Cranial Nerve Palsies in the Setting of Arachnoid Cysts: A Case Series and Literature Review.

BACKGROUND: Arachnoid cysts (ACs) are benign lesions typically believed to not cause neurologic defects in the adult population and are most often found incidentally on imaging. We describe 2 patients with ACs potentially leading to isolated cranial nerve (CN) dysfunction. METHODS: We describe 2 patients, 1 with a fourth nerve palsy and the other with a sixth nerve palsy found to have ACs on MRI brain imaging in locations that potentially caused a compressive CN palsy. We review previous literature of ACs presenting with CN III, IV, or VI palsy. RESULTS: Patient 1 was a 62-year-old man who presented with a 22-year history of diplopia with strabismus examination consistent with a congenital CN IV palsy. Despite multiple surgeries, his CN IV palsy insidiously worsened. An AC in the posterior fossa with mass effect on the quadrigeminal plate and asymmetric atrophy of the right superior oblique was identified on imaging. Patient 2 was an 80-year-old man who presented with an 18-year history of diplopia and was found to have a left esotropia and abduction deficit consistent with complete CN VI palsy. An AC in the left cavernous sinus was identified on imaging. He underwent strabismus surgery with satisfactory resolution of diplopia. We identified a total of 18 previously published cases: 8 reports of CN III palsy, 4 reports of CN IV palsy, and 6 reports of CN VI palsy. Patient ages ranged from 1 to 67 with a median of 34.5. In 16/18 (89%) cases, the diagnosis of ACs was made within 1 year of symptom onset. Surgical removal of the AC was successful in resolving diplopia in 7/12 (58%) cases. In no case was strabismus surgery performed as primary treatment. CONCLUSIONS: Although ACs are typically congenital asymptomatic lesions, we present a case series of 2 patients with ACs in anatomic locations that potentially caused chronic, progressive, isolated CN palsies leading to strabismus. Our literature review revealed that most published cases detailing this clinical scenario resulted in neurosurgical fenestration of ACs with mixed results. Our cases represent 2 patients with AC-associated CN palsy treated with strabismus surgery.

Neuro-ophthalmic manifestations of cancer: a narrative review.

BACKGROUND AND OBJECTIVE: Neuro-ophthalmic manifestations of cancer are vast and early recognition of a serious ocular condition due to either cancer or its therapy is important for both vision preservation as well as providing valuable treatment and prognostic information regarding the underlying malignancy. This review focuses on direct and indirect effects of cancer on the eye and its adnexa, hematologic malignancy, complications of traditional and novel oncologic therapies, and paraneoplastic syndromes as they relate to the eye as these disorders can lead to potentially devastating or irreversible vision loss. METHODS: PubMed was searched primarily for the following topics: optic nerve infiltration, primary vitreoretinal lymphoma (PVRL), ocular paraneoplastic disorders, and ophthalmic complications of cancer therapeutics. Literature was selected based on historical significance and landmark studies (e.g., Cross et al. series of paraneoplastic optic neuritis patients; Chan's textbook on primary intraocular lymphoma) as well as publications published after 2000. References from select studies were additionally included. Given the sparsity of literature on many subjects, most publications were included during this time frame in our review. KEY CONTENT AND FINDINGS: There are several ophthalmic entities that the oncologist should be aware of including leukemic optic nerve infiltration, PVRL, paraneoplastic syndromes as they related to the eye, and adverse effects of therapeutics. Unfortunately, given the rarity of some of these entities [e.g., paraneoplastic optic neuropathy (PON), cancer-associated retinopathy (CAR)], diagnosis can be difficult and treatment options are often limited. CONCLUSIONS: Oncologists can develop a set of basic ophthalmology examination skills that will help to triage and manage patient eye complaints. In certain instances, oncologists have the potential to avert devastating vision loss with early recognition of neuro-ophthalmic complications.

Eye Disease in Medical Practice: What You Should Know and Why You Should Know It.

