Unilateral CRAO as the presenting clinical sign of systemic lupus erythematosus.

Purpose: This report describes the case of unilateral central retinal artery occlusion (CRAO) as a patient's presenting clinical sign of systemic lupus erythematosus (SLE). Observations: Though the patient knew of her SLE diagnosis through incidental abnormal lab work, she never pursued treatment because she never exhibited signs of the disease. Despite her asymptomatic course, she presented with a sudden and severe thrombotic event that left her with no light perception in her affected eye. Laboratory work-up was consistent with SLE and anti-phospholipid syndrome (APS). Conclusions and importance: This case draws attention to the possibility of CRAO as a presenting sign of SLE, rather than a sequela of active disease. Awareness of this risk may play a factor in future discussions between patients and their rheumatologists when considering initiation of treatment at the time of diagnosis.

Orbital Compartment Syndrome After High-speed Air-Gasoline Blast Injury.

Orbital compartment syndrome is an ophthalmologic emergency that requires timely surgical intervention. The authors present a rare case of orbital compartment syndrome in a 30-year-old male injured by forceful entry of air-gasoline mixture into the orbit, secondary to inadvertent firing of the piston from running mechanical diagnostics on an automobile internal combustion engine. Orbital CT revealed extensive orbital emphysema with both pre- and postseptal involvement and diffuse chemical cellulitis. Serial exams revealed rapid deterioration of vision with elevated intraocular pressure and development of eyelid, corneal, and orbital edema; a relative afferent pupillary defect and optic nerve hypoperfusion. He was started on intravenous steroids and underwent an emergent lateral canthotomy with cantholysis, which temporarily reduced the intraocular pressure. However, a second rapid increase in soft tissue swelling resulted in another episode of ocular hypertension and compressive optic neuropathy, requiring emergent orbital bony decompression, which was followed by decreased intraocular and orbital pressure. The patient later developed progressive corneal opacification indicating delayed chemical injury. This was managed with a 10-day course of aggressive topical and systemic antiinflammatory agents with significant improvement in visual acuity. At last follow up, the vision was 20/30 and the corneal and eyelid edema had cleared.

Irreversible Blindness Following Periocular Autologous Platelet-Rich Plasma Skin Rejuvenation Treatment.

A 49-year-old woman developed acute visual loss in the right eye following bilateral cosmetic platelet-rich plasma injections to rhytids in the glabellar region. External exam showed skin necrosis in the region over the right rhytids and restricted right ocular motility. Dilated fundus exam was significant for ophthalmic artery occlusion. Imaging revealed right eye extraocular muscle ischemia and optic nerve infarction, along with right frontal, parietal, and occipital lobe infarction. Work-up for thromboembolic and vascular etiologies were negative. To our knowledge, this is the first case reported of extensive ischemia following autologous platelet-rich plasma therapy.

Delayed bilateral fibrinous anterior chamber reaction following autologous bone marrow transplant and cataract surgery.

PURPOSE: Immune reconstitution uveitis (IRU) is a well-described phenomenon that by definition occurs in patients with AIDS who undergo highly active antiretroviral therapy resulting in a rebound inflammatory response to the presence of clinically latent cytomegalovirus (CMV). We hypothesize that similar phenomena may exist in other cohorts who undergo transient immunosuppression with rapid white blood cell count recovery. OBSERVATIONS: A patient developed rebound inflammation a few months after cataract surgery with intraocular lens placement characterized by photophobia, significant anterior chamber cell and fibrinous deposits. She had a history of multiple myeloma treated with chemotherapy and a recovery of white blood cell counts following autologous bone marrow transplant. She underwent a thorough work-up for infectious etiologies, as well as the presence of intraocular CMV, which were negative. Her vision and symptoms improved to baseline with the use of topical steroids and at one year her exam remained stable. CONCLUSIONS AND IMPORTANCE: With a negative work-up for infectious etiologies, and the timing and clinical presentation, the patient's inflammation was likely the result of rapid white blood cell count recovery following iatrogenic immunosuppression similar to the mechanism described for IRU.

Visual recovery following open globe injury with initial no light perception.

