Pentosan Polysulfate Maculopathy With 13 Years of Follow-up Imaging.

Purpose: To describe a case of pentosan polysulfate maculopathy progression with 13 years of follow-up imaging. Methods: A case was analyzed and a literature review performed. Results: A 65-year-old woman was referred to the retina service for a second opinion of a bilateral progressive pigmentary maculopathy. Her medical history was significant for interstitial cystitis that was actively treated with daily pentosan polysulfate since 2003. Multimodal imaging and fundus examination were consistent with pentosan polysulfate maculopathy. A review of records showed previous fundus imaging dating back 13 years that permitted longitudinal assessment of the disease course. Imaging findings were more prominent than the fundus examination findings. There was a 5-year period from the onset of parafoveal atrophy to foveal involvement. A pseudopodial pattern of disease expansion was seen on fundus autofluorescence. Conclusions: To our knowledge, this case represents the longest documented follow-up imaging of the progression of pentosan polysulfate maculopathy in the literature.

Multimodal imaging of white preretinal lesions in atypical familial exudative vitreoretinopathy: Case report and literature review.

Purpose: To report a rare clinical finding of preretinal granules associated with atypical familial exudative vitreoretinopathy (FEVR) and perform a review of the literature. Observations: An asymptomatic 18-year-old male was referred for unilateral peripheral avascular retina evaluation in association with presumed FEVR. He was first noted to have white preretinal granules on fundus examination at five years of age. The lesions remained unchanged over the subsequent years. Genetic testing did not reveal a pathogenic or likely pathogenic variant in a known FEVR gene. A review of the literature revealed five other cases of FEVR with similar findings. Conclusions and Importance: Literature review suggests preretinal granules may present rarely in FEVR. Negative genetic screening of known FEVR genes in our patient with atypical FEVR suggests either a molecularly distinct etiology supporting the rarity of this association with FEVR or, alternatively, the presence of granules in developmental retinal vascular anomalies that are not specific to FEVR. Future study and genetic testing is necessary to better understand the cause of these preretinal granules and the clinical manifestations of FEVR.

Pars Plana Vitrectomy for Vitreomacular Traction Resulting in Persistent Postoperative Loculated Foveal Subretinal Fluid.

Purpose: To describe a case of postoperative persistent loculated subretinal fluid (SRF) that developed after pars plana vitrectomy (PPV) for vitreomacular traction (VMT) syndrome. Methods: A case was analyzed and a literature review performed. Results: A healthy 64-year-old man with no significant ocular history presented with persistent, symptomatic VMT. Combined phacoemulsification and PPV, epiretinal membrane and internal limiting membrane peeling, and gas-fluid exchange were performed. Postoperative spectral-domain optical coherence tomography imaging showed loculated foveal SRF. The SRF persisted for 8 months, with minimal change in size and little best-corrected visual acuity improvement. Conclusions: Although persistent loculated SRF has been reported after vitrectomy for rhegmatogenous retinal detachment (RD) in high myopia and tractional RD in diabetes, it has not yet been reported postoperatively after PPV for VMT. Studies of the pathophysiology and long-term course of persistent SRF after PPV for VMT are needed to inform management decisions for this rare complication.

UTILIZATION OF PREOPERATIVE MIDPERIPHERAL OPTICAL COHERENCE TOMOGRAPHY FOR TAILORED SURGICAL MANAGEMENT OF PROLIFERATIVE VITREORETINOPATHY.

PURPOSE: The purpose of this study was to report on the use of preoperative spectral domain optical coherence tomography to assess retinal pathology and guide the surgical approach to proliferative vitreoretinopathy. METHODS: A case report was discussed. RESULTS: A 70-year-old man developed proliferative vitreoretinopathy after surgical repair of a macula-off rhegmatogenous retinal detachment. In preparation for further surgery, inferior preretinal fibrosis and membranes were identified on preoperative optical coherence tomography. The patient underwent successful vitrectomy with peeling of the membranes resulting in markedly improved visual acuity. CONCLUSION: Widely available spectral domain optical coherence tomography can be used preoperatively to image the midperipheral retina and guide surgical decision-making in the management of proliferative vitreoretinopathy.

Internal Plugging of a Traumatic Posterior Perforation Site Using Donor Sclera, Human Amniotic Membrane, and Fibrin Glue: A Novel Surgical Technique.

Purpose: To describe a novel surgical approach to treat traumatic posterior perforating injuries. Methods: A case and its findings were analyzed. Results: A 21-year-old man presented with bilateral intraocular foreign bodies that were sustained while hammering a metal railway pin. In the left eye, the foreign body was embedded in the posterior scleral wall, resulting in a 2 mm × 6 mm posterior perforation and partial retinal detachment. Silicone oil tamponade could optimize the chances for retinal reattachment; however, there was concern that the silicone oil would migrate through the posterior defect and into the orbit. Therefore, the perforation site was filled using a 3-layer plug consisting of donor sclera, human amniotic membrane, and fibrin glue. The silicone oil was successfully maintained within the globe; however, the final visual acuity was limited due to proliferative vitreoretinopathy. Conclusions: The efficacy and safety of the 3-layer plug technique should be further validated in similar cases.

