Keratoconus in a patient with Alport syndrome: A case report

BACKGROUND Known ocular manifestations of Alport syndrome include features such as anterior lenticonus and fleck retinopathy. Reports of keratoconus in such patients are limited. We report tomographic findings consistent with keratoconus in a patient with Alport syndrome.CASE SUMMARY A 52-year-old female was referred to our ophthalmology clinic with decreased vision and increased tearing. She was diagnosed with stage Ⅲ Alport syndrome two years prior. Upon examination she was found to have average keratometries of 48D bilaterally with tomographic evidence of keratoconus.CONCLUSION Although a rare presentation, concurrent Alport syndrome and keratoconus should be considered when reviewing the ocular health of Alport syndrome patients and appropriate management steps should be taken upon the diagnosis.

Pseudophacomorphic Glaucoma Along with Pupillary Block after Visian^TMImplantable Collamer Lens Implantation for High Myopia

Purpose: To report a case of bilateral glaucoma related to pseudophacomorphic mechanism in one eye and pupillary block in the other eye after Visian Implantable Collamer Lens (ICL;STAAR Surgical) insertion. Methods: A 44-year-old female with high myopia underwent bilateral ICL implantation of MICL12.6 after sulcus diameter measurements were performed by Pentacam. Results: Pseudophacomorphic glaucoma-related angle closure occurred due to lens oversizing in the right eye. The mechanism was relieved via ICL explantation. In the left eye, pupillary block developed in a subacute manner after closure of the Peripheral Iridotomy (PI). The attack was ameliorated by reestablishing patency of the iridotomy. Conclusions: ICL-related glaucomatous attacks may result from improper sizing as well as from placement of a single PI. Identification of the proper mechanism is vital as treatments differ significantly. In pseudo phacomorphic glaucoma, explantation is needed. In pupillary block glaucoma, treatment involves establishment of a patent PI.

Astigmatic correction with implantation of a light adjustable vs monofocal lens: a single site analysis of a randomized controlled trial

AIM: To evaluate the light adjustable lens(LAL) vs a standard monofocal lens in achieving target astigmatic refraction and improving postoperative uncorrected distance visual acuity(UDVA). METHODS: This randomized controlled clinical trial included 40 patients with pre-existing astigmatism and visually significant cataract. Twenty-eight patients received the LAL and 12 control patients received a monofocal intraocular lens(IOL) after cataract extraction at a single institution. The patients with the LAL underwent adjustment by ultraviolet(UV) light postoperatively plus subsequent lock-in procedures and all patients returned to clinic for follow up of study parameters at 6, 9, and 12 mo. Manifest refraction, distance visual acuity, and adverse events were recorded at each visit.RESULTS: The mean cylinder before adjustment in eyes with the LAL was-0.89±0.58 D(-2.00 to 0.00 D) and-0.34±0.34 D(-1.25 to 0.00 D) after lock-in(P=1.68 x10-8). The mean cylinder in patients with the monofocal lens was-1.00±0.32 D(-1.50 to-0.50 D) at 17-21 d postoperatively, which was statistically different from the LAL cylinder postlock-in(P=1.43 x10-6). UDVA in the LAL group was 20/20 or better in 79% of patients post lock-in with good stability over 12 mo compared with 33% of the control patients with UDVA of 20/20 or better. CONCLUSION: These results demonstrate that the LAL is more effective in achieving target refractions and improving postoperative UDVA in patients with pre-existing corneal astigmatism than a standard monofocal lens.

