Mary E. Smith

Idiopathic inflammatory orbital pseudotumor in childhood. II. Results of diagnostic tests and biopsies.

Twenty-nine patients with pediatric orbital pseudotumor underwent a wide variety of diagnostic tests including biopsies. The following abnormalities were discovered: peripheral blood eosinophilia (9/29 patients); elevated ESR (17/27); elevated antinuclear antibody titres in the Tolosa-Hunt variant (2/2); hypercomplementemia (2/3); and mild CSF pleocytosis (2/6). Thyroid function tests were normal in nine patients so studied. B-mode ultrasonography performed on 12 patients displayed abnormalities in all cases (some combination of Tenonitis, myositis, perioptic inflammation, or mass effect). Computed tomography in seven patients provided higher resolution confirmation of these findings. Orbital bone changes and serious sinus disease were absent on routine radiographic studies. Biopsies performed on 16 patients disclosed mild lymphocytic inflammation in all cases, fibrosis and tissue eosinophilia in 9 biopsies (6 correlating with peripheral blood eosinophilia). Nine biopsies demonstrated a lipogranulomatous response to damaged fat cells. A true vasculitis or extensive lymphoid hyperplasia was not identified in any biopsy specimen.

Multifocal Static Creamy Choroidal Infiltrates: An Early Sign of Lymphoid Neoplasia

Three patients, each more than 60 years of age at initial presentation, had early stage lymphoid infiltrates (formerly called reactive lymphoid hyperplasia) of the choroid and contiguous extraocular tissues. There were multifocal, confluent, and nonconfluent creamy patches in the choroid. These lesions changed very little over periods of observation ranging from 1 to 4 years and failed to produce ophthalmoscopically visible disturbances of the retinal pigment epithelium (RPE). Fluorescein angiography demonstrated dye collection within the lesions without leakage into the subretinal or subpigment epithelial spaces. Both ultrasonography and computed tomography (CT) showed thickening of the choroid with either anterior or posterior episcleral extensions of lymphoid tissue. Pathologic evaluation of biopsy specimens of extraocular portions of the lesions showed low-grade tumors that were diffuse infiltrates of mature lymphocytes, which exhibited lymphoplasmacytoid features, Dutcher bodies, or small residual germinal centers (so-called borderline lesions). Two patients had uniocular localized disease, whereas the third had bilateral ocular lesions, hypogammaglobulinemia, and another extranodal chest wall lymphoid tumor. Therapy for the localized ocular condition consisted either of oral administration of corticosteroids or low doses of radiotherapy.

Retinal Detachment in the Morning Glory Anomaly

The morning glory anomaly is a rare congenital clinical entity that results from abnormal optic nerve development. In our series of 30 patients, followed for a mean duration of 10.3 years, 11 developed associated retinal detachments. These detachments followed a variable clinical course, including spontaneous reattachment and even redetachment, and may result from an abnormal communication between the subarachnoid space of the optic nerve and the subretinal space, allowing cerebrospinal fluid to accumulate subretinally. The clinical findings (including ultrasonography, computed tomography, and electrophysiologic testing), natural history, complications, and therapeutic indications are discussed.

Manetic Resonance, Imaging in the Evaluation of Optic Nerve Gliomas

Five patients with optic nerve gliomas were evaluated by magnetic resonance imaging (MRI). Images obtained with MRI were equal to computed tomography (CT) in evaluation of the orbital components of these tumors and superior to CT in evaluating the intracanalicular, chiasmal, and post-chiasmal extension of tumor. MRI is unimpeded by artifacts produced by surrounding bone, as encountered in CT, and also proves to be more sensitive in delineating subtle differences in fat content and hydration of neural tissues. Thus, MRI is extremely valuable in furthering our understanding of the natural and therapeutic response of optic nerve gliomas.

