Systemic Lupus Erythematosus-associated Retinal Vasculitis Treated with Adalimumab.

PURPOSE: To present a case of refractory systemic lupus erythematosus (SLE)-associated retinal vasculitis that responded to the anti-tumor necrosis factor (TNF)-alpha inhibitor adalimumab as corticosteroid-sparing therapy. METHODS: Descriptive case report of a patient with SLE with retinal vasculitis complicated by an ischemic retinal vein occlusion and cystoid macular edema. RESULTS: A 30-year-old female patient with a history of SLE presented with retinal vasculitis and an ischemic, branch retinal vein occlusion with macular edema in the left eye. Oral corticosteroid was administered along with mycophenolate mofetil (MMF) as a corticosteroid-sparing agent. Despite MMF therapy, the patient developed an exacerbation of her vasculitis with the involvement of both eyes. Adalimumab was initiated with a resultant resolution of retinal vasculitis as a corticosteroid-sparing strategy with over 2 years of follow-up. CONCLUSION: Anti-TNF-alpha therapy with adalimumab may be effective as a corticosteroid-sparing agent in select patients with ocular inflammation associated with SLE.

Pentosan polysulfate maculopathy versus age-related macular degeneration: comparative assessment with multimodal imaging.

OBJECTIVE: To evaluate whether pentosan polysulfate maculopathy manifests distinctive imaging features that can be differentiated from those found in age-related macular degeneration (AMD). METHODS: Local databases were queried to identify patients with a diagnosis of interstitial cystitis who were seen at the Emory Eye Center between May 2014 and January 2019 and who had fundus imaging available for review. Ninety patients met the eligibility criteria. Masked graders categorized patients based on imaging characteristics as follows: category 1: pentosan polysulfate maculopathy; category 2: AMD or drusen; category 3: neither; and category 4: unsure. Pentosan polysulfate exposure characteristics were compared among groups. RESULTS: Of the 90 subjects evaluated, 79 (88%) were female and the median age was 61.5 years (range, 30-89). Seventeen patients were placed in category 1; 25 in category 2; 47 in category 3, and; 1 in category 4. Among categories 1 to 4, respectively, 17 (100%), 15 (60%), 28 (60%), and 0 patients had exposure to pentosan polysulfate (p = 0.007). Mean cumulative exposure to pentosan polysulfate across the four categories was 2.1, 0.36, 0.34, and 0 kg, respectively (p < 0.00001). Eyes with pentosan polysulfate maculopathy did not have typical drusen in the macula. CONCLUSION: Although pentosan polysulfate maculopathy resembles some aspects of AMD, the two conditions can be differentiated with the use of multimodal fundus imaging.

Clinical management of an outbreak of nutritionally variant streptococcus endophthalmitis following intravitreal bevacizumab injection.

BACKGROUND: The management of an outbreak of endophthalmitis associated with intravitreal bevacizumab represents a challenging real-time process involving identification of cases, treatment and mitigation measures during the outbreak. We summarize the clinical presentation and management of a cluster of endophthalmitis cases from contaminated bevacizumab, in addition to mathematical probabilistic assessment of the number of cases that define an outbreak. METHODS: A retrospective study was conducted to assess the management of an endophthalmitis outbreak after intravitreal bevacizumab (IVB) administration. Demographic data, clinical information, individual patient management and public health reporting measures were reviewed. Outcomes of patients who received prophylactic antibiotics for endophthalmitis prevention were also reviewed. Binomial tail probability calculations were performed to determine the likelihood of clusters of endophthalmitis that could inform when an outbreak was evolving that would warrant more public health notification measures and communication. RESULTS: Forty-five eyes of 42 patients who received IVB from a single batch were reviewed. Four cases of endophthalmitis from Granulicatella adiacens, a nutritionally-variant Streptococcus species, were treated successfully with intravitreal antibiotics ± vitrectomy. Thirty-four of the remaining 41 eyes were treated with prophylactic intravitreal vancomycin with no additional cases of endophthalmitis. Outbreak management also included CDC, ASRS and public health authority notification. Binominal tail probabilities demonstrated the rarity of clusters from a single batch (i.e. ~ 1/10,000 for 2 cases; 1/2 million for 3 cases). However, given the U.S. scale of IVB administration, there is an 87% chance of a cluster ≧ 2 and a 1% chance of a cluster ≧ 3 cases annually, which may guide outbreak management. A process diagram was developed to incorporate patient management and public health measures when an outbreak is suspected. CONCLUSION: Intravitreal antibiotics and vitrectomy were effective in the individual management of cases of endophthalmitis, and no serious adverse events occurred with prophylactic intravitreal vancomycin for at-risk eyes. Best practices for outbreaks should be evaluated, given their likelihood within the U.S. and the sight-threatening consequences of endophthalmitis.

