Larry P. Frohman

CT of orbital trauma

In a patient with acute orbital trauma, visual acuity and extraocular muscle motility are the two most important ophthalmologic functions to be evaluated emergently. The assessment of these capabilities may sometimes be difficult due to the severity of the head injury, the extent of periorbital soft tissue edema, inadequate cooperation in alert patients, and a reduced level of consciousness in obtunded individuals. Consequently, computed tomography (CT) has come to play a major role in the orbital examination of acute trauma patients. In this study, in conjunction with clinical evaluation, we have sought to utilize CT to determine the various prevalences of the causes of decreased visual acuity and extraocular muscle motility resulting from orbital trauma. We retrospectively reviewed the records of all patients admitted to our emergency facility who, having suffered head trauma, underwent a CT study for diagnosis. CT examinations of the head using a multidetector scanner were performed from the base of the skull to the vertex at 5-mm intervals. Orbital CT was obtained when a routine CT of the head showed periorbital soft tissue edema and/or facial bone fractures. The orbital CT examination was performed using axial 1-mm and coronal 3-mm slices. Coronal reformation images were prepared if the patient was unable to tolerate the prone position for direct coronal imaging. The imaging findings were correlated with ophthalmologic observations. The orbit floor was the most common and the orbital roof the least common site of fracture of the bony coverings of the eye. Twenty-three patients suffered decreased visual acuity. In order of declining frequency, the causes of reduced vision consequent to trauma were retrobulbar hemorrhage, optic nerve thickening presumably secondary to edema, intraorbital emphysema, optic nerve impingement, detached retina and ruptured globe. Five patients had visual impairment without demonstratable radiographic abnormalities. The most common finding associated with diminished extraocular muscle motility was muscle impingement by fracture fragments, followed in decreasing frequency by thickened muscle due to edema or contusion, intraconal emphysema, muscle entrapment, and retrobulbar fat herniation. Six patients with decreased extraocular muscle activity had no abnormalities demonstrated on CT images. The overwhelming majority of patients with decreased visual acuity or reduced extraocular muscle motility consequent to trauma had abnormalities demonstrated by orbital CT. Hence, CT examinations should play a major role in the evaluation of the intraorbital contents in patients with orbital trauma.

Aspirin reduces the incidence of second eye NAION: a retrospective study.

The objective of this study was to determine if aspirin reduces the incidence of second eye involvement after nonarteritic anterior ischemic optic neuropathy (NAION) in one eye. Records were reviewed of 131 patients who sustained unilateral NAION. Of these, the 33 patients who sustained second eye NAION were compared to those followed for a minimum of 2 years without sustaining a second eye NAION (67). Thirty-one of the 131 patients were excluded because of inadequate follow-up. Except for diabetes (relative risk [RR] 1.43, p = 0.05), the incidence of second eye NAION was independent of gender, age, cup/disk, hypertension, anemia, and migraine. The degree of visual acuity or field dysfunction in the first eye correlated poorly with the acuity (r = 0.28) and field (r =0.33) loss in the second eye. Aspirin (65–1,300 mg) taken two or more times per week decreased the incidence (17.5% vs. 53.5%) and relative risk (RR = 0.44, p = 0.0002) of second eye AION regardless of the usual risk factors. Even after eliminating those patients who had bilateral disease when first referred, ASA still reduced the incidence of second eye involvement (35% vs. 13%, RR = 0.74, p = 0.01). Aspirin may be an effective means of reducing second eye NAION.

Adalimumab-associated optic neuritis

We present, to our knowledge, the first published cases of optic neuritis associated with adalimumab, a medication in the class of anti-tumor necrosis factor-alpha (TNF-alpha) antagonists. Approved in recent years by the FDA, adalimumab (Humira, Abbott Laboratories; Abbott Park, IL) is a recombinant monoclonal antibody that targets and blocks the physiologic effects of TNF. Other TNF antagonists have had associations with optic neuritis and demyelinating events.

Sarcoidosis of the anterior visual pathway: 24 new cases.

