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PubMed2005

Radiation therapy for orbital lymphoma.

PURPOSE: To describe radiation techniques and evaluate outcomes for orbital lymphoma. METHODS AND MATERIALS: Forty-six patients (and 62 eyes) with orbital lymphoma treated with radiotherapy between 1987 and 2003 were included. The majority had mucosa-associated lymphoid tissue (48%) or follicular (30%) lymphoma. Seventeen patients had prior lymphoma at other sites, and 29 had primary orbital lymphoma. Median follow-up was 46 months. RESULTS: The median dose was 30.6 Gy; one-third received <30 Gy. Electrons were used in 9 eyes with disease confined to the conjunctiva or eyelid, and photons in 53 eyes with involvement of intraorbital tissues to cover entire orbit. Local control rate was 98% for all patients and 100% for those with indolent lymphoma. Three of the 26 patients with localized primary lymphoma failed distantly, resulting in a 5-year freedom-from-distant-relapse rate of 89%. The 5-year disease-specific and overall survival rates were 95% and 88%, respectively. Late toxicity was mainly cataract formation in patients who received radiation without lens block. CONCLUSIONS: A dose of 30 Gy is sufficient for indolent orbital lymphoma. Distant relapse rate in patients with localized orbital lymphoma was lower than that reported for low-grade lymphoma presenting in other sites. Orbital radiotherapy can be used for salvage of recurrent indolent lymphoma.

Adult↗
PubMed2005

Proton radiotherapy for orbital rhabdomyosarcoma: clinical outcome and a dosimetric comparison with photons.

BACKGROUND: Over 85% of pediatric orbital rhabdomyosarcoma (RMS) are cured with combined chemotherapy and radiation. However, the late effects of photon radiation compromise function and cosmetic outcome. Proton radiation can provide excellent tumor dose distributions while sparing normal tissues better than photon irradiation. METHODS AND MATERIALS: Conformal 3D photon and proton radiotherapy plans were generated for children treated with proton irradiation for orbital RMS at Massachusetts General Hospital. Dose-volume histograms (90%, 50%, 10%) were generated and compared for important orbital and central nervous system structures. Average percentages of total dose prescribed were calculated based on the 3 dose-volume histogram levels for normal orbital structures for both the proton and photon plans. The percent of normal tissue spared by using protons was calculated. RESULTS: Seven children were treated for orbital rhabdomyosarcoma with proton irradiation and standard chemotherapy. The median follow-up is 6.3 years (range, 3.5-9.7 years). Local and distant controls compare favorably to those in other published accounts. There was an advantage in limiting the dose to the brain, pituitary, hypothalamus, temporal lobes, and ipsilateral and contralateral orbital structures. Tumor size and location affect the degree of sparing of normal structures. CONCLUSIONS: Fractionated proton radiotherapy is superior to 3D conformal photon radiation in the treatment of orbital RMS. Proton therapy maintains excellent tumor coverage while reducing the radiation dose to adjacent normal structures. Proton radiation therapy minimizes long-term side effects.

Brain↗
PubMed2003

Metastatic melanoma in the eye and orbit.