BACKGROUND: The purpose of this study was to analyze eyes presenting with no light perception (NLP) after open globe injury (OGI) to determine visual outcomes and prognostic indicators for visual recovery. METHODS: The records of consecutive patients with at least 6 months of follow-up presenting with OGI and NLP to a single institution between January 1, 2003 and December 31, 2013 were reviewed for demographics, ophthalmic history, context and characteristics of injury, ocular examination findings, surgical interventions, and follow-up visual acuity. Unpaired t-tests and Fisher's Exact tests were used for statistical analysis. RESULTS: Twenty-five patients met our inclusion criteria. The mean age was 50.4±25.5 (range 8-91) years. Four patients (16%) regained vision (hand motion in three patients and light perception in one patient) while 21 patients (84%) remained with NLP or had a prosthesis at final follow-up. Fourteen eyes (56%) were enucleated; nine (36%) were secondary enucleations. Although the sample sizes were small, neither ocular trauma score nor wound size was found to predict visual recovery. CONCLUSION: Four patients regained some vision after presenting with NLP due to OGI. These findings suggest that, in select cases, physicians should discuss the possibility of regaining some vision.

Genetic Anticipation in Familial Neuromyelitis Optica: Case and Literature Review.

OBJECTIVES: To describe genetic anticipation in a mother and daughter with antiaquaporin 4 (AQP4) antibody-positive neuromyelitisoptica (NMO). METHODS: Retrospective case review. RESULTS: A woman with onset of transverse myelitis at age 38 was found to have a positive AQP4 antibody during work-up of recurrent symptoms. Subsequently, she developed intermittent episodes of monocular vision loss with optic nerve involvement that were treated with intravenous methylprednisolone and chronic rituximab. Eighteen years after initial presentation, her 78-year-old mother, with a history of recurrent urinary tract infections, also developed monocular vision loss and her anti-AQP4 antibody was positive. Previous reports of genetic anticipation in familial NMO are identified and discussed. CONCLUSIONS: These cases highlight genetic anticipation in familial NMO. Disease onset can occur with a chronological age difference of as much as 40 years between parent and child. Patients with NMO should be counseled regarding the possibility of subsequent disease onset in family members, particularly parents, with significant differences in calendar or chronological year of onset.

Central retinal vein occlusion resulting from anomalous retinal vascular anatomy in a 24-year-old man.

An otherwise healthy 24-year-old man presented with a painless decrease of vision in the left eye for 2 days. Best-corrected visual acuity was 20/20 in the right eye and 20/80 in the left eye. Anterior exam was unremarkable and funduscopic exam in the left eye revealed retinal hemorrhages in all four quadrants with venous dilation and tortuosity consistent with central retinal vein occlusion. Fluorescein angiography revealed delayed venous filling with neither leakage nor vasculitis. A comprehensive work-up that included infectious, inflammatory, and hypercoagulability studies was unremarkable, and magnetic resonance imaging of the orbits was unrevealing. After 2 months, best-corrected visual acuity returned to 20/20-2 in the left eye. Upon closer review of the vascular anatomy in the left eye, a bifurcation of the central retinal artery at the level of the optic disc was tightly intertwined with an undilated nasal retinal vein in a manner that appeared to compress the underlying central retinal vein, resulting in dilation and tortuosity of the remaining venous branches. The vessel wall damage, turbulent venous flow, and compressive mass effect resulting from the anomalous retinal vasculature relationship is the proposed mechanism of the central retinal vein occlusion. Careful attention to the retinal vascular anatomy is suggested to aid in assessing the risk of retinal vein occlusion in any age group.

Characteristics and outcomes of fall-related open-globe injuries in pseudophakic patients.

AIM: We aimed to identify the characteristics and prognostic indicators of poor visual and anatomic outcome in pseudophakic patients with fall-related open-globe (OG) injuries. We used a case series design, for a total of 26 patients. METHODS: Charts of consecutive pseudophakic patients with fall-related OG injury at a single institution were reviewed. Demographics, ophthalmic history, circumstances of injury, initial best-corrected visual acuity (BCVA), examination findings, surgical interventions, and follow-up BCVA were tabulated for statistical analysis with unpaired t-tests and Fisher's exact tests. RESULTS: Nineteen patients (73%) were women. Mean (± standard deviation) age was 80.6±4.6 years (range: 61-97 years). Initial BCVA was <20/400 in 24 of 25 patients (96%). Mean ocular trauma score (OTS) was 38.54±10.95. OTS was lower (P=0.0017, P<0.0001, and P=0.0240) and wound size was larger (P=0.0440, 0.0145, and 0.0026) in patients with final BCVA <20/40, <20/400, and phthisis at final follow-up, respectively; compared to patients with BCVA ≥20/40, 20/400, and no phthisis at final follow-up, respectively. Final BCVA <20/400 was associated with 360° subconjunctival hemorrhage (SCH), retinal detachment, and proliferative vitreoretinopathy (P=0.0498, 0.0181, and 0.0310, respectively). Total hyphema, intraocular lens (IOL) damage, and IOL expulsion were associated with needing multiple surgical interventions (P=0.0345, P<0.0001, and P=0.0023, respectively). CONCLUSION: Large wound size, low OTS, 360° SCH, total hyphema, posterior injury, and IOL damage are common findings that are also prognostic of poor visual and anatomic outcome in pseudophakic patients with fall-related OG injuries. Ophthalmologists dealing primarily with geriatric populations should contribute to the discussion of fall risk.

