Interdisciplinary Protocol for the Management of Vision-Threatening Papilledema.

BACKGROUND: This study evaluates the effectiveness of a multidisciplinary protocol for management of patients with papilledema and vision loss secondary to increased intracranial pressure. METHODS: Retrospective record review of all adult patients who presented with vision-threatening papilledema (VTPE) and were treated under this protocol. Patients are admitted for lumbar drain placement and diuretics and followed daily to determine if they may be managed medically or require surgery (optic nerve sheath fenestration [ONSF] and/or cerebrospinal fluid [CSF] shunting). RESULTS: Nineteen patients were included. Twelve had body mass index in the obese range and 6 were morbidly obese. Fourteen had idiopathic intracranial hypertension. Five had secondary pseudotumor cerebri syndrome related to medication use, dural venous sinus thrombosis, hypothyroidism, end-stage renal disease, pulmonary disease, and diastolic heart failure. Three patients did not require surgery and were discharged on oral diuretics; 3 patients underwent unilateral ONSF, 9 underwent bilateral ONSF, and 4 underwent bilateral ONSF followed by ventriculoperitoneal shunt placement. The average follow-up was 10.1 months. The visual acuity improved bilaterally in 12 patients and unilaterally in 4 patients. The remaining 3 patients had worsened vision in both eyes. Fifteen patients had bilateral improvement in their visual fields. Five eyes in 3 patients showed further constriction of the visual field at follow-up. CONCLUSIONS: We demonstrate how a multidisciplinary complex care protocol for treating VTPE can expedite and streamline treatment and restore vision. We found that most patients had improved symptoms and signs, including visual acuity, visual fields, and papilledema. We encourage institutions that manage VTPE to adopt similar institutional protocols.

Increased Incidence of Pseudotumor Cerebri Syndrome Among Users of Tetracycline Antibiotics.

BACKGROUND: To determine whether the use of a tetracycline-class antibiotic is associated with an increased risk of developing pseudotumor cerebri syndrome (PTCS). METHODS: We identified patients in the University of Utah Health system who were prescribed a tetracycline-class antibiotic and determined what percentage of those individuals were subsequently diagnosed with PTCS secondary to tetracycline use. We compared this calculation to the number of patients with PTCS unrelated to tetracycline use. RESULTS: Between 2007 and 2014, a total of 960 patients in the University system between the ages of 12 and 50 were prescribed a tetracycline antibiotic. Among those, 45 were diagnosed with tetracycline-induced PTCS. We estimate the incidence of tetracycline-induced PTCS to be 63.9 per 100,000 person-years. By comparison, the incidence of idiopathic intracranial hypertension (IIH) is estimated to be less than one per 100,000 person-years (Calculated Risk Ratio = 178). CONCLUSIONS: Although a causative link between tetracycline use and pseudotumor cerebri has yet to be firmly established, our study suggests that the incidence of pseudotumor cerebri among tetracycline users is significantly higher than the incidence of IIH in the general population.

Multiple Cranial Neuropathies and Pachymeningitis in a Patient With a Pathogenic Nucleotide-Binding Oligomerization Domain 2 Polymorphism.

