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Aspiration cytology of metastatic chordoma to the orbit.

PURPOSE: To report a case of metastatic tumor to the left orbit from a sacrococcygeal chordoma. METHODS: A 48-year-old man with a sacrococcygeal chordoma developed left orbit swelling, left eye proptosis with deteriorating vision, and the inability to walk. Fine needle aspiration cytology (FNAC) of the orbital tumor and sacrococcygeal tumor was performed. RESULTS: Fine needle aspiration cytology (FNAC) showed features of sacrococcygeal chordoma with metastatic tumor to the left orbit. CONCLUSION: Although chordoma rarely metastasizes, this case demonstrates chordoma metastatic to the orbit.

Biopsy, Needle↗

A new surgical technique for the management of orbital fat prolapse.

PURPOSE: To present a new surgical technique for the management of orbital fat prolapse. METHODS: Four eyes of three patients (average age +/- SD: 73.0 +/- 6.9 years; range, 69 to 81 years) who presented with orbital fat prolapse were treated with conjunctival fixation to the sclera approximately 8 to 10 mm posterior to the corneoscleral limbs in the area of orbital fat prolapse with four to seven 6.0 Vicryl (polyglactin 910, Johnson & Johnson, New Brunswick, New Jersey) stitches. RESULTS: The treatment with conjunctival fixation to the sclera was successful in all four eyes, and during a mean follow-up period of 357.5 days (+/-SD: +/-189 days; range, 641 to 263 days), there has been no recurrence of orbital fat prolapse. CONCLUSION: We successfully treated orbital fat prolapse with conjunctival fixation to the sclera, a new surgical approach for this condition.

Adipose Tissue↗

Orbital granulocytic sarcoma in an elderly patient.

PURPOSE: To report a 71-year-old woman with acute myelogenous leukemia in remission who developed orbital granulocytic sarcoma. METHODS: The patient was referred for acute proptosis and decreased vision of the right eye. Computed tomography of the orbits demonstrated a right extraconal mass compressing the optic nerve. A right lateral orbitotomy was performed, and a portion of the mass was excised for diagnostic purposes and orbital decompression. RESULTS: Histopathologic and immunohistochemical evaluation disclosed orbital granulocytic sarcoma. With chemotherapy and radiation, vision remained stable and right proptosis resolved. CONCLUSIONS: Orbital granulocytic sarcoma is usually diagnosed in children with a history of acute myelogenous leukemia. This case demonstrated that this entity may also occur rarely in older patients with a history of acute myelogenous leukemia.

Aged↗

Clinical characteristics associated with orbital invasion of cutaneous basal cell and squamous cell tumors of the eyelid.

Over a six-year period, between 1984 and 1990, 622 patients with basal cell and squamous cell carcinoma of the eyelids were examined at our institution. Thirteen patients had orbital invasion at initial examination. The average age of patients at orbital invasion was 75.8 years. Ten patients were men, eight of whom had basal cell carcinoma and two of whom had squamous cell carcinoma. Most patients had an orbital mass and incomitant strabismus at initial examination. Invasive basal cell carcinoma developed in 11 patients, and squamous cell carcinoma developed in two patients. Ten patients were treated for cutaneous carcinoma at the site of invasion before examination at our institution. The average duration between onset of a cutaneous lesion and our examination for orbital invasion was 9.8 years for basal cell carcinoma and one year for squamous cell carcinoma. Radiologic and histopathologic features were reviewed. The clinical characteristics of these patients were reviewed and orbital exenteration was recommended to all 13 patients. Nine patients underwent exenteration and four refused the operation.

Aged↗

Non-African Burkitt lymphoma presenting with oral thrush and an orbital mass in a child.

