|Year : 2013 | Volume
| Issue : 1 | Page : 20-22
Massive ossifying fibroma of the mandible in a child
Minu Bajpai1, P Goel1, O Bhutia2, Anand Gupta2, A Seth3, AK Gupta3, DK Pawar4
1 Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Dental Surgery, All India Institute of Medical Sciences, New Delhi, India
3 Department of Radio-diagnosis, All India Institute of Medical Sciences, New Delhi, India
4 Department of Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||7-Feb-2013|
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
| Abstract|| |
An interesting case of large ossifying fibroma of the mandible in a child with a sickle-cell trait is reported.
Keywords: Mandible, ossifying fibroma, osteofibroma, sickle-cell trait
|How to cite this article:|
Bajpai M, Goel P, Bhutia O, Gupta A, Seth A, Gupta A K, Pawar D K. Massive ossifying fibroma of the mandible in a child. J Indian Assoc Pediatr Surg 2013;18:20-2
|How to cite this URL:|
Bajpai M, Goel P, Bhutia O, Gupta A, Seth A, Gupta A K, Pawar D K. Massive ossifying fibroma of the mandible in a child. J Indian Assoc Pediatr Surg [serial online] 2013 [cited 2018 May 28];18:20-2. Available from: http://www.jiaps.com/text.asp?2013/18/1/20/107011
| Introduction|| |
Ossifying fibroma is a benign, locally aggressive fibro-osseous neoplasm of the jaw in humans and animals  that most frequently affects the mandible, causing distortion of the normal contour, displacement and loss of teeth and difficulty in mastication. The synonyms are osteofibrous dysplasia, osteo-fibroma and fibro-osteoma. Cases of multiple ossifying fibromas are usually sporadic; there are rare reports of multiple synchronous lesions  and familial tendency  as well.
| Case Report|| |
A 10-year-old boy with a known sickle-cell trait, had a four-years history of a painless, slowly enlarging tumor of the lower jaw [Figure 1] was referred from the department of dental surgery where incision biopsy and digital subtraction angiography (DSA) was done. The mass was non-tender, non-fluctuant, hard and covered with intact and uninvolved mucosa inside the oral cavity and skin externally. There was no paresthesia or hypoesthesia of lower lip or face. Severe microcytic and hypochromic anemia with hemoglobin of 4.9 g/dL with anisopoikilocytosis was reported. Pencil cells could be demonstrated in the peripheral smear. Hb HPLC was suggestive of a sickle-cell trait (Hb A 0 =62.6%, Hb F=0.7%, Hb S=27.1%, Hb A 2 =3.2%).
|Figure 1: Clinical photograph of a huge ossifying fibroma of the mandible: Massive size, involvement of the body of the mandible as a whole with expansion of the outer cortex only|
Click here to view
On computed tomography (CT) scan, it was a large, very well defined, single, expansile lesion, 17×9.5×12 cm, involving the whole of the body and the antero-inferior hemi-ramus on the right side. The matrix was calcified, and the teeth were displaced to the periphery of the lesion. The inner cortex of the mandible was thin but intact. Bilateral temporo-mandibular joints and the maxilla were spared. There was no cervical adenopathy. The CT angiography showed that the tumor was being fed by bilateral facial and lingual arteries. The incision biopsy was consistent with an ossifying fibroma. It revealed the collagenous nature of the stroma with contained spindle fibroblastic cells. The stroma was highly vascular. The partially interconnecting trabeculae of woven bone and the presence of osteoblasts on their surfaces were distinguishing.
The patient was intubated by the nasal route and positioned supine with a roll under the upper back to extend the neck slightly. The incision given was completely intra oral extending from the right retromolar region to the left retromolar region at mucogingival junction. The lower lip was gradually dissected off the tumor surface circumferentially till we reached all around the mass. Both the rami were divided proximal to the tumor margins with a gigley saw, and the tumor removed by gradually dissecting it off the remaining soft-tissue attachments. The inner cortex of the mandible was thinned out but free of the tumor mass. A U shaped piece of bone was removed from the inner cortex of resected specimen as free bone graft and used to reconstruct the mandible. Reconstruction of the mandible was done using 2.0 mm high profile titanium plate (Synthes; ) and free bone graft from the uninvolved mandibular bone. Tongue musculature (hyoglossus and genioglossus) and mylohyoid muscles were tied around the reconstructed mandible using 2-0 proline suture to provide normal function of mandible and associated structures and importantly preventing the fall back of the tongue.
The patient was kept intubated for 2 days to prevent asphyxia due to fall back of tongue and pharyngeal edema. The drain was removed on the fourth post-operative day. The lower jaw was supported by dynaplast strapping. The patient was started on physiotherapy. Drooling of saliva was present in immediate post-op due to boggy lower lip but gradually the oral seal was achieved in 6 weeks.