ABSTRACT: An 11-year-old boy presented with 2 weeks of intermittent headache, right orbital pain, and constant diplopia. Brain MRI showed dural thickening and enhancement of the right lateral cavernous sinus, right orbital apex, and tentorium. Initial cerebral spinal fluid analysis showed only mild pleocytosis, and serum diagnostics were unrevealing. The working diagnosis was Tolosa-Hunt syndrome. His pain and sixth nerve palsy resolved with corticosteroids. Five months after initial presentation, he developed new numbness of the right cheek, complete right ophthalmoplegia, and weakness and numbness of his right hand and leg, all of which were responsive to steroids. Fifteen months later, he returned to the emergency department with 2 weeks of left-sided headaches and acute diplopia. On examination, he had a left cranial nerve 6 palsy. Dural biopsy showed diffuse mononuclear inflammatory cell reaction consisting mostly of lymphocytes with no signs of granuloma formation, nor any epithelioid or giant cells. His clinical course was consistent with an autoinflammatory condition of unknown etiology. Genetic testing with an immunodeficiency panel showed a risk allele in NOD2 (nucleotide-binding oligomerization domain 2) c.3019dup (p.Leu1007Prof*2) that is associated with an increased risk for Crohn disease. His clinical condition had similarities to central nervous system sarcoidosis. Because of the similarities between our patient's clinical, imaging, and genetic findings and neurosarcoidosis, he was switched to a more targeted therapy-infliximab. His condition has since been stable for nearly 2 years. In conclusion, genetic testing should be considered in patients with suspected occult autoimmunity.

Adjunctive techniques to dacryocystorhinostomy: an evidence-based review with recommendations.

BACKGROUND: Many adjunctive techniques exist for dacryocystorhinostomy (DCR), with varying levels of supportive evidence. METHODS: Literature from PubMed, EMBASE, and Cochrane Databases was reviewed between January 1990 and January 2020 to examine evidence regarding the utility of adjunctive techniques to DCR, including mucosal flap preservation, concurrent septoplasty, stenting, topical mitomycin C (MMC), and 5-fluorouracil (5-FU), as well as perioperative antibiotics and steroids. Recommendations were made based on the evidence found. RESULTS: Seven adjunctive techniques used in DCR were examined. The literature supported concurrent septoplasty when septal deviation is present. Silicone stents are recommended for external DCR approaches. MMC use is optional in external DCR and revision cases. The literature does not support the routine use of silicone stents or MMC in primary endoscopic DCR. Mucosal flap preservation is optional, with evidence showing comparable results with or without utilization. The level of evidence is limited for topical 5-FU, as well as perioperative antibiotic and steroid use. CONCLUSION: Recommendations for adjunctive techniques to external and endoscopic DCR surgeries can be made based on the current literature. Higher-level studies are needed to better optimize perioperative approaches to DCR.

A Comparison Between the Clinical Features of Pseudotumor Cerebri Secondary to Tetracyclines and Idiopathic Intracranial Hypertension.

PURPOSE: Comparisons between clinical features of tetracycline-induced pseudotumor cerebri (PTC-T) and those of idiopathic intracranial hypertension (IIH) are absent in the literature. We hypothesized that significant clinical differences between these etiologies exist and could be better understood by retrospective analysis. DESIGN: Retrospective cohort study. METHODS: We reviewed patients diagnosed with pseudotumor cerebri syndrome (PTCS) at our center and identified those who developed PTC-T after treatment with a tetracycline-class antibiotic and those with IIH. Groups were compared by demographics, body mass index, ophthalmic examination, treatment, clinical course, and visual outcomes. RESULTS: We identified 52 cases of PTC-T and 302 cases of IIH. Obesity rates were significantly different (43.8% for PTC-T vs 79.2% for IIH, P < .001). The mean age at diagnosis was younger for PTC-T (19.8 years vs 28.1 years for IIH, P < .001). Diplopia was more common with PTC-T (40.4% vs 20.1% for IIH, P = .001). The mean illness duration was shorter for PTC-T (18.3 weeks vs 62.9 weeks for IIH, P <.0001). Recurrence rates were significantly different (4.0% for PTC-T vs 16.5% for IIH, P <.001). The frequency of surgical intervention was similar. Vision loss was uncommon but occurred with similar frequency. CONCLUSION: We identified significant clinical differences but also identified important similarities between the 2 groups. There appear to be nonobese patients who develop PTC-T, discontinue the antibiotic, and never develop PTCS again. There are other patients who develop PTC-T, discontinue the antibiotic, and later develop IIH. We conclude that PTC-T represents a spectrum of disease in susceptible individuals.

