Decreased Ocular Perfusion Pressure Associated With Reverse Ophthalmic Artery Flow on Transcranial Doppler Ultrasonography.

Innovative applications of clinical ocular diagnostic tools are emerging to help identify systemic disorders that extend beyond ocular diseases. Ophthalmodynamometry (ODM) is a screening tool that non-invasively determines mean central retinal artery pressure (MCRAP) and ocular perfusion pressure (OPP). Decreased OPP and MCRAP on Falck Medical Multifunctional Device (FMD, Falck Medical, Inc., Mystic, CT), along with reverse ophthalmic artery flow (ROAF) on transcranial Doppler ultrasonography (TCD), indicate increased collateral brain perfusion and possible stenosis of the ophthalmic artery or internal carotid artery (ICA). In this case report, we describe the case of a 78-year-old female with ROAF, reduced MCRAP, and OPP in the right eye, confirmed by carotid duplex of 50-79% right ICA stenosis. Early application of ODM and TCD allowed for prompt diagnosis and management with a vascular specialist.

Urrets-Zavalia Syndrome of Unresolving Mydriasis Following Endocyclophotocoagulation Combined with Phacoemulsification.

Aim and background: Combined endocyclophotocoagulation and phacoemulsification (ECP/Phaco) are uncommonly associated with complications. We present the first case of a rare complication following ECP/Phaco. Case description: A 72-year-old patient with dense nuclear sclerotic cataracts and primary open-angle glaucoma (POAG) underwent bilateral surgery uneventfully. He experienced a brief episode of postoperative elevated intraocular pressure (IOP), but only one eye with a lower baseline IOP developed a dilated pupil. No pupillary response was observed after applying 4% pilocarpine. The fixed mydriasis persisted without reaction to light or near stimulus, and the best-corrected vision (BCVA) was 20/30 in the affected eye. Conclusion: This case reports a possible rare complication when undergoing ECP/Phaco therapy. The pathogenesis of Urrets-Zavalia syndrome is unknown, but we hypothesized that eyes with more pronounced increases in IOP from baseline may be more susceptible to ischemic injury to the pupillary sphincter, resulting in a chronically dilated pupil. Clinical significance: Even a modest transient rise in postoperative IOP in a glaucomatous eye with normal baseline IOP could result in a chronically dilated pupil. How to cite this article: Cheng AMS, Vedula GG, Kubal AA, et al. Urrets-Zavalia Syndrome of Unresolving Mydriasis Following Endocyclophotocoagulation Combined with Phacoemulsification. J Curr Glaucoma Pract 2024;18(1):28-30.

Epithelial ingrowth in descemet membrane endothelial keratoplasty associated with vitreous loss.

BACKGROUND: Epithelial ingrowth is a rare but potentially sight-threatening complication caused by the invasion of corneal or conjunctival epithelial cells into the eye during ocular surgeries. DMEK is emerging as a widely used surgery for endothelial keratoplasty with its improved safety profile. We describe a case of epithelial ingrowth in the graft-host interface after uneventful DMEK associated with vitreous prolapse in the anterior chamber. CASE PRESENTATION: An 81-year-old female with Fuchs endothelial dystrophy underwent DMEK for corneal decompensation following cataract surgery. During the DMEK procedure, vitreous prolapse was observed around the intraocular lens (IOL). Her early postoperative course was unremarkable, but a dense paracentral interface opacity was observed during the 3-month follow-up. The area of epithelial ingrowth was imaged with optical coherence tomography (OCT) as a uniform nodule with a discrete increase in interface hyperreflectivity. A low-energy YAG laser was applied to remove the opacity. She maintained good vision and clear cornea without reoccurrence after treatment. CONCLUSIONS: We propose that, in addition to the introduction of epithelial cells during surgery, vitreous retention in the anterior chamber may be a risk factor by providing a scaffold that potentially aggravates epithelial ingrowth in DMEK. Our case demonstrated that early YAG intervention may disrupt interface epithelial cell growth, and the transmitted laser energy may fragment the scaffold vitreous noninvasively.