PURPOSE: To report an unusual case of orbital involvement with non-African Burkitt lymphoma in a child. METHODS: A 26-month-old boy developed oral thrush and painless right proptosis with eyelid swelling. Magnetic resonance imaging disclosed a diffuse mass involving the inferior, lateral, and superior right orbit and both maxillary sinuses. Incisional biopsy of the orbital mass was performed. RESULTS: Histopathology disclosed diffuse infiltration of the orbital tissues by a high-grade lymphoid neoplasm that showed positive immunoreactivity for B-cell markers. Immunoglobulin gene rearrangement studies disclosed a characteristic t(8;14) translocation consistent with a small, noncleaved malignant lymphoma of the Burkitt type. CONCLUSION: Non-African Burkitt lymphoma may manifest with orbital involvement.

Antineoplastic Combined Chemotherapy Protocols↗

Combined embolization and surgical treatment of arteriovenous malformation of the orbit.

We examined three patients with arteriovenous malformation of the orbit. At initial examination, all patients had a slowly progressive mass effect combined with congestive symptoms and clinical signs typical of an orbital vascular lesion. Arteriovenous malformation of the orbit was diagnosed on the basis of clinical and radiographic assessment, including the use of digital subtraction angiography and in one patient, magnetic resonance imaging. Treatment of each patient involved neuroradiologic embolization of the vascular lesion followed by surgical excision. Transient complications of endovascular management occurred. In one patient particulate emboli migrated into the central retinal artery after postembolization orbital surgical excision; consequent focal ischemic retinopathy contributed to short-term visual field deficits, which eventually resolved spontaneously. In another patient, spasm of the ophthalmic artery occurred during embolization, resulting in a transient afferent pupillary defect and loss in visual acuity to 20/60, which resolved over the subsequent week. High-flow orbital vascular lesions represent a considerable treatment challenge because of the complex anatomic and hemorrhagic characteristics of the malformation. Neuroradiologic characterization and embolization as a preoperative adjuvant is not without risk but offers an important advantage in the treatment of these formidable lesions. A combination of endovascular and surgical management may allow otherwise inoperable lesions to be treated successfully.

Adolescent↗

Orbital meningoencephalocele manifesting as a conjunctival mass.

Orbital meningoencephalocele is a rare congenital abnormality caused by a defect of the cranio-orbital bones that usually manifests soon after birth as a soft, cystic fullness in the superomedial canthal area with associated exophthalmos. We managed an unusual case of congenital orbital meningoencephalocele that manifested as a cystic conjunctival mass without proptosis or periorbital changes. Preoperative computed tomographic scans failed to demonstrate a bone defect. After suture ligature of the posterior stalk, excision of the lesion yielded an ependymal cyst surrounded by neuroglial and meningeal tissue and filled with cerebrospinal fluid. The patient had normal results of ophthalmic and neurologic examinations after transconjunctival resection of the lesion after three years of follow-up. An orbital approach may be appropriate for a few selected cases in which no bone defect is found on computed tomography. Orbital meningoencephalocele should be included in the differential diagnosis of isolated congenital conjunctival cystic masses.

Conjunctival Diseases↗

Immunofluorescent and immunoperoxidase characteristics of IgDlambda myeloma involving the orbit.

A biopsy specimen from a 59-year-old man with unilateral proptosis revealed an orbital plasmacytic tumor. In vitro immunoperoxidase and immunofluorescence studies and direct immunoperoxidase staining of Bouin's fixed, paraffin-embedded tissue sections demonstrated that the tumor consisted of plasma cells with surface and intracellular IgDlambda immunoglobulin. Systemic evaluation demonstrated multiple myeloma. A review of previously published reports combined with the case presented here suggests that IgD myeloma has a predilection for orbital involvement. Thus, while IgD myeloma accounts for only 2% of all myelomas, it may account for a significant percentage of myelomas with orbital involvement. The distinctive clinical course of IgD myeloma, particularly its poor prognosis, warrants consideration of the immunologic and serologic findings in patients with orbital involvement by myeloma. The application of immunofluorescence and immunoperoxidase techniques to the study of orbital lymphoreticular neoplasms should result in a clearer understanding of these neoplasms.

Diplopia↗

Increased expression of adrenomedullin mRNA in the tissues of intraocular and orbital tumors.