At 6-week follow-up after surgery, the patient could elevate and depress his lower jaw over amplitude of 3.5 cm. Normal tongue movements were achieved together with adequate competency of lips [Figure 2]. The soft tissues started contracting to give a more normal appearance. The plain radiograph showed no signs of resorption of the bone graft. Reconstruction is planned in two stages. Stage one (temporary) with bone plates and free bone graft from uninvolved bone as a patient had sickle-cell anemia and another surgical site for permanent reconstruction may lead to increased anesthesia time and more blood loss. Stage two (permanent) is planned at the age of 18-20 years when his growth seizes, and better esthetics should be planned to correct the residual deformity. Permanent reconstruction will be done using free fibula transfer (microvascular surgery) and dental implants for final oral rehabilitation.
|Figure 2: Post-operative results at 6 weeks after surgery: The patient could elevate and depress his lower jaw over amplitude of 3.5 cm. Normal tongue movements were achieved together with adequate competency of lips|
Click here to view
| Discussion|| |
Ossifying fibroma is classically a slow growing, expansile, neoplastic lesion that replaces the normal bone as it enlarges. The precise pathogenesis of the lesion is still unknown. Wenig  et al. suggested the role of trauma induced stimulation. The origin of this neoplasm lies in the multipotent, undifferentiated, mesenchymal cells of the periodontal ligament tissues capable of forming both bone and cementum. Based on the microscopic similarity with fibrous dysplasia, some investigators consider it to be a localized dysplastic process in which bone metabolism has been altered. The neoplastic etiology is supported by its persistent, locally aggressive growth characteristics and the occasional reports of local recurrences.
Most lesions are asymptomatic at the time of initial presentation. The most common site of origin is the teeth bearing portion of the jaws; the lower jaw being more commonly involved. Most affected patients are in the third or the fourth decade of life. The females are more commonly affected than the males (5:1). The well-defined border between the tumor and the surrounding uninvolved bone tissue differentiates it from fibrous dysplasia. On radiography, the early lesions are radiolucent. The teeth are gradually displaced to the periphery of the mass or rarely; the roots may be resorbed. There is no evidence for the potential for malignant transformation. Complete surgical excision followed by reconstruction is the standard management protocol. Recurrence if any should be managed conservatively until it becomes too large or causes bone destruction.
The following peculiarities make this case unusual (a) such large lesions are not frequently encountered. (b) The coexistence of a sickle-cell trait in a patient of ossifying fibroma has, probably never been reported, although certain chromosomal translocations have been described. (c) The basic principle behind this surgery was the successful excision of the whole tumor mass with whole of the body of the mandible followed by the reconstruction of the mandible with the lingual cortex of the bone. The realignment of the lingual muscles and the muscles of mastication around the reconstructed mandible could pave the pathway for successful rehabilitation of the patient with his lost capabilities. The ultimate goal behind treating this condition is excision of the mass and restoration of the normal structure, function and cosmesis. (d) The intra oral approach used carries with it the advantages of avoiding any external scar, preservation of extra oral tissues for reconstruction, muscular reconstruction and excision of the mucosa if involved. However, it leads to contamination of the surgical wound by the oral flora, pooling of the ooze and dead space infection due to lack of dependent drainage, increases the risk of iatrogenic damage to the various branches of the internal maxillary artery traversing this area, and finally, the immediate bone graft reconstruction by the oral route caries a high risk of infection and loss of graft occurs in as high as 30% of the cases. (e) While going in for surgery we had several options in mind as a source for bone graft, including the ribs or the iliac crest. After complete dental rehabilitation the patient should be in a position to lead a good social life with minimum restrictions. (f) The tedious physiotherapy schedules coupled with the lack of cooperation peculiar to the patients in the pediatric age group, and the long process of dental reconstruction requires great patience, commitment and dedication on part of the parents. Further permanent mandibular reconstruction is planned to give better esthetics and function after the final growth spurt.
August et al.  reported 70 patients who were treated by resection and immediate reconstruction. 22 grafts (18.6%) failed, and the most common cause of early graft failure was infection in 13 patients (59.1%). Lawson et al.  reviewed 54 patients who had undergone mandibular resection for tumors. They found that in 24 patients treated by resection with immediate reconstruction, 11 grafts failed due to infection (46%). In contrast, in the 30 patients treated by resection and delayed reconstruction, the infection rate was only 10% (3 of 30). In view of the above studies, two staged protocol was devised in this patient with the advantage of better observation for recurrence, less surgical time, decreased blood loss, and better esthetics and functional results after final growth spurt.
| References|| |
|1.||Morse CC, Saik JE, Richardson DW, Fetter AW. Equine juvenile mandibular ossifying fibroma. Vet Pathol 1988;25:415-21. |
|2.||Yih WY, Pederson GT, Bartley MH Jr. Multiple familial ossifying fibromas: Relationship to other osseous lesions of the jaws. Oral Surg Oral Med Oral Pathol 1989;68:754-8. |
|3.||Canger EM, Celenk P, Kayipmaz S, Alkant A, Gunhan O. Familial ossifying fibromas: Report of two cases. J Oral Sci 2004;46:61-4. |
|4.||August M, Tompach P, Chang Y, Kaban L. Factors influencing the long-term outcome of mandibular reconstruction. J Oral Maxillofac Surg 2000;58:731-7; discussion 738. |
|5.||Lawson W, Loscalzo LJ, Baek SM, Biller HF, Krespi YP. Experience with immediate and delayed mandibular reconstruction. Laryngoscope 1982;92:5-10. |
[Figure 1], [Figure 2]
|This article has been cited by|
||Psammomatoid juvenile ossifying fibroma of the maxilla: Radical surgery with maxillary resection in a 7-year-old girl
| ||Halama, D., Hierl, T., Wickenhauser, C., Pausch, N.C. |
| ||Klinische Padiatrie. 2013; 225(7): 418-419 |