William T. Shults

Combined Clinical and Computed Tomographic Diagnosis of Orbital Glioma end Meningioma

The clinical information on 22 patients with orbital optic nerve gliomas and 47 patients with meningiomas was correlated with computed tomographic findings obtained in both axial and coronal studies. Most of the gliomas occurred in children, although 7 patients presented after 20 years of age. Among the patients with meningiomas, the majority were women in early middle age, although two tumors occurred in children less than 20 years of age. Low grades of proptosis (median, 2 mm for both tumors), frequent significant visual field obscurations with eye movements, and opto-ciliary shunt vessels pointed toward the diagnosis of an optic nerve tumor. Patients with gliomas generally manifested massively swollen fusiform optic nerves with clear-cut margins due to circumscription by an intact dura. Kinks and bucklings of the optic nerve as well as infarctive cysts distinguished the glioma CT-scan patterns from the meningiomas. Distinctive axial CT-scan features of the meningiomas not shared by the gliomas were narrowly and diffusely enlarged nerves with polar expansions either at the orbital apex or immediately behind the globe; calcification; irregular excrescent margins signifying extradural invasion into the orbital soft tissues; a negative optic nerve shadow running down the center of the lesion; and bone erosion near the orbital apex. Coronal studies often revealed irregular margins signifying transgression of the dura. A diffusely and narrowly enlarged optic nerve shadow with regular margins (intrasheath lesions) was the one morphologically overlapping pattern displayed by 11 meningiomas and three gliomas. In these cases there tended to be more profound visual loss in the gliomas compared with the meningiomas, as well as the more frequent presence of opto-ciliary vessels in the meningiomas. Arteriography may be helpful in this particular category by demonstrating a tumor blush for the meningiomas, whereas this finding is typically absent with optic nerve gliomas. Meningiomas may be very closely simulated by dural or intraneural inflammations.

Features of amiodarone-induced optic neuropathy

PURPOSE To report clinical features of amiodarone-induced optic neuropathy and outline the differentiation of amiodarone optic neuropathy from nonarteritic anterior ischemic optic neuropathy. METHOD We reviewed data from 73 patients reported to have developed an optic neuropathy while taking amiodarone. RESULTS Amiodarone optic neuropathy is characterized by an insidious onset, slow progression, bilateral visual loss, and protracted disk swelling that tends to stabilize within several months of discontinuing the medication. Nonarteritic ischemic optic neuropathy is characterized by acute, unilateral visual loss that is usually complete at onset, with resolution of disk edema over several weeks. CONCLUSION Unique clinical features of amiodarone-induced optic neuropathy may help clinicians diagnose and distinguish between amiodarone-induced optic neuropathy and nonarteritic anterior ischemic optic neuropathy.

Clinical features and natural history of the acute idiopathic enlarged blind spot syndrome

OBJECTIVE To study the clinical pattern and natural history of patients with the symptom of an enlarged blind spot. DESIGN A retrospective case series. Twenty-one patients were collected from a neuro-ophthalmologic practice during the period January 14, 1983, to July 1, 1996, and four consecutive patients were added from three vitreoretinal practices during the period April 14, 1986, to June 7, 1999. PARTICIPANTS Twenty-six eyes of 25 patients were studied at onset and at repeat visits from 1 year and 7 months to 15 years and 6 months later. METHODS The first visit was composed of a complete neuro-ophthalmologic examination with fundus photos and fluorescein angiography in 12 of the 26 eyes. Follow-up examination consisted of an interval history, ophthalmologic examination, visual fields, fundus photographs, fluorescein angiograms in eight eyes, indocyanine green angiograms in seven eyes, and multifocal electroretinograms (mfERG) in both eyes of seven patients. MAIN OUTCOME MEASURES The major outcome measures were onset and long-term visual field characteristics, disc and peripapillary features, association with other chorioretinal diseases, and mfERG features. RESULTS Twenty-one eyes had clinical features of chorioretinal syndromes, which are usually associated with an enlarged blind spot. Five eyes were examined too late after onset to expect such features. The visual field defect regressed in all but 12 eyes but never to an unequivocally normal-sized blind spot. Four of the 12 had chorioretinal scarring that corresponded to the permanent field defect. Twenty-one of 26 eyes had peripapillary scarring. The peripapillary scarring appeared the same no matter what the associated chorioretinal disease or type or size of field defect was. mfERG testing of seven patients at follow-up revealed first-order and second-order abnormalities long after clinical recovery, abnormalities that were bilateral even when the clinical signs had been unilateral. CONCLUSIONS If an eye with an enlarged blind spot is examined within 2 weeks of onset, signs of a chorioretinal disease will usually be present. Beyond that period, signs such as disc congestion, disc staining, peripapillary retinitis, foveal changes, and peripheral retinal spots may not be present. Although the patient usually becomes asymptomatic, the blind spot is slightly and permanently enlarged, and there is usually peripapillary disc scarring. mfERG testing indicates that retinal damage is more widespread, bilateral, and permanent than the visual field and clinical features would indicate. Chorioretinal syndromes that are associated with a temporal field defect have some features in common and others that are distinctive.