Supraperiosteal fat repositioning with midface lift in lower eyelid blepharoplasty: an 18-year experience.

PURPOSE: To assess the clinical outcomes of fat repositioning via supraperiosteal dissection with midface lift for correction of tear trough deformity in a large Asian patient population. METHODS: Retrospectively review 1152 Asian patients who underwent fat repositioning to the supraperiosteal plane with a midface lift between 2005 and 2022. Surgical technique, postoperative course, and complications were recorded. At the 6-month postoperative follow-up, the degree of patient satisfaction was assessed. RESULTS: A total of 2304 eyes from 1152 patients with an average follow-up of 10 months. These procedures were performed using a transforniceal approach in 185 patients (16%) or a transcutaneous skin excision approach in 967 patients (84%). Among the patients who underwent the transcutaneous technique, seven individuals (0.6%) experienced effective treatment of lower lid ectropion through lateral tarsal strip procedures. Nine patients (0.7%) required revision surgery to address the remaining lateral fat pad due to inadequate lateral orbital fat excision during the initial procedure. At the 6-month follow-up, most patients reported a high level of satisfaction, with 800 patients (78%) expressing extreme satisfaction and 196 patients (19.1%) reporting satisfaction with the improvement in their appearance. No one reported facial numbness, lower eyelid or cheek paralysis, newly developed diplopia or granuloma formation. CONCLUSION: The procedure of fat repositioning involving supraperiosteal dissection and a midface lift, whether performed using a transforniceal approach or a transcutaneous skin excision approach, in lower eyelid blepharoplasty proves to be a secure and auspicious surgical technique for rectifying tear trough deformity and attaining a pleasing aesthetic outcome.

Role of Topical 5-Fluorouracil in Demodex -Associated Blepharitis.

PURPOSE: The aim of this study was to report clinical observations suggesting the efficacy of topical 1% 5-fluorouracil (5-FU) in treating Demodex -associated blepharitis. METHODS: An observational retrospective review of 13 eyes from 13 individuals with conjunctival neoplastic lesions and concomitant Demodex lash infestation that received topical 1% 5-FU eye drops. Patients underwent slit-lamp examination at each follow-up visit. Clinical photographs of the lash line were obtained after treatment initiation. In a subset of patients, lashes were epilated bilaterally and microscopically analyzed for presence of Demodex mites before and after treatment initiation. RESULTS: The mean age of the population was 68 ± 14 years (range: 30-84 years) and 92% were male. In all 13 patients, a marked reduction in cylindrical dandruff was noted in the treated eye by slit-lamp examination after 2 cycles of 5-FU. There was complete resolution of cylindrical dandruff in 10 of 13 treated eyes compared with 0 resolution of cylindrical dandruff in untreated eyes ( P = 0.0001). In the 6 patients who received epilation, the lashes from the treated eye showed no Demodex , whereas lashes from the fellow untreated eye revealed persistent Demodex . CONCLUSIONS: Topical 1% 5-FU shows efficacy in treating Demodex -associated blepharitis. Further studies are indicated to reproduce our findings and evaluate the potential use of 5-FU as a treatment ingredient.

Recent Advances in Imaging Macular Atrophy for Late-Stage Age-Related Macular Degeneration.

Age-related macular degeneration (AMD) is a leading cause of blindness worldwide. In late-stage AMD, geographic atrophy (GA) of dry AMD or choroidal neovascularization (CNV) of neovascular AMD eventually results in macular atrophy (MA), leading to significant visual loss. Despite the development of innovative therapies, there are currently no established effective treatments for MA. As a result, early detection of MA is critical in identifying later central macular involvement throughout time. Accurate and early diagnosis is achieved through a combination of clinical examination and imaging techniques. Our review of the literature depicts advances in retinal imaging to identify biomarkers of progression and risk factors for late AMD. Imaging methods like fundus photography; dye-based angiography; fundus autofluorescence (FAF); near-infrared reflectance (NIR); optical coherence tomography (OCT); and optical coherence tomography angiography (OCTA) can be used to detect and monitor the progression of retinal atrophy. These evolving diverse imaging modalities optimize detection of pathologic anatomy and measurement of visual function; they may also contribute to the understanding of underlying mechanistic pathways, particularly the underlying MA changes in late AMD.

