Yufei Tu

Placement of ProKera in the management of ocular manifestations of acute Stevens-Johnson syndrome in an outpatient.

Purpose: To report the clinical use of ProKera (Bio-Tissue, Inc., Miami, FL) under topical anesthesia in an outpatient for the management of ocular manifestations of acute Stevens-Johnson syndrome (SJS). Methods: Interventional case report. Results: A 19-year-old woman developed acute SJS with ocular involvement after ingestion of oral antibiotics. Slit-lamp examination performed 2 weeks later showed severe inflammation and areas of ulceration along all 4 lids and complete, bilateral sloughing of bulbar and palpebral conjunctivae, including the limbus. ProKera was placed under topical anesthesia without sedation in both eyes instead of an amniotic membrane due to excess risk of general anesthesia. Three weeks after placement, slit-lamp examination showed complete re-epithelialization of both corneas and conjunctivae, with only trace conjunctival injection and minor limbal epithelial irregularities. Three months postprocedure, there were no signs of clinically significant scarring, and the visual acuity (VA) was 20/20 bilaterally. Fourteen months postprocedure, VA remained stable, and the patient did not have dry eye, photophobia, clinically significant scarring, or symblepharon. Conclusions: ProKera placement performed under topical anesthesia may be appropriate for the treatment of ocular surface manifestations of acute SJS particularly in those patients followed in an outpatient setting with milder forms of disease and/or with contraindications to general anesthesia.

Ranibizumab in patients with dense cataract and proliferative diabetic retinopathy with rubeosis

Background: To evaluate the safety of ranibizumab as a surgical adjunct during cataract surgery in patients with proliferative diabetic retinopathy (PDR) with rubeosis, and to evaluate the efficacy and adverse effects of ranibizumab in treating PDR with rubeosis. Materials and Methods: Three intravitreal injections of 0.5 mg ranibizumab were administered on day-1, months-1 and -2 with cataract surgery 6-16 days after first injection. Retreatments with ranibizumab injections and pan-retinal photocoagulation (PRP) were given if recurrence or persistence of PDR was noted between months-3 and -11. Safety observation visits occurred at months-12, -18 and -24. Primary end points were incidence and severity of adverse events (AEs) that were related to both cataract surgery and treatment of PDR with rubeosis through month -12. Results: Of six patients screened, four (mean age 61.3 years) were enrolled. No AEs were noted with either cataract surgery or treatment of PDR. Neovascularization of iris (NVI) promptly regressed by 4 days after first ranibizumab injection, prior to cataract surgery in three of four patients (one had significantly regressed NVI by post-injection day-3 visit); NVI was not noted in any patient at 2 weeks after first ranibizumab injection. Recurrence of rubeosis or NVA after 3 monthly injections was not observed in any. At month-12, PDR was not present when assessed clinically and by fluorescein angiogram (FA). Only one patient developed neovascularization of disc and neovascularization elsewhere and required retreatments at months-5 and -9. Conclusions: Multiple intravitreal injections of ranibizumab may be a safe, effective treatment adjunct for PDR and diabetes-related rubeosis.

Epibulbar Rosai-Dorfman Disease: Novel Manifestation and Treatment

S inus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease [RDD]) is a benign, idiopathic, self-limiting lymphoproliferative disease, described by Rosai and Dorfman in 1969. Extranodal manifestations represent about 43% of cases. The disease is classically accompanied by fever, malaise, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. Rosai-Dorfman disease is diagnosed histopathologically, with abundant histiocytes often engulfing lymphocytes and lymphoid cells, known as emperipolesis. The histiocytes are characteristically reactive to CD68 and S-100 proteins. Herein, we discuss the unique case and treatment of a woman who had RDD with bilateral epibulbar lesions concurrent with anterior uveitis.

Chronic Non-infectious Uveitis in Patients with Juvenile Idiopathic Arthritis

ABSTRACT Purpose: To describe clinical findings and analyze treatment evolution of chronic, non-infectious uveitis in patients with juvenile idiopathic arthritis (JIA). Methods: A total of 82 patients (147 eyes) with JIA-related uveitis treated for ≥2 months were included (78% females; 79% bilateral uveitis; 74% anterior uveitis). Outcome measures were visual acuity (VA), inflammation control, side-effects, and surgical procedures. Results: Mean ± SD age at diagnosis was 4.9 ± 3.8 years; mean ± SD follow-up time was 8.7 ± 7.8 years. Mean VA did not significantly change throughout the study. Three (2%) eyes resulted in no light perception (NLP) vision. Thirty (37%) patients underwent 69 procedures. In total, 41 (50%) patients achieved inflammation control. TNF-α inhibitors were significantly associated with inflammation control. Seven (8.5%) patients stopped treatment due to side-effects. Conclusions: JIA is a cause of significant ocular morbidity. TNF-α inhibitor use was associated with inflammation control. Prospective, randomized, double blind clinical trials in this regard are warranted.

Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature

Abstract Purpose: To report a novel plasminogen gene mutation and detection of anti-plasminogen antibodies in a patient with ligneous conjunctivitis successfully treated with 60% fresh frozen plasma (FFP). Methods: Retrospective data collected on a 45-year-old Caucasian female presenting with unilateral chronic membranous lesions. Results: Laboratory investigation demonstrated decreased plasminogen antigen level, plasminogen activity, and rate of plasminogen activation by u-PA or t-PA, and elevated plasminogen activator inhibitor-1. Anti-plasminogen IgG and IgA antibodies were detected. DNA analysis revealed a novel Asp432Asn heterozygous missense mutation in the plasminogen gene (exon 11). The patient was treated with topical 60% FFP, achieved complete remission after four months, and remained membrane-free for over five years of follow-up. Conclusions: A novel plasminogen gene mutation, deficiency of plasminogen antigen and activity, and anti-plasminogen IgG and IgA antibodies were identified in a patient with adult-onset ligneous conjunctivitis. Sixty percent FFP maintained this patient disease-free for over five years.

Adult patients with uveitis associated with juvenile idiopathic arthritis: a retrospective review

OBJECTIVE To describe clinical characteristics and outcomes of adults with uveitis associated with juvenile idiopathic arthritis (JIA). METHODS Retrospective chart review from 2001 to 2014 of adult patients with JIA and follow-up ≥2 months. Outcome measures included visual acuity, intraocular pressure (IOP), inflammation grade, ocular findings/complications, immunosuppressive therapies, and surgeries. RESULTS Nineteen patients were included (95% female, 84% bilateral uveitis, 47% anterior uveitis). Mean (SD) ages at presentation and JIA diagnosis were 25.6 (7.8) years and 8.9 (5.6) years, respectively. Visual acuity and IOP did not significantly change throughout the study. Mean (SD) presenting inflammation grade was significantly decreased at 6 and 12 months. Fifteen (79%) patients were on topical medications, and 17 (89%) were on systemic immunosuppression. Two (12%) patients developed side effects requiring medication cessation. CONCLUSION Uveitis associated with JIA may extend into adulthood despite the use of topical medications and/or systemic immunosuppression and result in significant ocular morbidity, including the need for surgical intervention.

Characteristics of open-globe eye injuries with respect to zone of injury.

Aims: To describe the demographics, characteristics and outcomes of open -globe injuries (OGI) with respect to zone of injury. Methods: Medical records of all patients presenting with OGIs to University Hospita l, Newark, NJ between January 2001 and December 2008 with a follow-up of at least 3 months were reviewed. Demographics, characteristics of the trauma and outcomes were compared with respect to the zone of injury; location of injury is confined to the corn ea and limbus in zone 1 (Z1), 5mm posterior to the limbus in zone 3 (Z3). Results:Of the 309 patients (310 eyes) identified, 228 (74%) were male. The mean age at presentationwas 35.3years (1-96). Meanfollow-up was 22.8 months (3-108 months). Most of the eyes presented with Z1 injury: 141 eyes (46%) Z1 injury, 83 (27%)Z2 and 86 (28%) Z3. Rupture was the most common type of injury in Z2 and Z3 injuries. Nineteen (86%) of 22 eyes with an intraocular fore ign body(IOFB) had a Z1 injury. 32 (42%) of 77 eyes with Z3 injury had no light perception (NLP) at presentation, compared with 9 (8%) of 119 Z1 and 13 (17%) of 78 Z2 -injured eyes. Four percent of Z1, 11% of Z2 and 18% of Z3-injured eyes had a final visi

A Case of Vortex Vein Aplasia and Recurrent Idiopathic Uveal fusion Syndrome

A 46-year-old Hispanic man with a history of retinal detachment (RD) in the left eye presented ten years later with worsening superior field visual defect due to inferior serous RD in the right eye. Choroidal and ciliary body detachments were present. A diagnosis of idiopathic uveal effusion syndrome (IUES) was made after exclusion of other etiologies, and he underwent placement of sclera windows. Absence of vortex veins was noted intra-operatively. Histological analysis of the sclera specimen showed abnormal arrangement of collagen fibrils with the deposition of glycosoaminoglycan (GAG). His vision gradually improved postoperatively, and the retina completely attached within four months. Four years later, however, patient developed recurrence of IUES and subsequently underwent debridement of previouslyconstructed scleral windows that were noted to be covered with ingrowth of fibrous tissue. Resolution of subretinal fluid and recovery of baseline vision were achieved postoperatively within 4 months, and no relapse has occurred during 27-month follow-up.