Risk for Surgical Team Hearing Loss With Vitrectomy.

Purpose: To assess sound-level exposure during vitrectomy using 3 of the most common commercially available machines. Methods: This noninterventional cross-sectional study examined sound emission from the Constellation, Stellaris, and EVA vitrector systems. For each machine, a noise dosimeter was used to measure the sound-level exposure of the surgeon during 3 surgical cases in which vitrectomy was performed. Sound levels associated with progressively increasing cut rates and vacuum pressures were also measured. Finally, sound measurements were taken during the use of various additional functions of each machine, including diathermy, laser, and extrusion. Sound levels were compared with occupational health guidelines in Canada and the United States. Results: The maximum sound level recorded during vitrectomy surgery was 88.2 dBA. The mean sound level during vitrectomy surgical cases ranged from 58.5 to 66.8 dBA. A strong positive linear correlation was found between the cut rate and sound level (r = 0.88-0.98) and the vacuum pressure and sound level (r = 0.83-0.97). This relationship was consistent across the 3 vitrector systems (P < .001). Conclusions: Noise exposure during vitrectomy procedures was acceptable but may be sufficient for surgical team activity interference, as described by World Health Organization recommendations. A strong correlation was found between the cut rate and noise exposure. If cut rates continue to increase, attention should be given to ensure that the resulting noise exposure does not threaten the hearing of vitreoretinal surgeons and the operating room staff.

Management and Complications of Dexamethasone Intravitreal Implant Migration Into the Anterior Chamber.

Purpose: To report patient demographics, the clinical course, and visual outcomes across a case series of patients who experienced dexamethasone (DEX) intravitreal implant (Ozurdex, Allergan, Inc) migration into the anterior chamber (AC), with a focus on the corneal transplantation rate. Methods: In this retrospective cross-sectional study, a chart review of all cases was performed. For numerical responses, means and SDs were calculated. Percentages and absolute numbers were used to report the proportion of patients who experienced various outcomes of interest. Results: The study comprised 32 cases. All cases occurred in pseudophakic eyes; of those, 8 (25.0%) had posterior chamber intraocular lenses in the capsular bag with no known capsular or zonular issues. The mean duration from DEX implant injection to detection of migration was 19.4 ± 14.5 days. The DEX implant was explanted in 21 patients (65.6%) and repositioned in the vitreous cavity or subconjunctival space in 6 patients (18.8%). Twelve patients (37.5%) ultimately required corneal transplantation. Conclusions: To our knowledge, this is the largest case series of DEX intravitreal implant migration into the AC compiled to date. Cases of migration occurred in individuals with no known history of significant prior zonule disruption. This potential complication should be discussed with all patients undergoing DEX implant injection, which may aid in earlier presentation and improved visual outcomes.

Extensive serous ciliochoroidal detachments and macular subretinal and intraretinal fluid following laser peripheral iridotomy.

Purpose: We present multimodal imaging of an interesting case of a 78-year-old man who developed large ciliochoroidal detachments and macular subretinal and intraretinal fluid in the right eye following bilateral neodymium-doped yttrium aluminium garnet (Nd:YAG) laser peripheral iridotomies (LPIs). Observations: The ciliochoroidal detachments developed in the absence of documented post-procedure hypotony or intraocular pressure fluctuation. Ultrasound biomicroscopy (UBM) confirmed serous ciliochoroidal detachment. There are a small number of cases of ciliochoroidal detachments developing after peripheral iridotomy, but these have involved either argon laser, significant decrease in intraocular pressure, or underlying ocular conditions or structural abnormalities, such as Vogt-Koyanagi-Harada (VKH) or nanophthalmos. Conclusions: Serous ciliochoroidal detachments following the relatively non-invasive procedure of LPI are rare occurrences. We present our case in hopes of increasing awareness of this potential acute complication. We also discuss the diagnostic challenges of this unique case, the extensive work up, and current status of the patient.

Relentlessly Progressive Sweet Syndrome of the Eye with Scleritis and Choroidal Infiltration.

PURPOSE: To describe a case of Sweet syndrome, a dermatologic inflammatory disease, with progressive, unrelenting ocular findings. METHODS: Case report. RESULTS: A 73-year-old male was evaluated with a six-month history of Sweet syndrome, manifesting as cutaneous erythematous edematous papules on the dorsal arms and shins and confirmed with biopsy demonstrating neutrophil infiltration with nuclei fragmentation and lack of vasculitis. He initially noted a unilateral red eye with ocular pain and was found to have scleritis and choroidal infiltration. The patient's ocular disease progressed despite treatment with systemic corticosteroids, intraocular Ozurdex ®, systemic dapsone, and subtenons triamcinolone. Systemic evaluation was negative for malignancy or other inflammatory syndromes. Following 7 months of non-manageable ocular pain enucleation was offered to the patient, but he declined. CONCLUSION: Sweet syndrome, a dermatologic condition, can be associated with unilateral scleritis and choroidal infiltration that are relentlessly progressive despite maximal systemic and ocular corticosteroid therapy.