Faricimab Effectively Resolves Intraretinal Fluid and Preserves Vision in Refractory, Recalcitrant, and Nonresponsive Neovascular Age-Related Macular Degeneration.

PURPOSE: To evaluate the functional and anatomic outcomes of faricimab treatment in patients with neovascular age-related macular degeneration (nAMD) who are unresponsive to other anti-vascular endothelial growth factor (VEGF) therapies. METHODS: A retrospective interventional study was conducted on patients with refractory nAMD who were initially treated with intravitreal bevacizumab, ranibizumab, or aflibercept. These patients were switched to monthly faricimab injections. The central subfield thickness (CST), intraretinal fluid (IRF) or subretinal fluid (SRF) height, and visual acuities were compared before and after faricimab treatment. RESULTS: A total of 13 eyes (eight right eyes and five left eyes) from 11 patients were followed for 10.4 ± 6.9 months after bevacizumab treatment and 40.3 ± 28.7 months after aflibercept treatment before switching to faricimab. The follow-up time for patients receiving a mean number of 3.7 ± 1.3 faricimab injections was 3.4 ± 1.2 months. The overall median CST was reduced by 18µm (p=0.001) from 342µm to 318µm, along with a reduction of 89µm (p=0.03) in IRF/SRF height from 97µm to 40µm. Following three consecutive injections, the CST showed a significant reduction of 21.5µm (p=0.004) from 344µm to 322.5µm, and IRF/SRF height was reduced by 89µm (p=0.03) from 104µm to 18.5µm. The intraretinal fluid size decreased and leakage stopped, as seen on fluorescein angiography. Visual acuity remained stable after switching to faricimab treatment (0.59 ± 0.45 logMAR vs 0.58 ± 0.45 logMAR, p=1). CONCLUSIONS: Faricimab has proven to be an effective treatment for nAMD patients resistant to other anti-VEGF agents. It demonstrates significant anatomical improvement and vision preservation in this challenging patient population.

Pituitary Macroadenoma with Optic Cupping Masquerading as Normal Tension Glaucoma.

When non-glaucomatous disease with disc cupping mimics normal-tension glaucoma (NTG), diagnosis is challenging. The typical optic disc features of glaucomatous disease are often subjective, and often overlap with disc changes in compressive intracranial lesions. Ancillary diagnostic testing such as retinal nerve fiber layer (RNFL) analysis and visual field testing can elevate the index of suspicion of an underlying non-glaucomatous process. We present a case of a nonfunctional macroadenoma coexisting with NTG, although it is unclear if the concurrent brain lesion aggravated or caused it. This case highlights the diagnostic challenge of recognizing optic cupping and non-matching abnormalities in the visual field from a coexisting intracranial lesion, even in the absence of other neurological signs.

Fornix deepening reconstruction in conjunctivochalasis surgery.

PURPOSE: To assess the extent of inferior fornix shortening in conjunctivochalasis (CCh) and to evaluate whether fornix deepening reconstruction can restore the fornix tear reservoir in patients with CCh. MATERIALS AND METHODS: This was a retrospective review of five patients (3 unilateral and 2 bilateral eyes, total 7 eyes) with CCh who underwent fornix deepening reconstruction with conjunctival recession and amniotic membrane transplantation. Postsurgical outcome measures included changes in fornix depth with correlation to basal tear volumes, symptoms, corneal staining, and conjunctival inflammation. RESULTS: For the three patients with unilateral surgery, both the fornix depth (8.3 ± 1.5 mm) and wetting length (9.3 ± 8.5 mm) of the operative eyes were less than the fellow eyes (10.3 ± 1.5 mm and 10.3 ± 8.5 mm, respectively). At 5.3 ± 2.7 months (range 1.7-8.7) postoperatively, the fornix depth increased significantly by 2.0 ± 1.1 mm (P = 0.02). Deepening of the fornix depth was accompanied by overwhelming symptomatic relief (91.5%) that could be subdivided into complete relief (87.5%) and partial relief (4%) of symptoms, with blurred vision being the most notably relieved symptom (P = 0.03). Furthermore, superficial punctate keratitis and conjunctival inflammation were significantly improved at follow-up (P = 0.008 and 0.05, respectively). CONCLUSION: Deepening of the fornix to restore the tear reservoir is an important surgical objective that may change the tear hydrodynamic state to provide a stable tear film and improve outcomes in CCh.