OBJECTIVE: Presentation of a large series of patients with metastatic melanoma involving the eye and orbit. DESIGN: Retrospective clinical study. PARTICIPANTS: Thirteen cases of metastatic melanoma involving intraocular tissues, 6 cases of metastatic melanoma in the orbit, and 1 case of metastatic melanoma involving both the eye and the orbit, treated in Lausanne between 1986 and 2002, were identified from the computer files of the ocular oncology and orbitopalpebral surgery units of Jules Gonin Hospital. METHODS: The analysis is based on the demographic data concerning these patients, clinical data concerning the tumor, the treatment applied, the outcome, and the follow-up. MAIN OUTCOME MEASURES: Clinical presentation of intraocular and orbital metastasis, interval between primary tumor and ocular metastasis, survival of the patients, evaluation of various therapeutic protocols. RESULTS: Intraocular metastases (14 cases, 15 eyes) were situated in the choroid in 11 cases (isolated lesion, 6 cases; multiple lesions, 3 cases; diffuse involvement, 2 cases), in the iris and ciliary body in 2 cases, and in the retina and vitreous in 2 other cases. The primary tumor was a cutaneous melanoma in 8 cases, a melanoma of the contralateral eye in 3 cases, a mucosal melanoma in 1 case, and was unknown in 2 cases. The mean interval between the diagnosis of ocular metastases and the patient's death was 8.8 months (range, 1-48 months). The primary tumor in the 7 cases of orbital metastases was a cutaneous melanoma in 5 cases, a uveal melanoma in the contralateral eye in 1 case, and was unknown in 1 case. The mean interval between the diagnosis of orbital metastases and death was 19.7 months (range, 5-48 months). The patients were treated by various protocols. The best results, in terms of both local tumor control and preservation of visual function, were obtained with circumscribed proton beam radiotherapy or external beam irradiation, depending on the site and extent of the tumor. CONCLUSIONS: Metastatic melanomas to the eye and orbit are rare and generally occur in patients with disseminated metastases during the terminal stages of the disease, with a short life expectancy. Treatment is palliative and, among the various possible treatment options, circumscribed proton beam radiotherapy or global photon beam radiotherapy, at relatively high irradiation doses, seems to achieve the most favorable results.

Adult↗
PubMed2006

Oral chlorambucil for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue of the orbit.

PURPOSE: To report the outcome of oral chlorambucil as a single treatment in patients with orbital mucosa-associated lymphoid tissue (MALT) lymphoma. DESIGN: Retrospective nonrandomized clinical study. PARTICIPANTS: Thirty-three patients with isolated orbital MALT lymphoma. METHODS: Medical records of all patients with histology-verified orbital MALT lymphoma treated with oral chlorambucil at the Royal Melbourne Hospital were reviewed. MAIN OUTCOME MEASURES: Complete clinical response, partial response, local relapse, systemic extension (distant relapse), and survival. RESULTS: Thirty-three patients (19 female; mean age, 69 years) participated in the study. Patients received an average of 4 courses of oral chlorambucil with a mean total dose of 600 mg. The lacrimal gland was the most frequent site of occurrence (24%), followed by the conjunctiva, eyelid, and superior orbit. Orbital mass, swelling, and diplopia were common presenting signs. Complete response was noted in 26 patients (79%). In 2 of the patients with complete clinical response, mild residual thickening was noted on follow-up orbital imaging studies. Four patients (12%) showed disease recurrence or relapse. Mean follow-up time was 32 (+/-20) months (range, 8 months-6 years; median, 26 months). None of the patients developed granulocytopenia secondary to chemotherapy, and none suffered significant nausea or vomiting. One patient with malignant transformation died 12 months after diagnosis and initial treatment. CONCLUSIONS: Systemic chemotherapy with chlorambucil is a reasonable option in patients with orbital MALT lymphoma. It is associated with minimal to no side effects. Additionally, it may be well tolerated by elderly patients and also may treat subclinical disease elsewhere.

Administration, Oral↗
PubMed2006

Orbital invasion by recurrent maxillary ameloblastoma.

PURPOSE: To describe 2 patients with orbital invasion by maxillary ameloblastoma, a rare odontogenic tumor that is not commonly encountered in ophthalmic practice. DESIGN: Retrospective, interventional case report. METHODS: Two patients who were diagnosed with maxillary ameloblastoma several years ago sought treatment for new-onset ocular and orbital signs and symptoms. MAIN OUTCOME MEASURES: Clinical and radiological findings and outcome. RESULTS: In the first patient, tumor recurrence with orbital invasion was diagnosed, and the patient underwent a total orbital exenteration. No recurrence was noted after 18 months of follow-up. The second patient had intracranial involvement with orbital invasion and underwent an extensive resection through an intracranial approach. No recurrence was noted after a 6-month follow-up period. CONCLUSIONS: Although a slow-growing tumor, maxillary ameloblastoma can recur after surgical excision and can be locally aggressive; it can invade the orbit and result in significant ocular morbidity. Ophthalmologists should be aware of this tumor and should monitor these patients closely when orbital invasion is suspected.