PURPOSE: To explore the possible involvement of adrenomedullin in the pathophysiology of intraocular and orbital tumors. METHODS: Competitive reverse transcription-polymerase chain reaction was used to determine adrenomedullin mRNA levels in the tissues from 40 consecutive patients (40 eyes) undergoing vitrectomy, orbital tissue biopsy, or enucleation for various ocular diseases, including intraocular (n = 4) and orbital (n = 3) tumors, proliferative vitreoretinopathy (n = 8), proliferative diabetic retinopathy (n = 8), age-related macular degeneration (n = 4), preretinal macular fibrosis (n = 9), and acute retinal necrosis (n = 4). RESULTS: Adrenomedullin mRNA levels in the tissues obtained from patients with intraocular or orbital tumors were significantly higher than those of patients with proliferative vitreoretinopathy (P <.05), proliferative diabetic retinopathy (P <.05), preretinal macular fibrosis (P <.005), and acute retinal necrosis (P <.01). CONCLUSION: Adrenomedullin may play a role in the pathophysiology of intraocular and orbital tumors.

Adrenomedullin↗

Primary orbital meningioma: a review of 41 patients investigated radiologically.

The X-ray features in 41 patients with histologically verified primary meningiomas in the orbit are reviewed. These tumours arise from the sheath of the optic nerve (78%) or extradurally within the orbit, remote from the nerve (22%). Sheath meningiomas occur predominantly in middle-aged women. Characteristically they give little sign of their presence on plain X-rays, but may show minor variations in the size of the optic canal (22%) or calcification in the tumour (12.5%). The radiological diagnosis is based upon the computed tomography (CT) findings. Visual deterioration in a middle-aged or elderly patient with obvious optic nerve enlargement on CT and minimal or no enlargement of the optic canal is virtually diagnostic of a sheath meningioma. Primary extradural meningiomas in the orbit usually present with proptosis before compressing the optic nerve. The majority of these show some plain X-ray evidence of their presence such as localised or generalised orbital enlargement, hyperostosis of the orbital walls, or changes in the adjacent sinuses. In this group the tumour may be located inside or outside the rectus muscle cone and the CT changes are usually non-specific.

Adult↗

CT findings in orbital tumours of nerve sheath origin.

The clinical and CT features of orbital nerve sheath tumours (13 neurofibromas and 17 neurilemmomas, including one malignant neurilemmoma) have been reviewed. The commonest clinical findings were proptosis, mild impairment of visual acuity and disorders of eye movements. No significant correlation with intra- or extraconal location and these symptoms was found. The two types of tumour were morphologically similar, most being smooth retrobulbar masses, elongated in the long axis of the orbit; lobulation was slightly more common with neurofibromas. The neurilemmomas were generally larger than the neurofibromas. The distribution of neurilemmomas in the retrobulbar space, and in its intra- and extraconal compartments appeared random, but most neurofibromas occurred in the upper orbit and three quarters lay extraconally. The apex of the orbit was seen to be clear of tumour in all intraconal neoplasms (except the one case of malignant neurilemmoma); this observation may be useful in differentiation from intrinsic optic nerve tumours and pseudotumour. Focal or generalised enlargement of the bony orbit was common, being seen in four-fifths of neurilemmomas and three-quarters of neurofibromas. Tumour density was homogeneous for all the neurilemmomas and in the majority of neurofibromas: attenuation coefficients were slightly higher for the neurofibromas. Subcutaneous extension was more common in neurofibromas, occurring in approximately one third of cases but was observed in about a tenth of the neurilemmomas. Enhancement with intravenous contrast medium was variable; contrast medium was helpful in showing intracranial extension. Although the CT findings did not allow unequivocal presurgical diagnosis, they were suggestive in many cases and facilitated biopsy.

Humans↗

Growth of the fetal lens and orbit.