Clinical outcome of diffractive multifocal lens versus monofocal lens in post-laser in situ keratomileusis patients: A retrospective, comparative study.

Purpose: To compare the clinical outcomes of diffractive multifocal and monofocal lenses in post-laser in situ keratomileusis (LASIK) patients who underwent cataract surgery. Methods: This was a retrospective, comparative study of clinical outcomes that was conducted at a referral medical center. Post-LASIK patients who underwent uncomplicated cataract surgery and received either diffractive multifocal or monofocal lens were studied. Visual acuities were compared at baseline and postoperatively. The intraocular lens (IOL) power was calculated with Barrett True-K Formula only. Results: At baseline, both groups had comparable age, gender, and an equal distribution hyperopic and myopic LASIK. A significantly higher percentage of patients receiving diffractive lenses achieved uncorrected distance visual acuity (UCDVA) of 20/25 or better (80 of 93 eyes, 86% vs. 36 of 82 eyes, 43.9%, P = 1.0 x 105) and uncorrected near vision of J1 or better (63% vs. 0) compared to the monofocal group. The residual refractive error had no significant difference (0.37 ± 0.39 vs. 0.44 ± 0.39, respectively, P = 0.16) in these two groups. However, more eyes in the diffractive group achieved UCDVA of 20/25 or better with residual refractive error of 0.25-0.5 D (36 of 42 eyes, 86% vs. 15 of 24 eyes, 63%, P = 0.032) or 0.75-1.5 D (15 of 21 eyes, 23% vs. 0 of 22 eyes, P = 1.0 x 10-5) compared to the monofocal group. Conclusion: This pilot study shows that patients with a history of LASIK who undergo cataract surgery with a diffractive multifocal lens are not inferior to those who receive monofocal lens. Post-LASIK patients with diffractive lens are more likely to achieve not only excellent near vision, but also potentially better UCDVA, regardless of the residual refractive error.

Successful management of chronic Blepharo-rosacea associated demodex by lid scrub with terpinen-4-ol.

PURPOSE: To report a successful treatment of chronic rosacea associated ocular demodicosis with lid scrub containing terpinen-4-ol (T4O). OBSERVATIONS: A 72-year old woman presented with recurrent and refractory ocular erythema, irritation, dryness, and photophobia despite conventional medical treatment (artificial tears, hypochlorous acid lid hygiene, doxycycline, and erythromycin) for 5 years. Examination revealed facial erythema, telangiectasias on cheeks, nose and lids, and cylindrical dandruff (CD) on bilateral upper and lower lashes. Epilation sampling confirmed demodicosis. After treatment with lid wipe containing T4O (Cliradex, Biotissue, Miami, FL) over face and lids, ocular discomfort, CD, facial and eyelid erythema, telangiectatic vessels were significantly reduced. Complete eradication of demodex mites and resolution of symptoms and signs lasted 8 months of follow-up. CONCLUSIONS: This case suggests that T4O is effective in treating chronic rosacea associated ocular demodex blepharitis.

Prevalence of Ocular Demodicosis in an Older Population and Its Association With Symptoms and Signs of Dry Eye.

