The Potential Role of the Light Adjustable Lens in Flanged Intrascleral Haptic Fixation.

PURPOSE: To compare maximum tensile strength between commonly used 3-piece intraocular lens (IOL) for flanged intrascleral haptic fixation (FISHF). SETTING: Willis-Knight Eye Institute. DESIGN: Laboratory investigation. METHODS: Haptic tensile strength was compared with MA60AC, CT Lucia 602, AR40E, and the light-adjustable lens (LAL). Haptic strength with a 24D (diopter) IOL was compared across all lenses, as well as a range of 10-30D with the MA60AC. A custom device was created to hold the IOL in correct haptic orientation. The max tension (mean +/- standard deviation) was recorded in newtons (N) when the haptic lost tension or broke. RESULTS: CT Lucia was the strongest at 1.53 +/- 0.11 N vs 1.00 +/- 0.15 (MA60AC), 0.87 +/- 0.19 (AR40E), and 0.83 +/- 0.14 N (LAL), P<0.001. The LAL and AR40E were similar to a 9-0 polypropylene suture while being significantly stronger than 10-0 polypropylene suture, P<0.001. No difference in haptic tension for the MA60AC from 10-30D, P>0.05. High magnification revealed the highest haptic fractures for MA60AC at 40% compared to LAL, AR40E, and CT Lucia at 0%. CT Lucia and AR40E had 100% of haptics disinserted from the IOL without any damage compared to 60% LAL and 60% MA60AC. CT Lucia, AR40E, and LAL have a flatter haptic angulation at 5 degrees. CONCLUSION: Haptic strength, durability, and angulation of the LAL may support the possibility of FISHF in the hands of experienced surgeons. However, further testing is strongly recommended to verify if physiologic conditions or light treatments may compromise long-term haptic stability.

Preventing the Argentinian flag sign and managing anterior capsular tears: A review.

The Argentinian flag sign (AFS) is a feared complication during cataract extraction. Intralenticular pressures, especially excessive posterior pressure, have been identified as potential mechanisms for capsular stress and tearing associated with AFS. Capsular tension is created by positive intralenticular pressures, which cause the irido-lens diaphragm to move anteriorly once the manual capsulorhexis has been initiated. This tension can cause inadvertent tears that self-propagate to the lens equator, causing an AFS, among other intraoperative complications. Thus, this review highlights the importance of identifying intumescent cataracts as well as a combination of techniques to relieve intracapsular pressures needed to prevent AFS. However, some instances of anterior capsular tears are unavoidable. Therefore, focus will also be placed on techniques during cataract extraction used to manage anterior capsular tears, mitigating extension to the posterior capsule.

The Role of Early Nd:YAG Laser Capsulotomy in Improving Visual Performance in Mild to Moderately Symptomatic Trifocal Patients.

Purpose: To determine the effect of early Nd:YAG (neodymium:yttrium-aluminum-garnet) laser capsulotomy on objective and subjective visual quality in symptomatic trifocal intraocular lens (IOL) patients. Methods: A single-center, prospective study examined symptomatic patients after bilateral cataract extraction with trifocal IOL implantation. A ten-question survey was conducted one month after surgery. Study endpoints included the assessment of monocular and binocular uncorrected distance visual acuity (UDVA), uncorrected near visual acuity (UNVA), contrast sensitivity (CS), and subjective visual quality before and after Nd:YAG capsulotomy prior to 4 months after cataract surgery. Results: A total of 38 eyes from 21 patients were included with a TFAT00 (n = 23) or TFAT30-60 (n = 15). Overall satisfaction with the IOL was 8.55 ± 1.77 (range 5-10). A Nd:YAG capsulotomy was performed at 55 ± 26 days. Monocular UDVA and UNVA > 20/25 before Nd:YAG were 53.0% and 42.0%, which improved post-Nd:YAG to 63.0% and 66.0%, respectively (P = 0.41, P = 0.051). Binocular UDVA and UNVA >20/25 before Nd:YAG were 82.0% and 63.0%, which increased to 97% and 97%, respectively (P < 0.05, P < 0.001). CS increased in all post-Nd:YAG capsulotomies (P < 0.01). The presence of glare was documented at 74% pre-Nd:YAG, which decreased to 41% post-Nd:YAG (P < 0.01). Glare which limited activities was documented at 24%, which decreased to 5% post-Nd:YAG (P = 0.21). Conclusion: Early treatment of posterior capsule opacities in mild to moderately dissatisfied trifocal IOL patients may be beneficial in improving CS, visual quality, and reducing the presence and severity of dysphotopsias.

