Sep 9, 2016 - Abstract. Benign fibro-osseous lesions are common in the maxillofacial skeleton. However, ... progressive hearing loss on the side of the swelling and sought medical ... (Figures 1,2). Due to the absence of signs of nerve entrapment, and the high .... Craniofacial fibrous dysplasia and ossifying fibroma. Oral.
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Annals of Otolaryngology and Rhinology
Case Report
*Corresponding author
Fibro-Osseous Dysplasia of the Temporal Bone: Review and Case Presentations
A 10 years old male presented a painless swelling of the left mastoid region of two years duration. At that time there was no hearing loss, ear discharge, pain, facial nerve affection or
ISSN: 2379-948X
Keywords
Benign fibro-osseous lesions are common in the maxillofacial skeleton. However, their incidence in the temporal bone is rare. They are usually silent but may mimic other temporal bone pathologies. The radiographic features suggest the diagnosis. In some cases, histological confirmation may be needed. Fibro-osseous lesions of the temporal bone should be considered in the differential diagnosis of lesions with atypical otological presentations. In most cases no intervention is planned except when there is a functional deficit. This paper reviews this condition with a presentation of three cases.
Case 1
Published: 09 September 2016
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Abstract
CASE PRESENTATION
Accepted: 07 September 2016
© 2016 Mostafa et al.
Department of Otorhinolaryngology-Head and Neck Surgery, Ain-Shams Faculty of Medicine, Cairo-Egypt
Benign fibro-osseous lesions (BFOLs) of the maxillofacial bones comprise a diverse group of pathologic conditions ranging from developmental lesions to neoplasms [1]. Regardless of subtype, all BFOLs demonstrate replacement of normal bone by fibrous connective tissue with an admixture of mineralized product, including osteoid, mature bone, and/or cementumlike calcifications. Clinically they may be asymptomatic or may cause significant cosmetic and functional disturbances [1-3]. Radiographically, BFOLs may manifest as solitary, multifocal, or multi quadrant disease. They may be defined ill or well; they may have a radiolucent, mixed radiolucent-radio paque, predominantly radio paque, or ground glass appearance; they may be monostotic or polyostotic lesions rarely cross the midline [4]. Fibrous dysplasia usually arises within the first or second decades of life, manifesting clinically as a slow-growing, painless expansion of the involved bone [5]. Facial asymmetry may be apparent. In rare cases, the expansion may be more rapid or begin to accelerate after a period of slow growth, resulting in marked facial deformity and potential nerve entrapment [6]. Active growth typically slows or ceases around the time of puberty or after skeletal maturation; however, sporadic periods of re growth may occur in adulthood [7]. Temporal bone dysplasia is rare [811] and usually asymptomatic. This paper presented three cases
Submitted: 30 July 2016
Copyright
Badr Eldin Mostafa* and Lobna El Fiky
INTRODUCTION
Badr Eldin Mostafa, Department of OtorhinolaryngologyHead and Neck Surgery, Ain-Shams Faculty of Medicine, 75 El Nozha Street, 11351,Cairo-Egypt, Email:
• Fibro-osseous dysplasia • Mastoid swelling • Hearing loss
vertigo. Five months prior to presentation, the parents noticed progressive hearing loss on the side of the swelling and sought medical advice. The child was healthy without obvious morbidity. There was a painless smooth diffuse swelling of the left mastoid region blending with the rest of the skull. The auricle was slightly pushed outwards. The external auditory canal is obliterated by firm swellings of the anterior and posterior walls with intact skin. There were no other swellings all over the body, no sites of abnormal pigmentation. There was no evidence of any endocrinological abnormalities. Audiological evaluation revealed left-sided conductive hearing loss of 45 dB. The right ear has a normal audiogram.
Diffuse affection of all parts of the temporal bone by a homogenous appearance of ground glass appearance. The middle ear space is preserved but restricted. Ossicles appear intact but entrapped by the restricted space. The lateral two-thirds of the external auditory meatus were obliterated by swellings of all the walls. The drum is obscured by the swellings. The otic capsule is spared but looks as a hyperdense island within the ground glass bone. The internal auditory meatus looks normal with a comparable diameter as the opposite side although in some cuts it looks narrower at its porus. There is apparently no neural compression with a normally appearing fundus and facial canal (Figures 1,2).
Due to the absence of signs of nerve entrapment, and the high risk on the facial nerve in the event of exploration, the patient and his parents were counseled to wait and observe. They were also instructed to report immediately whenever there were signs of hearing deterioration, facial twitches of facial weakness. A repeat scan is scheduled 6 months after the initial presentation.
Cite this article: Mostafa BE, El Fiky L (2016) Fibro-Osseous Dysplasia of the Temporal Bone: Review and Case Presentations. Ann Otolaryngol Rhinol 3(10): 1134.
Mostafa et al. (2016) Email:
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Case 2 This was an 18 years old boy presenting with an attack of acute suppurative otitis media with fever, otalgia and otorrhea. He was treated medically at another facility by intravenous ceftriaxone, local ear drops and analgesics. His symptoms abated but there was still some tenderness over the mastoid and hearing loss. A CT scan was ordered and showed cloudiness of the middle ear and mastoid cells. There was also a suspicious cavity in the mastoid (Figure 3,4). A congenital cholesteatoma was suspected and an MRI was also performed. It showed a rounded mass with a hyperintense signal on T1 and a hypo intense signal on T2 (Figure 5,6). Consequently, an exploratory mastoidectomy was scheduled. It showed a smooth cavity full of fibrous tissue and bony spicules. The mastoid was otherwise healthy and the middle ear full of seromucous fluid. Biopsy revealed a fibroosseous lesion.