Incidence of cataract, diabetic retinopathy, macular degeneration, and glaucoma will significantly increase by 2050. Visual impairment can increase morbidity and mortality in nonocular disease. There are different patterns of vision loss in cataract, diabetic retinopathy, age-related macular degeneration, and glaucoma. Internists and medical subspecialists play an important role in prevention, detection, and early treatment of eye disease. Awareness of screening guidelines for eye disease as well as a basic ocular history and simple penlight examination can decrease incidence of vision loss and its impact. Visual impairment places a significant financial burden on society.

Nailfold Capillary Hemorrhages: Microvascular Risk Factors for Primary Open-Angle Glaucoma.

BACKGROUND: Primary open-angle glaucoma (POAG) is associated with systemic microvascular dysfunction including hemorrhages and other abnormalities of the nailfold capillary bed. This study aimed to verify the specificity of nailfold capillary hemorrhages and other abnormalities as risk factors for POAG. METHODS: Nailfold video capillaroscopy was performed using a JH-1004 capillaroscope on the fourth and fifth digits of the nondominant hand in control (n = 277), POAG (n = 206), OHT (n = 57), and SG (n = 29) subjects. The number of hemorrhages, dilated capillaries >50 µm, and avascular zones ≥200 µm were counted and adjusted to counts per 100 capillaries. Descriptive analyses as well as univariate- and multivariable-adjusted logistic regression were performed comparing all groups with controls and POAG with OHT and SG. Subanalyses were conducted in POAG patients examining the association between nailfold capillary outcomes and previous glaucoma surgery, successful IOP control, or disease severity. RESULTS: All nailfold capillary outcomes were significantly increased in POAG, no outcomes were increased in SG, and only hemorrhages were mildly increased in OHT. Hemorrhages were significantly more frequent in POAG compared with both OHT (P < 0.0001) and SG (P=0.001). There were significant trends between higher numbers of hemorrhages and POAG compared with controls, OHT, and SG, with odds ratios of 18.3 (8.5-39.4), 9.1 (1.9-13.4), and 11.8 (1.7-7.3), respectively, for the presence of two or more hemorrhages per 100 capillaries. Hemorrhages were not significantly associated with previous glaucoma surgery, successful postoperative IOP control, or disease severity in POAG. CONCLUSIONS: These findings suggest that systemic microvascular dysfunction is frequent in POAG and occurs early in the disease process. The high specificity of nailfold hemorrhages makes them viable clinical risk factors for POAG.

National Study of Ocular Hospitalizations in Medicare Beneficiaries.

PURPOSE: To study the characteristics of Medicare beneficiaries hospitalized for ophthalmic conditions. DESIGN: Cross-sectional study. METHODS: The 2015 National Medicare 100% Inpatient Limited Dataset was analyzed to identify all patients with either an admitting or primary diagnosis for an ophthalmic condition using ICD-9-CM codes. All other hospitalized Medicare patients served for comparison. Comorbidities were calculated using the Elixhauser Comorbidity Index. Multivariable logistic regression was used to determine odds of primary ophthalmic hospitalization after controlling for patient characteristics and medical comorbidities. RESULTS: For 2015, there were a total of 13 152 Medicare patients with ocular hospitalizations compared to 6 621 005 patients with nonophthalmic events. Most ophthalmic patients were emergent admissions (73.19%) with routine discharges (75.50%) and low rates of inpatient mortality (0.62%). The top admitting diagnoses for nontraumatic and traumatic eye conditions were diplopia (11.69%) and closed fracture of the orbital floor (3.76%), respectively. Patients admitted for eye conditions were more likely to be younger, to be African American, and to have hypertension, valvular heart disease, diabetes, hypothyroidism, AIDS, lymphoma, solid tumor without metastasis, rheumatologic diseases, alcohol and drug abuse, psychoses, and depression compared to the general Medicare inpatient population. CONCLUSIONS: Most inpatient admissions for US Medicare beneficiaries with primary ophthalmic diagnoses were for nontraumatic disorders of the eye and adnexa. Ophthalmic admissions were on average shorter in duration and had lower rates of inpatient mortality compared to nonophthalmic admissions. Patients admitted for eye conditions were more likely to have comorbidities such as hypertension, diabetes, and depression compared to the general Medicare inpatient population.