OBJECTIVES: To present normal lens and orbital measurement ranges by gestational age. METHODS: Three hundred and thirty-five women with uneventful pregnancies were evaluated from the 15th to the 40th week of gestation. Lens and orbital measurements were added to routine biometric measurements for normal fetuses. RESULTS: A strong linear correlation was observed between gestational age (GA) and lens diameter (R(2)=0.89; P<0.0001), circumference (R(2)=0.89; P<0.0001), and surface (R(2)=0.90; P<0.0001). A linear correlation was also found between GA and orbital diameter (R(2)=0.92; P<0.0001), circumference (R(2)=0.92; P<0.0001) and surface (R(2)=0.95; P<0.0001). A linear growth function was observed between biparietal diameter and both lens diameter (R(2)=0.90; P<0.0001) and orbital diameter (R(2)=0.94; P<0.0001). CONCLUSION: Lens and orbital measurements provide normative data for fetal growth and development. These data may be also helpful in detecting fetal ocular anomalies.

Embryonic and Fetal Development↗

Management of orbital tumors.

Orbital tumors are uncommon. In children, both malignant and benign causes of orbital proptosis necessitate urgent assessment; in many cases, emergent intervention is necessary to avoid blindness. In adults, proptosis is most commonly associated with thyroid orbitopathy. Orbital tumors in adults rarely are characterized by the explosive growth and damage that can occur with childhood lesions. In both age-groups, the evolution of better scanning modalities, such as magnetic resonance imaging with fat saturation and gadolinium enhancement, has improved diagnostic accuracy, especially in patients with loss of vision. In more than 95% of cases, noninvasive techniques yield a correct diagnosis. In patients who require nonsurgical intervention, especially if the diagnosis is uncertain, fine-needle aspiration biopsy has an accuracy that exceeds 95%. Combined-modality therapy has improved the control of and decreased the morbidity associated with several orbital tumors. Surgical advances, such as the ancillary use of the CO2 laser, have enhanced the management of some orbital tumors.

Adolescent↗

Predictors of response to transantral orbital decompression in severe Graves' ophthalmopathy.

OBJECTIVE: To identify factors that may help predict the outcome after transantral orbital decompression in Graves' ophthalmopathy. DESIGN: A retrospective study was conducted of 428 patients who had undergone an initial transantral orbital decompression for severe Graves' ophthalmopathy at the Mayo Clinic between November 1969 and May 1989. MATERIAL AND METHODS: With use of logistic regression analysis, we assessed the preoperative characteristics, the early postoperative results, and the follow-up questionnaire data (obtained a median of 9.5 years postoperatively) from 304 female and 124 male patients with Graves' ophthalmopathy who had undergone transantral orbital decompression at a median age of 53 years. RESULTS: On multivariate stepwise regression analysis, young age, male sex, and long duration of eye symptoms were predictors of severe initial proptosis (P < 0.001). The only independent predictors of greater postoperative recession of proptosis were severity of initial proptosis and longer interval between operation and postoperative examination (P < 0.001). Patients with the most reduction of proptosis had the greatest improvement in visual acuity but more chance for postoperative development of continuous diplopia. Failure of prior corticosteroid or orbital radiation therapy did not affect the degree of recession of proptosis or improvement in visual acuity. On multivariate analysis for predictors of long-term overall patient satisfaction, only young age of the patient was of borderline significance (P = 0.05), and the only significant predictor of satisfaction with the postoperative eye appearance was an operation done primarily for cosmetic purposes (P = 0.012). CONCLUSION: Although various factors may influence the outcome of orbital decompression in patients with Graves' ophthalmopathy, this study showed that the more pronounced the initial proptosis, the greater the degree of recession postoperatively. A higher degree of reduction of proptosis is associated with better visual acuity but also a greater likelihood of development of continuous diplopia.

Adolescent↗

[Relapsing ophthalmoplegia as a presenting symptom of orbital myositis].

