Epiretinal proliferation after rhegmatogenous retinal detachment.

PURPOSE: To determine the characteristics and appearance rate of epiretinal proliferation (ERP) on SD-OCT after surgery for rhegmatogenous retinal detachment (RRD) repair. METHODS: One hundred eight eyes of 108 patients who underwent one or more surgeries for RRD were enrolled. The eyes with other maculopathies that were directly related to RRD were excluded. Image acquisition was performed with SD-OCT (Heidelberg Engineering, Germany). Clinical charts were reviewed to assess clinical and surgical findings. Statistical analyses were performed using XLSTAT (Assinsoft, Paris, France). RESULTS: ERP was found in 9.3% eyes (n = 10). The mean initial visual acuity (logMAR) was 1.34 ± 0.82 in the ERP group compared to 0.49 ± 0.70 in the non-ERP group. PVR was present in 70.0% and chronic macular edema was found in 80.0% of eyes which developed ERP. The mean number of vitreoretinal surgeries in eyes with ERP was 3.3 ± 1.19 and only 1.44 ± 1.02 in eyes without. Silicone oil was used in 60.0% of eyes which developed ERP compared to 13.9% in the non-ERP group. CONCLUSION: ERP is a late-onset postoperative finding in eyes with RRD and can occur in absence of macular holes. Overall, ERP is more frequent in eyes with complicated courses of RRD including multiple operations, PVR, usage of silicone oil, and chronic macular edema.

RETINAL DETACHMENT IN EYES WITH BOSTON TYPE 1 KERATOPROSTHESIS: Surgical Techniques and Mid-Term Outcomes.

PURPOSE: To evaluate the mid-term outcomes of pars plana vitrectomy performed for retinal detachment (RD) repair after Boston Type 1 keratoprosthesis (KPro) implantation. METHODS: Retrospective review of medical records of KPro implanted at the Stein Eye Institute presenting with RD and treated by pars plana vitrectomy. Functional success was defined as a postoperative visual acuity maintained within 2 Snellen lines of the corrected distance visual acuity measured before the development of the RD (baseline) and anatomical success as an attached retina after the pars plana vitrectomy. Kaplan-Meyer survival analyses were performed. RESULTS: Among the 224 KPro performed, 28 (15.2%) RD were identified; of which, 21 (9.4%) were included. The mean follow-up was 42.5 ± 27.3 months. Vitreoretinal proliferation was present in 18 of 21 eyes (85.7%). Surgical techniques were adapted to the complex anterior segment anatomy of KPro eyes. Anatomical success was achieved in 18 of 21 eyes (85.7%). Functional success occurred in 17 of 21 eyes (81.0%), and 5 of 21 eyes (23.8%) reached 20/400 or better visual acuity at the final follow-up. The KPro was retained in 11 in 21 eyes (52.4%). The retention rate decreased from 94.7% at 1 year to 53.5% at 5 years. The most frequent complications were retroprosthetic membrane (47.6%) and corneal melt (23.8%). CONCLUSION: Modified pars plana vitrectomy techniques resulted in relatively good mid-term anatomical, functional, and retention rate outcomes, given the severity of RD at presentation and the numerous preoperative comorbidities of KPro eyes.

PERIPAPILLARY PACHYCHOROID SYNDROME.

PURPOSE: To describe the features of peripapillary pachychoroid syndrome (PPS), a novel pachychoroid disease spectrum (PDS) entity. METHODS: Medical records of 31 eyes (16 patients) with choroidal thickening associated with intraretinal and/or subretinal fluid in the nasal macula extending from the disk were reviewed (patients with PPS). Choroidal thickness was compared with 2 age-matched cohorts: typical PDS (17 eyes with central serous chorioretinopathy or pachychoroid neovasculopathy) and 19 normal eyes. RESULTS: The patients with PPS were 81% men aged 71 ± 7 years. Peripapillary pachychoroid syndrome eyes displayed thicker nasal versus temporal macular choroids, unlike PDS eyes with thicker temporal macular choroids (P < 0.0001). Peripapillary intraretinal and/or subretinal fluid was often overlying dilated Haller layer vessels (pachyvessels). Fundus autofluorescence and fluorescein angiography illustrated peripapillary pigmentary mottling without focal leakage. Most PPS eyes (70%) exhibited other PDS findings including serous pigment epithelial detachment or gravitational tracks. Indocyanine green angiography illustrated dilated peripapillary pachyvessels and choroidal hyperpermeability. The disk was usually crowded, with edema noted in 4/31 (13%) eyes and mild late fluorescein disk leakage identified in half of the cases. Choroidal folds (77%), short axial lengths (39% less than 23 mm), and hyperopia (86%) were common. CONCLUSION: Peripapillary pachychoroid syndrome is a distinct PDS variant, in which peripapillary choroidal thickening is associated with nasal macular intraretinal and/or subretinal fluid and occasional disk edema. Recognition of PPS is important to distinguish it from disorders with overlapping features such as posterior uveitis and neuro-ophthalmologic conditions.