Comparison of FDA safety and efficacy data for KAMRA and Raindrop corneal inlays

AIM：To provide a side-by-side analysis of the summary of safety and effectiveness data（SSED）submitted to the FDA for the KAMRA and Raindrop corneal inlays for the correction of presbyopia.METHODS：SSED reports submitted to the FDA for KAMRA and Raindrop were compared with respect to loss of corrected distance visual acuity（CDVA）,adverse event rates,induction of astigmatism,retention of contrast sensitivity,stability of manifest refractive spherical equivalent（MRSE）,and achieved monocular uncorrected near visual acuity（UNVA）at 24mo.RESULTS：Totally 442/508 of KAMRA patients and344/373 Raindrop patients remained enrolled in the clinical trials at 24mo.The proportion of KAMRA and Raindrop patients who lost≥2 lines of CDVA at 24mo was 3.4%and1%,respectively.The adverse event rate was comparable between the devices.No significant inductions of astigmatism were noted.Both technologies induced a transient myopic shift in MRSE followed by a hyperopic shift and subsequent stabilization.Totally 87%of KAMRA and 98%of Raindrop patients attained a monocular UNVA of J5（20/40）or better at 24mo,28%of KAMRA and 67%of Raindrop patients attained a monocular UNVA of J1（20/20）or better at 24mo.CONCLUSION：Both devices can be considered safe and effective,however,the results of corneal inlay implantation are mixed,and long-term patient satisfaction will likely depend on subjective expectations about the capabilities of the inlays.Variability in surgical technique and postoperative care within and between the two clinical trials diminishes the comparative power of this article.

Teaching in the operating room:trends in surgical skills transfer in ophthalmology

Cataract surgery is arguably the most commonly performed operation in ophthalmology.Surgical skills transfer from experienced surgeons to resident surgeons is complicated by the fact that the teaching surgeon primarily acts as an observer rather than directly performing the procedure.Therefore,wet lab and simulator training are utilized to reduce the learning curve of the novice surgeons,which establishes tissue awareness,dexterity and muscle memory required to perform each step of the procedure,safely.Access to a wet lab and simulator environment is accomplished by establishing a surgical training curriculum in residency programs.In the operating room,topical anesthesia is a safe alternative for teaching cataract surgery.There are three well-described approaches to teaching individual steps of cataract surgery:forward,“backwards”,and deconstructed step-by-step instruction.Simulator training can be incorporated prior to live patient experience or integrated concurrently with learner presence in the operating room.The trend towards a competency-based instruction model has necessitated appropriate evaluation tools that include Objective Assessment of Skills in Intraocular Surgery(OASIS),Global Rating Assessment of Skills in Intraocular Surgery(GRASIS),and the International Council of Ophthalmology’s Ophthalmology Surgical Competency Assessment Rubrics(ICO-OSCAR).We review the literature on trends in surgical teaching in ophthalmology,with the focus on cataract surgery instruction to the novice surgeon.

埋藏性视神经玻璃膜疣所致的视野缺损和视网膜神经纤维层缺失

PURPOSE: To evaluate the visual field with the use of automated perimetry and to evaluate the retinal nerve fiber layer (RNFL) with optical coherence tomography (OCT) in patients with buried optic nerve drusen (OND). DESIGN: Observational case control study. METHODS: Eyes with buried OND were defined as eyes with ultrasound-proved drusen that were not visible with indirect slit-lamp biomicroscopy. All eyes underwent automated perimetry. Some eyes underwent OCT to evaluate the RNFL. RESULTS: Fifty-eight eyes of 41 patients with buried OND were evaluated. Three eyes (5%) had inferior arcuate scotomas. The other 55 eyes did not have visual field defects. Twenty-one of the eyes without visual field defects underwent RNFL analysis with OCT. All 21 eyes had normal average RNFL thickness. Some eyes had focal RNFL defects, but it is not clear whether these defects were clinically significant. CONCLUSION: Visual field defects are uncommon in eyes with buried OND. Eyes with buried OND may have focal RNFL defects but have normal average RNFL thickness. In patients with buried OND and a visual field defect, consideration should be given to searching for other causes of the defect, especially if the defect is substantial.