Young Adults With Anterior Ischemic Optic Neuropathy: A Multicenter Optic Disc Drusen Study.

PURPOSE: Optic disc drusen (ODD), present in 2% of the general population, have occasionally been reported in patients with nonarteritic anterior ischemic optic neuropathy (NA-AION). The purpose of this study was to examine the prevalence of ODD in young patients with NA-AION. DESIGN: Retrospective, cross-sectional multicenter study. METHODS: All patients with NA-AION 50 years old or younger, seen in neuro-ophthalmology clinics of the international ODDS (Optic Disc Drusen Studies) Consortium between April 1, 2017, and March 31, 2019, were identified. Patients were included if ODD were diagnosed by any method, or if ODD were excluded by enhanced-depth imaging optical coherence tomography (EDI-OCT) using ODDS Consortium guidelines. NA-AION eyes with ODD were termed "ODD-AION"; those without were termed "NODD-AION". RESULTS: A total of 65 patients (127 eyes) with NA-AION were included (mean 41 years old). Of the 74 eyes with NA-AION, 51% had ODD-AION, whereas 43% of fellow eyes without NA-AION had ODD (P = .36). No significant differences were found between ODD-AION and NODD-AION eyes in terms of Snellen best-corrected VA or perimetric mean deviation. According to EDI-OCT results, 28% of eyes with NODD-AION had peripapillary hyperreflective ovoid mass-like structures (PHOMS); 7% had hyperreflective lines, whereas 54% with ODD-AION had PHOMS; and 66% had hyperreflective lines (P = .006 and P < .001, respectively). CONCLUSIONS: Most of these young NA-AION patients had ODD. This indicates that ODD may be an independent risk factor for the development of NA-AION, at least in younger patients. This study suggests ODD-AION be recognized as a novel diagnosis.

Multirater Validation of Peripapillary Hyperreflective Ovoid Mass-like Structures (PHOMS).

Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a new retinal optical coherence tomography (OCT) finding. The Optic Disc Drusen Studies Consortium had made recommendations to distinguish PHOMS from true optic disc drusen (ODD) in 2018. While publications on PHOMS have increased since then, the accuracy of the definition of PHOMS and reliability of detection is unknown. In this multi-rater study, we demonstrate that the 2018 definition of PHOMS resulted in a poor multi-rater kappa of 0.356. We performed a Delphi consensus process to develop a consistent and refined definition of PHOMS with clear principles around the nature of PHOMS and how they differ from normal anatomy. Fifty explanatory teaching slides, provided as supplementary material, allowed our expert group of raters to achieve a good level of agreement (kappa 0.701, 50 OCT scans, 21 raters). We recommend adopting the refined definition for PHOMS.

Patients With Migraine Have Substantial Reductions in Measures of Visual Quality of Life.

