Sarcomatous transformation of the orbit in a patient with Paget's disease.

OBJECTIVE: To report the clinical and pathologic features of sarcomatous transformation of the skull with involvement of the orbit, in Paget's disease. DESIGN: Interventional case report. PARTICIPANT: An 83-year-old woman with Paget's disease who experienced progressive proptosis of the left eye. INTERVENTION: Fine-needle aspiration biopsy of the orbital mass. RESULTS: Cytologic examination revealed round to oval malignant cells with wispy cytoplasm, consistent with the diagnosis of sarcoma. The patient died shortly after institution of orbital radiation therapy and systemic steroid therapy. CONCLUSIONS: Proptosis related to Paget's disease is uncommon, and only rarely is it a result of sarcomatous transformation. Sarcomatous transformation of the skull involving the orbit should be included in the differential diagnosis of progressive proptosis in patients with Paget's disease.

Calcified scleral plaques imaged on orbital computed tomography.

PURPOSE: To identify calcified scleral plaques on orbital computed tomography. METHODS: We identified all orbital computed tomographic scans at one center from January 1, 1994, through December 31, 1994. Orbital computed tomographic scans of 145 patients were reviewed to determine the presence or absence of the typical appearance of calcified scleral plaques on film. RESULTS: Of the 145 scans reviewed, nine (6.2%) demonstrated calcified scleral plaques. Seven (22.6%) of 31 scans obtained on patients older than 70 years demonstrated calcified scleral plaques. CONCLUSIONS: Calcified plaques of the sclera commonly appear on orbital computed tomographic scans, especially in elderly patients. This entity should be included in the differential diagnosis of ocular calcifications. Familiarity with the computed tomographic findings of calcified scleral plaques may help ophthalmologists and radiologists distinguish them from intrascleral metallic foreign bodies.

Fine needle aspiration cytology of orbital masses.

OBJECTIVE: To review our experience with fine needle aspiration of orbital lesions. STUDY DESIGN: Over an eight-year period, 24 orbital fine needle aspiration (FNA) samples were recorded, 22 of them adequate for interpretation. RESULTS: There were two benign tumors. There were 14 malignant tumors, 5 primary, 8 metastatic and 1 malignant and originating in the nasal cavity, invading the orbit. In two cases the original tissue diagnosis was incorrect and was revised by the fine needle aspirate. There were six nonneoplastic cases. In one case an infectious agent was identified, and another showed atypical lymphoid cells. Of the two nondiagnostic cases, one was compatible clinically with an orbital pseudotumor, and the other had no available follow-up. A specific diagnosis was achieved in 18 of 24 instances, or 75% of the cases. CONCLUSION: FNA is useful in the workup of an orbital lesion, leading to prompt diagnosis and treatment.

Neuro-ophthalmic manifestations of meningocerebral inflammation from the limited form of Wegener's granulomatosis.

PURPOSE: Wegener's granulomatosis is classically a systemic necrotizing granulomatous vasculitis that involves the upper and lower respiratory tracts and kidneys. Ophthalmologic and neurologic manifestations are common. The limited form of Wegener's granulomatosis may have pathologic characteristics consistent with the disease but lacks the complete clinical triad. We studied the clinical, pathologic, laboratory, and radiologic findings of four patients with Wegener's granulomatosis so that others will recognize the disease, even when it occurs in its limited form. METHODS: From three clinical centers, a chart review disclosed four patients with neuro-ophthalmic findings and the limited form of Wegener's granulomatosis. RESULTS: Three men and one woman, ranging in age from 40 to 73 years, were studied. All four patients had ocular motor abnormalities (one with oculomotor and trochlear nerve palsies, one with oculomotor nerve palsy, one with trochlear and abducens nerve palsies, and one with horizontal gaze deviation) in addition to other cranial nerve and cerebral abnormalities. Neuroimaging showed prominent meningeal, as well as intraparenchymal, abnormalities. In all patients, results of antineutrophil cytoplasmic antibody tests were initially negative but in one patient were positive at a late stage of the disease. In all patients, results of a biopsy demonstrated necrotizing granulomatous inflammation consistent with Wegener's granulomatosis. CONCLUSIONS: Neuro-ophthalmic findings may be the earliest manifestations of the limited form of Wegener's granulomatosis. Extensive meningocerebral inflammation can occur before systemic involvement or laboratory confirmation. Early diagnosis by biopsy of affected tissues may facilitate appropriate treatment and prevent progression of the disease.

Visual loss following treatment of sphenoid sinus carcinoma.