Spontaneous hyphema and pupillary block in a patient with a left ventricular assist device.

The left ventricular assist device (LVAD) has been a standard of care for the management of patients with advanced heart failure since the 1990s. An increased risk of spontaneous bleeding related to the device has been noted, ranging from minor epistaxis to major thoracic and mediastinal hemorrhages. To our knowledge, intraocular hemorrhage has not been previously reported. We report a 72-year-old patient with an LVAD who subsequently developed a spontaneous intraocular hemorrhage that manifested as hyphema, pupillary block, and acute intraocular pressure elevation.

Clinical and histologic findings in patients with uveal melanomas after taking tumor necrosis factor-α inhibitors.

OBJECTIVE: To describe the progression of uveal melanocytic lesions to melanomas after initiation of tumor necrosis factor-α (TNF-α) inhibitors. PATIENTS AND METHODS: We report 3 cases of uveal melanoma occurring after treatment with TNF-α inhibitors, 2 from Mayo Clinic and 1 from Yale University. The study took place from February 27, 2009, through July 15, 2013. RESULTS: Two women and one man with inflammatory disease who received TNF-α inhibitors had subsequent development of uveal melanomas. The 2 women had inflammatory bowel disease and had been followed up for melanocytic tumors that grew markedly within 1 year after beginning treatment with TNF-α inhibitors to the point of requiring treatment. One had histologic confirmation of the melanoma. The male patient had rheumatoid arthritis that was being treated with TNF-α inhibitors. Serial ultrasonography was performed to monitor bilateral diffuse scleritis, and within 16 months of initiation of TNF-α inhibitor therapy, a choroidal mass was detected that continued to grow over the next 3 months. The patient elected to have enucleation, which revealed uveal melanoma and thinning of the sclera from the previous scleritis. CONCLUSION: Our 3 cases of uveal melanocytic tumors occurring after the use of TNF-α inhibitors add to the growing literature suggesting a correlation between TNF-α inhibitors and the development of malignant neoplasms. Considering the association between cutaneous melanoma and TNF-α inhibitors, we recommend that patients have an eye examination before initiation of TNF-α inhibitors, and those with preexisting nevi should be followed up at regular intervals.

HIPAA for physicians in the information age.

The increased prominence of electronic health records, email, mobile devices, and social media has transformed the health care environment by providing both physicians and patients with opportunities for rapid communication and knowledge exchange. However, these technological advances require increased attention to patient privacy under the Health Insurance Portability and Accountability Act (HIPAA). Instant access to large amounts of electronic protected health information (PHI) merits the highest standard of network security and HIPAA training for all staff members. Physicians are responsible for protecting PHI stored on portable devices. Personal, residential, and public wireless connections are not certified with HIPAA-compliant Business Associate Agreements and are unsuitablefor PHI. A professional and privacy-oriented approach to electronic communication, online activity, and social media is imperative to maintaining public trust in physician integrity. As new technologies are integrated into health care practice, the assurance of privacy will encourage patients to continue to seek medical care.

The relationship between sex and symmetry in thyroid eye disease.

