| Abstract|| |
Rationale: Ossifying fibromas are mainly found in the jaws and are rare in other parts of the cranium. Orbital involvement is relatively rare. Patient Details: A case of an otherwise healthy 38-year-old overseas patient with gradual onset of a large growth in the right ocular area involving adjacent bone and operated twice over the last eight years is being presented. The lesion is involving the entire medial wall. Treatment: Considering the complex loco-regional anatomy as well as the restriction of overseas residence, preservation of a thin margin of bone was performed. Take-Away Lessons: The need for repeat radiological surveillance was stressed.
Keywords: Exophthalmos, medical tourism, orbital pathology, ossifying fibroma, telecanthus
|How to cite this article:|
Balaji S M, Balaji P. Recurrent ossifying fibroma of the orbit – A case report. Indian J Dent Res 2021;32:411-4
| Background|| |
Ossifying fibroma (OF) is a rare, benign fibro-osseous tumour involving cranial elements. It is characterized by the replacement of normal bone by a fibrous cellular stroma including numerous mineralization or ossification foci. Based on clinical, morphological, and radiological features, it is classified as cemento-OF (COF), juvenile psammomatoid OF (JPOF) and juvenile trabecular OF (JTOF). A condition often noted early in life, it could cause disfigurement and lead to decrease in quality of life. Additionally, depending on the location, they may cause headaches, blindness, hearing loss, cognitive/neurological impairment, ulcerations while some patients experience little to no symptoms beyond the skull protrusion that contributes to the delay in seeking treatment.,,
When occurring in the midfacial region, compression by this tumour may lead to disastrous outcomes. Hence a watch and wait option may be a less desirable option. The goal of treatment for OF would always be to achieve full resection and/or decompression with minimal damage., The aim of the manuscript is to present surgical management of a case of long standing OF in a middle-aged adult who had been managed twice for a large, slow growing craniofacial tumour.
| Case Report|| |
This is a 38 years old, otherwise healthy male from Africa who presented with a growth and swelling of the right forehead and eye since the past 20 years. The swelling was insidious, gradually progressive and had no other associated symptoms including pain or visual disturbances. The skin over the area was normal. Previous biopsy revealed the lesion to be OF. History revealed that 8 years ago, he underwent frontal extradural osteoplastic craniotomy and resection with micro neurosurgical technique. A year later, he underwent mid-facial degloving and decompression of right orbit at 31 years of age. At present, general physical and systemic examination revealed no abnormality.
On clinical examination, a well-defined bony hard swelling was noted below the right eyebrow. The right eyebrow was at a lower level than the left eyebrow. Other notable findings included lowered right canthal plane, exophthalmos of right eyeball and telecanthus. Ophthalmologic examination revealed normal visual acuity in both eyes.
The computed tomography (CT) scan showed a radiodense mass with ground glass appearance involving the frontal bone, medial wall and roof of the orbit and its expansion causing exophthalmos of right eye. Based on clinical history, radiographic assessment, CT and 3D skull model, a diagnosis of OF was reconfirmed and orbital wall decompression was planned for proptosis correction [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d.
|Figure 1: (a) Preoperative view showing a well-defined bony hard swelling below the right eyebrow, lowered right canthal plane, exophthalmos of right eyeball and telecanthus, (b and c) 3DCT scan showing radiodense mass with ground glass appearance involving the frontal bone, medial wall and roof of the orbit and its expansion causing exophthalmos of right eye, (d) 3D stereo-lithographic model showing bony hard swelling|
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| Surgical Procedure|| |
After standard preparation, in sitting position, a lumbar puncture was performed in between L3-L4 area and free flow of cerebrospinal fluid was confirmed and drain placed. Under general anaesthesia, an orotracheal intubation was done. The patient was positioned supine with the end elevated. A temporary tarsorrhaphy was performed to protect the right eye. After scrubbing and draping, a standard bicoronal incision was placed over the previous surgical scar on the vertex. The incision is extended over the scalp layers and a flap was raised. Raney clips and electrocautery wires were used to control bleeding. Dissection was carried to expose the superolateral rim of orbit and nasal bone. Periorbital dissection was done to expose the roof of orbit. Lateral dissection was carried out to visualize the orbital margin. Right frontal craniotomy defect was seen fixed by a mesh, which was removed along with bone. Craniotomy was done using burr holes and craniotome. The frontal bone flap was reflected to expose the protective membrane of the brain (Dura).
During this procedure, the globe was depressed with malleable retractor, which was made easy by draining CSF through the lumbar drain. The right supraorbital rim and roof of orbit were exposed. An intraorbital whitish firm hard tumour was seen attached to the medial and superior wall of orbit extending posteriorly up to the superior orbital fissure. The hard bony mass was drilled and the lesional mass was removed in toto. After removal, only a thin layer of bone was left attached to the roof of the orbit and medial wall. The excised specimen was given for histopathological examination. On checking the patient, his proptosis was corrected. Haemostasis was achieved. Antibiotic irrigation was done. The bone flap was replaced and fixed with mini plates, titanium mesh and screws. Subgaleal drain was placed and closure done in layers with vicryl and skin staplers [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d.
|Figure 2: (a and b) The frontal bone flap was reflected to expose the protective membrane of brain (Dura). Right supraorbital rim and roof of orbit exposed, (c) The frontal bone specimen, (d) Bone flap replaced and fixed with mini plates, titanium mesh and screws|
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Histopathological report showed numerous small spherical ossicles or psammoma bodies that are embedded in a densely cellular and vascularized fibrous stroma and cancellous bony spicules with osteoblastic rimming interspersed with loose fibrovascular tissue and diagnosed as psammomatoid ossifying fibroma [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e, [Figure 3]f.