A 71-year-old woman developed complete third nerve palsy and total blindness of the right eye one month after completing a course of radiotherapy for sphenoid sinus carcinoma over a 13-month period. Differential diagnosis included recurrence of the tumor, radiation-induced second neoplasm, empty sella with chiasmal prolapse and secondary chiasmal arachnoid adhesions, and radionecrosis. Magnetic resonance imaging demonstrated gadolinium contrast enhancement of the right intracranial optic nerve and chiasm, suggesting a radionecrosis process.

Fascicular arrangement in partial oculomotor paresis.

We treated two patients with partial oculomotor paresis who had pupillary mydriasis, marked inferior rectus muscle weakness, and medial rectus muscle paresis, which were attributed to an ipsilateral fascicular lesion, demonstrated on neuroimaging studies. These cases support the fascicular proximity of inferior rectus muscle and pupillary fibers and suggest that fascicular medial rectus and inferior rectus muscle fibers are adjacent to each other.

Transient worsening of optic neuropathy as a sequela of the Jarisch-Herxheimer reaction in the treatment of Lyme disease.

A 58-year-old woman developed neurologic and neuroophthalmologic manifestations of Lyme disease, including a radiculomyelitis, cranial neuritis and mild right optic neuropathy. Upon treatment with intravenous ceftriaxone a Jarisch-Herxheimer reaction occurred with encephalopathy, mild fever, worsening radiculomyelitis, and deterioration of her visual acuity. Intravenous methylprednisolone was given, and the visual acuity recovered over 72 hours. This case suggests that transient worsening of optic neuropathy can develop as a sequela of the Jarisch-Herxheimer reaction in the treatment of Lyme disease.

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.

Posterior chorioretinopathy and retinal detachment after organ transplantation.

Four patients, three after renal transplantation and one after heart-lung transplantation, developed visual loss in both eyes associated with geographic zones of disruption and coarse clumping of the pigment epithelium in the posterior fundi. Secondary retinal detachment occurred bilaterally in three patients. Localized choroidal intravascular coagulation is the suspected but unproven cause.

Phakomatous choristoma of the eyelid. Immunohistochemical and electron microscopic observations.

BACKGROUND: A 13-month-old Hispanic boy underwent excision of a congenital inferonasal orbital mass arising from the right lower lid. Results of histopathologic examination of the tumor showed a phakomatous choristoma of the eyelid. An immunohistochemical and electron microscopic study of this rare, benign, congenital tumor of lenticular anlage was performed. METHODS: Immunohistochemistry was performed on 4-microns thick sections from paraffin-embedded tissue. Electron microscopy was performed on thin sections stained with uranyl acetate and lead citrate. FINDINGS: The cuboidal epithelial cells that comprise this choristoma showed strongly positive cytoplasmic staining with S-100 protein and vimentin and focally positive staining with a keratin cocktail (AE1/AE3). Electron microscopy showed the presence of numerous 10-nm whorled cytoplasmic microfilaments within degenerating epithelial cells. CONCLUSION: The immunoreactivity of this tumor to keratin and vimentin are newly described in this detailed clinicopathologic report and, together with its S-100 positivity, support the proposal that this tumor is of lenticular anlage. The authors hypothesize that the intracytoplasmic 10-nm intermediate filaments observed with electron microscopic examination within the epithelial cells that comprise this choristoma represent vimentin as detected by immunohistochemistry.

Disc swelling: a tall tail?

A 30-year-old man presented with monocular visual loss secondary to chronic papilledema, due to an ependymoma involving the spinal cord. No other neurological symptoms were present at the time. Initial neuroradiologic tests as well as laboratory investigations were negative, except for elevated pressure and protein concentration of his cerebrospinal fluid. In spite of intensive investigation, the diagnosis of a spinal cord tumor was delayed for approximately 12 months until he presented with neurologic symptoms attributable to a spinal cord lesion. This is only the fourth case reported of a spinal cord tumor associated with papilledema presenting with visual loss, without any other manifestations of either elevated intracranial pressure, or spinal disease. Possible mechanisms for elevated intracranial pressure in cases of spinal cord tumors are reviewed.

Visual recovery in two patients after intravenous methylprednisolone treatment of central retinal artery occlusion secondary to giant-cell arteritis.

Two patients with central retinal artery occlusions secondary to biopsy-proven giant-cell arteritis lost visual acuity to no light perception but recovered to baseline acuity after treatment with intravenous methylprednisolone at a dose of 15 to 30 mg/kg/day. The potential advantages and theoretical basis of early and aggressive treatment with large-dose intravenous corticosteroids in arteritic central retinal artery occlusion are discussed.

