Cases reported "Orbital Neoplasms"

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1/21. Transnasal endoscopic removal of an orbital cavernoma.

    The approach to posterior and medial orbital tumors is still a challenge, since poor functional results are frequent. We report a case of cavernoma successfully removed by a modified transnasal endoscopic procedure. The patient, a 56-year-old woman, complained of a decrease in vision of the left eye. magnetic resonance imaging evidenced a lesion in the posterior part of the orbital cavity, inferior to the optic nerve, extending to the sphenoidal cleft. The lesion was isodense on T1-weighted images and showed contrast enhancement. Because of the medial location of the tumor, the patient was referred to the otolaryngology department by the neurosurgeons, and a transnasal endoscopic approach was chosen. A large exposure of the operative field was obtained, and a cavernoma was removed. Rapid relief of the symptoms was obtained. In view of this good result, we advocate the transnasal endoscopic approach in cases of inferomedial and posterior intraconal lesions as an alternative and addition to the standard techniques of orbital surgery.
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2/21. hypnosis instead of general anaesthesia in paediatric radiotherapy: report of three cases.

    PURPOSE: This report proposes hypnosis as a valid alternative to general anaesthesia for immobilisation and set-up in certain cases in paediatric radiotherapy. methods: We report three cases of children who underwent radiotherapy in 1994 and were treated using hypnosis for set-up during irradiation. The first and the second were two cases of macroscopic resection of cerebellar medulloblastoma in which craniospinal irradiation was necessary, while the third patient suffered of an endorbitary relapse of retinoblastoma previously treated with bilateral enucleation, radiotherapy and chemotherapy; in this last situation the child needed radiation as palliative therapy. hypnosis was used during treatment to obtain the indispensable immobility. Hypnotic conditioning was obtained by our expert psychotherapist while the induction during every single treatment was made by the clinician, whose voice was presented to the children during the conditioning. RESULTS: Every single fraction of the radiation therapy was delivered in hypnosis and without the need for narcosis. CONCLUSIONS: hypnosis may be useful in particular situations to prepare paediatric cancer patients during irradiation, when lack of child collaboration might necessitate the use of general anaesthesia and when anaesthesia itself is not possible.
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3/21. Multiple recurrences in malignant peripheral nerve sheath tumor of the orbit: a case report and a review of the literature.

    PURPOSE: To report the onset of malignant peripheral nerve sheath tumor of the orbit 8 years after irradiation in a patient with neurofibromatosis type-1. methods: Case report of a young man with neurofibromatosis type-1 who received irradiation for presumed bilateral optic nerve and chiasmal gliomas and in whom a malignant peripheral nerve sheath tumor later developed. Exenteration with extirpation of the entire contents of the orbit was performed 6 times. RESULTS: Complete recurrence of the tumor occurred after each surgical procedure until the patient died of malignancy. CONCLUSIONS: Our case underscores the risk of irradiation, especially in children with neurofibromatosis type-1, and emphasizes that radiotherapy should never be given as an empirical therapy. The authors believe that irradiation and neurofibromatosis type-1 may, in combination, pose a significant risk for the development of malignancies. Clear-cut indications and a precise tissue diagnosis are desirable before the initiation of radiotherapy, particularly in the pediatric population. We recommend that if irradiation is necessary in persons with neurofibromatosis type-1, regular follow-up is imperative. In view of the hostile nature of malignant peripheral nerve sheath tumor, early aggressive treatment appears to be the only viable alternative at present.
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4/21. Surgical sectors of the orbit: using the lower fornix approach for large, medial intraconal tumors.

    PURPOSE: The orbit can be viewed as comprising four surgical sectors, with several alternative incisions and dissection paths to each. Large tumors in the medial intraconal sector pose particular problems of access, exposure, and removal without functional compromise. We report the use of the lower fornix approach for excision of large tumors in this high-risk sector. methods: The surgical technique is described. Its application to biopsy or subtotal resection of infiltrative lesions in the medial intraconal sector is noted. Its use for complete excision of solid, circumscribed lesions in this sector is presented in a retrospective, consecutive, noncomparative interventional case series of 5 large, medial intraconal cavernous hemangiomas. RESULTS: The lower fornix approach provided adequate exposure for the goal of surgery in each case. Among the 5 patients with large, medial intraconal lesions, 4 had postoperative improvement in vision and 1 maintained his preoperative central acuity of 20/20. No patient had persistent symptomatic diplopia after surgery. Ease or difficulty of tumor dissection was related to the tenacity of apical fibrous attachments, a factor that appeared to be independent of the surgical approach. CONCLUSIONS: The lower fornix approach to the orbit, an established technique for other indications, can be applied to the removal of large, medial intraconal tumors whose inferolateral poles protrude in the inferior surgical sector.
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5/21. Tegaderm transparent dressing (3M) for the treatment of chronic exposure keratopathy.