INTRODUCTION: We report a case of orbital myositis revealed by a relapsing and initially painless ophthalmoplegia. CASE REPORT: A 50-year-old patient suddenly presented with an isolated palsy of the right lateral rectus muscle which recovered in 3 weeks after a short-lasting corticosteroid therapy but relapsed. Cerebral and orbital imaging were normal. Orbital pain with a fluctuating course occurred 6 months later. Two years after the clinical onset, orbital CT scan revealed a fusiform hypertrophy of the right lateral rectus muscle which was enhanced by contrast. After corticosteroid treatment, the symptoms improved and CT scan became normal. CONCLUSION: Orbital myositis may not be ruled out in case of painless ophthalmoplegia and systematic and repeated imaging is necessary.

Adrenal Cortex Hormones↗

[Bilateral idiopathic orbital myositis].

INTRODUCTION: Orbital myositis is an idiopathic inflammation of the extraoccular muscles. It is often included under the broad description of orbital pseudotumor. It is commonly a unilateral affection. CASE REPORT: We report one case of bilateral orbital myositis in a 45-year-old woman. The first symptoms were diplopia and acute bilateral orbital pain exacerbated by eye movements. Physical examination especially neurological was normal. Serum creatine kinase and biological thyroid function were normal. CT scan found a bilateral enlargement of rectus oculi medialis and lateralis. Clinical improvement was obtained a few days after oral corticosteroid treatment with remission of all signs. CONCLUSION: Orbital myositis is a rare entity. 90-95p.cent of cases are unilateral. Bilateral forms are exceptional and in this case require search for specific etiologies particularly thyroid ophthalmopathy. There is controversy concerning treatment options but corticosteroids are still the most common first choice therapy with good outcome.

Adult↗

Cystic schwannoma of the orbit: case report.

BACKGROUND: Cystic lesions of the orbit have diverse clinical findings and histopathological features. We report an orbital schwannoma composed of a single cyst. CASE DESCRIPTION: A 62-year-old woman with an intraconal cystic tumor in the left orbital cavity is described. The cystic tumor was totally removed and the pathological diagnosis of the cyst wall was schwannoma composed of both Antoni type A and B. CONCLUSIONS: A rare case of cystic schwannoma of the orbit is reported. This case may contribute to an improved preoperative differential diagnosis for cystic lesions of the orbit.

Cysts↗

Periorbital and orbital cellulitis before and after the advent of Haemophilus influenzae type B vaccination.

OBJECTIVE: To evaluate the effect of the introduction of the Haemophilus influenzae B (Hib) vaccine (introduced first in 1985, then extended in 1990 to children at least 2 months of age) on the epidemiologic features of periorbital and orbital cellulitis. DESIGN: Retrospective, comparative case series. PARTICIPANTS: Three hundred fifteen pediatric inpatients. METHODS: Children at Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary with discharge diagnosis of periorbital or orbital cellulitis from 1980 through 1998 were reviewed. MAIN OUTCOME MEASURES: Case rate, culture-positive isolates, and associated conditions. RESULTS: A total of 297 cases of periorbital cellulitis and 18 cases of orbital cellulitis were reviewed. Before 1990, there were 27 cases of Hib-related cellulitis (11.7% of total in that period), whereas after 1990, there were only three (3.5% of total; P = 0.028). The number of cases per year was significantly lower after 1990 (21.2 +/- 10.4 vs. 8.7 +/- 3.9; P = 0.008), as were the number of positive culture isolates (for any organism) after 1990 (76 [33. 0%] vs. 9 [10.6%]; P < 0.001). The medical conditions most commonly associated with periorbital cellulitis were sinusitis (44 [14.5%]) and upper respiratory infections (73 [26.6%]). All cases of orbital cellulitis were associated with sinusitis. CONCLUSIONS: The introduction of the Hib vaccine coincided with a sharp decline not only in the number of periorbital and orbital cellulitis cases related to H. influenzae, but also in the annual case rate. These data are consistent with a facilitative role for H. influenzae in the development of cellulitis secondary to other pathogens. They also may support restriction of the spectrum of antibiotics used to manage these conditions.

Cellulitis↗