Aged↗
PubMedDate not supplied

Orbital cysts of childhood--classification, clinical features, and management.

There is little in the literature about the clinical spectrum of orbital cysts of childhood and no comprehensive classification has been proposed. The authors propose a classification of orbital cysts of childhood and review their clinical features, pathology, and management. The major categories in the classification include cysts of surface epithelium, teratomatous cysts, neural cysts, secondary cysts, inflammatory cysts, and noncystic lesions with cystic component. Cysts of the surface epithelium are further divided into simple epithelial cyst (epidermal, conjunctival, respiratory, and apocrine gland), and dermoid cyst (epidermal and conjunctival). Epidermal dermoid cyst (dermoid) is by far the most common orbital cystic lesion in children, accounting for over 40% of all orbital lesions of childhood and for 89% of all orbital cystic lesions of childhood that come to biopsy or surgical removal. Neural cysts include those associated with ocular maldevelopment (congenital cystic eye and colobomatous cyst) and those associated with brain and meningeal tissue (cephalocele and optic nerve meningocele). The most important secondary cyst is mucocele that can occur in children with cystic fibrosis. Inflammatory cysts are generally due to parasitic infestations and are more common in tropical areas of the world. Noncystic lesions that can have a cystic component include adenoid cystic carcinoma, rhabdomyosarcoma, lymphangioma, and others. Each type of cyst has rather characteristic, but not pathognomonic, clinical features. Computed tomography and magnetic resonance imaging can help differentiate a cystic lesion from a solid tumor, suggest the type of cyst, and help in planning management. The pathology varies with the cells that line the cyst and with the inflammatory agent. Management varies from local excision to observation, depending on the location and type of cyst. Orbital cysts of childhood can be classified into categories, based mainly on their histopathology. The clinical, radiologic, and histopathologic features can be correlated with the classification in order to better evaluate a child with a cystic lesion in the orbit.

Adolescent↗
PubMed1994

A review of 340 orbital tumors in children during a 60-year period.

We reviewed cases of histopathologically verified orbital tumors in children at our institution over 60 years to determine the distribution of various pathologic processes and trends over time. We studied the medical records and pathology specimens from 340 patients aged 18 years or younger who underwent biopsy for orbital mass from 1932 through 1991. The most common tumors were cysts (79 of 340, 23.2%), vascular lesions (60 of 340, 17.6%), optic nerve and meningeal neoplasms (56 of 340, 16.5%), inflammatory masses (29 of 340, 8.5%), osseous and fibrocystic lesions (27 of 340, 7.9%), and rhabdomyosarcomas (24 of 340, 7.1%). The overall frequency of malignancies was 18.2% (62 of 340): 11.5% (39) were primary tumors and 6.8% (23) were secondary and metastatic. The frequency of orbital malignancies was 25.4% (36 of 142) in the first 30 years (1932 through 1961) and 13.1% (26 of 198) in the second 30 years (1962 through 1991). The percentage of primary orbital malignancies was almost identical during the first and second periods (11.9% [17 of 142] and 11.1% [22 of 198], respectively). However, secondary and metastatic orbital neoplasms occurred in 13.4% (19 of 142) of the patients during the first 30 years and in only 2.0% (four of 198) during the second 30 years. The frequency of primary orbital malignancies in biopsy material remained the same over 60 years. However, the overall incidence of orbital biopsy specimens containing a malignancy decreased as a result of a reduction in the number of secondary and metastatic neoplasms that underwent biopsy.

Child↗
PubMed1992

Supervoltage orbital radiotherapy in 36 cases of Graves' disease.