OBJECTIVE: Migraine is associated with several important visual symptoms, during both acute attacks and headache-free intervals. The purpose of this investigation was to use validated vision-related quality of life instruments to assess the effect of migraine on visual quality of life. BACKGROUND: Many migraineurs experience visual aura, increased photophobia during and between headache attacks, and increased symptoms of dry eye with structural changes in corneal nerve endings. Other visual symptoms associated with migraine include positive persistent visual phenomenon (visual snow) and transient vision changes. Previous research looking at the disability associated with migraine has shown that patient-reported quality of life data can be useful in determining the severity of disease burden. Recent published literature has suggested that visual symptoms related to migraine represent a proportionally minor burden to patients, compared to other manifestations of migraine, but no previous studies have assessed how migraine affects visual quality of life. METHODS: In this cross-sectional quantitative survey, visual quality of life in individuals with chronic and episodic migraine was assessed using the National Eye Institute Visual Function Questionnaire-25, and the 10-item National Eye Institute Visual Function Questionnaire-25 Neuro-Ophthalmic Supplement. Overall headache severity and impact was assessed using the Migraine-specific Quality of Life Questionnaire (Version 2.1) and the Headache Impact Test-6. Participants were recruited from Headache and Neuro-ophthalmology subspecialty clinics. The target sample size was 30 participants per subgroup. The results were compared to those from disease-free controls and to results from other neuro-ophthalmic disease quality of life studies. RESULTS: Among 29 participants with chronic migraine, vision-specific quality of life scores were all statistically significantly decreased compared to disease-free controls. The National Eye Institute Visual Function Questionnaire-25 median composite score was 85 for chronic migraineurs and 96 for controls (P < .001). The 10-item National Eye Institute Visual Function Questionnaire-25 Neuro-Ophthalmic Supplement median score was 72 for chronic migraineurs and 95 for controls (P < .001). Among 37 participants with episodic migraine, vision-specific quality of life scores were also decreased compared to disease-free controls. In the episodic migraine group, decreases in the National Eye Institute Visual Function Questionnaire-25 scores were not statistically significant (median score 91, P = .01 compared to the control group), but decreases in the 10-item National Eye Institute Visual Function Questionnaire-25 Neuro-Ophthalmic Supplement remained statistically significant (median score 85, P = .003 compared to the control group). Chronic migraineurs had decreased visual quality of life scores compared to those with episodic migraines. Participants with chronic migraine had visual quality of life scores that were as poor as those previously published for patients with other neuro-ophthalmic disorders such as multiple sclerosis, myasthenia gravis, and ischemic optic neuropathy. CONCLUSIONS: Visual quality of life is significantly adversely affected in migraine sufferers. In fact, patients with chronic migraine may have visual quality of life impacts that are as significant as those associated with other common neuro-ophthalmic disorders. Future studies of the overall disease burden in patients with migraine should include an evaluation of the effects on visual functioning.

The Most Common Causes of Eye Pain at 2 Tertiary Ophthalmology and Neurology Clinics.

BACKGROUND: Eye pain is a common complaint, but no previous studies have determined the most common causes of this presenting symptom. Our objective was to determine the most common causes of eye pain in 2 ophthalmology and neurology departments at academic medical centers. METHODS: This was a retrospective cross-sectional analysis and chart review at the departments of ophthalmology and neurology at the University Hospital Zurich (USZ), University of Zürich, Switzerland, and the University of Utah (UU), USA. Data were analyzed from January 2012 to December 2013. We included patients aged 18 years or older presenting with eye pain as a major complaint. RESULTS: Two thousand six hundred three patient charts met inclusion criteria; 742 were included from USZ and 1,861 were included from UU. Of these, 2,407 had been seen in an ophthalmology clinic and 196 had been seen in a neurology clinic. Inflammatory eye disease (conjunctivitis, blepharitis, keratitis, uveitis, dry eye, chalazion, and scleritis) was the underlying cause of eye pain in 1,801 (69.1%) of all patients analyzed. Although only 71 (3%) of 2,407 patients had migraine diagnosed in an ophthalmology clinic as the cause of eye pain, migraine was the predominant cause of eye pain in the neurology clinics (100/196; 51%). Other causes of eye pain in the neurology clinics included optic neuritis (44 patients), trigeminal neuralgia, and other cranial nerve disorders (8 patients). CONCLUSIONS: Eye pain may be associated with a number of different causes, some benign and others sight- or life-threatening. Because patients with eye pain may present to either a neurology or an ophthalmology clinic and because the causes of eye pain may be primarily ophthalmic or neurologic, the diagnosis and management of these patients often requires collaboration and consultation between the 2 specialties.

Shingles as the underlying cause of orbital myositis in an adolescent: A case report.