PURPOSE: To evaluate associations between Demodex and its symptoms and ocular surface parameters in individuals with dry eye (DE). METHODS: A cross-sectional study of 119 individuals with DE symptoms or signs, grouped by the presence of ocular demodicosis, was performed. All individuals filled out questionnaires and underwent an ocular surface examination. Demographics, comorbidities, symptoms profiles, and ocular surface parameters were compared between the 2 groups. Multivariable regression analyses were used to determine which factors were associated with DE symptoms. Receiver operating curves analyses were performed to evaluate relationships between Demodex quantity and specific clinical phenotypes. RESULTS: Demodex was highly prevalent in our population (68.9%) with average counts of 4.2 ± 3.9. Individuals with and without Demodex had comparable demographics. Overall, symptoms profiles were similar between individuals with and without Demodex using the Dry Eye Questionnaire 5 and Ocular Surface Disease Index. Individuals with Demodex, however, were more likely to report itching (58.5% vs. 35.1%, P = 0.03), with increasing frequency in those with higher Demodex counts. Individuals with Demodex also had more severe eyelid abnormalities [cylindrical dandruff (CD) and lid vascularity] and a more unstable tear film but similar tear production and corneal staining when compared with individuals without Demodex. Neither Demodex nor ocular surface findings related to DE symptoms in multivariable analyses. Receiver operating curves analysis revealed Demodex counts ≥2 maximized sensitivity and specificity for both itching (64.7% and 59.0%, respectively) and CD (80.4% and 60.4%, respectively). CONCLUSIONS: Demodex is highly prevalent in an older population with DE. Symptom of itching and CD were the findings most specific for Demodex.

Self-retained cryopreserved amniotic membrane for treating severe corneal ulcers: a comparative, retrospective control study.

To compare the effectiveness of self- retained cryopreserved AM as an adjuvant therapy for infectious corneal ulcers. Retrospective, case-control study of 24 eyes of 24 consecutive patients with central and paracentral corneal infectious ulcers and initial visual acuity worse than 20/200. Among them, 11 eyes of 11 patients received additional placement of self-retained cryopreserved AM. Epithelialization and Best Corrected Snellen Visual Acuity (BCSVA) were compared between the two groups. At baseline, both groups had comparable age, gender, visual acuity (VA), size and location of corneal ulcer. Patients receiving additional placement of cryopreserved AM had significantly faster epithelialization within 3.56 ± 1.78 weeks vs 5.87 ± 2.20 weeks (p = 0.01) and achieved complete epithelialization in significantly more patients (72.7% vs 23.1% p = 0.04) despite overall larger baseline defect size (32.7 ± 19.5 mm2 vs 21.5 ± 10.7 mm2, p = 0.11). Consequently, the AM group had clinically significant BCSVA (> 3 lines) (81.8% vs 38.4%, p = 0.047) and total VA improvement (log MAR 0.7 ± 0.6 vs 1.6 ± 0.9, p = 0.016) compared to the control group at the time of complete epithelialization. In-office sutureless AM may be an effective adjuvant therapy in treating sight-threatening infectious corneal ulcers by promoting faster corneal epithelialization and overall better recovery of the VA.

Strategies in Surgical Decompression for Thyroid Eye Disease.

Surgical management of thyroid eye disease- (TED-) associated morbidity has been plagued by the complex interplay of different operative techniques. Orbital decompression is the well-recognized procedure for disfiguring exophthalmos and dysthyroid optic neuropathy (DON). There are numerous published techniques described for the removal of the orbital bone, fat, or a combination. The diverse studies are noncomparative as they include different indications, stages of disease, and methods of evaluation. Thus, it is difficult to conclude the most efficient decompression technique. To obtain effective and predictable results, it is therefore important to propose a logical and acceptable clinical guideline to customize patient treatment. Herein, we developed an algorithm based on the presence of DON, preoperative existing diplopia, and severity of proptosis which were defined by patient's disabling symptoms together with a set of ocular signs reflecting visual function or cosmesis. More specifically, we aimed to assess the minimal but effective surgical technique with acceptable potential complications to achieve therapeutic efficacy. Transcaruncular or inferomedial decompressions are indicated in restoring optic nerve function in patients with DON associated with mild or moderate to severe proptosis, respectively. Inferomedial or fatty decompressions are effective to treat patients with existing diplopia associated with mild or moderate to severe proptosis, respectively. Fatty or balanced decompressions can improve disfiguring exophthalmos in patients without existing diplopia associated with mild to moderate or severe proptosis, respectively. Inferomedial or 3-wall decompressions are preferred to address facial rehabilitation in patients associated with very severe proptosis but without preoperative diplopia.