Clinical Features Associated with Acute Elevated Intraocular Pressure After Intravitreal Anti-VEGF Injections.

Purpose: To study the effects of intravitreal injection (IVI) of anti-VEGF (vascular endothelial growth factor) agents on intraocular pressure (IOP) and find associations with acute pressure spikes. Methods: This was a three-month, prospective study of patients receiving outpatient IVI of anti-VEGF agents for diabetic retinopathy (DR), age-related macular degeneration (AMD), and retinal vein occlusion (RVO) at the Acuity Eye Group Medical Centers. IOP was measured pre- and post-injection at 10-minute intervals up to 50 minutes after injection with a handheld tonometer. Patients with an IOP greater than 35 mmHg at 30 minutes received an anterior chamber paracentesis (ACP), while patients below 35 mmHg were monitored without intervention. Results: A total of 617 patients (51% female, 49% male) received IVI for DR (n = 199), AMD (n = 355), and RVO (n = 63). ACP was performed in 17 patients. Average pre-injection IOP was 16 ± 4 compared to 24 ± 7 mmHg for the non-ACP vs ACP group, respectively (mean ± standard deviation), p < 0.0001. IOP returned to baseline in 98% of patients at 50 minutes. A diagnosis of glaucoma and glaucoma suspect was more prevalent in the ACP group compared to the non-ACP group, 82.3% vs 14.2% and 17.6% vs 9.0%, respectively, p < 0.0001 and p > 0.05. Patients with a pre-injection IOP >25 mmHg and a history of glaucoma had a 58.3% rate of ACP. A 31-gauge needle had a higher mean increase in IOP from baseline compared to 30-gauge needle, p < 0.0001. Conclusion: IOP spikes are most significant in the first 10 minutes after IVI but typically resolve within the first hour. However, utilizing a smaller 31-gauge IVI in patients with a glaucoma history and pre-injection IOP >25 mmHg may be associated with significant IOP spikes lasting longer than 30 minutes.

The Role of Multifocal Intraocular Lens Implantation in Extreme Axial Myopia: A Case Report and Literature Review.

The prevalence of high myopia is rising globally. In addition to an increased risk of retinal detachment, high myopia is associated with earlier cataract formation. Patients with myopia are also often more motivated to become spectacle-independent after a lens procedure. However, the use of multifocal intraocular lens (MfIOL) remains controversial for patients with extreme myopia, which is classified as patients with an axial length >28 mm. Here, we present the case of a 64-year-old patient with visually significant cataract and extreme axial myopia >31 mm in both eyes who desired to be spectacle-independent. A preoperative workup revealed a normal macula with peripheral lattice degeneration. On optical coherence tomography, the macula had a normal fovea contour without the presence of a staphyloma. A thorough peripheral examination was performed by a retina specialist which required no prophylactic treatment. Pentacam analysis demonstrated a low spherical aberration and minimal ectasia risk. Cataract surgery was uneventful with a 5 mm laser capsulotomy centered over the visual axis with the placement of a trifocal intraocular lens. Two months after the cataract surgery, the remaining refractive error was corrected with a laser-assisted in situ keratomileusis enhancement. The patient achieved an uncorrected distance visual acuity of 20/15- and uncorrected near visual acuity of J1+ in both eyes. Overall, this case report and review aims to highlight important preoperative, intraoperative, and postoperative techniques to improve patient outcomes with MfIOL in patients with extreme myopia.

Activation of Ocular Syphilis After Small-Incision Lenticule Extraction.