PURPOSE: Orbital myositis is characterized by pain with eye movements, gaze restriction, diplopia, and enlargement of extraocular muscles on imaging. Varicella zoster virus (VZV) is an extremely rare cause of the disease in the elderly and has never been reported in a patient younger than forty-five years old such as the adolescent described herein. We present this case to raise awareness of an entity that will likely become more prevalent due to current vaccine strategies. OBSERVATION: We present the case of a 13-year-old girl with VZV-associated orbital myositis and meningitis that had a quick and complete recovery following IV acyclovir and oral steroids. CONCLUSIONS AND IMPORTANCE: In conclusion, orbital myositis is an extremely rare complication of facial VZV infections. Our case highlights the importance of prompt detection and treatment in the pediatric population.

An Estimation of the Risk of Pseudotumor Cerebri among Users of the Levonorgestrel Intrauterine Device.

Because of a previous association of pseudotumor cerebri (PTC) with levonorgestrel, we wished to evaluate the use of levonorgestrel-eluting intrauterine devices ("levonorgestrel intrauterine systems", LNG-IUS) in our University of Utah and Rigshospitalet PTC patients. In our retrospective series, PTC prevalence was approximately 0.18% and 0.15% in the LNG-IUS population versus 0.02% and 0.04% in the non-LNG-IUS population (Utah and Rigshospitalet, respectively), with no significant differences in PTC signs and symptoms among the two groups. Our investigation suggests that women with an LNG-IUS may have increased risk of developing PTC but does not suggest an LNG-IUS can cause PTC.

Simbu Ptosis: An Outreach Approach to Myogenic Ptosis in Eastern Highlands of Papua New Guinea-Experience and Results From a High-Volume Oculoplastic Surgical Camp.

PURPOSE: To present the results of a high-volume oculoplastic surgical outreach in a remote region of Simbu Province in the Eastern Highlands of Papua New Guinea. The authors describe the clinical features and evaluation and treatment of a novel ptosis syndrome found in this area. DESIGN: A team of 4 international ophthalmologists and 3 local doctors and 3 local nurses involved in a high-volume field intervention for all patients presenting with a bilateral ptosis to Mingende Rural Hospital. METHODS: Patients were systematically evaluated and treated during a 6-day surgical outreach and followed daily for 1 week and as needed via telemedicine. Visiting surgeons provided skills-transfer instruction for 3 local doctors and 3 nurses. Data collected included demographic information, history of present illness, past medical history, family history, social history, and a complete ophthalmologic and targeted neurologic evaluation. Patients were offered surgical intervention if they met criteria for safe eyelid elevation and could present for follow ups. Detailed notes of patient geographic location, history, and risk factors were collected in addition to pre- and postoperative photos. MAIN OUTCOME MEASURES: Efficient triage and treatment of all patients referred to the remote oculoplastic clinic. RESULTS: A total of 97 patients presented to the camp; of these, 87 underwent complete evaluation. There were 72 patients with ptosis, of which 60 were found to be of Simbu-type. These patients were grouped clinically by degree of ptosis: mild, moderate, and severe. Thirty-eight patients had moderate ptosis of which 34 underwent surgical intervention. Eleven patients with mild ptosis were counseled and observed. The 10 patients with severe ptosis and 2 with moderate ptosis were treated medically with ptosis crutches manufactured on site. A new technique for creating ptosis crutch glasses was developed. CONCLUSIONS: A new variant of progressive myogenic ptosis was identified. A high-volume oculoplastic surgical camp is an efficient way to systematically evaluate and treat this new entity. Skills-transfer training for local doctors and staff ensured continuity of care for the surgical patients.

Increased prevalence of depression and anxiety in patients with migraine and interictal photophobia.