Orbital ultrasonography in the diagnosis of neoplastic extraocular muscle enlargement.

Neoplastic infiltration of the extraocular muscle (EOM) is a rare condition which can pose a diagnostic dilemma due to its rarity and overlapping ultrasonographic features with orbital myositis. The ultrasonographic features of neoplastic enlargement of EOM have not been systematically studied and previously have been described in only a few case reports. Orbital ultrasonography, in conjunction with the pattern of ocular misalignment, was assessed for its potential role in identifying patients with neoplastic EOM enlargement. Retrospective chart review of patients with neoplasm and myositis. The clinical features of 8 patients with neoplastic infiltration of the EOM were compared to 15 patients with myositis. In the neoplastic group the width of the EOM was (10.5 mm) almost twice the normal width of the muscle with myositis (p < 0.001). All the muscles in the neoplastic category were low to medium reflective. Paretic deviation was seen in 4/8(50%), purely restrictive in 2/8 (25%) and combined pattern in 2/8 (25%) were noted. In the myositis group the average EOM enlargement was 5.8 mm and all muscles showed low reflectivity. Although ultrasonographic features overlapped between the 2 groups paretic deviations were more common in the neoplastic group versus the myositis group (50% versus 7%). Neoplastic muscle enlargement tends to be larger with paretic deviations of ocular motility seen clinically. These findings in a patient with EOM enlargement should raise the suspicion of neoplasm as the etiology and further work up should be considered.

Integrating the Internship into Ophthalmology Residency Programs: Association of University Professors of Ophthalmology American Academy of Ophthalmology White Paper.

Future ophthalmologists will need to have broad skills to thrive in complex health care organizations. However, training for ophthalmologists does not take advantage of all of the postgraduate years (PGYs). Although the traditional residency years seem to have little excess capacity, enhancing the internship year does offer an opportunity to expand the time for ophthalmology training in the same 4 PGYs. Integrating the internship year into residency would allow control of all of the PGYs, allowing our profession to optimize training for ophthalmology. In this white paper, we propose that we could capture an additional 6 months of training time by integrating basic ophthalmology training into the intern year. This would allow 6 additional months to expand training in areas such as quality improvement or time for "mini-fellowships" to allow graduates to develop a deeper set of skills.

A school of red herring.

A 66-year-old man with chronic kidney disease presented with painless unilateral vision loss and bilateral optic disk swelling. Vision loss progressed in the affected eye and developed in the fellow eye. Evaluation for infectious, neoplastic, ischemic, and inflammatory disorders was unrevealing. High-dose corticosteroids appeared to stabilize his vision temporarily. Eventually he was diagnosed with papilledema in the setting of transverse venous sinus stenosis and dural arteriovenous fistula. His papilledema resolved, and his vision improved following stenting of the venous sinus stenosis and embolization of the fistula.

Effectiveness of medial rectus advancement alone or in combination with resection or lateral rectus recession in the management of consecutive exotropia.

PURPOSE: To prospectively determine the long-term success of medial rectus advancement alone or in combination with other procedures in the management of consecutive exotropia. METHODS: All patients with consecutive exotropia who underwent medial rectus advancement alone or in combination with medial rectus resection or lateral rectus recession or both, performed by a single surgeon between 1999 and 2010, were included. Initially, a retrospective review was performed. Patients were then recalled and examined by a masked observer. Good results were defined as final alignment within 10(Δ) of orthotropia. RESULTS: A total of 46 patients were enrolled in the study with a mean age of 43 ± 15.5 years (range, 14-76) and a mean exotropia of 32 ± 18(Δ) (range, 10-90). Good results were achieved in 33 patients (72%) at a mean follow-up time of 2.5 years. Of the 21 patients returning for prospective examination, 15 (71%) had good alignment at an average follow-up of 4.2 years (range, 6.9 months to 8.6 years). Adduction deficits were improved in 30 of 33 (91%) patients at final follow-up. CONCLUSIONS: In patients with consecutive exotropia, surgery including medial rectus advancement alone or in combination with resection or lateral rectus recession or both usually is effective.

Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes: a prospective study.

PURPOSE: To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes. DESIGN: Prospective, multicenter, observational case series. PARTICIPANTS: A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy. TESTING: Magnetic resonance imaging (MRI) of the brain. MAIN OUTCOME MEASURES: Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment. RESULTS: Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7-24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause (P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64). CONCLUSIONS: In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present.

Optic nerve biopsy in the management of progressive optic neuropathy.

BACKGROUND: In cases of progressive optic neuropathy, diagnostic uncertainty often persists despite extensive work-up. Optic nerve biopsy (ONB) can be considered, especially when visual decline of the affected or fellow eye ensues despite empiric therapy. We aimed to evaluate both diagnostic and therapeutic utilities of ONB based on the long-term experience at a tertiary care institution. METHODS: This was a retrospective chart review of biopsies over 20 years at a single institution involving intrinsic or adherent optic nerve masses. Main outcome measures included the impact of tissue sampling on reaching a diagnosis and on guiding treatment. Secondary measures included vision in the eye of the ONB and the fellow eye. RESULTS: Fifteen patients with a mean age of 51.7 ± 17.4 years underwent biopsies. At the time of biopsy, visual acuity was no light perception in 8 (53%) eyes, light perception to counting fingers in 5 (33%), and 20/400 or better in 2 (13%). The fellow eye of 7 patients (47%) experienced some degree of sequential vision loss before biopsy. Seven specimens included en bloc biopsy of the nerve, 7 contained the dural sheath (usually with a portion of the optic nerve), and 1 only of the compressive mass. Six patients (40%) had tumors. Six of 8 inflammatory lesions biopsied required further clinical data to arrive at specific diagnoses. In one case, a clinical diagnosis could not be made. No patients experienced further vision loss in the fellow eye at last follow-up (median, 8 months). CONCLUSIONS: In diverse circumstances of progressive optic neuropathy, ONB can be beneficial in establishing the diagnosis. ONB can help direct specific local or systemic treatment, particularly when infectious or inflammatory etiologies are identified. ONB, if considered early in the disease course, can potentially halt or prevent vision loss when the fellow eye is threatened.

The diagnostic and economic yield of neuroimaging in neuro-ophthalmology.

BACKGROUND: Diagnostic studies such as computed tomography scans (CT) and magnetic resonance imaging (MRI) are ordered frequently in neuro-ophthalmic practice, although the diagnostic yield and cost-effectiveness of these tests have been studied for only a few conditions. We assessed the diagnostic and economic yield of CT and MRI across all patients evaluated in a neuro-ophthalmology practice. METHODS: This retrospective review included all patients referred by the division of neuro-ophthalmology at the Scheie Eye Institute for CT, CT angiography, MRI, MRA, or magnetic resonance venography over a 12-month period. Abnormal imaging findings were categorized as significant (one that elicited changes in management) and/or relevant (one that related to the patient's neuro-ophthalmic complaint or examination findings). The diagnostic yield of the test ordered was analyzed according to the patient's chief complaint, neuro-ophthalmic examination findings, and indication for imaging. The total costs for each diagnostic group and costs per significant finding were calculated using the global Resource-Based Relative Value Units for each examination from the Centers for Medicare and Medicaid Services Web site. RESULTS: Two hundred eleven imaging studies in 157 patients were evaluated. 28.9% (95% confidence interval, 22.5%-36.2%) of imaging studies had significant abnormalities relevant to the neuro-ophthalmic complaint. Imaging obtained for evaluation of progressive optic nerve dysfunction and cranial nerve palsy had statistically significant higher diagnostic yield than studies performed for other reasons. Total cost of all imaging studies performed was $107,615.72. Cost per clinically significant and relevant finding was $1,764.19. CONCLUSIONS: In comparison to the diagnostic yield of neuroimaging studies in other specialties, CT and MRI of the brain requested by neuro-ophthalmologists provide significant and relevant data at a reasonable cost.