A 41-year-old female presented to the Willis-Knighton Eye Institute to undergo evaluation for refractive surgery. The patient had a best-corrected visual acuity for a distance of 20/15-1 of the right eye (OD) and 20/15-1 of the left eye (OS) with a manifest refraction of -2.75 OD and -1.75 OS. Near visual acuity was J1+ in both eyes (OU). A trial of a monovision contact lens was successful with the dominant eye selected for distance. The patient was then planned for small-incision lenticule extraction (SMILE) OD only with a plano target. SMILE was performed and was uncomplicated with uncorrected visual acuity of 20/15- on postoperative day one. Two weeks after the initial SMILE procedure, the anterior segment was notable for 1-2+ cells OD. Topical prednisone was changed to difluprednate 0.05% TID OD with improvement in symptoms. However, the anterior chamber cell was never fully resolved by month three. A systemic workup revealed a positive rapid plasma reagin with 1:64 titer and a positive fluorescent treponemal antibody absorption in a patient never treated for syphilis. The patient was diagnosed with ocular syphilis and received a two-week course of intravenous penicillin G. A slow topical prednisone tapper was performed with the resolution of inflammation by one year. Anterior uveitis after refractive surgery is uncommon. The incidence of anterior uveitis after SMILE is even rarer with no previously documented incidence in the literature. As a result, persistent cell seen in refractive procedures, especially SMILE, is a concerning finding, warranting further workup to rule out underlying systemic diseases including syphilis.

Bilateral Cavernous Sinus Thrombosis in Presumed COVID-19 Infection.

Cavernous sinus thrombosis (CST) is a rare life-threatening condition where a blood clot develops within the cavernous sinus secondary to various etiologies, ranging from infection to aseptic causes (e.g., trauma or surgery). The dural sinuses and the cerebral veins have no valves, which allow retrograde blood flow according to pressure gradients. As a result, cavernous sinuses are vulnerable to septic thrombosis from infection at various sites including sphenoid and ethmoid sinuses. Less commonly, infections of the face, ears, nose, tonsils, soft palate, and teeth may lead to CST if treatment is delayed. Clinical findings of CST extending to the opposite cavernous sinus typically requires 24-48 hours after the initial presentation of orbital signs. However, we present a patient with facial and orbital cellulitis that was immediately treated with high-dose IV antibiotics within one hour of presentation and IV heparin six hours after admission and CST diagnosis. However, the patient developed a rapid progression of bilateral CST within six hours, unresponsive to treatment. Although facial cellulitis may lead to septic CST if untreated, the rapid progression of bilateral CST in the setting of acute hypoxic respiratory failure, renal failure, and coagulation abnormalities suggests a possible underlying infection and complications similar to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection.

Sterile iris abscess associated with herpes zoster ophthalmicus.

PURPOSE: To report a case of a sterile iris abscess associated with herpes zoster ophthalmicus (HZO). OBSERVATIONS: A 69-year-old African American female presented to SUNY Downstate Medical Center complaining of left-sided eye pain for two weeks. The patient had a best-corrected visual acuity of 20/30 OD and 20/200 OS. On external exam, vesicles were noted on the left upper lid and tip of the nose. Anterior segment exam was notable for decreased sensation without epithelial defects. The patient had 2+ stromal edema with 3+ cell and flare. The iris was flat with 1+ nuclear sclerosis OU. The intraocular pressure (IOP) was 14 mmHg OD and 40 mmHg OS. The patient was diagnosed with HZO with secondary uveitic glaucoma.At ten weeks, anterior segment inflammation resolved and IOP stabilized. However, an iris nodule was noted superior nasal which continued to enlarge by 16 weeks follow up. Iris ultrasound revealed a 3 × 3 mm elevated lesion with internal homogeneity suggestive of an abscess.At five months, a dense, mature cataract developed. The patient underwent cataract extraction with sector iridectomy. Gram staining and cultures were negative for organisms but positive for polymorphic neutrophils. Histopathology revealed fibrosis, surface necrosis, and stromal infiltration with chronic inflammatory cells consistent with chronic iritis and a sterile abscess secondary to HZO. CONCLUSIONS AND IMPORTANCE: HZO is associated with a range of ocular sequelae with acquired iris nodule only mentioned once in the literature. As the second documented case, our findings will add to the general fund of knowledge regarding iris lesions and HZO.