Thirty-six patients who had clinically progressive Graves' dysthyroid orbitopathy were treated with supervoltage orbital radiotherapy. Twenty-three of the patients had discontinued the use of orally administered prednisone after developing intolerable side effects. Medical contraindications prevented seven patients from receiving systemic corticosteroid treatment. Six additional patients declined to take prednisone and chose orbital radiotherapy as their primary treatment. All patients were treated with one radiotherapy protocol wherein a 6 MV linear accelerator delivered 2,000 cGy to the midplane of both orbits in ten fractions. None of the 36 patients was treated with corticosteroids during the orbital radiotherapy treatment interval. We encountered three patients who failed to respond to orbital radiotherapy and required supplemental immunosuppression to arrest progression of Graves' disease. The remaining 33 patients experienced stabilization or clinical improvement of their condition. None of the patients experienced complications from orbital irradiation. We believe supervoltage orbital radiotherapy is an effective means of treating Graves' dysthyroid orbitopathy.

Adult↗
PubMed1983

Increase in orbital volume obtained by decompression in dried skulls.

We totally or partially removed the medial wall, floor, lateral wall, or a combination of the three, in four orbits and measured the increased orbital volume that results from the more common orbital decompression techniques. Removal of medial wall alone added 6 cc of volume to the orbit. Removing the floor of the orbit while preserving bone lateral to the infraorbital groove added 7 cc of volume. Removing the lateral floor added 1 cc and removing the lateral wall added 2 cc. Effective lateral decompression, therefore, requires creation of space within the temporal fossa. Removing the wall and the medial floor together added 13 and 14 cc of volume in two separate skulls. The largest volume gained by orbital decompression was 15 cc. This occurred when the entire orbital floor and medial wall were removed. Little volume is lost, however, when the lateral floor is preserved.

Cadaver↗
PubMed1999

Wrapping hydroxyapatite orbital implants with posterior auricular muscle complex grafts.

PURPOSE: To report the use of posterior auricular muscle complex grafts as a wrapping material for hydroxyapatite orbital implants in enucleation surgery. METHOD: In a retrospective multicenter study, autogenous posterior auricular muscle complex grafts were used to cover hydroxyapatite orbital implants in 83 patients with a mean age of 38.6 years (range, 1 to 85 years), of whom 63 had primary unilateral enucleation and 20 had secondary orbital implants following enucleation. The mean follow-up period after posterior auricular muscle complex grafts was 36 months (range, 14 to 60 months). RESULTS: Of the 83 patients, two (2.4%) developed limited orbital implant exposure, which was treated with a second posterior auricular muscle complex "patch graft." No patient developed postoperative orbital infection or implant extrusion. All patients were fitted with an acceptable prosthesis and had satisfactory cosmetic and functional results. No patient developed vascular compromise or a wound defect associated with the posterior auricular donor site. CONCLUSION: Autogenous posterior auricular muscle complex grafts should be considered as an appropriate wrapping material for hydroxyapatite orbital implants for primary enucleation and for secondary orbital implants after enucleation.

Adolescent↗
PubMed2001

Impairment of ocular blood flow during regional orbital anesthesia.

BACKGROUND: Regional anesthesia for ophthalmic surgery has been associated with ischemic complications, such as central retinal vascular occlusion, optic atrophy and ischemic optic neuropathy. Impairment of pulsatile ocular blood flow (POBF) may occur with regional orbital anesthesia. In this study we quantified POBF in patients undergoing regional orbital anesthesia. METHODS: Eleven patients (12 eyes) with a mean age of 76.5 years having regional orbital anesthesia for cataract or retinal surgery at a private refractive surgical centre in Calgary had POBF monitoring before, during and 15 minutes after induction of anesthesia. RESULTS: There were no significant changes in intraocular pressure or heart rate during the induction phase or 15 minutes after induction of regional orbital anesthesia. However, ocular blood flow indices, including pulse amplitude, pulse volume and POBF, were significantly reduced following attainment of regional orbital blockade (p < 0.05). With time there was recovery in these variables, but they all remained significantly reduced from baseline 15 minutes later. INTERPRETATION: Ocular blood flow appears to be significantly impaired during regional orbital anesthesia, induced as described. There could be benefit in monitoring POBF to reveal otherwise undetectable deleterious effects on retinal circulation in patients having retrobulbar injections, orbital compression or digital manipulation of the globe.

Aged↗
PubMed1993

Hydroxyapatite orbital implant after enucleation--experience with 200 cases.

