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Purpose Prosthetic iris devices have recently been used to improve cosmesis and reduce glare in aniridia. There is currently no consensus on which prosthetic iris device or which surgical approach is preferred for managing large iris defects. Methods A novel surgical approach with Gore-Tex polytetrafluoroethylene sutures was used to achieve scleral fixation of an intraocular lens and artificial iris complex in a 19-year-old Caucasian female patient with aniridia, nystagmus, cataracts, and ectopia lentis. Results Six weeks postoperatively, the intraocular lens-artificial iris complex remained well centered, and the vision in the left eye improved from 20/400 to 20/70. Two years after prosthetic iris device implantation, there have been no complications. Conclusion This case demonstrates a promising proof-of-concept for long-term management of complicated aniridia cases using an intraocular lens and artificial iris complex prosthetic iris devices. Gore-Tex sutures may be preferable to conventional polypropylene sutures because of their improved durability.

Purpose Paracentral acute middle maculopathy (PAMM) is a recently identified clinical entity with numerous retinal vascular and systemic associations. To our knowledge, this is the first reported case of PAMM associated with idiopathic intracranial hypertension. Methods A case is presented with multimodal imaging. Results A patient with idiopathic intracranial hypertension is found to have the characteristic clinical, spectral domain optical coherence tomography, and OCT angiography findings associated with PAMM. Conclusion Paracentral acute middle maculopathy is believed to arise from a microvascular insult to the retinal deep capillary plexus. We add a previously undescribed association with idiopathic intracranial hypertension to the list of etiologies that can cause PAMM.

Purpose To present a novel case of immune retinopathy associated with nivolumab therapy for non-small cell lung cancer. Methods Retrospective chart review. Results A 64-year-old woman presented with photoreceptor injury evidenced by hypoautofluorescent and hyperautofluorescent patches on fundus autofluorescence, loss of the ellipsoid zone on optical coherence tomography, and dysfunction of the rods and cones on electroretinogram. She had a history of Stage IV lung adenocarcinoma, treated with nivolumab, a checkpoint inhibitor. Serology testing was negative for paraneoplastic antibody panel, antirecoverin and antienolase antibodies, but positive for antiretinal antibodies against 30-kDa (carbonic anhydrase II), 35-kDa (GADPH), 38-kDA, 58-kDa (PKM2), and 112-kDa proteins. Cessation of the medication and high-dose oral steroids resulted in resolution of her symptoms and stability of ocular findings. Conclusion The checkpoint inhibitors, including nivolumab, have significant ocular side effects. All patients receiving nivolumab should undergo a baseline comprehensive eye examination and should be counseled to seek medical attention immediately if visual changes occur.

Purpose:This study examines treatment-based outcomes of endophthalmitis due to antivascular endothelial growth factor (anti-VEGF) intravitreal injection and its effect on subsequent management of neovascular disease.Methods:A retrospective multicenter study was conducted of 157 patients with a diagnosis of endophthalmitis following anti-VEGF intravitreal injection at 10 major ophthalmic centers.Results:The median number of injections before endophthalmitis was 10 (range, 1 to 84 injections). Initial treatment with tap and inject with or without subsequent vitrectomy trended toward smaller visual acuity changes from baseline (4 ETDRS [Early Treatment Diabetic Retinopathy Study] letter difference vs 19 ETDRS letter difference) compared with initial vitrectomy, but the difference was not statistically significant. There was no significant change in medication choice among injections after endophthalmitis. There was a statistically significant shift away from regular interval (1- to 2-month) injections and a shift toward treat-and-extend and as-needed injection algorithms.Conclusions:The visual outcomes were not significantly different between patients who initially underwent tap and injection of antibiotics and those who underwent vitrectomy. There was no significant change in medication choice before and after endophthalmitis but there was a shift toward lower-frequency injection algorithms after postintravitreal injection endophthalmitis compared with prior.

Purpose To describe a patient with confirmed transthyretin V30M form of familial amyloidosis who presented initially with isolated retinal angiopathy. Methods Retrospective chart review. Results A 66-year-old woman presented with bilateral retinal angiopathy. Extensive workup for an infectious, inflammatory, or hypercoagulable cause was unrevealing. The patient subsequently developed bilateral neovascularization of the optic nerve and iris complicated by recurrent vitreous hemorrhages, which were treated with intravitreal bevacizumab and panretinal photocoagulation. The development of cardiac and gastrointestinal symptoms 5 years after presentation led to tissue biopsies that revealed both Congo red staining and apple-green birefringence in polarized light, confirming the diagnosis of systemic amyloidosis. Sequencing of the transthyretin gene confirmed the patient to be heterozygous for the common amyloidogenic V30M mutation. Conclusion The common transthyretin V30M form of familial amyloidotic polyneuropathy can rarely present with retinal angiopathy. Recurrent vitreous hemorrhages were treated successfully with intravitreal bevacizumab and panretinal photocoagulation.

Background Autosomal recessive bestrophinopathy is part of the diverse spectrum of retinal diseases caused by mutations in the BEST1 gene. Methods A case report. Results We present a case that highlights the classic retinal findings of autosomal recessive bestrophinopathy with an emphasis on modern multimodal imaging. Conclusion We describe modern multimodal imaging in an individual with a BEST1 gene mutation and clinical findings consistent with an autosomal recessive bestrophinopathy.