Self-retained cryopreserved amniotic membrane in treating acute ocular graft-versus-host-disease (oGVHD).

PURPOSE: To report successful management of acute ocular graft-versus-host disease (oGVHD) by self-retained cryopreserved amniotic membrane (AM). OBSERVATIONS: A 69-year-old male developed acute oGVHD following hematopoietic stem cell transplantation (HSCT) with severe dryness, pain, photophobia, and blurred vision, the right eye worse than the left eye. Despite topical artificial tears, corticosteroids, and bandage contact lens (BCL) for two weeks, his right eye deteriorated with increasing redness and blurred vision and presented with diffuse conjunctival inflammation, severe superficial punctate keratitis, and corneal and limbal epithelial defect. Hence, self-retained cryopreserved AM was applied in the right eye while conventional therapy was continued in the left eye. Placement of self-retained AM for 3-days resulted in resolution of symptoms, reduction in inflammation, and complete re-epithelialization of the corneal and limbal defect with the visual acuity improving from 20/500 to 20/70 in the right eye. In contrast, the visual acuity improved from 20/300 to 20/150 and remained inflamed with conventional therapy in the left eye. One-month post-AM placement, the right eye remained asymptomatic with the visual acuity improved to 20/30 without any additional therapy, whereas the left eye improved to 20/70 with conventional treatment of BCL, loteprednol, and artificial tears. CONCLUSIONS AND IMPORTANCE: This case suggests application of self-retained AM can be an effective treatment to accelerate the restoration of vision and corneal surface health for acute oGVHD.

Severe Inflammation and Hyphema After Micropulse Diode Transscleral Cyclophotocoagulation.

PURPOSE: To describe vision-threatening complications after micropulse diode transscleral cyclophotocoagulation (MP-TSCPC). METHODS: Retrospective case series. Medical charts of patients who underwent MP-TSCPC and developed visually significant inflammation and hyphema after the procedure were reviewed. Patients were seen at the Upstate Medical University Ophthalmology clinic between 2017 and 2019. RESULTS: Out of 64 patients who underwent MP-TSCPC in the defined time period, 2 patients with postprocedure severe inflammation and hyphema were identified. One patient had severe-stage primary open-angle glaucoma (POAG). After MP-TSCPC, the patient was noted to have a large visually significant anterior chamber reaction and hyphema that occupied 80% of the anterior chamber on postprocedure day 1. The patient was treated with topical and oral corticosteroids, and topical atropine. The second patient had uncontrolled severe-stage POAG. The patient underwent MP-TSCPC. After the procedure, the patient was noted to have significant inflammation with fibrin and hyphema that filled 80% of the anterior chamber. The patient was treated with topical steroids. CONCLUSIONS: As with any intervention, complications are inevitable. Recognition of complications and its possible associations allow for better and more individualized risk versus benefit analysis of an intervention. In this case series, exuberant anterior segment inflammation and hyphema occurred in 2 patients who underwent MP-TSCPC. The authors' goal is to raise awareness of severe anterior chamber inflammation and hyphema as complications occurring after laser treatment with a reputation of minimal or no side-effect profile and to better understand this relatively new laser advancement in the treatment of glaucoma.

Effect of non-invasive intranasal neurostimulation on tear volume, dryness and ocular pain.

