Isolated progressive visual loss after coiling of paraclinoid aneurysms.

BACKGROUND AND PURPOSE: The proximity of the paraclinoid segment of the internal carotid artery to the visual pathways may result in visual deficits when patients present with aneurysms in this segment. Although surgical clip ligation of these aneurysms has been the standard of care for decades, the advent of coil embolization has permitted endovascular therapy in those aneurysms with favorable dome-to-neck ratios. Although immediate nonprogressive visual loss after coil embolization of paraclinoid aneurysms has been well described, isolated progressive visual loss immediately or shortly following coil embolization, to our knowledge, has not. We have identified 8 patients who experienced progressive loss of vision, unassociated with any other neurologic deficits, developing immediately or shortly after apparently uncomplicated coil embolization of a paraclinoid aneurysm. MATERIALS AND METHODS: This study is a retrospective case series of 8 patients seen at 4 separate academic institutions. Inpatient and outpatient records were examined to determine patient demographics, previous ocular and medical history, and ophthalmic status before endovascular embolization. In addition, details of the primary endovascular therapy and subsequent surgical and nonsurgical interventions were recorded. Follow-up data, including most recent best-corrected visual acuity, postoperative course, and duration of follow-up were documented. RESULTS: Eight patients developed progressive visual loss in 1 or both eyes immediately or shortly after apparently uncomplicated coiling of a paraclinoid aneurysm. MR imaging findings suggested that the visual loss was most likely caused by perianeurysmal inflammation related to the coils used to embolize the aneurysm, enlargement or persistence of the aneurysm despite coiling, or a combination of these mechanisms. Most patients experienced improvement in vision, 2 apparently related to treatment with systemic corticosteroids. CONCLUSION: Patients in whom endovascular treatment of a paraclinoid aneurysm is contemplated should be warned about the potential for both isolated nonprogressive and progressive visual loss in 1 or both eyes. Patients in whom progressive visual loss occurs may benefit from treatment with systemic corticosteroids.

Symptomatic corneal topographic change induced by reading in downgaze.

OBJECTIVE: To elucidate the cause of monocular blur or diplopia after reading in downgaze. METHODS: Corneal topography was obtained before and after a 15-to 30-minute reading effort in downgaze in three symptomatic patients and in nine asymptomatic control subjects. RESULTS: Changes in corneal topographic color maps, corneal uniformity index, and predicted corneal acuity were found in the symptomatic patients but not in the control subjects before and after reading. CONCLUSION: Changes in corneal topography can occur after prolonged reading in downgaze and may produce symptoms of blur or monocular diplopia.

Atypical features prompting neuroimaging in acute optic neuropathy in adults.

BACKGROUND: Acute optic neuropathy due to an intracranial lesion may masquerade as optic neuritis or nonarteritic anterior ischemic optic neuropathy (NAION). We reviewed the records of patients who presented with acute unilateral optic neuropathy that was initially diagnosed as optic neuritis or NAION but who ultimately proved to have an underlying structural lesion. METHODS: Retrospective observational case series. We reviewed the records of patients with the initial diagnosis of optic neuritis or NAION in whom the diagnosis was changed to an intracranial etiology at four tertiary care neuro-ophthalmology centres between 1995 and 1998. RESULTS: Eight cases were identified in which atypical features prompted further investigation, including neuroimaging, leading to the diagnosis of an intracranial etiology for the optic neuropathy. Five patients were discovered to have neoplasms (a tuberculum sellae meningioma in two cases, an optic nerve sheath meningioma in two cases and a metastatic lesion in one case), and three patients had intracranial sarcoidosis. Atypical features for optic neuritis included a progressive course, absence of pain, optic atrophy at presentation, lack of significant visual improvement and age over 40 years. For NAION, the atypical features included progressive course, optic atrophy on presentation, absence of vasculopathic risk factors and preceding transient visual loss. INTERPRETATION: Clinicians should be aware that patients with intracranial lesions may present with acute optic neuropathy mimicking optic neuritis or NAION and that certain atypical features should warrant consideration for neuroimaging.

Incidence of ocular misalignment and diplopia after uneventful cataract surgery.