Case report: The use of netarsudil to improve corneal edema after laser peripheral iridotomy and Descemet's membrane endothelial keratoplasty.

PURPOSE: To report a case in which netarsudil ophthalmic solution 0.02% improved refractory corneal edema after laser peripheral iridotomy (LPI) and Descemet's membrane endothelial keratoplasty (DMEK). OBSERVATIONS: A 63-year-old female presented with decreased vision due to corneal edema secondary to iatrogenic endothelial cell loss from previous YAG and argon laser peripheral iridotomy. Initial treatment with topical sodium chloride 5% solution was unsuccessful in resolving the edema. As a result, topical netarsudil was initiated off-label. Improvement in corneal thickness and visual acuity was noted, but after a few months, the left eye decompensated with worsening edema. Cataract surgery with DMEK was performed. Surgery was prolonged and intraoperative floppy iris was encountered. Post-operatively, the patient's best-corrected visual acuity (VA) fluctuated between 20/30 to 20/70 with persistent corneal edema. The central corneal thickness (CCT) ranged from 758 to 779 three months after surgery. Topical netarsudil was started again off-label for cornea edema once nightly. Over the next two months, visual acuity and CCT improved to 20/25 and 650, respectively. Stabilization of visual acuity and cornea edema has been maintained for eight months after initiation of topical netarsudil. CONCLUSIONS: Netarsudil, a commercially available rho-kinase inhibitor, may be an effective, non-invasive adjunctive therapy for refractory corneal edema. Our case demonstrates improvement in BCVA and CCT using topical netarsudil, which has been maintained without any vision threatening side effects.

Transient Reticular Cystic Corneal Epithelial Edema With Topical Netarsudil: A Case Series and Review.

PURPOSE: To describe cases in which netarsudil ophthalmic solution 0.02% precipitated reversible, reticular cystic epithelial edema. METHODS: A retrospective case review at the Brooklyn Veteran's Association Hospital of patients with corneal stromal edema that were treated with netarsudil and subsequently developed cystic epithelial edema. RESULTS: Four male patients with a mean age of 72 ± 8.0 years developed a reticular, honeycomb-like pattern of epithelial edema located in the interpalpebral and inferior cornea. In 3 of 4 patients, epithelial edema arose within 1 month compared with 2 months in 1 patient. New epithelial cysts did not correlate with worsening central corneal thickness and best spectacle-corrected visual acuity in every patient, which was likely due to the location of the cysts. Two of 4 patients developed increased central corneal thickness with worsening best spectacle-corrected visual acuity. In comparison, 1 patient had improvement in both parameters, whereas 1 patient had no significant change. In all cases, there was resolution of the epithelial cysts after discontinuation of netarsudil. CONCLUSIONS: Although rho-kinase inhibitors have been suggested to improve endothelial function, we have documented worsening epithelial cysts in a subset of patients with pre-existing corneal edema. These effects of netarsudil were transient and resolved after discontinuing treatment within 2 weeks in most patients. We hypothesize that the incidence of this adverse finding is more common than previously believed. Nevertheless, large-scale studies are needed to accurately report on the incidence and clinical significance of this novel finding.

Impact of posterior vitreous detachment on contrast sensitivity in patients with multifocal intraocular lens.

PURPOSE: To investigate posterior vitreous detachment (PVD) and pars plana vitrectomy (PPV) effects on contrast sensitivity function (CSF) in patients with a multifocal intraocular lens (MfIOL). METHODS: This single-center prospective case-control study analyzed 27 patients with 43 consecutive eyes. Twenty patients with 36 consecutive eyes received MfIOL implantation with either ZLB00 or ZMB00. CSF was measured as the area under the log contrast sensitivity function (AULCSF) in the presence and absence of PVD (PVD+ group and PVD- group, respectively). Seven eyes associated with a symptomatic PVD and severe visual dissatisfaction after MfIOL implantation underwent PPV (symptomatic PVD+ group). CSF was measured prior to and after PPV. RESULTS: The mean AULCSF was significantly lower in the PVD+ group (1.5 ± 0.1) versus the PVD- group (1.7 ± 0.1, p < 0.0001). Major complaints in the symptomatic PVD+ group included floaters (n = 2) and blurry vision (n = 5). The preoperative AULCSF (1.4 ± 0.1) was significantly lower in the symptomatic PVD+ group versus the PVD- group (p < 0.0001) and PVD+ group (p = 0.02). The preoperative AULCSF in the symptomatic PVD+ group was significantly improved after PPV (1.4 vs. 1.7, respectively, p = 0.002). CONCLUSIONS: PVD significantly decreased CSF in patients with MfIOL. Patients with symptomatic PVD exhibited the greatest decrease in CSF, which was significantly improved after PPV. Measurement of CSF and careful assessment of PVD may be useful in determining the appropriateness of surgical intervention for improving visual performance and satisfaction in MfIOL patients with symptomatic PVD.