PURPOSE: To evaluate the effect of one TrueTear session on change in tear volume and symptoms of dryness and ocular pain. METHODS: Retrospective interventional case series of patients seen in a dry eye clinic. Seventy-five individuals underwent an ocular surface examination and one session of neurostimulation. Outcome measures included objective change in tear volume measured via phenol red test, and subjective change in sensations of dryness and ocular pain measured on a 0-10 Numerical Rating Scale. RESULTS: The mean age of the 75 individuals was 59±13 years, and the majority were male (73%). Intranasal neurostimulation increased tear volume (mean 13.40±8.00 mm, p<0.0005) and reduced intensities of dryness (mean -2.85±2.79, p<0.0005) and ocular pain (mean -1.48±2.41, p<0.0005 for both). However, these effects were independent of one another as change in symptom report did not correlate with change in tear volume (r=-0.13, p=0.25 for dryness; r=0.07, p=0.56 for pain). In a multivariable model, the strongest predictors for increased tear volume were lower baseline tear volume (standardised beta (ß)=-0.50, p<0.0005) and absence of an autoimmune disease (ß=-0.36, p=0.001) (R2=0.30). The strongest predictors for reduced dryness and pain scores were lower baseline dryness and ocular pain scores. No complications related to neurostimulation were noted. CONCLUSION: Intranasal neurostimulation increased tear volume and reduced intensities of dryness and ocular pain, independently of one another.

Pax 6 Controls Neural Crest Potential of Limbal Niche Cells to Support Self-Renewal of Limbal Epithelial Stem Cells.

On ocular surface, corneal epithelial stem cells (SC) reside in limbus between cornea and conjunctiva. Pax6, an evolutionally conserved transcription factor essential for eye development, is expressed in post-natal corneal and limbal epithelia progenitors (LEPC) but not in underlying stroma. Because Pax6 is transiently expressed in developing corneal stroma and a subset of limbal and corneal stromal progenitors, we examined the role of Pax6 in limbal niche cells (LNC) in maintaining the phenotype of neural crest (NC) progenitors to support LEPC. Our results showed that nuclear Pax6 staining was found in freshly isolated LNC but not corneal stromal cells. Serial passaged LNC resulted in gradual loss of nuclear Pax6 (46 kDa) staining and neural crest progenitor status defined by the expression of embryonic SCs and NC markers, neurosphere formation, and differentiation into neurons, oligodendrocytes and astrocytes. Gain of function of 46 kDa Pax6 in late-passaged LNC resulted in nuclear Pax6 staining and promotion of the aforementioned NC progenitor status. In an in vitro reunion assay, early passaged LNC and late passaged LNC with overexpression of Pax6 inhibited the expression of corneal epithelial differentiation marker and promoted holoclone by LEPC. Therefore, expression of nuclear 46 kDa Pax6 in LNC plays an important developmental role in maintaining NC progenitor status to support self-renewal of corneal epithelial SCs in the limbal niche.

IgG4-related Ophthalmic Disease in Idiopathic Sclerosing and Non-Sclerosing Orbital Inflammation: A 25-Year Experience.

Purpose: To determine the prevalence, clinical manifestations, and treatment outcomes of IgG4-related ophthalmic disease (IgG4-ROD) in previously diagnosed idiopathic orbital inflammation (IOI).Materials and Methods: Thirty one surgically treated patients with IOI between 1990 and 2015 were divided into sclerosing (SIOI) and non-sclerosing (NSIOI) to identify IgG4-ROD. Preserved pathological specimens were immunostained for IgG4 cells. Changes in clinical features were evaluated before and after treatment.Results: Out of the 31 patients, 15 (48.4%) had IgG4-ROD, consisting of 7 of the 16 SIOI (43.8%) and 8 of the 15 NSIOI patients (53.3%). Among 15 patients with IgG4-ROD, 4 (26.7%) achieved complete remission, which was significantly less than among IgG4-unrelated patients (11/16, 68.8%, p = .03). Furthermore, 3 out of 15 IgG4-ROD patients (20%) experienced recurrence, all of whom had SIOI, compared to 0% among IgG4-unrelated patients (p = .1).Conclusions: IgG4-ROD is common among previously identified IOI in our study. Treatment response is modest in IgG4-ROD patients.