PURPOSE: To evaluate the incidence of ocular misalignment and diplopia after uneventful cataract surgery. SETTING: An outpatient private practice eye institute. METHODS: One hundred thirty-eight patients referred to 1 cataract surgeon were prospectively evaluated. Orthoptic evaluations were performed within 1 month before and then 1 day, 1 week, and 1 month after cataract surgery. Anesthesia was by retrobulbar injection, and cataract extraction was done by phacoemulsification. RESULTS: Cataract surgery was performed in 118 patients. Preoperatively, 16 patients had ocular misalignment; 10 were phoric, 4 were intermittently tropic, and 2 were tropic. Follow-up evaluation was obtained for 101 patients (86%) at 1 day, 91 (77%) at 1 week, and 88 (75%) at 1 month. A change in ocular alignment occurred in 22 of 101 patients (22%) at 1 day, 9 of 91 (10%) at 1 week, and 6 of 88 (7%) at 1 month. Only 1 patient who had a change in alignment at 1 month was symptomatic. CONCLUSIONS: A persistent change in ocular alignment after uneventful cataract surgery occurred in 7% of patients. However, symptomatic diplopia was uncommon (1 in 118; 0.85%) in this relatively small series.

Treatment of optic nerve sheath meningioma with three-dimensional conformal radiation.

PURPOSE: To report a patient recovering vision after three-dimensional conformal radiotherapy for optic nerve sheath meningioma. METHODS: Radiotherapy was delivered by a three-dimensional conformal technique in 28-180-cGy fractions. RESULTS: Visual acuity improved from 20/200 to 20/30, and the visual field defect resolved. CONCLUSION: Visual loss from optic nerve sheath meningioma can be reversed by three-dimensional conformal radiotherapy.

Ophthalmoplegia associated with the anti-Ri antibody.

Anti-Ri antibodies most often occur in patients with breast cancer and typically are associated with the paraneoplastic syndrome of opsoclonus-myoclonus-ataxia. This study reports a patient with diplopia and ophthalmoplegia. She had anti-Ri antibodies, and despite an exhaustive search for malignancy at presentation, breast cancer was not detected for six months.

Visual loss in idiopathic intracranial hypertension after resolution of papilledema.

PURPOSE: To demonstrate that progressive visual field loss may occur after resolution of papilledema in patients with idiopathic intracranial hypertension and persistently elevated intracranial pressure. METHODS: A patient with idiopathic intracranial hypertension was evaluated with serial Humphrey automated static perimetry after initial treatment and resolution of papilledema. RESULTS: The patient developed recurrent headache and elevated cerebrospinal fluid pressure. Optic nerve head appearance did not change. Automated perimetry demonstrated reproducible, worsening visual field loss; mean deviation decreased 11 dB in each eye. Visual field defects resolved after optic nerve sheath fenestration. CONCLUSIONS: Increased intracranial pressure caused visual field loss after resolution of papilledema. Optic nerve sheath fenestration improved visual function in this patient.

An ice test for the diagnosis of myasthenia gravis.

OBJECTIVE: To determine whether ice application to a ptotic eyelid can differentiate myasthenic from nonmyasthenic ptosis. DESIGN: Prospective, multicenter, nonrandomized, comparative trial. PARTICIPANTS: Twenty patients with myasthenia gravis (MG) and ptosis were evaluated in the neuro-ophthalmology service. CONTROL SUBJECTS: Twenty patients with nonmyasthenic ptosis evaluated in the same locale. METHODS: Palpebral fissures were measured before and immediately after a 2-minute application of ice to the ptotic eyelid. MAIN OUTCOME MEASURES: The difference in palpebral fissures in millimeters before and after ice application. Two or more millimeters of improvement after ice application was considered a positive ice test result. RESULTS: A positive ice test result was noted in 16 of the 20 (80%) patients with MG and in none of the 20 patients without MG (P < 0.001). Of the 4 patients with MG and complete ptosis, 3 had a negative ice test result. CONCLUSIONS: The ice test is a simple, short, specific, and relatively sensitive test for the diagnosis of myasthenic ptosis. The sensitivity of the ice test in patients with complete ptosis decreases considerably.

Optic nerve head swelling in the Hadju-Cheney syndrome.