Diabetic tractional retinal detachment repair by vitreoretinal fellows in a county health system.

PURPOSE: To investigate anatomical and functional outcomes of vitreoretinal fellow-performed vitrectomy for tractional retinal detachment secondary to proliferative diabetic retinopathy in a county hospital system. METHODS: Consecutive retrospective review of patients surgically treated for diabetic tractional retinal detachment at Olive View-UCLA County Medical Center (Sylmar, CA) during a 2-year training period. RESULTS: Sixty-two eyes of 58 patients met inclusion criteria with a mean age of 48 ± 9 years and preoperative hemoglobin A1c of 8.4 ± 1.9%. Previous panretinal photocoagulation had been performed in 34 eyes (54.8%). Mean surgery duration was 153 ± 54 minutes. There was no significant time difference observed between first-year fellow surgeries (159.5 ± 52.3 minutes) and those performed by second-year fellows (146.8 ± 56.4 minutes, P = 0.35), although there was a trend toward longer first-year surgical times. After a mean follow-up of 11.2 months, successful retinal reattachment was achieved in 56 eyes (90.3%). Overall, mean logMAR visual acuity improved from 2.0 ± 0.5 to 1.4 ± 0.8 (P = 0.0007). Final visual acuity had improved in 33 eyes (53.2%), was unchanged in 11 eyes (17.7%), and decreased in 18 eyes (29%). Postoperative complications encountered included early vitreous hemorrhage in 10 eyes (16.1%), delayed vitreous hemorrhage in 3 eyes (4.8%), secondary rhegmatogenous retinal detachment in 11 eyes (17.7%), and neovascular glaucoma in 5 eyes (8%). Second-year fellows had a lower incidence of rhegmatogenous retinal detachment than first-year fellows (P = 0.016). CONCLUSION: Patients with diabetic tractional retinal detachment present to county hospitals with more complex retinal pathology, yet surgical outcomes as performed by vitreoretinal fellows compare favorably to previously reported series.

Chronic hypotony and uveitis managed with pars plana vitrectomy, silicone oil tamponade, and permanent keratoprosthesis.

PURPOSE: We report a case of silicone oil placement after Boston Type 1 keratoprosthesis implantation for improvement and maintenance of intraocular pressure in a patient with chronic hypotony secondary to chronic uveitis. METHODS: Observational case report. RESULTS: A 54-year-old female with a history of bilateral chronic panuveitis and subsequent hypotony presented with progressive corneal decompensation and band keratopathy in her better-seeing left eye. Her CDVA in her left eye declined from count fingers at 3' to hand motion. In an effort to clear the visual axis and stabilize intraocular pressure, implantation of a Boston Type 1 Keratoprosthesis combined with pars plana vitrectomy and silicone oil injection was performed. Post operatively, the intraocular pressure improved to the mid-teens and vision improved to count fingers at 4'with a follow-up of 2 years. CONCLUSION: Pars plana vitrectomy and injection of silicone oil after placement of a Boston Type 1 keratoprosthesis can successfully improve and maintain intraocular pressure in eyes affected by chronic uveitis with ciliary body atrophy and chronic hypotony. This strategy effectively reverses such problems as hypotony maculopathy, disk swelling, scleral infolding, and corneal failure.

Macular Sling: A Customizable Method for Macular Buckling using Available Elements.