Figure 4 Axial CT of Case 2.
Figure 5 MRI T2W of case 2.
Figure 1 Axial CT of case 1.
Figure 6 MRI of case 2. Coronal and axial.
Case 3
Figure 2 Coronal CT of case 1.
This was a 27 years old male patient presenting with a painless swelling of the mastoid region with diffuse headache. Imaging revealed a large heterogeneous mass involving the temporal bone and squama. The external auditory canal was stenosed but the middle ear, ossicles and otic capsule were intact. There were no other or otological or neurological complaints. As there were no further complaints and wait and watch policy was adopted.
DISCUSSION
Figure 3 Axial CT of case 2. Ann Otolaryngol Rhinol 3(10): 1134 (2016)
Fibrous dysplasia is a rare benign intra medullary fibroosseous lesion, which may present in either monostotic or polyostotic forms [1,12-14].It is a genetic non inherited condition caused by missense mutation in the GNAS1 gene on chromosome 20 [15,16]. It is characterized by abnormal proliferation of fibrous tissue interspersed with normal or immature bone. It occurs in equal proportions in males and females, most often during the first two decades of life. In 3-5% of cases it may be
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Mostafa et al. (2016) Email:
Central associated with endocrine dysfunction, abnormal pigmentation, and precocious puberty in girls. The initial clinical sign is usually a painless enlargement of the affected bone. Other signs and symptoms include bone pain, pathologic fractures, and bone deformities [17]. The common sites of involvement, in decreasing order of frequency, are the femur, tibia, skull and facial bones, pelvis, ribs, upper extremities, lumbar spine, clavicle, and cervical spine [12,17,18]. The dysplasia may be unilateral or less commonly, bilateral. In patients with polyostotic disease, the most commonly involved bones are the craniofacial bones, ribs, and metaphysis or diaphysis of the proximal femur or tibia, and the lesions are often found on one side of the body [3,4]. The abnormal skin pigmentation also tends to be present on the same side. The craniofacial bones are affected in about 10% of cases of monostotic fibrous dysplasia and in 50%–100% of cases of polyostotic forms [19,20]. When only the cranial and facial bones are affected, the term craniofacial fibrous dysplasia is used.
The diagnosis of fibrous dysplasia can be made by radiologic imaging and confirmed by bone biopsies. CT is the study of choice for diagnosis and follow-up of fibrous dysplasia because of its superior bony detail and accurate assessment of the extent of the lesion. Furthermore, CT can often assist with differentiating fibrous dysplasia from other osteodystrophies of the skull base, including otosclerosis, osteogenesis imperfecta, Paget’s disease, osteopetrosis or aneurysmal [19]. The radiological features of temporal bone involvement include an increase in size and bone density associated with areas of sclerosis and radiolucency. They may be ill or well defined; they may have a radiolucent, mixed radiolucent-radiopaque (21 %), predominantly radiopaque or sclerotic (23%), ground glass or pagetoid (56%) appearance. Lesions rarely cross the midline [4,20]. The characteristic signs are the expansion of bone, thinned cortical layer and displacement of surrounding structures. Generally, the otic capsule is preserved and the labyrinth can be seen as a solitary isle within the lesion [2,19-21]. MRI establishes the lesion’s shape and content and size. The mass is usually hypointense on T1 and hyperintense on T2. Signal intensity on T1- and T2-weighted images and the degree of contrast enhancement on T1-weighted images depend on the amount and degree of fibrous tissue, bone trabeculae, cellularity, collagen, and cystic and hemorrhagic changes [22,23]. Bone scintigraphy can determine the extent and activity of the bone lesion and influence decision-making [24].
Fibrous dysplasia of the temporal bone presents frequently as a progressive painless swelling of the squama or mastoid. Involvement of the middle ear, ossicles, otic capsule or facial nerve has been reported [25-27]. Medical treatment with biphosphonates is limited and indicated in cases with bone pains [18]. Surgery is only indicated in cases with marked cosmetic or functional affection [9,11,28]. Careful assessment of each case and weighing of the risk of surgery is mandatory in skull base lesions. Definitive indications include external canal stenosis with cholesteatoma formation, facial nerve entrapment or internal auditory canal stenosis causing sensorineural loss. In cases with slow progression or minimal symptoms, close radiological follow-up is recommended especially during periods of suspected growth such as during puberty. Ann Otolaryngol Rhinol 3(10): 1134 (2016)
CONCLUSION Fibroosseous lesions of the temporal bone are uncommon. They usually present with minor symptoms and signs and in most cases do not warrant any management. However follow up is mandatory and surgical treatment may be necessary if there are any functional or cosmetic effects.
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Cite this article Mostafa BE, El Fiky L (2016) Fibro-Osseous Dysplasia of the Temporal Bone: Review and Case Presentations. Ann Otolaryngol Rhinol 3(10): 1134.
Ann Otolaryngol Rhinol 3(10): 1134 (2016)
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