BACKGROUND: Most patients with migraine report photophobia associated with headache; a subset report interictal photophobia. These patients are light sensitive even during headache-free periods. The objective of this case-control study was to assess the prevalence of symptoms of anxiety and depression in migraine patients with and without interictal photophobia. METHODS: We recruited 16 subjects with migraine and interictal photophobia, 16 age- and gender-matched migraine subjects without interictal photophobia, and 16 age- and gender- matched controls. Migraine subjects met International Headache Society classification criteria. Participants completed a photophobia questionnaire, Beck Depression Inventory (BDI-II), and Beck Anxiety Inventory (BAI). Chi-square analyses and two-tailed Wilcoxon rank sum tests were used for the analyses. RESULTS: Subjects with interictal photophobia had significantly higher scores on the photophobia questionnaire compared to subjects without interictal photophobia. Subjects with interictal photophobia had significantly higher scores on the BDI-II and BAI compared to subjects without interictal photophobia. CONCLUSIONS: Migraine patients with interictal photophobia are more likely to manifest symptoms of depression and anxiety compared to migraine patients without interictal photophobia. Care providers should be aware of increased prevalence of these symptoms in this population and consider appropriate referrals. Future research could assess whether treatment of photophobia leads to improvements in symptoms of depression and anxiety in migraine patients.

Thin-film optical notch filter spectacle coatings for the treatment of migraine and photophobia.

Previous evidence suggests optical treatments hold promise for treating migraine and photophobia. We designed an optical notch filter, centered at 480nm to reduce direct stimulation of intrinsically photosensitive retinal ganglion cells. We used thin-film technology to integrate the filter into spectacle lenses. Our objective was to determine if an optical notch filter, designed to attenuate activity of intrinsically photosensitive retinal ganglion cells, could reduce headache impact in chronic migraine subjects. For this randomized, double-masked study, our primary endpoint was the Headache Impact Test (HIT-6; GlaxoSmithKline, Brentford, Middlesex, UK). We developed two filters: the therapeutic filter blocked visible light at 480nm; a 620nm filter was designed as a sham. Participants were asked to wear lenses with one of the filters for 2weeks; after 2weeks when no lenses were worn, they wore lenses with the other filter for 2weeks. Of 48 subjects, 37 completed the study. Wearing either the 480 or 620nm lenses resulted in clinically and statistically significant HIT-6 reductions. However, there was no significant difference when comparing overall effect of the 480 and 620nm lenses. Although the 620nm filter was designed as a sham intervention, research published following the trial indicated that melanopsin, the photopigment in intrinsically photosensitive retinal ganglion cells, is bi-stable. This molecular property may explain the unexpected efficacy of the 620nm filter. These preliminary findings indicate that lenses outfitted with a thin-film optical notch filter may be useful in treating chronic migraine.

Blepharospasm in children and adolescents.

PURPOSE: Benign essential blepharospasm (BEB) generally is considered a disorder of adults; however, it rarely can present in childhood or adolescence. The main purpose of this study was to determine the prevalence of BEB in children and adolescents. Our research question was whether blepharospasm is seen in children or adolescents as well as in the adult population. METHODS: We conducted a retrospective chart review at the University of Utah and Johns Hopkins University. We reviewed our databases for diagnoses of blepharospasm and tic disorder over the past 10 years in patients of all ages. Charts then were reviewed to confirm the diagnosis, and a questionnaire was sent to subjects whose blepharospasm had apparently begun before age 20 years. RESULTS: We identified 26 patients diagnosed with eyelid spasms that had begun while under the age of 20. We confirmed BEB in four of these cases. Of these individuals, all had developed symptoms in adolescence or before and all were still symptomatic but had noted improvement in the severity and frequency of their symptoms. CONCLUSIONS: Although rare, BEB can develop in the first decade of life, producing symptoms and signs that are similar to adults, with persistence into adulthood.

Chronic migraine is associated with reduced corneal nerve fiber density and symptoms of dry eye.