Neuroblastoma presenting as acute lymphoblastic leukemia but correctly diagnosed after orbital fine-needle aspiration biopsy.

A 3 1/2-year-old girl with a diagnosis of common acute lymphoblastic leukemia antigen (CALLA)-positive acute lymphoblastic leukemia was noted to be hypertensive and developed a tonic-clonic seizure. Computed tomography scan of the head revealed a right orbital mass. Orbital fine needle aspiration biopsy demonstrated rosette-like arrangements of cells with fibrillar cytoplasmic processes suggesting neuroblastoma. The tumor cells were antineuron-specific enolase positive. The cytologic findings suggested neuroblastoma, a diagnosis confirmed on subsequent work-up. The difficulty in distinguishing neuroblastoma from acute lymphoblastic leukemia in the pediatric patient is discussed in terms of clinical and cytologic features.

What to tell the patient with optic neuritis about multiple sclerosis.

If the ophthalmologist tells the patient with an initial isolated attack of optic neuritis that multiple sclerosis may develop, will he/she worry the patient needlessly or provide useful information that the patient is rightfully entitled to have? In this set of articles, the philosophies and individual circumstances that should influence the ophthalmologist in deciding what information to supply are discussed.

Discrete metastasis of solid tumors to extraocular muscles.

We report five cases of discrete solid tumor metastasis to extraocular muscles. Computed tomography confirmed that orbital involvement in all cases was confined to the extraocular muscles; there was no tumor in the bony orbit or in the adjacent paranasal sinuses or intracranial space. In two of our five cases, ophthalmic signs were the first evidence of metastatic disease; in the three other cases, there was a known history of cancer prior to orbital involvement. Pain, diplopia, and proptosis were the most common presenting manifestations. Neuroimaging demonstrated bilateral focal, nodular enlargement of multiple extraocular muscles in three cases. Diffuse enlargement of a single muscle occurred in the two other cases, inclusive of the tendinous insertion in one instance. Fine-needle aspiration biopsy provided a diagnosis of undifferentiated malignancy in all four cases in which it was performed.

Bilateral optic disc colobomas with orbital cysts and hypoplastic optic nerves and chiasm.

A 3-month-old boy with bilateral optic disc colobomas and orbital cysts is presented. Both eyes appeared relatively normal in size. Ultrasonography on one side demonstrated a communication between the globe and cyst by way of the optic disc coloboma. On computed tomography and magnetic resonance imaging, the cysts' contents were similar to vitreous. The optic nerves and chiasm appeared markedly hypoplastic on neuroimaging tests. Fluorescein angiography, electroretinography, and visual evoked responses suggested that the visual deficit primarily was ascribable to optic nerve or anterior visual pathway dysfunction.

Neuroimaging in neuro-ophthalmology.

Computed tomographic scanning is an excellent modality for evaluation of most orbital and intracranial tumors and strokes. However, MR scanning is definitely superior to CT when evaluating for multiple sclerosis, posterior fossa lesions (evaluation of gaze palsies, internuclear ophthalmoplegia, and downbeat nystagmus), or when assessing an area where sagittal scanning is important (chiasmal lesions). Magnetic resonance has increased specificity when the CT is equivocal. Although MR and CT may be complementary in the information they provide, many clinical studies have shown MR to be superior to CT in evaluating cerebral infarctions, hematomas, the intracanalicular optic nerve, optic chiasm, sella turcica, and the cavernous sinus. Magnetic resonance generally has replaced metrizamide CT cisternography as the procedure of choice for evaluating the suprasellar cistern and posterior fossa. Computed tomography is still preferable for major trauma, especially involving acute fractures and hematomas, although MR is more sensitive to the more subtle intracerebral lesions, such as shear injuries and subdural hematomas, that may provide prognostic information. Computed tomography also is preferred in situations where detecting small amounts of calcification is important for the differential diagnosis. As the development of MR imaging continues with faster scan times, finer spatial resolution, the use of paramagnetic contrast agents, and with increased availability and decreased cost, MR imaging may become preferable to CT as the imaging modality of choice for the CNS and orbit.

Scleritis as the presenting manifestation of procainamide-induced lupus.

Scleritis developed in a patient using procainamide as part of a drug-induced lupus syndrome. Systemic findings, which developed after the onset of ocular signs and symptoms, included arthralgias, myalgias, weight loss, and markedly elevated antinuclear antibody (ANA) titers and antihistone titers. The clinical picture and laboratory abnormalities improved after discontinuation of the drug. Although systemic findings secondary to drug-induced lupus have been well described in the medical literature, this case represents the first detailed ophthalmologic documentation of scleritis as the presenting manifestation of procainamide-induced lupus.