PURPOSE: Macular buckling surgery is used to treat certain cases of myopic traction maculopathy but is rarely performed in the United States. One of the main factors limiting its use is the lack of commercially available buckling elements. Here we describe a novel technique for creating an effective macular buckle using readily obtainable buckling materials. METHODS: By using a traditional circumferential 41 band as the anchoring point around the globe, a 240 band can then be attached and oriented posteriorly along the superonasal-infertemporal axis. This posterior 240 band is then used to guide a grooved sponge (509G) under the macula to create a customizable and titratable tamponade effect along the posterior pole. This approach was used to provide external support in the case of a recurrent, complex tractional retinal detachment which had failed multiple prior vitrectomy-based repairs. RESULTS: Placement of the macular sling resolved the patient's recurrent retinal detachment with return of visual acuity to her pre-operative baseline. There have been no adverse effects related to the surgery aside from a large hyperopic shift due to the buckle effect on the macula. We believe the technical and material complexity of this technique is comparable to that of more common scleral buckling techniques. CONCLUSIONS: This macular sling technique can be used to create an effective posterior buckle without requiring specialized materials.

Long-term visual and anatomic outcomes of patients with peripapillary pachychoroid syndrome.

BACKGROUND/AIMS: To analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum. METHODS: This study retrospectively included patients from several retina centres worldwide. Visual acuity (VA), retinal thickness and choroidal thickness at baseline, 6 months and final follow-up were assessed. Temporal trends in VA and anatomic characteristics were evaluated. Visual and anatomic outcomes in eyes that were observed versus those that were treated were analysed. RESULTS: Fifty-six eyes of 35 patients were included with mean follow-up of 27±17 months. Median VA was 20/36 at baseline and remained stable through follow-up (p=0.77). Retinal thickness significantly decreased subfoveally (p=0.012), 1.5 mm nasal to the fovea (p=0.002) and 3.0 mm nasal to the fovea (p=0.0035) corresponding to areas of increased thickening at baseline. Choroidal thickness significantly decreased subfoveally (p=0.0030) and 1.5 mm nasal to the fovea (p=0.0030). Forty-three eyes were treated with modalities including antivascular endothelial growth factor injection, photodynamic therapy, and others. VA remained stable in treated eyes over follow-up (p=0.67). An isolated peripapillary fluid pocket in the outer nuclear layer was characteristic of PPS. CONCLUSION: Patients with PPS experienced decreased retinal oedema and decreased choroidal thickening throughout the course of disease. While some patients experienced visual decline, the overall visual outcome was relatively favourable and independent of trends in retinal or choroidal thickening.

SUPERFICIAL AND DEEP CAPILLARY ISCHEMIA AS A PRESENTING SIGN OF RETINAL VASCULOPATHY WITH CEREBRAL LEUKOENCEPHALOPATHY AND SYSTEMIC MANIFESTATIONS.

PURPOSE: The aim of this study was to investigate the presenting sign of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations, a rare autosomal dominant condition caused by mutations in the TREX1 gene, and to explore the potential efficacy of bevacizumab in preventing capillary occlusions. METHODS: Observational case report with the use of ultra-widefield fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography. RESULTS: A 31-year-old man with a family history of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations presented with a scotoma in his left eye. The visual acuity was 20/20 in both eyes, and his examination was notable for scattered cotton wool spots in the retina of both eyes as well as an area of paracentral acute middle maculopathy in the left eye. Ultra-widefield fluorescein angiography revealed peripheral capillary nonperfusion and vascular leakage corresponding to the cotton wool spots. Spectral domain optical coherence tomography and optical coherence tomography angiography confirmed the presence and distribution of superficial capillary plexus and deep capillary plexus ischemia. Neurologic examination and imaging were normal. A trial of monthly intravitreal bevacizumab injections to the left eye over 6 months resulted in diminished capillary leakage. CONCLUSION: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations is a rare genetic condition manifested most commonly by cerebral and retinal ischemia. This retinal vasculopathy leads to occlusions of small-caliber retinal vessels in the superficial plexus and deep plexus with resulting cotton wool spots and paracentral acute middle maculopathy, respectively. Recognition of the retinal findings by ophthalmologists and neurologists may avoid unnecessary brain biopsies in diagnosing this rare disorder.

Central serous chorioretinopathy after solid organ transplantation.