The recently introduced hydroxyapatite orbital implant was designed to provide improved motility of the prosthesis in patients who have undergone enucleation. To date, little has been written about the use of the hydroxyapatite orbital implant in eyes that harbored malignant neoplasms. We have performed hydroxyapatite orbital implantation in approximately 200 patients, including children as young as 1 month of age, who underwent enucleation primarily for malignant intraocular tumors. During a median follow-up of 24 months, no cases of orbital hemorrhage, orbital infection, implant extrusion, or implant migration have occurred. Three patients have had conjunctival erosion, which was successfully repaired. Motility of the socket and fornices has been excellent, and all patients have cosmetically satisfactory motility of the prosthesis. Use of the hydroxyapatite implant has had no adverse effect on the clinical course of the intraocular neoplasms, and it has not hindered postoperative evaluation for recurrent orbital tumor. Overall, the hydroxyapatite orbital implant seems to be well tolerated, provides improved motility of the prosthesis, and is associated with few complications.

Adult↗
PubMed2003

Surgical treatment of orbital cavernomas.

BACKGROUND: There are numerous descriptions for the operative techniques applied in orbital lesions. We present a systematic overview of the surgical approaches, as determined by the location and extension of orbital cavernomas. METHODS: In our study, 37 patients (12 m, 25 f, aged 21-74 years, mean 51.22 years) underwent surgical removal of their orbital cavernoma at our clinic between 1988 and 2002. RESULTS: In ten cases the cavernoma was located in the lateral part of the intraconal space and a lateral orbitotomy was performed. Nine cavernomas were located more inferiorly and a transconjunctival approach was used. Seven cavernomas involving the optic canal, orbital apex and superior orbital fissure were operated on via an ipsilateral intradural approach. Four cavernomas of the superior orbital fissure or of the laterobasal intraconal space were approached extradurally. In one patient, a combined extra- intradural pterional operation was performed. Four patients with a lesion located medially in the posterior intraconal space were operated on via a contralateral pterional approach. One superior, medial, intraconal cavernoma was approached supraorbitally. In one cavernoma with direct contact to the sinus a transantral approach was used. The postoperative visual acuity improved in 14 of 19 cases with visual impairment, but in none with primary visual loss. Proptosis resolved completely in 21 of 27 patients. Ocular motility recovered in four of six patients, two patients with involvement of the superior orbital fissure presented with new oculomotor palsy postoperatively. CONCLUSIONS: The location of the cavernoma determines the recommended surgical approach. Because of their tendency to lead to irreversible loss of visual acuity due to the mass effect, we favor early surgery after the onset of symptoms.

Adult↗
PubMed2001

Neonatal orbital abscess.

PURPOSE: To describe two cases of orbital abscess in neonates and to review the literature of orbital cellulitis in neonates. DESIGN: Two interventional case reports. METHODS: Photographs, orbital computed tomography scans, and full pediatric examination were obtained in two cases of orbital abscess in neonates. RESULTS: Acute ethmoiditis with orbital abscess formation was found in both infants. In one of them, Staphylococcus aureus was identified as the source of infection. CONCLUSIONS: The clinical findings of our cases concur with the literature (eight cases) indicating that orbital abscess caused by Staphylococcal ethmoiditis is the most common form of orbital cellulitis in neonates.

Abscess↗
PubMed2003

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.

Aged↗
PubMed1992

Microanatomy of the orbital apex. Computed tomography and microcryoplaning of soft and hard tissue.