Thirteen Years' Progression of Macular Atrophy in a Patient With Jeune Syndrome.

Jeune syndrome is a rare skeletal dysplasia with an associated retinal dystrophy. The authors describe a case of progressive bilateral macular atrophy (with multimodal imaging) in a patient with Jeune syndrome who was followed over 13 years. This case, confirmed with genetic testing, highlights the importance of characterizing the relationship between phenotype and genotype in this genetically heterogenous condition. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:107-109.].

Pentosan Polysulfate Maculopathy versus Inherited Macular Dystrophies: Comparative Assessment with Multimodal Imaging.

PURPOSE: To evaluate whether pentosan polysulfate (PPS) maculopathy manifests distinctive characteristics that permit differentiation from hereditary maculopathies with multimodal fundus imaging. DESIGN: Retrospective review. PARTICIPANTS: Emory Eye Center databases were queried for the following International Classification of Diseases codes from May 20, 2014, through October 22, 2019: 362.70 (unspecified hereditary retinal dystrophy), 362.74 + H35.52 (pigmentary retinal dystrophy), 362.76 + H35.54 (dystrophies primarily involving the retinal pigment epithelium), and H35.50 (unspecified macular degeneration). METHODS: Fundus images for each patient were evaluated, including color fundus photographs, fundus autofluorescence images, and spectral-domain OCT images. Cases with imaging sufficient for diagnostic classification were analyzed. Masked graders classified patient images as follows: highly suggestive of PPS maculopathy; some features resembling PPS maculopathy, but not classic disease; and clearly distinct from PPS maculopathy. MAIN OUTCOME MEASURES: Sensitivity and specificity for identification of PPS maculopathy by masked reviewers. RESULTS: A total of 1394 patients were evaluated, and 1131 had imaging sufficient for classification. Fifteen patients were categorized as having findings highly suggestive of PPS maculopathy; 25 patients showed some features resembling PPS maculopathy but not classic disease; and 1091 patients showed evidence of disease clearly distinct from PPS maculopathy. All 10 patients with PPS maculopathy in this dataset were correctly categorized as having PPS maculopathy. Five patients without PPS exposure were categorized incorrectly as having PPS maculopathy. This represented a 100% sensitivity and 99.6% specificity for identification of PPS maculopathy by masked review of fundus imaging in this dataset. CONCLUSIONS: The imaging characteristics of PPS maculopathy allow for differentiation from hereditary maculopathies even in the absence of known exposure to the drug.

Treatment of Acute Zonal Occult Outer Retinopathy With Intravitreal Steroids.

BACKGROUND AND OBJECTIVE: To report on the use of intravitreal steroids in the management of acute zonal occult outer retinopathy (AZOOR), a rare disorder affecting the outer retina. PATIENTS AND METHODS: Retrospective case series of nine eyes of five patients with AZOOR who received intravitreal triamcinolone acetonide (IVTA), dexamethasone intravitreal implant, and/or fluocinolone acetonide implant. Treatment response was determined by reported symptoms and multimodal imaging findings. Patients were observed for at least 1 year following intravitreal steroid treatment (range: 14 months to 63 months). RESULTS: Seven eyes received IVTA, six eyes received the dexamethasone intravitreal implant, and one eye received the fluocinolone acetonide implant. All patients experienced disease stability or improvement based on symptomatic response and multimodal imaging findings after intravitreal steroids. One eye developed central serous retinopathy, and another eye a choroidal neovascular membrane. Five of nine eyes experienced ocular hypertension. All phakic eyes developed cataracts. CONCLUSION: Intravitreal steroids effectively achieved disease stability in patients with AZOOR. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:504-509.].

Microanatomical Location of Hyaluronic Acid Gel Following Injection of the Upper Lip Vermillion Border: Comparison of Needle and Microcannula Injection Technique.