星状玻璃体变性患者眼内3片式硅树脂人工晶体表面钙化:临床病理报道

Objective: To illustrate the laboratory findings in a patient with bilateral asteroid hyalosiswho presentedwith calcified deposits on a 3- piece silicone intraocular lens (IOL). Design: Observational case report. Methods: A 76- year- old diabetic woman underwent uneventful cataract surgery in 1994 with implantation of a silicone- optic polypropylene- haptic IOL in the left eye. A neodymium:yttrium- aluminum- garnet (Nd:YAG) laser posterior capsulotomy was performed 2 years after cataract surgery, but persistent whitish deposits were observed on the posterior optic surface of the lens. Over the next 3 years, the opacification increased in the region corresponding to the capsulotomy. The IOL was explanted/exchanged. The right eye had cataract surgery in 1995. The acrylic lens implanted in this eye developed no opacities after 6 years. Main Outcome Measures: Gross, microscopic, and surface analyses of the explanted IOL. Results: Gross and light microscopic analyses revealed the presence of confluent crustlike deposits on the central area of the posterior optic surface, as well as Nd:YAG pits. Individual spherules exhibiting a Maltese- cross pattern under polarizing light were also observed. Energy dispersive x- ray spectroscopic analyses demonstrated the composition of the confluent deposits to be similar to hydroxyapatite. Conclusions: An association between asteroid hyalosis and dystrophic calcification of IOLs has been reported only with silicone plate haptic designs. The material opacifying the 3- piece silicone lens probably was derived from the asteroid bodies or from the process that results in this vitreous condition. Clinical evaluation of other pseudophakic patients with asteroid hyalosis will confirm if this phenomenon is restricted to silicone IOLs.

Phlyctenulosis-Like Presentation Secondary to Embedded Corneal Foreign Body

Case Presentation: A nine-year-old boy presented to the general ophthalmologist with a several weeks history of redness, photophobia and intermittent foreign body sensation in the right eye. A pigmented lesion with anterior chamber inflammation was noted on examination. B-scan ultrasound was performed and revealed no foreign body. The patient was diagnosed with anterior uveitis, which did not completely respond to treatment. The differential diagnosis was expanded to include peripheral ulcerative keratitis, phlyctenulosis, pigmented neoplasm, and corneal foreign body. Upon referral to a cornea specialist, an exam under anesthesia revealed a large foreign body consistent with a rock fragment in the peripheral cornea, which was subsequently removed without complication. Conclusion: This case highlights an atypical presentation of foreign body as well as a differential diagnosis of pigmented peripheral corneal lesions. Foreign bodies represent the most common cause of urgent ophthalmic evaluation. When evaluating lesions of the cornea, it is imperative to keep an extensive differential diagnosis, giving the potential for severe and rapid development of visually threatening complications.

Diagnosis and management of intraocular lymphoma:a narrative review

Background and Objective:Intraocular lymphoma(IOL)is a heterogenous category of rare malignancies that are often misdiagnosed and underrecognized.The rarity of IOL impedes clinical research and contributes to difficulty in standardizing its management.In this article we review the existing scientific literature to identify the current diagnostic tools and discuss comprehensive management of various categories of IOL.Our objective is to increase disease recognition of IOL as a whole and explore updated management options for each subtype.Methods:PubMed and Embase were searched for publications using the terms‘intraocular lymphoma’,‘vitreoretinal lymphoma’,‘uveal lymphoma’,‘iris lymphoma’,‘choroidal lymphoma’and‘ciliary body lymphoma’published from 1990 to June 2021.Inclusion criteria were English language articles.Exclusion criteria were non-English language articles,case reports and animal studies.Key Content and Findings:IOL often presents in middle-aged and older patients with symptoms of floaters and vision changes,but a broad array of clinical signs and symptoms are possible depending upon subtype.IOL can be subdivided by location of involvement into vitreoretinal and uveal lymphoma.These subtypes express key differences in their pathophysiology,clinical presentation,histology,prognosis,and treatment.Primary vitreoretinal lymphomas(PVRL)generally originate from B-lymphocytes and are associated with central nervous system(CNS)lymphoma.Ophthalmic findings include retinal pigment epithelium changes with yellow subretinal deposits known as“leopard spotting.”Primary uveal lymphomas generally originate from low-grade B-lymphocytes invading the choroid and carry an improved prognosis compared to vitreoretinal lymphomas.Funduscopic findings of primary uveal lymphoma include yellow to pink-yellow choroidal swelling with infiltrative subconjunctival“salmon-patch”lesions.Diagnosis for IOL is often delayed due to insidious onset,low prevalence,and tendency to mimic diseases such as uveitis.Diagnosis may be challenging,often relying on biopsy with specialized laboratory testing for confirmation of IOL.Optimal treatment regimens are currently debated among experts.Management of IOL is best coordinated in association with neuro-oncology clinicians due to the tendency for intracranial involvement.Conclusions:IOL represents a group of multiple malignancies with distinct clinicopathologic features.Future outlook for treatment and prognosis of IOL is likely to improve with less invasive molecular diagnostic techniques and increased awareness.Clinicians should be circumspect in all patients with possible IOL and promptly refer to oncologic specialists for rapid evaluation and treatment.

