Progressive outer retinal necrosis presenting with isolated optic neuropathy.

Progressive outer retinal necrosis is a necrotizing herpetic retinopathy usually seen in immunocompromised patients. The authors describe two patients with this disease who initially had findings suggestive of an optic neuropathy. Vision declined after treatment with methylprednisolone, after which fundus examination became consistent with progressive outer retinal necrosis. These cases underscore the importance of careful examination of the retinal periphery before management of any presumed optic neuropathy with steroids.

Orbital presentations of giant cell arteritis.

BACKGROUND: giant cell arteritis (GCA) is a systemic vasculitis that may affect the optic nerve and cause blindness (e.g. ischemic optic neuropathy). Orbital inflammatory disease, however, is an uncommon presentation of GCA. PURPOSE: to alert clinicians to the orbital presentations of GCA. PATIENTS AND METHODS: a retrospective case series from tertiary care academic ophthalmic referral centers of four patients with orbital manifestations of giant cell arteritis. RESULTS: presentation of cases and review of the literature. In three cases, a temporal artery biopsy was diagnostic of GCA, but in one case, an orbital biopsy was needed to confirm the diagnosis. CONCLUSION: GCA can have orbital manifestations and clinicians should be aware of this unusual presentation of GCA in cases of presumed orbital inflammatory pseudotumor in the elderly.

Primary central nervous system lymphoma involving the optic chiasm in AIDS.

OBJECTIVE: To report visual loss resulting from chiasmal involvement by primary central nervous system lymphoma (PCNSL). MATERIALS AND METHODS: Case report. RESULTS: A patient with the acquired immune deficiency syndrome (AIDS) presented with visual loss resulting from PCNSL involving the optic chiasm. The clinical findings, neuroimaging, pathology, and treatment of this patient are described. CONCLUSIONS: Although rare, clinicians should consider PCNSL in the differential of a hypothalamic/chiasmal mass, especially in a patient with AIDS.

Orbital apex lesion as the presenting manifestation of sarcoidosis.

A 32-year-old black woman presented with progressive proptosis, diplopia, and optic disc edema of the right eye. A computed tomography scan of the orbit showed a right retroorbital mass. A gallium scan showed increased radiotracer activity in the right retroorbital region. Biopsy of the mass showed non-caseating granulomas that were compatible with sarcoidosis. The patient was treated with systemic steroids, and the proptosis and diplopia resolved. Seven months later, the patient presented with contralateral optic neuropathy. Neuroimaging showed enlargement of the left optic nerve. The patient again responded to systemic steroid treatment and experienced complete restoration of vision. An orbital apex lesion may be the presenting manifestation of sarcoidosis.

Third nerve palsy as the presenting manifestation of esthesioneuroblastoma.

A 52-year-old patient experienced unilateral third nerve palsy because of cavernous sinus involvement of biopsy-proven esthesioneuroblastoma. The patient experienced resolution of diplopia after surgical debulking of the tumor. Clinicians should be aware of the neuro-ophthalmologic manifestations of esthesioneuroblastoma.

A practice pathway for the initial diagnostic evaluation of isolated sixth cranial nerve palsies.

PURPOSE: To define a practice pathway for the evaluation of sixth-nerve palsies (SNPs) and to determine its cost-effectiveness and validity in a retrospective chart review. METHODS: A Medline search of the English-language literature from 1966 to 1995 was performed to define the available clinical evidence and develop the practice pathway. The authors retrospectively reviewed 407 charts with the diagnosis of SNP seen at three centers. Information obtained included: etiologic diagnosis if known; development of new neurologic or ophthalmologic findings; and results and costs of neuroimaging studies, if performed. RESULTS: Of the 407 patients, 98 underwent computed tomography scans and 212 underwent magnetic resonance imaging of the head. Eighty cases were non-isolated, 317 were isolated SNP, and ten could not be classified from chart information. Of the 317 cases of isolated SNP, 49 were classified as traumatic; 5, congenital; 158, vasculopathic; 63, nonvasculopathic; and 42, progressive or unresolved. Following the recommendations of the practice pathway, the 158 patients classified as having vasculopathic SNP would not have undergone neuroimaging studies, realizing a savings of $100,000 in this study population of 407 patients. CONCLUSION: The recommendations of the practice pathway are supported by review of the literature and the retrospective review of these cases. However, a prospective study with a matched control group is needed to demonstrate regional and specialty-specific variations in care and to strengthen the clinical certainty of the pathway recommendations.

Telemedicine screening of glaucoma.