PURPOSE: To describe the demographic and clinical characteristics of central serous chorioretinopathy (CSC) after solid organ transplantation. DESIGN: Case series. PARTICIPANTS: Fifteen patients who presented to the authors with CSC after solid organ transplantation. METHODS: We performed a retrospective chart review to identify patient demographics and clinical features of disease, including angiographic changes. MAIN OUTCOME MEASURES: Patterns of CSC. These patterns were compared with type of organ received, demographics, and visual outcome. RESULTS: We identified 25 eyes of 7 women (46.7%) and 8 men (53.3%) that developed CSC after solid organ transplantation. Patient ages ranged from 27 to 55 years (median, 40). Seven of the 15 patients (46.7%) were Caucasian, including 3 Hispanic patients (20%). Of the 8 remaining patients (53.3%), 2 were African American (13.3%), 2 were Filipino (13.3%), and 4 were Asian (26.7%). The organs received included 13 kidneys (86.7%), 1 liver (6.7%), and 1 heart (6.7%). Systemic hypertension was reported in 14 of 15 patients (93.3%). All patients were receiving systemic immunosuppressive drugs at presentation; 14 of 15 (93.3%) were also receiving systemic corticosteroids. Visual acuity at presentation ranged from 20/20 to counting fingers. Patterns of CSC included (1) geographic or diffuse alterations in the retinal pigment epithelium (5 eyes; 2 bilateral, 1 unilateral), (2) focal CSC (6 eyes, all unilateral), (3) multifocal CSC (6 eyes; 2 bilateral, 2 unilateral), and (4) CSC with bullous retinal detachment (8 eyes, all bilateral). Follow-up, available for 21 affected eyes of 13 patients, ranged from 1 month to 6 years (median, 12 months). Compared with other solid organ transplant recipients at our institutions, renal transplant recipients (P = 0.003), as well as Hispanic and Asian patients (P = 0.05), were more prevalent in this cohort. CONCLUSION: Central serous chorioretinopathy after solid organ transplantation varies in presentation and severity. Our observations support a role for choroidal vascular compromise in the pathogenesis of this disorder.

Outcomes of vitrectomy with inferior retinectomy in patients with recurrent rhegmatogenous retinal detachments and proliferative vitreoretinopathy.

PURPOSE: To report the anatomic and functional outcomes of patients treated with vitrectomy and inferior retinectomy for recurrent, rhegmatogenous retinal detachment complicated by proliferative vitreoretinopathy (PVR). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Fifty-six patients with recurrent, rhegmatogenous retinal detachments complicated by PVR who underwent an inferior retinectomy for repair. METHODS: Retrospective review over a 6-year period of patients treated with vitrectomy and inferior retinectomy. MAIN OUTCOME MEASURES: The primary outcome was anatomic success, defined as complete retinal reattachment. Secondary outcomes included change in visual acuity, the mean number of operations required for complete retinal reattachment, number of operations before retinectomy, use of silicone oil tamponade, location and extent of retinectomy, whether lensectomy was undertaken, and incidence of postoperative complications. RESULTS: Complete retinal reattachment was achieved in 52 of 56 patients (93%), with a mean follow-up of 25 months (range, 6-70 months). After retinal reattachment, visual acuity was improved or stabilized in 39 of 56 patients (70%). The mean number of operations for retinal detachment before diagnosis of PVR requiring retinectomy was 1.8 (range, 1-5). Patients undergoing radical anterior vitreous base dissection and lensectomy at the time of first retinectomy had a higher success rate than those who did not: 74% versus 38%, respectively (P = 0.011). Furthermore, tamponade with silicone oil had a higher success rate than tamponade with gas: 71% versus 18%, respectively (P = 0.002). Of the 56 patients, 9 (16%) had 1 or more of the following complications: keratopathy requiring penetrating keratoplasty (n = 4), glaucoma requiring aqueous shunt device (n = 3), and hypotony (n = 3). Silicone oil removal was performed in 26 of 45 patients (58%) before the last follow-up visit, with a 1 in 26 (4%) redetachment rate. CONCLUSIONS: When combined with anterior base dissection, inferior retinectomy may be useful in the surgical treatment of complex PVR-related retinal detachment. The authors show that with lensectomy, radical anterior base dissection, and inferior retinectomy, anatomic success rates are improved and visual function can be maintained. In addition, silicone oil offers an advantage over gas tamponade in these cases.

Severe visual loss caused by ocular perforation during chalazion removal.

PURPOSE: To report two cases of ocular perforation during chalazion removal procedures leading to severe vision loss. DESIGN: Observational case series. METHODS: Two patients presented with unilateral decreased vision after chalazion removal procedures. Complete ophthalmologic examinations were performed. RESULTS: Examination revealed a cherry red spot and perforation site in the first patient. In the second patient, there was an intraocular gas bubble and ischemic retina. CONCLUSIONS: Local anesthetic injections for procedures such as chalazia removal can result in ocular perforation. We postulate that the intraocular injections led to extremely high pressures, compromising the blood supply to the retina and optic nerve. Anesthetic injections for all procedures, even chalazia removal, should be done with great caution. It is imperative to avoid injection if ocular perforation is suspected, as the high pressure may cause the majority of the visual morbidity.