PURPOSE: To examine the relationships between sex and symmetry in the context of disease activity, severity, and thyroid status in thyroid eye disease. METHODS: Retrospective chart review of 31 men and 31 women with untreated thyroid eye disease. Subjective complaints, smoking status, thyroid status, and objective findings pertinent to the clinical activity score (CAS) and "NO SPECS" classification were recorded. Overall disease asymmetry was defined as having simultaneous asymmetry of both more than one symptom and more than one external finding. Asymmetry was compared across sex and thyroid status. CAS and NO SPECS severity were compared across sex, symmetry, and thyroid status. RESULTS: Asymmetric appearance was reported by 58% of men and 19% of women. Asymmetric proptosis (>2 mm difference) was seen in 45% of men and 23% of women (P=0.036). Overall asymmetry was seen in 55% of men and 19% of women (P=0.017). Thyroid status and sex had a combined effect on symmetry, as 15 of 16 hyperthyroid females (94%) demonstrated symmetric disease. Average NO SPECS severity was 3.5 (standard deviation [SD] 1.4) in men and 3.3 (SD 1.1) in women (P=0.51), and was 3.8 (SD 1.4) in asymmetric patients versus 3.2 (SD 1.3) in symmetric patients (P=0.08). The CAS was higher in asymmetric than symmetric patients (1.84 versus 0.97; P=0.012). CONCLUSION: Men demonstrated more asymmetric disease (proptosis and overall asymmetry) than women, while hyperthyroid females demonstrated more symmetry than euthyroid and hypothyroid males and females. NO SPECS severity score was unaffected by sex, thyroid status, or symmetry. Asymmetric patients demonstrated higher clinical activity scores.

Evaluation of a new single-piece 4% water content hydrophobic acrylic intraocular lens in the rabbit model.

PURPOSE: To evaluate uveal and capsular biocompatibility of a 1-piece intraocular lens (IOL) manufactured from a new hydrophobic acrylic material that incorporates a barrier step at the optic-haptic junctions. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: The study IOL (Eternity-Uni W-60) was implanted in the right eyes of 5 New Zealand rabbits and the control IOL (Acrysof SN60WF) in the left eyes. Slitlamp examination was performed 1 through 4 weeks postoperatively. After death, the globes were enucleated and fixed in formalin. Capsular bag opacification scoring (Miyake-Apple view) was then performed followed by complete histopathology. RESULTS: At the 4-week examination, the mean posterior capsule opacification (PCO) score was 1.5 ± 1.0 (SD) in the study group and 2.2 ± 1.09 in the control group (P=.02). Anterior capsule opacification (ACO) was not present in the study eyes and was mild in the control eyes. On gross examination, the mean central PCO score was 0.9 ± 0.65 in the study group and 1.7 ± 1.20 in the control group (P=.07); the mean peripheral PCO score was 1.3 ± 0.67 and 2.4 ± 1.14 (P=.01) and the mean Soemmerring ring score was 3.8 ± 0.44 and 4.2 ± 1.09, respectively (P=.47). Histopathology confirmed that both IOLs were equally tolerated by the rabbit eyes. CONCLUSIONS: In this rabbit study, the new hydrophobic acrylic material was biocompatible. The barrier step incorporated to the optic-haptic junctions has the potential to enhance PCO prevention.

Comparison of capsulorhexis resistance to tearing with and without trypan blue dye using a mechanized tensile strength model.

PURPOSE: To evaluate the influence of trypan blue staining on capsulorhexis tear resistance using a model for mechanical measurement of the entire capsular bag of fresh human cadaver eyes. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: After the cornea and iris were removed, a 5.0 to 5.5 mm anterior continuous curvilinear capsulorhexis (CCC) was created; the capsule in study eyes was stained with trypan blue. The nucleus was hydroexpressed and the capsular bag was evacuated by irrigation/aspiration. Two metal shoetree-shaped fixtures were implanted separately in the capsular bag and assembled using a screw nut. After zonulectomy, the fixture-capsular bag assembly was removed from the eye and loaded onto a mechanized tester. The fixtures were separated at a velocity of 7.0 mm/min in 0.15 µm intervals to stretch the capsulorhexis to its rupture point. A graph was generated for each eye. RESULTS: Ten study eyes were compared with 23 control eyes. The groups were comparable in donor age, time from death, and CCC diameter. The mean rupture load was 0.40 Newton (N) ± 0.13 (SD) in the trypan blue group and 0.39 ± 0.16 N in the control group (P=.94). The mean extension was 5.70 ± 0.99 mm and 5.85 ± 1.17 mm, respectively (P=.74). CONCLUSIONS: There was no difference in CCC strength between trypan blue-stained capsules and control capsules. Staining with trypan blue did not reduce CCC tear resistance. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Assessment of a single-piece hydrophilic acrylic IOL for piggyback sulcus fixation in pseudophakic cadaver eyes.

