by a physiotherapy section to retrain the masticatory muscles.


  Conclusion Top


Different types of lesions and conditions affect the TMJ to varying degrees. Accurate diagnosis is important for proper management considering the functional and aesthetic derangement that could result from poor management. CT scan evaluation of the TMJ is indispensable in arriving at an accurate diagnosis of TMJ lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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