PURPOSE: The anatomy of the orbital apex is characterized by a complex interplay between critical bony and neural structures. Traditional methods used to study this region include dissection, static sections, and computed tomography (CT). Tomographic techniques are very useful in understanding these complex relationships, but the resolution of conventional CT and magnetic resonance imaging (MRI) is not sufficient to recognize the intricate details of the optic nerve canal and associated structures. The purpose of this study is to determine the value of microcryoplaning and computer reconstruction in visualizing the orbital apex in detail not previously possible, at any orientation in three-dimensional space. METHODS: Microcryotomy of the orbital apex area was performed on cadaver specimens, and images of each cryosection were digitized. Subsequently, the lesser wing of sphenoid bone and optic nerve were outlined to allow for spatial manipulation and three-dimensional visualization of the orbital apex. RESULTS: The authors present reconstructed computer images of the orbital apex in coronal and axial planes with CT correlation. Clinically important anatomic points and landmarks as well as potential pitfalls are demonstrated. CONCLUSION: Microcryoplaning and computer reconstruction are useful techniques in viewing the detailed anatomy of the orbital apex. Although microcryoplaning has the limitation of poor soft tissue detail, the resolution of captured images is much greater than those obtained from CT or MRI scans; the improved resolution allows for accurate CT correlations. The technique has utility in education, surgical planning, and quantitative analysis of orbital apical anatomy.

Computer Simulation↗
PubMed1994

Simultaneous intraocular and orbital non-Hodgkin lymphoma in the acquired immune deficiency syndrome.

BACKGROUND: Non-Hodgkin lymphoma is more common in patients with the acquired immune deficiency syndrome (AIDS), and the incidence of non-Hodgkin lymphoma in the AIDS population has been increasing as the life expectancy of these patients has increased. Nevertheless, intraocular lymphoma, as part of primary central nervous system lymphoma and orbital non-Hodgkin lymphoma, has rarely been reported in patients with AIDS. Co-existent intraocular and orbital lymphoma is exceptionally unusual. The clinical and histopathologic findings are reported in a patient with AIDS who had simultaneous intraocular and orbital non-Hodgkin lymphoma. The clinical funduscopic findings initially were attributed to a viral retinitis. METHODS: A left orbital biopsy followed by enucleation of the left eye was performed and studied by light microscopy. Immunophenotyping of the orbital biopsy also was performed. FINDINGS: Simultaneous intraocular and orbital large cell malignant lymphoma was present with neoplastic spread directly along the scleral canal of the ciliary nerve. Massive necrosis involving the retina, retinal pigment epithelium, choroid, and optic nerve, as well as several solid retinal pigment epithelial detachments, were observed. Immunophenotyping of the orbital tumor disclosed positive staining for Leu 4 (T cells, 30%) and Leu 14 (B cells, 60%). Immunostaining for light and heavy chains was precluded by tumor necrosis. CONCLUSION: Non-Hodgkin lymphoma should be included in the differential diagnosis of acute retinitis, and proptosis in patients with AIDS.

Aged↗
PubMed1996

Are systemic corticosteroids useful in the management of orbital pseudotumors?

PURPOSE: Systemic corticosteroids are the established primary treatment for orbital pseudotumors, but patients with recurrent and refractory pseudotumors commonly are observed. In this retrospective study, the authors aimed to assess the value of corticosteroids in the management of orbital pseudotumor. METHODS: The clinical notes, computed tomography scans, and histologic specimens of patients with orbital pseudotumor seen at the Orbital Center, Amsterdam, between 1976 and 1994 were analyzed with particular regard to the clinical and visual outcome. The patients were categorized according to the anatomic location of the pseudotumor within the orbit. Patients with pseudotumors restricted to the lacrimal gland or an extraocular muscle were excluded. RESULTS: The authors studied 32 patients with orbital pseudotumor, 20 of whom had histopathologic confirmation. Twenty-seven of these 32 patients received systemic corticosteroids as an initial treatment. Of these 27 patients, 21 (78%) showed an initial response and 10 (37%) obtained a cure. After the initial response, 11 (52%) of the 21 patients recurred. Optic nerve involvement was present in 13 (41%) of the 32 patients, and all except 1 patient recovered with corticotherapy. The mean follow-up of therapy was 4.3 years (range, 5.3 months-21.5 years). CONCLUSION: The authors found a moderate response, a high recurrence, and a low cure rate in patients treated with corticosteroids for orbital pseudotumor, and they therefore challenge the value of corticosteroids in both diagnosing and treating this condition. However, in patients with pseudotumor-induced optic neuropathy, corticosteroids remain of value.

Adolescent↗