Pediatric rhegmatogenous retinal detachment: clinical features and surgical outcomes.

OBJECTIVE: To describe the characteristics of and surgical outcomes for primary pediatric rhegmatogenous retinal detachment (RRD) and to evaluate whether specific characteristics impact anatomical outcomes after surgical intervention. METHODS: Data for consecutive patients (18 years of age or younger) with RRD who required surgery over a 5-year period were studied retrospectively. Patients with recurrent retinal detachment or active retinopathy of prematurity were excluded. RESULTS: Forty-six eyes of 45 patients (median age, 9 years) were included. Ninety-eight percent of the patients had at least 1 predisposing factor, including prior surgery (61%), trauma (43%), developmental abnormality (35%), and myopia (17%). Retinal reattachment was attained in 78% of eyes. Younger age (P = 0.019), worse initial vision (P = 0.008), greater extent of retinal detachment (P = 0.007), and presence of proliferative vitreoretinopathy (grade C or worse) (P = 0.008) were associated with worse anatomical outcomes. Vision improved after surgery in 56% of patients, but overall visual results were modest. Thirty-seven percent of patients had vision-threatening lesions in the other eye, and 18% had a history of retinal detachment in the fellow eye. CONCLUSIONS: Pediatric RRD is often associated with a predisposing factor. Favorable anatomical and visual outcomes are possible, and lesions in the fellow eye are common.

Mucosa-associated lymphoid tissue lymphoma with intraocular involvement.

PURPOSE: To report the clinicopathologic features of a patient with mucosa-associated lymphoid tissue (MALT) lymphoma of the conjunctiva and associated intraocular involvement. METHODS: This study is a retrospective clinicopathologic correlative case report summarizing the clinical, radiologic, and histopathologic findings of a patient with conjunctival MALT lymphoma and associated intraocular involvement. RESULTS: Ophthalmic examination and fluorescein angiography demonstrated progressive conjunctival infiltration bilaterally, marked uveal effusions in the left eye, and cellular white infiltrates of the choroid in the right eye. MRI of the orbit revealed a diffusely infiltrating intra- and extraocular lesion extending around the globe and optic nerve in the left eye without evidence of intracranial extension. Conjunctival biopsy showed low-grade tumor cells, consistent with the diagnosis of MALT lymphoma. The patient was successfully treated with external beam radiation with marked clinical improvement. CONCLUSION: Conjunctival MALT lymphomas, typically indolent and localized tumors, may extend into the orbit and invade local tissues such as the choroid with devastating consequences. A conjunctival biopsy can provide an easy diagnosis of this treatable entity.

The fellow eye of patients with rhegmatogenous retinal detachment.

OBJECTIVE: To determine how often the fellow eyes of patients with rhegmatogenous retinal detachment (RRD) without proliferative vitreoretinopathy (PVR) harbor vision-threatening conditions at presentation and during follow-up. DESIGN: Retrospective, observational case series. PARTICIPANTS: Two hundred forty-eight patients with RRD without PVR were studied. INTERVENTION: The authors observed the fellow eye of patients with RRD for vision-threatening pathology. MAIN OUTCOME MEASURE: Detection of vision-threatening pathology in the fellow eye of patients with RRD without PVR. RESULTS: Two hundred forty-eight patients were observed for an average of 5.2 years. One hundred fifty-seven vision-threatening events or diagnoses occurred in the fellow eye, including pre-existing and newly diagnosed conditions, 97 (62%) of which were rhegmatogenous in nature. Fifty-six patients (23%) had retinal detachments in their fellow eye, including those with a history of RRD repair (28 eyes), those presenting with bilateral RRD (4 eyes), and those developing new RRD during follow-up (24 eyes). In addition, 1 patient developed recurrent retinal detachment in the fellow eye during follow-up. Retinal tears were diagnosed and treated with laser retinopexy or cryotherapy in 30 (12%) fellow eyes (4 before the initial examination, 7 upon initial examination, and 19 on follow-up). Lattice degeneration was present in 27 (11%) fellow eyes. CONCLUSIONS: Patients who develop RRD are at considerable risk for developing vision-threatening events, particularly rhegmatogenous events, in the fellow eye.