Objectives To describe the clinical spectrum and a rational approach to the diagnosis of anterior visual pathway sarcoidosis. Methods Retrospective chart review of all patients examined in neuro-ophthalmic consultation by 1 author from 1989 to 1998 with a diagnosis of sarcoidosis. Results There were 24 patients (17 female, 7 male, mean age 40 years) with anterior visual pathway sarcoidosis, 17 (71%) of whom were not previously known to have sarcoidosis. Visual acuity ranged from 20/20 to NLP. Normal fundi were observed in 15%. Among the 85% who had fundus abnormalities, pallor was present in 55%, disc edema in 26%, periphlebitis/sheathing in 14%, and optic disc granuloma in 10%. Ten patients (42%) had uveitis, active in only 3 (13%). An elevated angiotensin-converting enzyme (ACE) was present in 16 (76%) of 21 patients tested; evidence of sarcoidosis on chest radiograph was present in 13 (72%) of 18; gallium scanning was abnormal in 13 (93%) of 14; neuroimaging abnormalities of the optic nerves, chiasm, or tract were present in 16 (70%) of 23; lymphocytic pleocytosis or elevated cerebrospinal fluid protein was identified in 14 (88%) of 16 patients, with both values elevated in 7 (44%) patients. Histologic confirmation was obtained in 13 (81%) of 16 who underwent biopsy; in the remaining patients, diagnosis was based entirely on clinical and laboratory evidence. Conclusions Anterior visual pathway disease may be underrecognized as a presentation of sarcoidosis. Classic fundus findings of periphlebitis and optic granuloma are typically absent. An aggressive diagnostic evaluation may help establish the diagnosis early in its course.

Management of traumatic optic neuropathy--a study of 23 patients.

Twenty three patients with traumatic optic neuropathy were managed by medical and surgical treatment as follows. High dose intravenous steroids were initiated in all patients. If visions did not improve significantly after 24 to 48 hours decompression of an optic nerve sheath haematoma by medial orbitotomy and neurosurgical decompression of the optic canal were considered based on computed tomographic scan findings. Nine of 16 patients who received steroids only showed significant improvement. One of three showed improvement on optic nerve decompression after steroid failure; three or four showed improvement on optic nerve decompression after steroid failure; three or four showed improvement with combined optic nerve sheath decompression by the medial orbitotomy and decompression of the optic canal by frontal craniotomy. A lucid interval of vision after injury and an enlarged optic nerve sheath were associated with an improved prognosis. Five of the 23 patients had a lucid interval and all five had a final improved vision, while only five of 18 patients without a lucid interval improved. Similarly seven of the nine with an enlarged optic nerve sheath showed improvement while only three of 10 patients (three bilateral cases) who presented with no light perception improved with medical and surgical treatment. While a prospective controlled study of the management of traumatic optic neuropathy is necessary this preliminary study suggests that treatment of traumatic optic nerve sheath haematoma by optic nerve sheath decompression should be considered in selected patients.

Management of Visual Loss after Optic Nerve Sheath Decompression in Patients with Pseudotumor Cerebri

Purpose: To determine the appropriate management of patients with pseudotumor Cerebri with early, progressive visual loss after optic nerve sheath decompression (ONSD). Methods: The records of all patients with pseudotumor Cerebri who underwent ONSD were reviewed retrospectively. Patients who showed visual loss within 1 month of surgery were studied. Results: Five patients with pseudotumor Cerebri, including two with renal failure and hypertension, had visual loss within 1 month of ONSD. The first patient had an abrupt decrease in vision 6 days after ONSD. In this patient, a vessel on the nerve sheath bled into the surgical site. At the time of ONSD, this patient had a visual acuity of 20/20 1 day after surgery. Six days later, visual acuity decreased to 20/200. After high-dose intravenous corticosteroids failed to improve vision, emergency lumboperitoneal shunt resulted in full visual recovery. An apparent infectious optic neuropathy developed in the second patient 3 days after surgery. After 72 hours of intravenous antibiotics, visual acuity improved from 20/600 to 20/15. The other three patients had gradual visual loss after ONSD, which stabilized after lumboperitoneal shunts. Conclusions: Avoidance of bleeding during ONSD may prevent fibrous occlusion of the surgical site. Patients with no identifiable cause for visual loss after ONSD, who do not respond to intravenous corticosteroids, should be evaluated for emergency lumboperitoneal shunting. Postoperative infectious optic neuropathy should be considered in the differential diagnosis of abrupt visual loss after ONSD.

Resolution of papilledema after neurosurgical decompression for primary Chiari I malformation.