Retinoblastoma with Anterior Chamber Extension

Anterior chamber retinoblastoma is a rare clinical entity. The authors have reviewed the records of 1500 patients with retinoblastoma to determine the incidence and prognostic ramifications. Of 30 patients with anterior chamber involvement, 15 were noted on initial examination and 15 during observation after therapy. Despite aggressive multimodality treatment, total response was never achieved and all eyes were eventually lost. Results of pathologic examination showed ciliary body invasion, which was held accountable for the poor response to therapy. Anterior chamber retinoblastoma is a poor prognostic sign which cannot be controlled effectively with current techniques and should be considered an indication for enucleation without delay.

Magnetic Resonance Imaging in the Evaluation of Leukocoria

Leukocoria is an important clinical sign in ophthalmology. Conditions producing this white pupillary reflex must be differentiated from retinoblastoma to insure appropriate and timely treatment. Auxiliary diagnostic testing has been helpful in securing a clinical diagnosis. A new diagnostic modality, magnetic resonance imaging, provides similar morphologic information with the additional potential for biochemical characterization. A series consisting of 14 patients presenting with leukocoria as a result of retinoblastoma and simulating conditions was examined. The magnetic resonance imaging findings are discussed.

Regression of uveal malignant melanomas following cobalt-60 plaque. Correlates between acoustic spectrum analysis and tumor regression.

Parameters derived from computer analysis of digital radio-frequency (rf) ultrasound scan data of untreated uveal malignant melanomas were examined for correlations with tumor regression following cobalt-60 plaque. Parameters included tumor height, normalized power spectrum and acoustic tissue type (ATT). Acoustic tissue type was based upon discriminant analysis of tumor power spectra, with spectra of tumors of known pathology serving as a model. Results showed ATT to be correlated with tumor regression during the first 18 months following treatment. Tumors with ATT associated with spindle cell malignant melanoma showed over twice the percentage reduction in height as those with ATT associated with mixed/epithelioid melanomas. Pre-treatment height was only weakly correlated with regression. Additionally, significant spectral changes were observed following treatment. Ultrasonic spectrum analysis thus provides a noninvasive tool for classification, prediction and monitoring of tumor response to cobalt-60 plaque.

Computed Tomography of the Nonrhegmatogenous Retinal Detachment in the Pediatric Patient

Nonrhegmatogenous retinal detachments occur in the pediatric population secondary to other ocular disorders. The most important cause to identify is retinoblastoma. Computed tomography has proven to be the most reliable ancillary diagnostic test in this pediatric population. A series of eighty patients, presenting with nonrhegmatogenous retinal detachments, was examined. The role of computed tomography and characteristic clinical findings are discussed.

Immersion ultrasonography: simultaneous A-scan and B-scan.

In eyes with opaque media, ophthalmic ultrasound provides a unique source of information that can dramatically affect the course of patient management. In addition, when an ocular abnormality can be visualized, ultrasonography provides information that supplements and complements other diagnostic testing. It provides documentation and differentiation of abnormal states, such as vitreous hemorrhage and intraocular tumor, as well as differentiation of orbital tumors from inflammatory causes of exophthalmos. Additional capabilities of ultrasound are biometric determinations for calculation of intraocular lens implant powers and drug-effectiveness studies. Maximal information is derived from ultrasonography when A-scan and B-scan techniques are employed simultaneously. Flexibility of electronics, variable-frequency transducers, and the use of several different manual scanning patterns aid in detection and interpretation of results. The immersion system of ultrasonography provides these features optimally.

Diagnostic Ocular Ultrasonography

Since the 1960s ultrasonography has advanced from a research tool limited to the measurement of ocular dimensions to sophisticated imaging modality. The technique is now used extensively at all levels of ophthalmic evaluation. The most common application of diagnostic ultrasonography is for evaluation of eyes with opaque media (e.g., dense cataract or vitreous hemorrhage) or eyes that have sustained extensive ocular trauma with subsequent opacification of the ocular media. Additionally, ultrasonography is valuable for the characterization of ophthalmoscopically visible abnormalities such as choroidal tumors.