Biomedical subjects
Publications and source records attributed to T L Slamovits.
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.
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.
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.
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.
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.
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 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.
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.
Stereotaxic intracavitary irradiation with instillation of phosphorus-32 (32P) colloidal chromic phosphate was performed in nine patients with cystic craniopharyngiomas. Serial neurological, ophthalmological, neuroendocrinological, and radiological examinations were performed before and after treatment. Dosimetry was determined based on a computerized tomography (CT) estimation of tumor volume, and was calculated to provide a tumoricidal dose (200 to 300 Gy) to the cyst wall. The follow-up period ranged from 14 to 45 months (mean 27 months). After treatment, all nine patients showed improvement of symptoms and radiological evidence of cyst regression. Because of an expanding solid component producing recurrent symptoms, one patient required a craniotomy 14 months after isotope instillation. Three of five patients with impaired visual acuity before surgery had significant improvement in acuity after treatment. Preoperative visual field defects in eight patients improved in four after 32P therapy. Of seven patients with preoperative endocrine abnormalities, one individual showed almost complete normalization and another had improvement in endocrine function. Patients who exhibited residual neuroendocrine function before isotope instillation developed no significant deterioration in endocrine status during the follow-up period. The findings suggest that stereotaxic intracavitary irradiation is a safe and effective treatment which should be considered as the initial surgery for cystic craniopharyngiomas.
Three patients developed anisocoria as a result of third nerve palsies caused by intracranial lesions: two in the cavernous sinus and one in the mesencephalon. Pharmacologic testing using low concentration pilocarpine showed apparent preganglionic parasympathetic supersensitivity of the abnormal pupil. These findings are compatible with the existence of a proposed direct pathway from the midbrain to the eye, without synapse at the ciliary ganglion. Cholinergic hypersensitivity can occur, not only with postciliary ganglionic but also with preciliary ganglionic parasympathetic lesions.
We separated 24 patients with biopsy-proved giant cell arteritis into three groups based on erythrocyte sedimentation rates (ESRs) at clinical presentation: low, 1 to 40 mm/h; high, 41 to 80 mm/h; and very high, greater than 80 mm/h. The presence of anemia in the very high ESR group compared with the low ESR group was the only statistically identified difference. A linear regression analysis confirmed a high degree of inverse correlation between ESR and hematocrit in the subject population. There was no difference in ischemic ocular complications among the three groups. These findings emphasize that the diagnosis of giant cell arteritis should be made predominantly on clinical suspicion with less reliance on the ESR as a diagnostic criterion. Furthermore, the degree of ESR elevation does not predict which patients are at increased risk for the development of ocular complications. Finally, the ESR may not reliably indicate active disease in patients with normal hematocrit values.
Fine-needle aspiration was used as a primary orbital diagnostic technique in 156 patients. A 20-mm syringe, a 22-gauge, 3.75-cm needle, and a plastic pistol grip were used. Local anesthetic was not necessary. When the needle is in the lesion, small to-and-fro movements with a minimum of angulation are helpful. The aspirate is spread on slides fixed with alcohol. Positive cytologic identification was made in 125 of 156 cases. Commonly identified lesions included metastatic carcinoma, inflammatory lesions, and lymphoid lesions. Small posterior apical lesions are difficult to aspirate, but are often the most rewarding diagnostically. Fine-needle aspiration was not successful in tumors of fibrous consistency, in those located in the orbital apex, or in lymphocytic lesions with specimens inadequate for diagnosis.
Intrapapillary refractile bodies were observed in seven of 13 patients with chronic optic disc edema secondary to optic nerve sheath meningioma. The observation of refractile bodies preceded or coincided with decline in vision and disappeared as optic atrophy supervened. Intrapapillary refractile bodies appear to be a nonspecific sign of chronic optic disc edema.
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We report a case of thyroid carcinoma metastatic to the globe. In our survey we found thyroid carcinomas to metastasise to the eye rarely. In addition, we found metastases from the thyroid to be more common to the orbit than to the globe--a reverse of the pattern noted with tumours in general.
A 68-year-old woman experienced rapid progressive visual loss to blindness over a seven-week period. Inadequate clinical and diagnostic testing masked the diagnosis of a malignant glioma of adulthood.