The Hadju-Cheney syndrome is one of the idiopathic acroosteolyses. Associated neurologic abnormalities are often a result of progressive basilar invagination. A 48-year-old man with the Hadju-Cheney syndrome developed progressive bilateral visual loss. On examination, he had hyperopia, choroidal folds, optic nerve head swelling, and mild optic neuropathy. Computed tomographic scans showed massive enlargement of both intraorbital optic nerve sheaths. Improvement occurred after optic nerve sheath fenestration. Visual loss due to optic nerve meningocele can occur in the Hadju-Cheney syndrome. Optic nerve sheath fenestration can result in visual improvement. It is unclear whether the occurrence of optic nerve meningocele is causally or fortuitously related to the Hadju-Cheney syndrome.

Congenital optic nerve anomalies.

Congenital optic nerve anomalies are not as rare as was once thought. Visual prognosis depends on the type of anomaly present. Both central nervous system and systemic abnormalities have been associated with congenital optic nerve anomalies. Therefore, correct diagnosis has important visual and systemic implications. Congenital optic nerve anomalies are distinguished by the optic disc and peripapillary retinal appearance. In general, these anomalies can be grouped by abnormalities of optic disc size or conformation and by the presence of tissue not usually present at the disc.

Pneumosinus dilatans: a sign of intracranial meningioma.

BACKGROUND: Pneumosinus dilatans affecting the sphenoid and posterior ethmoid sinuses has been reported in association with spheno-orbital meningiomas and is believed by some authors be a sign of an adjacent meningioma. METHODS: We report the case of a 57-year-old man who developed progressive neurologic signs and symptoms consistent with a frontal lobe lesion. Neuroimaging studies revealed a large partially cystic mass at the base of the anterior cranial fossa that appeared to be invading the left frontal lobe and that was associated with pneumosinus dilatans of the adjacent left frontal sinus. RESULTS: Although the appearance of the mass by neuroimaging was thought to be most consistent with a malignant glioma, the lesion was found at craniotomy to be a benign meningothelial meningioma. CONCLUSIONS: Many previous cases of progressive optic neuropathy associated with pneumosinus dilatans affecting the sphenoid and posterior ethmoid sinuses have been found to be caused by adjacent optic nerve sheath meningiomas. This case provides further evidence that pneumosinus dilatans is a sign of intracranial meningioma.

Magnetic resonance imaging in patients with unexplained optic neuropathy.

PURPOSE: To investigate the anatomic relation between the optic nerve and the adjacent intracranial internal carotid artery (ICA) in patients with unexplained optic neuropathy. METHODS: Coronal magnetic resonance images (MRIs) of 20 patients with unexplained optic neuropathy and 20 age-matched controls were evaluated with digitizing software. RESULTS: The distance between the optic nerve and ICA was significantly less on the side ipsilateral to the atrophic optic nerve than either the study group's contralateral optic nerve-ICA distance (P<0.001) or the control group's optic nerve-ICA distance (P<0.001). The diameter of the atrophic optic nerves was significantly less than either the diameter of the study group's contralateral normal nerves (P=0.008) or the control group's nerves (P<0.001). There was no significant difference between the diameters of the ICA flow voids adjacent to the atrophic optic nerves versus either the study group's contralateral ICA flow voids (P=0.91) or the control group's ICA flow voids (P=0.74). CONCLUSIONS: The authors speculate that the intimate relation between the optic nerve and adjacent ICA may be important in the development of unexplained optic neuropathy. The ICA need not appear abnormal in MRIs.

Orbital infarction syndrome after surgery for intracranial aneurysms.

BACKGROUND: Global orbital infarction results from ischemia of the intraocular and intraorbital structures due to hypoperfusion of the ophthalmic artery and its branches. PATIENTS: The authors describe six patients in whom acute proptosis, ophthalmoplegia, and blindness developed immediately after surgery for intracranial aneurysms. RESULTS: All patients underwent standard frontotemporal craniotomies to clip their aneurysms. In all patients, proptosis, ophthalmoplegia, and blindness developed in the immediate postoperative period; fundus abnormalities included retinal edema, retinal arteriolar narrowing and other vascular abnormalities, and pale optic disc swelling. Some patients had facial and corneal anesthesia. Ophthalmoplegia and facial anesthesia improved in most patients, but none regained any vision in the affected eye. CONCLUSION: Orbital infarction syndrome is a rare complication of neurosurgical procedures. Increased orbital pressure probably reduced ophthalmic artery and collateral arterial perfusion, resulting in ischemia of the intraocular and intraorbital structures. There may be multiple factors that compound the risk for orbital infarction, and patients with subarachnoid hemorrhage, increased intracranial pressure, anomalous arterial or venous circulation, or impaired orbital venous outflow seem particularly vulnerable.

