231/1499. Melanotic neuroectodermal tumour of infancy involving the orbit and maxilla: surgical management and follow-up strategy. Melanotic neuroectodermal tumour (MNET) of infancy is a rare benign but locally aggressive tumour. We describe our surgical treatment of MNET of the orbital region. There was osteogenic relapse involving the bone of the orbit, 20 days after macroscopically complete excision of the primary tumour when the patient was 12 weeks old. This is only the second report of osteogenic relapse in MNET. The relapse was treated by excision of the involved orbital floor, preserving the orbital periosteum. The tumour has not recurred in 23 months of follow-up. Residual tumour islets may regress spontaneously after incomplete excision of MNET, but the relapse rate is between 15% and 45%. In our opinion, excising a safety margin of a few mm of apparently healthy bone reduces the risk of relapse. In contrast, the orbital contents should be preserved if they are macroscopically normal. Follow-up consisted of frequent physical examinations and CT scans. ( info) |
232/1499. Orbital exenteration for secondary orbital tumors: a series of seven cases. BACKGROUND: Exenteration is indicated in patients with malignant neoplasms of orbital contents. It entails the removal of the eyeball together with its extraocular muscles and other soft tissues. Exenterations can be classified into (1) total, (2) subtotal, and (3) supertotal exenteration. Retrospectively study, we reviewed 7 patients that had received exenteration/subtotal exenteration with spontaneous granulation/myocutaneous flap implantation or eyelid-sparing exenteration with myocutaneous flap. Primary lesions, histopathological examination results, treatments, and recurrences are discussed. methods: A retrospective study of the years 1987 through 2000 disclosed 7 patients that underwent exenteration/subtotal exenteration. The patients ranged in age from 41 to 68 years. Two patients underwent total exenteration without socket augmentation; 4 patients underwent exenteration/ subtotal exenteration with immediate facial reconstruction, and 1 with delayed facial reconstruction. RESULTS: classification of the 7 patients showed that 2 had basal cell carcinoma of the skin, 2 had squamous cell carcinoma of the conjunctiva, 1 had squamous cell carcinoma of the paranasal sinus, 1 had rhabdomyosarcoma of the paranasal sinus, and 1 had intracranial meningioma. radiotherapy was performed in 6 of the patients and chemotherapy in 2. Central nerve system invasion was noted in 2 patients, and 1 died due to it. CONCLUSION: Secondary orbital tumors involved the orbit from adjacent tissues: paranasal sinuses, nasopharynx, lacrimal sac, conjunctiva, eyelid, intraocular tissue, and intracranial tissues. Combined surgeries are necessary for complete tumor removal. And the imaging studies should include the field of the orbit, sinus, and brain to search for the primary lesions. ( info) |
233/1499. parvovirus-B19 infection preceding acute myeloid leukemia with orbital granulocytic sarcoma. We report here an unusual presentation of acute nonlymphoblastic leukemia with ocular granulocytic sarcoma who was firstly diagnosed iron deficiency anemia and acute parvovirus infection induced erythroid hypoplasia. To our knowledge this is the first paper of acute myeloblastic leukemia (AML) with granulocytic sarcoma, preceded by acute parvovirus B19 infection. ( info) |
234/1499. Bilateral exopthalmus due to retro-orbital chloromas in a boy with t(8;21)- positive acute myeloblastic acute leukemia. Exophthalmus is an infrequent finding in pediatrics. The differential diagnoses include chloromas, tumors of immature hematopoietic precursor cells. The case of a child with acute myeloid leukemia is reported. The presenting signs had included exophthalmus and nasal speech due to retro-orbital and sinusoidal chloromas, respectively. As in most cases of pediatric acute myeloid leukemia with chloroma, the AML1/ETO fusion gene was found, which portended a not unfavorable prognosis. This child was treated according to the AML-BFM 98 protocol and achieved an ongoing remission. The exophthalmus resolved completely. ( info) |