    We report an effective alternative treatment of profound chronic exposure keratopathy in a proptotic eye due to intraorbital extension of basal cell carcinoma. The corneal surface gradually reepithelialized in a 6-week period after the first application of Tegaderm transparent dressing (3M, St. Paul, MN, U.S.A.) with instillation of antibiotic ointment on the ocular surface. The patient has been comfortable with an intact epithelial surface, a vascularized cornea, and nonirritated surrounding skin after changing the Tegaderm dressing daily for 15 months. There was no evidence of recurrent corneal ulceration or infiltrations. We conclude that Tegaderm represents a useful treatment of exposure keratopathy due to chronic proptosis.
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6/21. Endonasal endoscopic resection of ethmoido-orbital osteoma compressing the optic nerve.

    Osteomas are the most common benign tumors of the paranasal sinuses. Open procedures for removal of ethmoid osteomas have been the method of choice, but debate over optimal treatment continues. We report resection of a large ethmoido-orbital osteoma via endonasal endoscopic approach with minimal morbidity. A 42-year-old man presented with a 1-year history of slowly progressive proptosis and lateral gaze diplopia. Imaging studies of orbits and sinuses revealed a large bony mass in left ethmoid sinus extending into the left orbit. The mass had compressed and slightly diverted the optic nerve. The patient had also bilateral extensive polyposis for which bilateral ethmoidectomy and sphenoidotomy were performed. Using a curved blunt elevator, the osteoma was gently and meticulously detached from adjacent structures. Finally, frontal recesses were cleaned form the polyps. The postoperative period was uneventful. After 18 months, he is still free of symptoms. The previously used management modality of symptomatic osteomas has mostly consisted of open approaches. Endonasal endoscopic approach provides a safe and effective alternative to open approaches, offering cosmetic advantages and lowering the morbidity. This approach could be judiciously used in large ethmoido-orbital osteomas in selected cases.
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7/21. Orbital decompression for gross proptosis associated with orbital lymphangioma.

    A 14-year-old boy with 11 mm of proptosis and exposure keratopathy secondary to an orbital lymphangioma underwent surgical debulking with a carbon dioxide laser through a lateral orbitotomy combined with a 3-wall orbital decompression. The proptosis was reduced by approximately 2 mm as a result of the debulking procedure, but a further 5 mm reduction was achieved with the orbital decompression. No serious adverse effects were encountered. Bony orbital decompression may be a useful alternative treatment in patients with severe proptosis secondary to orbital lymphangioma.
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8/21. Radiofrequency thermal ablation for recurrent meningioma extending extracranially.

    BACKGROUND: Image-guided and temperature-controlled radiofrequency thermal ablation techniques were applied to reduce tumor volume and relieve the symptoms caused by extracranial extension of recurrent meningioma. METHOD: We treated two patients with recurrent meningioma, an 81-year-old woman presenting with bulging of the temple and a 68-year-old woman presenting with visual disturbance, facial disfigurement, and sensory disturbance. neuroimaging in both patients, revealed a large tumor extending extracranially and involving the infratemporal fossa. To avoid injury to important anatomical structures either compressed or entrapped by the tumor, the spatial relation between the planned ablation volume and these structures was confirmed by 3-D reconstruction of the ablation target. During the ablation procedure, local temperatures over the tissue being cauterized were continuously monitored to limit the ablation area to that within the planned volume adjusting RF power. FINDING: Radiofrequency ablation produced tumor necrosis as planned without adverse effects and resulted in swift relief of symptoms and signs with shrinkage of the tumor. CONCLUSION: This technique may be an effective alternative for recurrent meningiomas extending extracranially and for which radical surgical procedures are not indicated.
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9/21. Lack of response to systemic corticosteroids in patients with lymphangioma.

    lymphangioma is a benign nonencapsulated orbital tumor that is difficult to treat. Recently, systemic corticosteroids were advocated as a safe and effective alternative to surgical drainage of the cysts. We describe our experience with 3 cases in which proptosis progressed or did not improve despite the exclusive use of systemic steroids for 1 week, necessitating urgent surgical intervention. These three cases demonstrate that at least in some cases, steroids alone may be ineffective, and alternative interventions must be undertaken.
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10/21. Combined positron emission tomography/computed tomography imaging of orbital lymphoma.

    PURPOSE: To report a case of orbital lymphoma with systemic dissemination that was identified and localized by combined positron emission tomography (PET)/computed tomography. DESIGN: Case report. methods: A 75-year-old woman presented with a 6-month history of painless, progressive fullness of her right upper eyelid. A PET/computed tomography was obtained to provide functional and anatomic assessment of the orbits. An orbitotomy was performed with sub-total excision of the lesion to obtain histologic diagnosis. RESULTS: The PET/computed tomography scan revealed intensive uptake of tracer in a right orbital mass measuring 2.3 x 1.8 x 2.6 cm. Foci of abnormal tracer uptake were also noted in the right neck, right hilar region, and central abdomen. Histopathologic findings were consistent with follicular cell lymphoma. CONCLUSIONS: The combination of PET/computed tomography provides accurate and precise information in an expedited manner not offered by alternative imaging modalities. PET/computed tomography imaging should be considered in the evaluation of orbital neoplasms, especially if lymphoma is suspected.
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