Conjunctival flaps for the treatment of advanced ocular surface disease—looking back and beyond

Conjunctival flaps have previously proven to be effective in preserving the globe for individuals with severe ocular surface disease.Infectious keratitis,neurotrophic keratitis,nontraumatic corneal melts,descemetoceles,perforations,and corneal burns are all indications for this procedure.The flaps promote nutrition,metabolism,structure,and vascularity,as well as reduce pain,irritation,inflammation,and infection.Furthermore,patients avoid the emotional and psychological repercussions of enucleation or evisceration,while requiring fewer postoperative medications and office visits.Currently,fewer flaps are performed due to the emergence of additional therapeutic techniques,such as serum tears,bandage lenses,corneal grafting,Oxervate,amniotic membrane,and umbilical cord grafting.However,despite newer conservative medical methods,conjunctival flaps have been demonstrated to be useful and advantageous.Moreover,future technologies and approaches for globe preservation and sight restoration after prior conjunctival flaps are anticipated.Herein,we review the history,advantages,and disadvantages of various surgical techniques:Gundersen’s bipedicle flap,partial limbal advancement flap,selective pedunculated conjunctival flap with or without Tenon’s capsule,and Mekonnen’s modified inferior palpebral-bulbar conjunctival flap.The surgical pearls and recommendations offered by the innovators are also reviewed,including restrictions and potential complications.Procedures for visual rehabilitation in selective cases after conjunctival flap are reviewed as well.

缝合前部撕囊的裂口

Aim:To describe a new technique of suturing a tear in the anterior capsulorhexis.Methods:Continuous curvilinear capsulorhexis(CCC)with lens removal was done in five fresh cadaver eyes.The diameter of the CCC was measured with a calliper.Using the same calliper a tear of the CCC was created while opening the calliper’ s arms.The distance between the calliper’ s arms needed to tear the CCC was documented.Using 9-0 Ethilon 9011,CS 160-6 sutures in two eyes,9-0 Prolene,D-8229,CTC-6L sutures in two eyes,and 10-0 Prolene,9090,CTC-6 suture in one eye,the tears were sutured.A tear in the CCC was created again in the same way as the first tear.The distance between the calliper’ s arms needed to tear the CCC was documented again.Results:Suturing of the tear restored some of the strength/elasticity of the CCC.Better results were found while using the 9-0 Prolene,D-8229,CTC-6L sutures than with the two others sutures.Conclusions:Suturing of a broken CCC can restore at least some of the strength/elasticity of the CCC.This can be important before intraocular lens(IOL)implantation for the safety of the implantation or after the implantation to ensure proper fixation of the IOL.

Epidemiology of age-related macular degeneration (AMD): associations with cardiovascular disease phenotypes and lipid factors

Age-related macular degeneration(AMD)is the leading cause of irreversible blindness in adults over 50 years old.Genetic,epidemiological,and molecular studies are beginning to unravel the intricate mechanisms underlying this complex disease,which implicate the lipid-cholesterol pathway in the pathophysiology of disease development and progression.Many of the genetic and environmental risk factors associated with AMD are also associated with other complex degenerative diseases of advanced age,including cardiovascular disease(CVD).In this review,we present epidemiological findings associating AMD with a variety of lipid pathway genes,cardiovascular phenotypes,and relevant environmental exposures.Despite a number of studies showing significant associations between AMD and these lipid/cardiovascular factors,results have been mixed and as such the relationships among these factors and AMD remain controversial.It is imperative that researchers not only tease out the various contributions of such factors to AMD development but also the connections between AMD and CVD to develop optimal precision medical care for aging adults.