Fibrin hydrogels are safe, degradable scaffolds for sub-retinal implantation.

Retinal pigment epithelium (RPE) transplantation for the treatment of macular degeneration has been studied for over 30 years. Human clinical trials have demonstrated that RPE monolayers exhibit improved cellular engraftment and survival compared to single cell suspensions. The use of a scaffold facilitates implantation of a flat, wrinkle-free, precisely placed monolayer. Scaffolds currently being investigated in human clinical trials are non-degradable which results in the introduction of a chronic foreign body. To improve RPE transplant technology, a degradable scaffold would be desirable. Using human fibrin, we have generated scaffolds that support the growth of an RPE monolayer in vitro. To determine whether these scaffolds are degraded in vivo, we developed a surgical approach that delivers a fibrin hydrogel implant to the sub-retinal space of the pig eye and determined whether and how fast they degraded. Using standard ophthalmic imaging techniques, the fibrin scaffolds were completely degraded by postoperative week 8 in 5 of 6 animals. Postmortem histologic analysis confirmed the absence of the scaffold from the subretinal space at 8 weeks, and demonstrated the reattachment of the neurosensory retina and a normal RPE-photoreceptor interface. When mechanical debridement of a region of native RPE was performed during implantation surgery degradation was accelerated and scaffolds were undetectable by 4 weeks. These data represent the first in situ demonstration of a fully biodegradable scaffold for use in the implantation of RPE and other cell types for treatment of macular degeneration and other retinal degenerative diseases.

Recurrent Herpes Zoster Ophthalmicus Preceded by Anabolic Steroids and High-Dose L-Arginine.

PURPOSE: To report a case of a 34-year-old male with recurrent herpes zoster ophthalmicus (HZO) preceded by a 6-week cycle of anabolic steroids and high-dose amino acid supplementation. Case Presentation. A 34-year-old man presented to our institution for left eye pain for one week associated with a vesicular rash in the V1 dermatome, respecting the midline. The patient had no significant past medical or past ocular history, including systemic immunosuppressive agents or HIV. However, prior to the onset of his symptoms the patient had completed a 6-week course of anabolic steroids including trenbolone, deca-durabolin, and testosterone as well as high-dose arginine supplementation averaging more than 40 grams a day. The best-corrected vision was 20/25 OS with slit-lamp examination remarkable for punctate staining and pseudodendrites at 6 o'clock, outside the visual axis. The patient was treated with oral acyclovir 800 mg five times a day for seven days along with prednisolone QID and moxifloxacin QID which was tapered over a month. Four months after resolution, the patient developed a recurrent HZO keratitis preceded by another cycle of anabolic steroids and amino acid supplementation. CONCLUSION: In vitro L-arginine supplementation has been associated with the proliferation and virulence of a variety of herpes viruses. Anabolic steroids have also been demonstrated by various studies to negatively affect cell-mediated immunity necessary to prevent viral infection. Thus, it is possible that anabolic steroids in conjunction with increased L-arginine intake may have precipitated a recurrent HZO in a previously healthy, immunocompetent individual.

A Simple, Novel Approach to Capsulorhexis Formation in the Setting of A Mature Cataract and Miotic Pupil.