Atonic pupil after cataract surgery.

We report and describe the clinical findings of three patients who developed atonic pupil after uncomplicated extracapsular cataract extraction with posterior chamber intraocular lens implantation. All patients had normally reactive pupils one day postoperatively, but after two weeks, the pupils were dilated and nonreactive to light, accommodation, and miotics. To estimate the incidence of atonic pupil after cataract surgery, we sent a survey on the frequency, clinical features, and possible etiologies of this syndrome to members of the American Society of Cataract and Refractive Surgery. Of the 567 respondents, 60% had seen at least one case of atonic pupil in the past five years; they reported a total of 1543 cases during that time. This is probably a conservative figure since many cases undoubtedly go unnoticed because of a lack of visual disturbance. We believe this condition occurs more frequently than previously reported. The atonic pupil should be recognized as a possible complication of cataract surgery that should be included in the preoperative consent form.

Magnetic resonance imaging in patients with low-tension glaucoma.

OBJECTIVE: To study diagnoses and anatomic findings found on magnetic resonance imaging in patients with low-tension glaucoma. PATIENTS: We included in this study magnetic resonance images of 20 consecutive patients with low-tension glaucoma. We individually matched each patient with low-tension glaucoma to a control with normal ocular findings who had magnetic resonance imaging for reasons unrelated to the visual pathway. DESIGN: We studied axial and coronal images of the orbit and optic nerve with digitizing software (Image-Pro Plus, Media Cybernetics, Silver Spring, Md). Statistical evaluation was with a Wilcoxon Signed Rank Test for anatomic findings and a McNemar Test for diagnosis. RESULTS: We found no difference between groups in the optic nerve diameter or length, the carotid artery area, or the distance from the optic nerve to the carotid artery (P > .05). Left optic nerve area was greater in the control patients than patients with low-tension glaucoma (P = .026). The prevalence of intracranial abnormalities, including meningioma, aneurysm, and arteriovenous abnormality, was similar between groups (P > .05). However, diffuse cerebral small-vessel ischemic changes were found more in patients with low-tension glaucoma (n = 8) than control patients (n = 1) (P = .0196). CONCLUSIONS: This study proposes a hypothesis that cerebral small-vessel ischemia is more common in patients with low-tension glaucoma and potentially reflects indirectly a vascular cause of the optic nerve head damage at least in a subgroup of patients. Importantly, further research still is required to provide direct evidence for a vascular cause involved in low-tension glaucoma.

Folate-responsive optic neuropathy.

We examined six patients who presented with bilateral, progressive visual loss. Each patient had a bilateral decrease in visual acuity, poor color vision, and central or cecocentral scotomas. Optic nerve head appearance varied from normal to diffusely pale. All patients consumed tobacco, ethanol, or both. Each patient was folate deficient and had normal vitamin B12 levels. Patients were treated with oral folic acid, 1 mg per day. Visual acuity improved bilaterally in all patients (mean: five lines in 2 months). Folate deficiency should be considered in any patient with progressive bilateral optic neuropathy of unknown etiology. Treatment with folic acid can result in significant improvement in visual function.

Ophthalmic manifestations of Rochalimaea species.

Rochalimaea henselae and R. quintana belong to the order Rickettsiales and are thought to be responsible for trench fever, bacillary angiomatosis, and cat scratch disease. We recently examined four patients with intraocular inflammation of unknown origin. Each patient had either unilateral or bilateral moderate loss of visual acuity ranging from 20/25 to counting fingers. Bilateral intraocular inflammation manifested by anterior and posterior segment cells, retinal lesions, macular exudate, and optic nerve head swelling was present to varying degrees. The R. henselae to R. quintana antibody titers were greater than or equal to 1:256 in each case. Marked improvement in vision occurred after treatment with either oral ciprofloxacin hydrochloride and prednisone or doxycycline hyclate. Rochalimaea species should be considered in the differential diagnosis of intraocular inflammation and inflammatory optic neuropathy. Appropriate treatment may result in marked improvement in visual acuity.