Prisms are effective in resolving diplopia from incomitant, large, and combined strabismus.

PURPOSE: To assess the effectiveness of prisms in the management of diplopia in patients with incomitant, large, and combined strabismus. MATERIALS AND METHODS: Data were collected retrospectively from the medical records of 64 patients who were prescribed prisms for symptomatic diplopia due to restrictive strabismus. The main outcome measure was resolution of diplopia (diplopia score 1 or 2). Data on the number of patients who continued wearing prisms versus those who opted for surgery were derived based on follow-up records. RESULTS: Among 64 patients, 72% of patients reported complete or partial resolution of diplopia after prism use. In patients who were prescribed =10 PD (n=18) and those with oblique prisms (n=18), 67% and 50%, respectively, reported resolution of diplopia. More patients with vertical prism reported improvement in double vision symptoms (83%) than those who were prescribed horizontal prism (70%, p=0.04). During follow-up, 73% of the cohort continued using prisms while 23% of patients opted for strabismus surgery. CONCLUSIONS: Prisms are an effective modality and should be considered in the initial or interim management of patients with diplopia from complex ocular misalignments.

Effectiveness of prisms in the management of diplopia in patients due to diverse etiologies.

PURPOSE: To study the effectiveness of prisms in the management of diplopia from different etiologies and over a broad range of ocular misalignment. METHODS: In this retrospective cohort study, resolution of diplopia in 94 patients who were prescribed prisms was studied. RESULTS: Among 94 patients, 88% reported complete or partial resolution of double vision (95% confidence interval: 84.1% to 95.6%) with highest improvement noted in the divergence insufficiency and skew deviation group (100%) compared to 64% improvement noted in patients with convergence insufficiency. More than 85% of patients who were prescribed greater than 10 diopters of prism and those with oblique prism prescriptions also reported resolution of diplopia. Eighty-nine percent of the cohort continued with prism use and 11% opted for strabismus surgery. CONCLUSION: In this study, prisms were effective in alleviation of diplopia over a broad range and for different etiologies of double vision.

Success of prisms in the management of diplopia due to fourth nerve palsy.

BACKGROUND: To analyze the success of prism use in alleviating diplopia in patients with fourth nerve palsy and to provide recommendations for prism prescription. METHODS: In this retrospective cohort study, the medical records of 83 patients who were prescribed prisms for symptomatic diplopia due to fourth nerve palsy were analyzed. Data on the nature and duration of diplopia, motility and alignment findings, and amount and type of prism prescribed were recorded. The success of prescribed prismatic correction was assessed by the patient's self-reporting of satisfaction with prism use and follow-up records. The main outcome measure was the satisfaction rate associated with the use of prisms (satisfaction score 1 or 2) in patients with fourth nerve palsy. RESULTS: There were 69 patients with congenital fourth nerve palsy and 14 patients with acquired fourth nerve palsy who received prisms. The mean primary position (± SD) deviation in this group was 7.8 (± 4.6) prism diopters (PD). The mean prism prescription was 6 (± 2.9) PD. Overall, 92% of patients were satisfied with the use of prisms. During the length of follow-up, which ranged from 2 months to more than 6 years (median: 18 months), 86% of the cohort continued using prisms while 14% of patients underwent strabismus surgery. Among 15 patients who had primary position deviation greater than 15 PD, 80% of the patients reported satisfaction with prisms, and in the 11 patients who received 10 PD or more of prismatic correction, 82% were satisfied. CONCLUSION: Prisms are an effective modality for the management of patients with symptomatic diplopia due to fourth nerve palsy. Even in patients with larger deviations including those who were prescribed greater than 10 PD of correction, the success rate of prisms in alleviating diplopia was high. Prisms should be considered as initial therapy in symptomatic patients with fourth nerve palsy.