PURPOSE To report a causal relationship between Chiari I malformation and its rare, but recognized manifestation of bilateral papilledema. DESIGN Interventional case series. METHODS Four adult female patients (mean age, 48, age range 25-59 years) with bilateral papilledema, signs and symptoms of increased intracranial pressure, and cranial magnetic resonance imaging (MRI) evidence of a Chiari I malformation ranging from 7 to 22 mm of tonsillar herniation underwent suboccipital decompression. RESULTS In all four patients, suboccipital decompression was followed by resolution of bilateral papilledema and signs and symptoms of increased intracranial pressure. CONCLUSION Patients with bilateral papilledema and presumed pseudotumor cerebri require a cranial MRI to determine if they have a Chiari I malformation, because patients with increased intracranial pressure and papilledema from a Chiari I malformation may benefit from suboccipital decompression.

Long-Term Results: Botulinum for Sixth Nerve Palsy

Eight patients with intracranial malignancies or vascular lesions and sixth nerve palsies were treated with botulinum toxin chemodenervation of the antagonist medial rectus muscle. Primary deviation ranged from 20 to 75 prism diopters (pd) of esotropia. Six were treated acutely (within 3 months of onset) and two, which demonstrated partial recovery of lateral rectus function but with residual esotropia and diplopia, were treated after 6 months. After a mean follow-up of 20.6 months, seven were diplopia-free with excellent rotations. Five had complete resolution of the esotropia and diplopia, with near complete recovery of abduction. One had 6 pd residual esotropia, while another, whose sixth nerve had been resected, required a modified Jensen procedure, resulting in full rotations. The single case of bilateral sixth nerve palsy had a functional improvement but was lost to follow-up. One patient had a vertical strabismus induced with the injection and had a gradual return of the esotropia.

Reversible blindness resulting from optic chiasmitis secondary to systemic lupus erythematosus

Objective To report the diagnosis, radiologic findings, and therapy of a 51-year-old female with systemic lupus erythematosus (SLE) who, while on hydroxychloroquine maintenance therapy, presented with a junctional scotoma indicative of chiasmal disease. This visual loss developed after she had been tapered off corticosteroids. Materials and Methods An interventional case report of a female that was given acute therapy with 1-gram daily of intravenous methylprednisolone sodium succinate for 5 days, followed by maintenance methotrexate and a slow taper of oral prednisone. Magnetic resonance imaging (MRI) scans, visual acuity, color vision, and threshold visual fields were performed. Results The MRI scan showed chiasmal involvement, which may occur in SLE in absence of any other evidence of systemic activity. Therapy led to visual function returning to 20/20 OD and 20/20 OS, with normal Ishihara plates OU and only minimal paracentral depressions OU. She has been able to be weaned off prednisone while on methotrexate maintenance. Conclusions Chiasmal involvement may occur in SLE in absence of any other evidence of systemic activity. Maintenance with hydroxychloroquine may not be adequate to prevent this rare cause of visual loss in SLE. Aggressive therapy of chiasmal involvement in SLE, even when the visual loss is profound, may lead to visual restoration, which was virtually complete in this case. Methotrexate may be an alternate agent for patients who break through with optic neuropathy while on hydroxychloroquine.

Neuro-ophthalmic manifestations of sarcoidosis : Clinical spectrum, evaluation, and management

Objective To familiarize the reader with the neuro-ophthalmic manifestations of sarcoidosis. Materials and Methods All patients underwent systemic evaluations (chest radiograph, magnetic resonance imaging and/or computed tomography, serum angiotensin-converting enzyme level, and gallium scan). Histologic confirmation was preferred (11 of 15 patients underwent biopsy, ten of whom [82%] had positive biopsies, and four refused). Otherwise, the diagnosis of clinical sarcoidosis was based on laboratory evaluation. Results We report our experience with 15 patients who had neuro-ophthalmic manifestations of sarcoidosis other than optic neuropathy or chiasmal disease. Eight of 15 (53%) did not have known sarcoidosis at the time of presentation. Thirteen of 15 (87%) patients demonstrated lesions consistent with sarcoidosis on magnetic resonance imaging of the brain. Treatment with corticosteroids and/or other immunomodulatory agents was necessary in all cases. Conclusions Neuro-ophthalmic manifestations of sarcoidosis are rare. They may be the presenting signs of otherwise occult disease. Suspicion and inclusion in the differential are a key to establishing the diagnosis. A strategy for the detection and evaluation of these cases is presented.