PURPOSE: To evaluate a single-piece hydrophilic acrylic intraocular lens (IOL) designed for sulcus fixation in a piggyback configuration in postmortem pseudophakic human eyes. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: Pseudophakic human cadaver eyes were imaged by high-frequency ultrasound (Artemis) to assess the overall position of the primary IOL and the sulcus diameter. The piggyback IOL (Sulcoflex) was then injected into the ciliary sulcus of these eyes. After fixation in formalin, they were reevaluated by high-frequency ultrasound for assessment of IOL fixation, fit, centration, tilt, and clearance from the primary IOL and intraocular structures and analyzed after sectioning. RESULTS: Data could be obtained from 11 eyes, all in which the primary IOL was located in the capsular bag. Different foldable IOLs and different degrees of Soemmerring ring formation were represented. The piggyback IOL could be injected and positioned in the ciliary sulcus and had overall appropriate centration and minimum or no tilt. Clearance between the 2 IOLs ranged from 232 to 779 µm, mostly depending on the thickness of the primary IOL. Direct assessment of the sulcus-fixated haptics showed no disturbances to the ciliary processes. CONCLUSIONS: The new IOL has large optic and overall diameters, smooth and undulating haptics, and a convex-concave optic profile. Results show that these characteristics minimize the possibility of interaction with the primary IOL and uveal tissues, decreasing the likelihood of optical aberrations and pigmentary dispersion.

Toxicity comparison of intraocular azithromycin with and without a bioadhesive delivery system in rabbit eyes.

PURPOSE: To determine whether the addition of a bioadhesive drug-delivery system to topical azithromycin induces intraocular inflammation and damage when introduced intraocularly by different approaches and in varying doses. SETTING: John A. Moran Eye Center, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: Commercial topical azithromycin 1.0% was duplicated, including the benzalkonium chloride, but without inclusion of the Durasite bioadhesive drug-delivery system. Injections of 50 µL, 25 µL, and 10 µL of the antibiotic solutions were administered in a masked fashion to 2 rabbits; 1 eye (study eye) in each rabbit was randomized to receive azithromycin with the delivery system and the fellow eye (control eye) to receive azithromycin without the delivery system. Two rabbits had topical drops of each solution placed after a 2.8 mm incision was created. Masked slitlamp examinations, pachymetry, and intraocular pressure (IOP) were determined 1 day and 2 days postoperatively. The animals were humanely killed, and the endothelial density and histopathology were examined. RESULTS: The IOP (P<.001), pachymetry (P<.001), and signs of inflammation (P=.38 to .003) were consistently higher in the study eye, especially at the 50 µL dose, than in the control eye. This was confirmed by histopathology. CONCLUSION: If the drug-delivery system gains access to the anterior chamber, it may cause substantial corneal edema and inflammation, even at low doses and after topical administration.

Prevention of capsular bag opacification with a new hydrophilic acrylic disk-shaped intraocular lens.

PURPOSE: To evaluate capsular bag opacification with a new disk-shaped single-piece hydrophilic acrylic intraocular lens (IOL) suspended between 2 haptic rings connected by a pillar of the haptic material and with a commercially available single-piece hydrophobic acrylic IOL in rabbits. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: The study IOL was implanted in the right eyes of 5 New Zealand rabbits and the control IOL in the left eyes. Slitlamp examination was performed at weeks 1 through 5. After the rabbits were humanely killed, the globes were enucleated and examined by ultrasound. Capsular bag opacification scoring from the posterior aspect (Miyake-Apple view) was then performed, followed by histopathology. RESULTS: Trace honeycomb posterior capsule opacification (PCO) was noted in some study eyes. All control eyes developed moderate to marked PCO, which was more pronounced at the level of the optic-haptic junction. The mean PCO score was 0.4 ± 0.22 (SD) in the study group and 3.4 ± 0.54 in the control group (P=.000179, paired t test). Minimal proliferative cortical material was confined to the space between the anterior and posterior rings of the study IOL haptics. Anterior capsule opacification was absent in study eyes and mild in control eyes. There was no contact between the anterior capsule and the anterior surface of study IOLs. CONCLUSION: The peripheral rings of the study IOL, by expanding the capsular bag and preventing IOL surface contact with the anterior capsule, appear to prevent opacification of the capsular bag. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.