BACKGROUND: We used in vivo corneal confocal microscopy to investigate structural differences in the sub-basal corneal nerve plexus in chronic migraine patients and a normal population. We used a validated questionnaire and tests of lacrimal function to determine the prevalence of dry eye in the same group of chronic migraine patients. Activation of the trigeminal system is involved in migraine. Corneal nociceptive sensation is mediated by trigeminal axons that synapse in the gasserian ganglion and the brainstem, and serve nociceptive, protective, and trophic functions. Noninvasive imaging of the corneal sub-basal nerve plexus is possible with in vivo corneal confocal microscopy. METHODS: For this case-control study, we recruited chronic migraine patients and compared them with a sex- and age-similar group of control subjects. Patients with peripheral neuropathy, a disease known to be associated with a peripheral neuropathy, or prior corneal or intraocular surgery were excluded. Participants underwent in vivo corneal confocal microscopy using a Heidelberg Retinal Tomography III confocal microscope with a Rostock Cornea Module. Nerve fiber length, nerve branch density, nerve fiber density, and tortuosity coefficient were measured using established methodologies. Migraine participants underwent testing of basal tear production with proparacaine, corneal sensitivity assessment with a cotton-tip applicator, measurement of tear break-up time, and completion of a validated dry eye questionnaire. RESULTS: A total of 19 chronic migraine patients and 30 control participants completed the study. There were no significant differences in age or sex. Nerve fiber density was significantly lower in migraine patients compared with controls (48.4 ± 23.5 vs. 71.0 ± 15.0 fibers/mm2 , P < .001). Nerve fiber length was decreased in the chronic migraine group compared with the control group, but this difference was not statistically significant (21.5 ± 11.8 vs. 26.8 ± 5.9 mm/mm2, P < .084). Nerve branch density was similar in the two groups (114.0 ± 92.4 vs. 118.1 ± 55.9 branches/mm2 , P < .864). Tortuosity coefficient and log tortuosity coefficient also were similar in the chronic migraine and control groups. All migraine subjects had symptoms consistent with a diagnosis of dry eye syndrome. CONCLUSIONS: We found that in the sample used in this study, the presence of structural changes in nociceptive corneal axons lends further support to the hypothesis that the trigeminal system plays a critical role in the pathogenesis of migraine. In vivo corneal confocal microscopy holds promise as a biomarker for future migraine research as well as for studies examining alterations of corneal innervation. Dry eye symptoms appear to be extremely prevalent in this population. The interrelationships between migraine, corneal nerve architecture, and dry eye will be the subject of future investigations.

Extramedullary hematopoietic lesion of the orbit presenting as a ring enhancing lesion.

A 47-year-old healthy man presented with symptoms of double vision and drooping of the right upper eyelid. Examination disclosed signs of vertical diplopia and right upper-eyelid ptosis. An MRI delineated a well circumscribed, ring enhancing mass within the right orbit. The mass with peripheral enhancement located within the orbit, generated a broad differential diagnosis. After an excisional biopsy was performed, the mass was revealed to be fibrosis with extramedullary hematopoiesis. Current differential diagnoses of ring enhancing orbital lesions do not include extramedullary hematopoiesis, but we present a unique presentation of extramedullary hematopoiesis presenting as a ring enhancing orbital mass.

Ocular anatomy and cross-sectional imaging of the eye.

Ocular cross-sectional imaging is usually obtained as an adjunct to clinical ophthalmologic examination and ocular ultrasound. Computed tomography/magnetic resonance imaging (CT/MRI) are complimentary for ocular imaging and are performed for evaluation of the vitreous cavity, choroid, retina, sclera, and potential spaces and for the assessment of extension of disease beyond the globe into the orbit or brain. CT has superior spatial resolution aided by the natural contrast between bone, soft tissues, air, and fat. The short scanning time is advantageous to reduce motion effects and the need for sedation. CT is also the modality of choice for evaluation of traumatic injury and for visualization of foreign bodies. Potential clinical indications for MRI include staging of retinoblastoma and other causes of leukocoria, assessment of retinal or choroidal detachments for underlying retinal mass or hemorrhage, uveal melanoma, ocular metastases, choroidal hemangioma, and buphthalmus, staphyloma, and coloboma. Last, but not least, MRI has the advantage of no ionizing radiation.