PURPOSE: To report a simple, effective technique for surgeons creating a capsulorhexis in patients with pupillary miosis and a dense, mature cataract. METHODS: A single center, two-year retrospective chart review examined 1408 phacoemulsification cataract surgeries. The criteria for inclusion involved a dense, mature cataract and a pupil that was unresponsive to pharmacologic dilation preoperatively. A standardized technique was used for all cases consisting of a 1mm corneal paracentesis and 2.4mm temporal clear corneal incision. Synecholysis was performed if present, followed by the insertion of a 6.25mm malyugin ring under cohesive viscoelastic. The cohesive viscoelastic was removed via the irrigation aspiration tip. The paracentesis was sealed with a small amount of viscoelastic and an air bubble was placed in the anterior chamber. The anterior capsule was then painted with trypan blue. The air bubble and trypan blue were then replaced by a dispersive viscoelastic. Curvolinear capsulorrhexis was performed followed by standard phacoemulsion. RESULTS: Nine patients ranging from 76 ± 12 years (mean ± standard deviation) met the criteria with a 4+ NS (n=5), white mature (n=3), or deep brunescent (n=1) cataract and 3mm pupil preoperatively. Pupillary miosis was caused by posterior synechia in 44.5% of the cases followed by pharmacologic interactions from tamsulosin and donepezil in 22.25% of cases respectively. One case involved idiopathic miosis likely from aging. Capsulorhexis formation was successful in all cases with no capsular tear, vitreous loss, or conversion to extracapsular cataract extraction (ECCE). However, one case had cortex retention requiring a second procedure for removal. CONCLUSION: Dense, mature cataracts and small pupils both compromise the view for the surgeon and may be significant risk factors for training ophthalmologists. Thus, good visualization of the anterior capsule and peripheral nucleus with our simple technique facilitates a reliable capsulorhexis, potentially limiting the risk of complications for ophthalmic surgeons.

Recurrent herpes zoster ophthalmicus in a young, healthy individual taking high doses of l-Arginine.

PURPOSE: To report a case of a healthy, young male with recurrent herpes zoster ophthalmicus (HZO) and high-dose l-arginine supplementation. OBSERVATIONS: A 39-year-old man presented to another institution with a HZO involving the right eye. He was treated with oral acyclovir with complete resolution. Four months later the patient had a recurrent HZO episode and was started again on oral acyclovir. After resolution of the episode, the patient was continued on 1 g of oral acyclovir daily as a prophylactic measure. He then presented to our institution for a second opinion. The patient was otherwise healthy, with no past medical history involving systemic immunosuppressant agents or HIV. However, the patient was an active weight lifter taking high doses of amino acids. A diet recall was performed prior to both HZO episodes, which calculated an average intake of 46.5 g of l-arginine a day. Examination revealed 20/40 best-corrected acuity, anterior stromal haze in the visual axis, and inferior superficial punctate keratitis in the right eye. The patient was treated for dry eye disease with punctual plugs and artificial tears. The patient was instructed to decrease all supplemental arginine consumption while continuing with oral acyclovir prophylaxis for one year. CONCLUSIONS AND IMPORTANCE: l-arginine is associated with the replication and virulence of a variety of viruses in vitro, including herpes simplex and varicella zoster. Although arginine consumption increased prior to the initial and recurrent HZO infection, further investigation needs to be performed to deem if a true association exists.

Multimodal imaging of retinal metastasis masquerading as an acute retinal necrosis.

BACKGROUND: To report the multimodal imaging and histology of a case of metastatic esophageal cancer with vitreoretinal involvement resembling acute retinal necrosis (ARN) in a patient receiving systemic chemotherapy. CASE PRESENTATION: A 69-year-old Japanese man with a history of stage 4 esophageal carcinoma, treated with three cycles of 5-fluorouracil (5-FU) and cisplatin (CDDP) chemotherapy as well as 30 sessions of radiation therapy, presented with new onset of blurry vision in the right eye (OD). Visual acuity was 20/200 OD. Fundus examination OD revealed 2+ nuclear cataract, veil-like vitreous opacity, a tractional retinal detachment, and white retinal lesions in the macula and periphery masquerading as an ARN. Due to the poor view and uncertainty regarding diagnosis, combined cataract extraction and 25 gauge pars plana vitrectomy was performed. Polymerase chain reaction and cytologic analysis were performed on the vitreous samples, which was negative for all infectious entities but positive for poorly differentiated malignant cells. The vitreous biopsy was consistent with the primary endoscopic esophageal biopsy. Ultra-wide view fundus imaging revealed multifocal white intraretinal lesions in the macula and periphery. Optical coherence tomography through these white opacities displayed hyper-reflective inner retinal lesions with no choroidal involvement, suggestive of retinal metastasis. Observation and palliative support was continued until the patient passed away 3 months after diagnosis. CONCLUSION: Retinal metastasis may mimic infectious syndromes such as ARN and are associated with a very poor prognosis. Outside of the retina, no further central nervous system metastasis was found. 5-FU is known to cross the blood-brain-barrier but may be inadequate in preventing retinal metastasis.