OBJECTIVES: Glaucoma is a major cause of blindness. More than 80,000 Americans suffer permanent vision loss from the disease. Widespread screening is fundamental in limiting the incidence of glaucoma-associated blindness. This pilot study explored the use of stereo digital images taken at a primary care center for telemedicine review by an off-site specialist as a means of screening for glaucoma. MATERIALS AND METHODS: Thirty-two diabetic patients were screened at a family medicine clinic. None had previously been diagnosed with glaucoma. A senior optometry student took stereoscopic digital and 35-mm optic disc photographs with a nonmydriatic retinal camera. The digital images were forwarded to a remote ophthalmologist for review. The conventional color stereo slide pairs of the same eyes were subsequently reviewed for comparison. Agreement on signs of glaucomatous disc changes between the two imaging systems was analyzed. RESULTS: Twenty-six of 32 eyes' digital and 35-mm photographs were analyzed. Six of 32 eyes (18.8%) could not be compared due to lack of matching 35-mm slides or digital images. Out of 26 eyes, lamina cribosa visibility was undeterminable in 8 eyes' digital images and 3 other eyes' 35-mm slides. Agreement among digital images and 35-mm slides of the remaining eyes was: 100%-vertical elongation, barring of vessels, bayoneting of vessels, and drance hemorrhage; 96.2%-focus notching of rim and rim pallor; 93.3%-lamina cribosa visability; 92. 3%-overpass cupping; 88.5%-focal enlargement; 84.6%-parapapillary halo; 80%-nerve fiber visibility; 65.4%-parapapillary atrophy. Parapapillary halo (p = 0.046) and nerve fiber layer visibility (p = 0.18) were detected on some 35-mm slides but not seen on matching digital views. CONCLUSION: Evaluations of cup-to-disc ratio (C/D) using both methods were in general agreement. However, some digital images were noted as too dark for assessing fine glaucomatous disc changes. Stereo digital images taken with a nonmydriatic camera by nonophthalmic photographers is a promising alternative for glaucoma screening in primary care settings. Telemedicine offers efficient communications with off-site glaucoma specialists. A larger study population is necessary to determine the overall effectiveness of using stereo digital imagery and teleophthalmology for glaucoma screening.

Telemedicine enters eye care: practical experience.

1. A combination of store-and-forward technology and videoconferencing technology can be used in eye care and other medical specialties. 2. Bandwidth is a rate-limiting factor for obtaining a high-resolution, real-time image. 3. Protocols, clinical and technical, must be used to implement telemedicine in an efficient and high-quality manner.

Optimal imaging protocol after intraocular silicone oil tamponade.

PURPOSE: Our purpose was to define the optimal protocol for imaging of the orbits after vitreous humor replacement with silicone oil. METHODS: Eleven eyes in 10 patients with tractional and/or rhegmatogenous retinal detachment were studied. Five CT scans and 18 high-field (1.5 T) MR images were obtained. Standard T1-weighted, T1-weighted with fat and silicone saturation, fast spin density-weighted, and T2-weighted orbital MR sequences were performed. Unique pulse sequences included fast spin density-weighted and T2-weighted imaging with and without fat saturation or silicone saturation, gradient-echo imaging, and short-tau inversion recovery imaging. RESULTS: The T1-weighted MR and CT studies were comparable in displaying the silicone. However, the fat- or silicone-saturated fast T2-weighted sequences always showed the fibrous bands and subretinal fluid to best advantage. In one case, the eye also contained inadvertently retained perfluorocarbon liquid, which blended with silicone oil on both saturated images, requiring companion T1-weighted sequences without saturation to demonstrate its presence. CONCLUSION: Simple, commonly available fat-saturated fast T2-weighted MR images supplemented by standard T1-weighted images are all that are needed to evaluate the eye efficiently after vitrectomy and tamponade.

Neuro-ophthalmologic manifestations of adenoid cystic carcinoma.

Intracranial adenoid cystic carcinoma is uncommon. We report two unusual cases of intracranial adenoid cystic carcinoma. The first patient presented with a steroid-responsive optic neuropathy from an orbital mass that simulated orbital pseudotumor, and subsequently developed intracranial involvement, presumably by contiguous perineural spread. The second patient presented with proptosis of the left eye, right facial weakness and numbness, and an intracranial mass, presumably from hematogenous metastatic spread.

A guide to the isolated dilated pupil.

The poorly reactive and dilated pupil observed in a comatose patient is often thought to represent an acute third nerve palsy owing to brain herniation or aneurysm. In the well patient, however, the isolated dilated pupil is unlikely to be owing to a third nerve palsy. It is more commonly owing to other benign causes such as local iris sphincter abnormalities, pharmacologic dilation, tonic pupil syndrome, or sympathetic irritation. This article presents a diagnostic flowchart to help the primary care physician analyze this problem and prevent costly and unnecessary imaging of these patients.

A phase I study of TNP-470 administered to patients with advanced squamous cell cancer of the cervix.

A Phase I study of the novel angiogenesis inhibitor TNP-470 was performed. Patients with inoperable recurring or metastatic squamous cell cancer of the cervix with evaluable disease, no coagulopathy, and adequate renal, hepatic, and hematological function were eligible. One course of treatment consisted of an i.v. infusion of TNP-470 over 60 min every other day for 28 days, followed by a 14-day rest period. The starting dose was 9.3 mg/m2. Eighteen evaluable patients were treated, with a median age of 48 years (range 27-55) and performance status Zubrod 1 (range 0-2). Grade 3 neurotoxicities consisting of weakness, nystagmus, diplopia, and ataxia were encountered in two patients receiving the 71.2 mg/m2 dose. An intermediate dose level of 60 mg/m2 was evaluated and found to be well tolerated by three patients. Only one patient experienced grade 3 nausea on the 60 mg/m2 dose level. No myelosuppression, retinal hemorrhage, weight loss, or significant alopecia were observed. One patient had a complete response, which continues for 26 months, and three patients with initially progressive disease stage had stable disease for 5, 7.7, and 19+ months. Other Phase I studies, including over 200 patients, were performed concurrently with this study. Based on this experience, the dose of TNP-470 recommended for further studies is 60 mg/m2 as a 60-min i.v. infusion every Monday, Wednesday, and Friday. Neurotoxicity was dose limiting, but appears to be reversible. Otherwise, the treatment was well tolerated. The drug may be active in squamous cell cancer of the cervix. Further studies of TNP-470 in squamous cell cancer of the cervix are warranted.