Cases reported "Bone Diseases"

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1/19. Congenital cholesteatoma of the mastoid region.

    Congenital cholesteatoma may arise in the petrous apex, mastoid, middle ear, or external auditory canal. The least common site being the mastoid process. We present one case of primary mastoid cholesteatoma confirmed by clinical examination, surgical findings and radiological evaluation.
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2/19. Epidermoids involving the temporal bone: clinical, radiological and pathological aspects.

    Epidermoids or congenital cholesteatomas arise from aberrant epithelial remnants and are, therefore, considerd blastomatous malformations. Their predilective sites are the intracranial cavity, the diploe of the skull and the spinal canal. In the base of the skull the temporal bone is the most frequent site. Epidermoids account for about 0.2-1.5 percent of all intracranial tumors. The majority originate in the cerebello-pontine angle where they account for 6-7 percent of all tumors. Their age incidence reveals a great scatter from birth to 80 years. The majority are recognized during the third and fourth decades with the onset of clinical symptoms occurring much earlier. They affect males more frequently than females. Their delicate capsule with a whitish mother-of-pearl sheen lends them a typical appearance. Epidermoids are generally slow growing lesions which may remain asymptomatic for years. The irritative effect of their content, however, can produce symptoms of dysfunction and intense inflammation. Malignant changes occur infrequently. Diploic epidermoids are easily recognized, whereas, intradural epidermoids are more difficult to identify. Epidermoids may arise in the vicinity, on the outer aspect or within the temporal bone. Epidermoids originating in any of these locations have certain characteristic features which may arouse suspicion of their presence. Examples of an epidermoid with origin in the typical locations within the temporal bone and cerebello-pontine angle are discussed to portray their individual characteristics.
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keywords = cholesteatoma
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3/19. endoscopy-assisted surgery of the petrous apex.

    OBJECTIVE: Five large petrous apex lesions, 4 cholesterol granulomas and 1 cholesteatoma, were managed with a combination of microsurgical and endoscopic techniques. STUDY DESIGN AND SETTING: Retrospective review of clinical experience in an academic medical center. Traditional microsurgical approaches, infracochlear and/or retrofacial, were used in all cases, followed by endoscopic examination and debridement of the interior of the cysts. In all cases residual disease or fibrous septae within the cysts were found endoscopically that were not evident through use of the operating microscope. RESULTS: In all cases, the lesions were successfully managed without operative complications. CONCLUSIONS: The addition of endoscopic visualization to traditional microsurgical approaches allowed exposure of recesses within the lesions, removal of debris and inspissated secretions, and identification and removal of septae and fibrous bands within the petrous apex lesions that would not have been identified with the operating microscope alone. No surgical or postsurgical complications occurred in any of the cases, including facial weakness, hearing loss, or cerebrospinal fluid (CSF) leak.
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keywords = cholesteatoma
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4/19. Epidermoid cysts of the cranial bones.

    Ectodermally derived tissue in ectopic sites is seen in the head and neck. Extracranial dermoid and epidermoid tumors are relatively rare. While most otolaryngologists are familiar with the cholesteatoma of the otic area, not very many are exposed to this lesion in other sites. Those of the cranial bones are even rarer still. These tumors may expand (1) laterally in the cranial bones, (2) externally to present as masses in the scalp or facial region, or (3) internally to involve the intracranial contents. A patient with a frontal bone epidermoid, whose tumor had expanded in all three directions to erode both inner and outer cortex of cranium and laterally to rupture into the frontal sinus, is discussed. The literature is reviewed and the management of these rare masses is discussed.
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5/19. Primary mucocele of the petrous apex: MR appearance.

    Mucoceles of the petrous apex are rare. Their MR appearance varies depending on the degree of hydration or inspissation of the contents. Concise preoperative diagnosis is helpful, since mucoceles are better drained to the mastoids via an infralabyrinthine approach rather than the more risky middle cranial fossa approach used for cholesteatomas.
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keywords = cholesteatoma
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6/19. Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion.