Visual Recovery in Combined Central Retinal Artery and Central Retinal Vein Occlusion

We observed two patients who had combined central retinal artery occlusion and central retinal vein occlusion with severely reduced visual acuity and characteristic retinal changes. Over the course of several months, visual acuity and ophthalmoscopic appearance returned to normal. Both patients had a transient visual loss before their occlusive events and a mild nonconcurrent central retinal vein occlusion in their fellow eye. There was no evidence of inflammatory, vascular, or myeloproliferative disease.

Severe ocular and orbital toxicity after intracarotid etoposide phosphate and carboplatin therapy

PURPOSE To report severe ocular and orbital toxicity after administration of intracarotid etoposide phosphate and carboplatin. METHOD Case report. RESULTS A 52-year-old man with glioblastoma multiforme underwent left intracarotid administration of eto poside phosphate and carboplatin inferior to the ophthalmic artery. Within 7 hours, a nonpupillary block angle-closure glaucoma developed secondary to uveal effusion in the ipsilateral eye, which was relieved by cycloplegia. Four days later, severe orbital inflammation resulted in a visual acuity of counting fingers, proptosis, optic neuropathy, and total external ophthalmoplegia in the eye. The patients condition improved after a lateral cantholysis and administration of high-dose intravenous corticosteroids. Two weeks later, an anterior uveitis occurred in the left eye, which responded to topical corticosteroids. During a 2-month period, the patient recovered to a visual acuity of 20/70, near normal motility, and normal intraocular pressure, and the ocular and orbital inflammation resolved. Preexisting ipsilateral chemotherapy-induced maculopathy became more pronounced. CONCLUSION Ocular and orbital toxicity after intracarotid etoposide phosphate and carboplatin therapy is infrequently reported.

Secondary fractures of Le Fort I osteotomy.

Purpose To report the ophthalmic complications of Le Fort I osteotomy for the correction of dentofacial deformities and to determine the maximal compressive loads applied during pterygomaxillary separation in a cadaver model. Methods Two cases of ophthalmic complications arising after Le Fort I osteotomy are reported. Le Fort I osteotomy was performed on five cadavers. The maximal compressive load applied during pterygomaxillary separation was recorded with a 10 kN (3,000 lbf) load cell of a MTS Mini-Bionix servohydraulic machine (MTS, Eden Prairie, MN, U.S.A.). A paired t test was used to compare forces applied to the right and left sides. Computed tomography scans of each specimen were obtained after Le Fort I osteotomy to document secondary fractures. The skulls were subsequently stained with 1% fuschin red to highlight secondary fractures. Results Maximum compressive loads during pterygomaxillary separation ranged from 22 N (5.0 lbf) to 162 N (36.5 lbf), with an average of 106 N (23.8 lbf) (SD 47.6 N [10.7 lbf]). Forces applied on the first operative side were significantly greater than forces applied on the second operative side (p = 0.0034). Secondary fractures were found in three specimens by computed tomography and in two specimens by 1% fuschin red. All secondary fractures occurred on the second operative side. Conclusion Secondary fractures in the Le Fort I osteotomy procedures occurred on the side opposite the greater maximal compressive load and on the second operative side.

Annular macular neuroretinopathy and multifocal electroretinographic and optical coherence tomographic findings.

A 61-year-old woman noted distorted near vision in November 2000. She soon recognized the distortion as a ring-shaped scotomasurrounding fixation in her left eye. She could not remember any unusual circumstances before the onset. Her local ophthalmologist found diminished central sensitivity in the left eye on visual field testing with the Humphrey apparatus (Allergan, San Leandro, CA) and referred her to one of us (WTS) in February 2002. A neurologic examination and magnetic resonance imaging in the interim were unremarkable She had a history of hypothyroidism and breast cancer treated by surgery and radiation in 1987. She did not smoke and consumed no more than two cups of coffee and caffeinated soft drinks daily at the time of onset of symptoms. Visual acuity was 20/20 in the right eye and 20/15 in the left eye with 1.75 diopter spheres. She drew an annular dark scotoma around fixation in the left eye on the Amsler grid (Figure 1). Results of color vision testing with Ishihara plates were 10 of 10 in the right eye and 0 of 10 in the left eye. Pupillary responses were normal. A 10-2 visual field program (Humphrey) confirmed diminished central sensitivity in the left eye. The anterior segments were healthy in both eyes, as were the fundus in the right eye and the optic nerves in both eyes. The fovea in the left eye had a circular zone of slightly pigmented and flat outer retina and retinal pigment epithelium within 500 m of the foveola (Figure 2). Fluorescein angiography was unremarkable. She was examined again in April 2002 (RCW). The ocular findings were unchanged. Multifocal electroretinography was performed in both eyes after pupillary dilation, and the data were analyzed using Veris 4.1 Science software (Electro Diagnostic Imaging, Inc., San Mateo, CA). It showed normal peripheral responses in rings 4, 5, and 6 but subnormal responses in amplitudes and implicit time in the central three rings in both eyes. The losses were more profound in the left eye. First-order and second-order responses were abnormal (Figure 3). She was examined again in February 2003 (RCW). The symptoms and ocular findings were unchanged. Optical coherence tomography was performed in both eyes with the OCT 3 Model (Humphrey). The right eye tracings were normal, but in the left eye, there were defects in the inner borders of the outer high signal band under the clivus (Figure 4).