235/1499. Ipsilateral orbital cavernous hemangioma and choroidal hemangioma. PURPOSE: A unique case of simultaneous ipsilateral choroidal and orbital hemangiomas is described. methods: A 71-year-old man was found to have a left orbital intraconal mass while being worked up for intractable headache. He also had a left juxtapapillary choroidal hemangioma which did not cause any leakage to macular area. RESULTS: The left intraconal tumor was removed by medial transconjunctival orbitotomy. Histopathological examination revealed cavernous hemangioma. The ipsilateral choroidal hemangioma was managed by observation only. During 15 months of follow-up, there has been no growth or accumulation of subretinal fluid. CONCLUSIONS: The occurrence of orbital cavernous and choroidal hemangiomas on the same side suggests common pathogenic derangement acting on two different sites. ( info) |
236/1499. Pleomorphic lipoma of the orbit: a case series and review of literature. OBJECTIVE: Pleomorphic lipomas are rare benign tumors that can resemble a variety of malignant soft tissue tumors on histologic examination. Six cases of patients with orbital pleomorphic lipoma, one of which was proven to be bilateral, are presented. DESIGN: Retrospective, noncomparative, interventional case series with clinicopathologic correlation. methods: Clinical and histologic review of 6 patients with pleomorphic lipomas of the orbit and histologic review of fat from 22 exenteration specimens and 20 other orbital procedures. MAIN OUTCOME MEASURES: Evidence of histologic abnormalities in histologic specimens. RESULTS: Pleomorphic spindle cells and multinucleated cells with nuclei arranged in a floret-like pattern were present in 7 specimens from 6 patients presenting with a clinical diagnosis of orbital fat prolapse, but there were no similar cell types present in the adipose tissue of 22 exenteration or 20 other orbital specimens. CONCLUSION: Pleomorphic lipoma may arise in the orbit, presenting as what was hitherto considered to be age-related epibulbar prolapse of orbital fat. ( info) |
237/1499. An unusual case of heminasal aplasia: proboscis lateralis or cystic teratoma? Unilateral aplasia of the nose is a rare congenital malformation. It is often associated with other malformations of the facial region, including abnormalities of the eye and lacrimal system, proboscis lateralis, and facial bone malformations. The authors present a case of heminasal aplasia in a 4-year-old boy who underwent surgery immediately after birth because of right-sided proboscis lateralis. A huge hemispheric tumefaction was occupying a large part of the right orbit, medial canthus, and frontonasal region of the face, causing lateroinferior protrusion of the eyeball. The right half of the external nose was aplastic. The patient was operated on by an external approach. The pathohistological findings from the top of tumefaction revealed cystic teratoma. The possible backgrounds for the onset of the tumefaction are discussed in two main directions: the cystic form of the regrowth of the remnants of inadequately excised proboscis lateralis and cystic teratoma of the ethmoidal sinus. ( info) |
238/1499. Echography as a useful adjunct in the diagnosis of orbital solitary fibrous tumor. PURPOSE: To describe the echographic features of solitary fibrous tumor of the orbit. methods: A retrospective review of 3 patients with solitary fibrous tumor of the orbit and a literature review were performed. RESULTS: Three patients with orbital solitary fibrous tumor were studied with standardized echography and 3 other cases of orbital solitary fibrous tumor with ultrasound descriptions were identified in a literature review. Consistent echographic features of the 6 orbital solitary fibrous tumors included firmness, low to medium internal reflectivity, regular internal structure, moderate sound attenuation, and some degree of vascularity. CONCLUSIONS: Standardized echography is a useful adjunct in the evaluation of orbital solitary fibrous tumors and can help differentiate these tumors from other orbital lesions. ( info) |
239/1499. 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. ( info) |
240/1499. Occult orbital neuroblastoma detected after administration of an antitumor vaccine. A 6-year-old girl with neuroblastoma developed swelling and erythema of her right upper eyelid following administration of an interleukin-2 and lymphotactin gene-modified allogeneic neuroblastoma cell vaccine. Computed tomography demonstrated a cystic lesion in the subperiosteal space. A biopsy of the mass showed necrotic neuroblastoma with minimal associated inflammation. To our knowledge, this case represents the first description of occult orbital metastases in a patient with neuroblastoma detected after administration of an antitumor vaccine. ( info) |