INTERNAL CHANDELIER-ASSISTED MACULAR BUCKLING FOR MYOPIC FOVEOSCHISIS.

PURPOSE: To present a surgical technique and case presentation of internal chandelier-assisted macular buckling for myopic foveoschisis. METHODS: Review of patient clinical features, visual acuity, and optical coherence tomography results after internal chandelier-assisted macular buckling for myopic foveoschisis. RESULTS: A 48-year-old highly myopic woman (axial length 29.85 mm) underwent internal chandelier-assisted macular buckling for myopic foveoschisis with macular detachment. The best-corrected visual acuity improved from 20/150 to 20/40. Postoperative optical coherence tomography confirmed central buckle positioning and demonstrated resolved foveoschisis and macular detachment. There were no complications. CONCLUSION: Internal chandelier-assisted macular buckling is a valuable tool to optimize buckle position and patient outcomes.

Henle Fiber Layer Hemorrhage in Macular Telangiectasia Type 2: Is Right Eye Dominance Coincidence or Consequence?

PURPOSE: To summarize all reported cases of Henle fiber layer (HFL) hemorrhage in the absence of subretinal neovascularization (SRNV) in patients with macular telangiectasia type 2 (MacTel2) and to propose a mechanism for the right-sided predominance of this unique presentation. DESIGN: Perspective. METHODS: Collection, review, and analysis of all cases in the literature and in the authors' databases of HFL hemorrhage in MacTel2, including analysis of baseline and follow-up multimodal retinal imaging findings of selected cases. Elucidation of the complex interplay of systemic venous pressure with the deep retinal capillary plexus and hypothesis regarding the right-sided predilection of HFL hemorrhage complicating MacTel2. RESULTS: Ten patients presented with a unilateral, characteristic radial macular hemorrhage within the HFL that affected only the right eye in all cases. Absence of SRNV was confirmed by fluorescein angiography and/or optical coherence tomography angiography. The hemorrhage resolved spontaneously in at least 7 of the 10 eyes. The HFL hemorrhage may plausibly be explained by dysfunction of the deep capillary plexus in MacTel2 combined with an acute rise in central venous pressure, for which the right side may be at increased risk. CONCLUSIONS: HFL hemorrhage can complicate MacTel2 in the absence of SRNV, and the radial pattern of blood affecting only the right eye is remarkable. The right eye predominance may be multifactorial in etiology. Related factors may include the right-sided predilection of MacTel2 and/or increased right-sided dural sinus drainage related to normal anatomical variation.

NOVEL APPROACH TO SCLERAL FIXATION OF A REPER INTRAOCULAR LENS AND ARTIFICIAL IRIS COMPLEX FOLLOWING PARS PLANA LENSECTOMY AND VITRECTOMY FOR ECTOPIA LENTIS AND CATARACT IN A PATIENT WITH ANIRIDIA AND NYSTAGMUS.

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.

Relentless Placoid Chorioretinitis With Branch Retinal Vein Occlusion and Secondary Peripheral Retinal Neovascularization.

Purpose: We report a case of relentless placoid chorioretinitis (RPC) that developed branch retinal vein occlusion and peripheral retinal neovascularization in one eye. Methods: A case report is presented. Results: A 33-year-old healthy man presented with decreased visual acuity (20/150) in both eyes. Slit-lamp examination revealed anterior chamber and vitreous inflammation. Multiple yellow-white lesions were evident in the macula and scattered throughout the fundus. Following workup, a diagnosis of RPC was made. The patient was started on Pred Forte (prednisolone acetate 1%) and atropine drops. Three months later, visual acuity improved to 20/70 and 20/100 in the right and left eyes, respectively. At this time, fundus examination and fluorescein angiography revealed peripheral retinal neovascularization. Sectoral scatter laser photocoagulation was performed in the areas of nonperfusion. Conclusions: We describe a novel presentation of RPC associated with branch retinal vein occlusion and retinal neovascularization. Although the pathophysiology of RPC is believed to be primarily a choroidal vasculitis, retinal vascular changes may also occur, as observed in other white dot syndromes.

Unilateral benign yellow dot maculopathy.

PURPOSE: To describe a unique case of unilateral benign yellow dot maculopathy. OBSERVATIONS: A 25-year-man was evaluated after incidental finding of yellow dots in the right macula. The findings of examination and multimodal imaging were in keeping with a diagnosis of benign yellow dot maculopathy. CONCLUSIONS AND IMPORTANCE: Benign yellow dot maculopathy is a recently described entity with either a sporadic or dominant inheritance pattern. This is the first known report of the characteristic findings of this phenotype presenting unilaterally.