A novel missense mutation in BEST1 associated with an autosomal-dominant vitreoretinochoroidopathy (ADVIRC) phenotype.

BACKGROUND: To report a 68-year-old female with an autosomal-dominant vitreoretinochoroidopathy (ADVIRC) phenotype associated with a subretinal hemorrhage (SRH) and novel BEST1 pathogenic variation p.Met571Thr. MATERIALS AND METHODS: The patient was assessed by fundus photography, fluorescence and indocyanine green angiography, spectral-domain optical coherence tomography, photopic and scotopic electroretinogram (ERG), and electrooculogram (EOG). Whole-exome and Sanger sequencing of the patient's and selected family members' DNA was performed. Ophthalmoscopic examinations were also performed on six patient's relatives. RESULTS: The patient presented moderate vitreous and SRH in the left eye. A distinct, annular hyperpigmented band was present in both eyes. Vitrectomy improved visual acuity, and the SRH gradually regressed without recurrence. Preserved macular function was shown by optical coherence tomography (OCT). Genetic analysis identified a novel heterozygous mutation, resulting in p.Met571Thr in BEST1. No mutations were observed in a panel of other eye disease genes, suggesting that this pathogenic variation in BEST1 is associated with an ADVIRC phenotype. No other evaluated family member had the variant or the fundus findings. CONCLUSIONS: We present a patient with a novel p.Met571Thr pathogenic variation associated with an ADVIRC phenotype. SRH is a unique finding in ADVIRC patients and may correspond to peripheral exudative hemorrhagic chorioretinopathy. The BEST1 pathogenic variation p.Met571Thr might be the likely cause of ADVIRC in this patient. However, further study is necessary to determine whether this mutation is causative.

Unilateral Multifocal Intraocular Lens Implantation in a Patient with Adie's Pupil.

PURPOSE: To report a case of a patient with unilateral Adie's pupil who underwent bilateral cataract extraction with multifocal and monofocal posterior chamber intraocular lens (IOL) implantation. METHODS: A 74-year-old woman presented to our institution complaining of worsening near vision. Gross examination revealed a 6-mm fixed pupil on the right eye (OD) and a 5-mm pupil reacting to 3 mm with light on the left eye (OS). Slit lamp examination revealed a tonic pupil with an exaggerated pupillary constriction to dilute pilocarpine OD. Dilated exam revealed 2-3+ nuclear and cortical lens changes bilaterally. The patient's active lifestyle, personality, and biometry measurements made her a good candidate for multifocal IOL (MfIOL) implantation OS. Femtosecond laser-assisted cataract extraction with a ReSTOR +3 ADD (SN60D1) implantation was performed OS. Monofocal lens implantation (SN60WF) was performed OD 6 months later. RESULTS: One year postoperatively, our patient had an uncorrected visual acuity (VAsc) of 20/15 for distance and J10 for near OD. Her VAsc was 20/25 +1 for distance and J1 for near OS. Visual acuity when using both eyes was 20/15 for distance and J1 for near. CONCLUSION: Optimizing success for MfIOL implantation is a multifactorial process. Large pupils preoperatively are of particular concern, as this may lead to increased dysphotopsia with pupil-dependent MfIOLs. Thus, patients with unilateral mydriasis, such as Aide's pupil, may have a beneficial outcome combining multifocal-monofocal implantation after bilateral cataract extraction, especially if they are not a candidate for monovision but desire spectacle independence.