    Thalassaemia intermedia should be considered in any chronically anaemic patient presenting from the middle east with hearing impairment. We report here three Saudi siblings with thalassaemia intermedia and features of severe bone marrow expansion, particularly invading the temporal bone. They were seen first for their otological problems before they had access to proper haematological evaluation. One member was admitted for surgical exploration of a cholesteatoma, which was then found to be marrow expansion of the temporal bone. Screening of the family revealed two more anaemic siblings with thalassaemia intermedia. Audiological examination of all the family members showed that only the two affected members had a high frequency sensori-neural hearing loss. bone marrow expansion into the temporal bone is a rare feature of thalassaemia intermedia. cholesteatoma-like lesion has not been previously described. It has to be considered in all cases of symptomatic thalassaemia intermedia manifesting with cavitation and lytic lesions in the mastoid system. The likelihood that sensorineural hearing loss may complicate the thalassaemias is raised and the possible mechanism for such involvement discussed. The proper management for different otological manifestations of the thalassaemias is suggested. These cases would suggest a more extensive involvement of the temporal bone in the thalassaemias than has been previously recognized. Further large scale studies are required to illuminate the subject.
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keywords = cholesteatoma
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7/19. Mode of growth of acquired cholesteatoma.

    A histopathological study of acquired cholesteatoma in four temporal bones from two adults and one child is presented. The findings suggest that the cholesteatoma originated from the retraction pockets of the tympanic membrane and there was active growth of the squamous epithelium of the retraction pockets, which may be enhanced in the presence of otitis media.
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keywords = cholesteatoma
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8/19. Petrous apex cholesteatoma.

    In this chapter we have reviewed the etiology, diagnosis, and surgical treatment of petrous apex cholesteatoma. The etiology is usually congenital for those tumors arising within the apex, but acquired for lesions secondarily invading the apex. diagnosis is primarily radiographic. CT remains an excellent modality for determining the extent of disease, whereas recent advances with MRI help in differential diagnosis. In our experience the middle fossa approach and the translabyrinthine-transcochlear approach allow the surgeon the best opportunity to effectively treat this rare and challenging tumor.
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keywords = cholesteatoma
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9/19. Management of petrous apex lesions.

    Advancements in imaging pose new diagnostic and therapeutic dilemmas as smaller lesions in the relatively inaccessible regions of the petrous apex and clivus are identified. Differentiation of true pathology from artifact and anatomic variants is critical in management of these lesions. We describe our experience with five patients diagnosed with petrous apex lesions: three, cholesteatomas; one, cholesterol granuloma; and one, false-positive. Soft-tissue obliteration of the temporal bone defect with postoperative follow-up using high-resolution computed tomographic scanning and magnetic resonance imaging is proposed as an alternative to exteriorization.
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keywords = cholesteatoma
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10/19. Bilateral cholesterol granuloma of the skull base: case report and review of the literature.

    A unique case of bilateral cholesterol granuloma of the skull base and its treatment is presented. cholesteatoma, a pathological entity often confused with cholesterol granuloma, is differentiated from cholesterol granuloma. cholesterol granuloma is not rare. This tumor seems to derive from an inflammatory process at the skull base that results in bony erosion surrounding a cyst wall of inflammatory tissue. Neurological abnormalities reflect the location of the tumor in relation to the brain stem. Radiographically, the cyst wall enhances with the administration of i.v. contrast agent, and the center of the lesion is isodense with brain on computed tomography, unlike cholesteatoma. magnetic resonance imaging characteristics are currently being defined. At operation, cholesterol granuloma consists primarily of a viscous fluid within a capsule of inflammatory tissue. Treatment requires establishing a pathway for drainage of the granuloma. The advantages of transsphenoidal, transclival drainage of such lesions are outlined.
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keywords = cholesteatoma
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