PURPOSE: To compare needle and microcannula injection techniques in regards to the microanatomical location of hyaluronic acid (HA) gel injected in the upper lip vermillion border of cadaver specimens. METHODS: The upper lip vermillion border was injected transcutaneously with HA gel in 8 fresh hemifaces of 4 female human cadavers. Each hemiface was injected by a single experienced injector, the right side using a 27-gauge microcannula and the left side using a 30-gauge needle. A 2-cm region of each lip was excised lateral to a point 1-cm lateral to the philtrum. Specimens were fixed in 95% alcohol, embedded in paraffin, and stained with hematoxylin-eosin for histologic examination. RESULTS: Most HA injected with either a needle or a microcannula was located within the orbicularis oris muscle, and the remaining HA resided within the subcutaneous fat. In 3/4 right (microcannula) hemifaces, 100% of the HA was located within the muscle. Only 2/4 left (needle) hemifaces had at least 95% of the HA located within the muscle. Overall, in right (microcannula) hemifaces, 93% of the filler was located within the muscle, and in left (needle) hemifaces, 79% of the filler was located within the muscle (p =0.14). CONCLUSIONS: Most HA filler injected into the vermillion border after either microcannula or needle injection resides within the orbicularis oris muscle rather than in a subcutaneous/submucosal location. Injection with a microcannula shows a trend for more uniform intramuscular location compared with needle injection.

Femtosecond laser-assisted cataract surgery in anterior lenticonus due to Alport syndrome.

PURPOSE: We describe a case of bilateral anterior lenticonus in a patient with Alport syndrome treated with femtosecond laser-assisted cataract surgery (FLACS). OBSERVATIONS: FLACS was performed without complication, and a desirable postoperative visual acuity was achieved. CONCLUSIONS AND IMPORTANCE: Femtosecond laser-assisted cataract surgery is an effective approach for managing patients with anterior lenticonus secondary to Alport syndrome.

Cicatricial ectropion in a patient treated with dupilumab.

PURPOSE: To describe a case of bilateral conjunctivitis and cicatricial ectropion associated with dupilumab therapy for atopic dermatitis. OBSERVATIONS: Severe hyperemia of the conjunctiva and eyelid margins, as well as cicatricial ectropion, began two months after starting weekly dupilumab injections for atopic dermatitis. The findings worsened over the next several months and improved after discontinuing dupilumab. CONCLUSIONS AND IMPORTANCE: Dupilumab is a promising intervention in the management of atopic dermatitis and asthma, however, little is known about its potential ocular adverse effects. We report the first case of dupilumab-associated ocular inflammation leading to cicatricial ectropion.

Leaking choroidal nevus treated with focal laser photocoagulation.

PURPOSE: To present a case of leaky choroidal nevus that responded favorably to treatment with focal laser photocoagulation, shown by spectral domain optical coherence tomography, fundus autofluorescence, and fundus photography. METHODS: Descriptive case report of a 40-year-old male patient with decreased visual acuity because of subretinal fluid associated with a choroidal nevus, which was treated with focal laser photocoagulation. RESULTS: Treatment with focal laser photocoagulation to the surface of the choroidal nevus resulted in the resolution of subretinal fluid by 6 weeks. The therapeutic effect remained after 1 year of follow-up with continued improvement in vision and with no growth of the lesion. CONCLUSION: Laser photocoagulation can be an effective treatment for symptomatic subretinal fluid associated with a choroidal nevus. Spectral domain optical coherence tomography, fundus autofluorescence, and fundus photography can be helpful in monitoring the treatment response.

Retinal telangiectasis detected during a vision screening examination in a child with hearing loss led to the diagnosis of facioscapulohumeral muscular dystrophy.

A 2-year-old girl with congenital sensorineural hearing loss was found to have retinal exudation and subretinal fluid in her left eye. Further investigation revealed leaking retinal telangiectasias in her left eye and extensive areas of peripheral retinal nonperfusion in both eyes. A clinical diagnosis of facioscapulohumeral muscular dystrophy (FSHD) was confirmed by genetic testing. The patient was followed with serial intraoperative optical coherence tomography (OCT) scans, which demonstrated subretinal fluid in the macula and its subsequent resolution after treatment. She underwent 6 rounds of panretinal photocoagulation and 2 injections of intravitreal bevacizumab, which resolved the subretinal fluid and exudates.