Purpose: Diffuse unilateral subacute neuroretinitis (DUSN) is often a challenging diagnosis to make. We present a DUSN case with its multimodal imaging to aid in the diagnosis, emphasizing the observations on optical coherence tomography angiography (OCTA). Observations: The evolution of a DUSN case is presented. Fundus photography and OCTA aided in the identification of the nematode. Conclusions and importance: DUSN is a difficult diagnosis to establish. We report the first case to our knowledge in which OCTA aided in the diagnosis of DUSN.

Purpose To report the association of rubeosis iridis with chronic bullous degenerative peripheral retinoschisis. Observations A 63-year-old female presented with acute hyphema and neovascularization of the iris in association with elevated intraocular pressure. Posterior segment examination including imaging revealed no vascular occlusion as a potential cause. However, large, peripheral bullous retinoschisis was noted in the right eye. No nonperfusion aside from that seen within the schism detachment, or neovascularization of the retina on wide-field fundus photography or fluorescein angiography was noted. Bullous retinoschisis was also found in the left eye. The patient was treated conservatively with prednisolone acetate and timolol eye drops. Conclusions and importance Chronic bullous retinoschisis can be associated with anterior segment neovascularization such as rubeosis iridis, presumably due to non-perfusion within the retinoschisis cavity.

Purpose To describe a case of an isolated Group 3 retinal arteriovenous malformation (AVM). Methods Observational case report. Results A 15-year-old girl with no significant medical history presented with no light perception vision in her right eye and gradually decreasing vision in her left eye over several years. She was found to have a large AVM in the posterior pole of the right eye with associated perivascular sheathing in the periphery. Her left eye seemed totally unremarkable. Neuroimaging revealed no intracranial or intraorbital abnormalities. A diagnosis of isolated Group 3 retinal AVM was made. Conclusion Retinal AVMs are congenital, nonhereditary, and sporadic vascular anomalies that develop in the posterior pole. A number of patients with this finding can have coexisting AVMs in the brain, mandible, skin, or orbit, which comprises the Wyburn-Mason syndrome. Because of these systemic associations, neuroimaging should always be considered when a retinal AVM is seen. Severe vision loss can occur from these AVMs secondary to a number of ocular complications, including central retinal vein occlusions.

BACKGROUND/PURPOSE We report the first two cases of postoperative opacification of scleral-sutured Akreos AO60 intraocular lens after vitrectomy with the gas tamponade. METHODS Two patients with ectopia lentis underwent pars plana vitrectomy, pars plana lensectomy, and scleral fixation of an Akreos AO60 intraocular lens with Gore-Tex suture. Retinal breaks were noted during vitrectomy and consequently intravitreal gas tamponade was used after endolaser retinopexy. Postoperatively, both patients developed intraocular lens opacification but maintained excellent visual acuity; however, one patient developed bothersome though tolerable photophobia. CONCLUSION Although still an excellent option for the management of surgical aphakia in the absence of capsular support, caution should be taken when using a hydrophilic secondary intraocular lens, such as Akreos AO60, during vitreoretinal surgery if gas is required.

Purpose To assess the spectrum of perivenular ischemia in eyes with retinal vascular obstruction (typically central or hemicentral retinal vein obstruction) using en face optical coherence tomography (OCT). Design Retrospective observational case series. Methods Eyes with recent retinal vascular occlusion illustrating paracentral acute middle maculopathy (PAMM) in a perivenular fern-like pattern with en face OCT were evaluated in this study. Multimodal retinal imaging including en face OCT segmentation of the inner nuclear layer was performed in all patients. Color fundus photography and fluorescein angiography (FA) images were used to create a vascular overlay of the retinal veins vs the retinal arteries to map the distribution of PAMM with en face OCT analysis. Results Multimodal retinal imaging was performed in 11 eyes with acute retinal vascular obstruction. While 7 eyes demonstrated obvious findings of retinal vein obstruction (5 with central and 2 with hemicentral retinal vein occlusion), 4 eyes were unremarkable at presentation. En face OCT analysis demonstrated a spectrum of perivenular PAMM illustrating a fern-like pattern with sparing of the periarteriolar area in all cases. Conclusion En face OCT may illustrate a remarkable perivenular pattern of PAMM in eyes with retinal vascular obstruction even in the absence of significant funduscopic findings. Perivenular PAMM with en face OCT demonstrates a wide spectrum of variation with narrow fern-like perivenular lesions at the mildest end and more diffuse lesions with only periarterial sparing at the most severe end of the spectrum. Arterial hypoperfusion secondary to outflow obstruction from a central retinal vein obstruction appears to be the most common cause of this presentation, although primary arterial hypoperfusion may also be an etiology.

Background Acute macular neuroretinopathy is a rare disease that with the help of multimodal imaging is being diagnosed more frequently. Methods An atypical case is presented and followed by multimodal imaging. Results A typical acute macular neuroretinopathy lesion was seen on biomicroscopic examination and spectral domain optical coherence tomography examination. However, near-infrared imaging revealed a lack of the characteristic finding along with a relative afferent pupillary defect and a widespread depressed multifocal electroretinogram which has not been reported previously. Conclusion This patient exhibits photoreceptor and outer retinal findings commonly described in acute macular neuroretinopathy lesion without classic near-infrared findings. This case may represent a severe form of acute macular neuroretinopathy.