|Figure 3: Histopathological images of the resected specimen, Hematoxylin, and Eosin stain. (a) Low power view. Image showing the cortical areas being replaced with fibrous tissues; (b) Low power view, Image depicting the calcification areas. Note the different stages of matrix formation and degree of calcification; (c) 10×, Image showing the mature area in the cancellous section of the bone. Note the bone-fibrous tissue interface; (d) 10×, Image showing active osteoblast activity; (e) High power view of the osteocytes; (f) High power view of the fibroblast component|
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Healing was uneventful and there were no residual neurological or ocular defects even after 36 months of follow-up. The patient is under regular follow-up in his country of residence [Figure 4]a and [Figure 4]b.
|Figure 4: (a) Post-operative view showing a near normal cosmetic result, (b) Post-operative 3DCT scan showing the margin of the lesion left back due to locoregional complexity|
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| Discussion|| |
Large disfiguring cranial tumours are rare owing to increased awareness, early tumour detection and treatment seeking behavior. The cases that are being reported generally occur when the patient often does not have access to treatment, especially during the active growth phase of such lesions. In restricted settings, surgery for this condition poses a challenge as it is associated with a high risk of peri-operative hemorrhage, difficulty associated with removing the large mass and need for reconstruction of the critical sized defects. Also, because of the complexity and rarity of large skull deforming tumours, each surgery must be carefully planned and carried out on an individual basis. The condition is benign but locally invasive and could cause destruction. Histopathological evidence and behavior has to be taken into account while planning surgical treatment. In the present case, the patient, being from the African continent, probably did not have early access to treatment and lacked proper medical guidance. As the lesion enlarged, he actively sought treatment. His early experiences in Africa were not congenial. At presentation, the lesion was involving his orbital bones. Adequate care has to be taken while removing the lesion such that the orbital architectural framework does not collapse causing complexity in reconstruction. As a thin margin was spared, reconstruction was relatively easy. Though there was no radiological evidence of recurrence within 36 months, there exists a remote possibility of the same as reported in previous literature.,, Sparing a thin margin of bone, especially for lesions in anatomically complex region, has been reported previously. In that particular tumour debulking case, the bone that was in lesional proximity was preserved.
Due to the complex loco-regional anatomy and rarity of the giant sized lesions, there is no protocol or treatment algorithm in place. Hence, this surgery was a highly customized one. Extensive imaging studies and 3D model was undertaken to help establish the extent of the tumour and guide surgical resection.,, At surgery, the goals were balanced between maximal safe surgical resection and desire to avoid permanent neurological deficit or disability, especially to the orbital apparatus. It was understood that this surgery is the only chance to achieve a cure. It was also understood that a permanent disability, especially of eye would be an unacceptable complication, particularly for individuals returning to countries with limited medical and social support resources. Before start of surgery, it was aimed to simply restoring a “near-normal” cosmetic result, which would allow the patient to re-enter society, radically improving his quality of life.
Though the lesion was removed, there is still need for continuous surveillance.,, The challenge of removing the lesion in toto, possibility of compromise of vision, eventuality of losing the orbital architecture complicates the management. The current approach would yield better quality of life to the patient.
| Conclusion|| |
The management of a large orbital OF is presented. The management of such lesions needs to be viewed holistically without compromising the quality of life or loss of vision. Effective options and solutions are presented.
The contribution and expertise of Dr. C.V. Shankar Ganesh, Neurosurgeon; in surgical management of the case is acknowledged.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bin Abdulqader S, Alluhaybi AA, Alotaibi FS, Almalki S, Ahmad M, Alzhrani G. Spheno-orbital juvenile psammomatoid ossifying fibroma: A case report and literature review. Childs Nerv Syst 2021;37:3251-5.
Nussbaum E, Graupman P, Lacey M, Harrison A, Lowary J, Touchette J, et al
. Transport of patients with giant disfiguring cranial tumors from Africa to the US for collaborative multidisciplinary treatment. Surg Neurol Int 2021;12:175.
Ta NH, Addison A, Beigi B, Philpott C. Unilateral visual loss resulting from orbital encroachment of an ethmoidal juvenile trabecular ossifying fibroma. Ann R Coll Surg Engl 2019;101:e111-4.
Wilson J, Kumar R, Goddard A, Liddington M, Carter L, Russell J, et al
. Recurrent ossifying fibroma of the sphenoid bone 26 years after primary surgical excision; a case report and review of the literature. Acta Neurochir 2013;155:549-51.
Yang B, Wang Y, Wang X, Wang Z. Imaging study of ossifying fibroma with associated aneurysmal bone cyst in the paranasal sinus. Eur J Radiol 2012;81:3450-5.
Yazici B, Yazici Z, Yalçinkaya U. Aneurysmal bone cyst secondary to ossifying fibroma in the orbit. Ophthalmic Plast Reconstr Surg 2011;27:e84-5.
Kawaguchi M, Kato H, Miyazaki T, Kato K, Hatakeyama D, Mizuta K, et al
. CT and MR imaging characteristics of histological subtypes of head and neck ossifying fibroma. Dentomaxillofac Radiol 2018;47:20180085.
López CB, Zabala AB, Bareño EÚ. Cranial juvenile psammomatoid ossifying fibroma: Case report. J Neurosurg Pediatr 2016;17:318-23.
Kaderbhai J, Lo W, Rodrigues D, White N, Evans M, Nishikawa H, et al
. Craniofacial approaches to pediatric orbital tumors. J Craniofac Surg 2019;30:1198-200.
Dr. S M Balaji
Director and Consultant Oral and Maxillofacial Surgeon, Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai - 600 018, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]