Long-term results of anterior chamber iris claw intraocular lens implantation in children with ectopia lentis in Marfan syndrome.

PURPOSE: To report the long-term clinical and endothelial cell count (ECC) results of lensectomy with primary anterior chamber iris claw lens implantation in the eyes of patients ≤18-year-old with ectopia lentis due to Marfan syndrome. METHODS: The medical records of Marfan patients operated on at a single institution from September 2007 to August 2020, with minimum follow-up of 2 years, were reviewed retrospectively. The following data were analyzed: sex, age at surgery, indication for surgery, the position of the lens in relation to the undilated and dilated pupil, corneal endothelial cell counts (ECC), peri- and postoperative complications, pre- and postoperative best-corrected visual acuity. RESULTS: A total of forty-two eyes of 23 patients (12 girls and 11 boys) were included. At least two or more postoperative ECCs were collected from 33 eyes (17 patients). Median age at IOL implantation was 6.1 years (range, 1.8-18). Median overall follow-up time was 6.2 years (range, 2-13.5). Median ECC follow-up time was 6.2 years (range, 2-10). Mean best-corrected visual acuity was 0.71 ± 0.38 logMAR before surgery and 0.02 ± 0.25 logMAR at final follow-up. The mean annual ECC decline was 0.71% ± 2.24. Total cell loss from first to last postoperative measurement was 150 cells ± 394 cells/mm2 (4.81%). Pre- and first postoperative data were available for 17 eyes of 10 patients, with a mean cell loss before and directly after surgery of 269 ± 268 cells (7.94%). Surgery related complications were iris bombé due to blockage of peripheral iridectomy in 3 eyes and claw dislocation due to direct impact trauma in 3 eyes. CONCLUSIONS: In our large, pediatric study cohort, anterior chamber iris claw IOL implantation resulted in an excellent visual outcome and normal endothelial cell loss compared with normative data. Safety measures are recommended to avoid traumatic dislocation of IOLs.

Visual acuity of 20/32, 13.5 years after a retinal pigment epithelium and choroid graft transplantation.

PURPOSE: To present the 13.5-year-survival of an autologous retinal pigment epithelium (RPE) and choroid graft transplantation with good visual acuity results. OBSERVATIONS: A 72-year old patient presented with a 5-weeks-old visual acuity deterioration to excentric finger counting at half a meter. Fundoscopy showed a fibrotic macular scar, a large subretinal hemorrhage, partly recent, combined with intraretinal fluid, blood, and hard exudates. RPE-choroid graft surgery was performed, and visual acuity improved to 20/32, and maintained up until 13.5 years postoperative. Microperimetry performed at the same time revealed a 3.4 dB sensitivity, with fixation on the graft. During the postoperative years glaucoma developed, an uveitis anterior was treated, and to treat a small Coats' like lesion; one bevacizumab injection was administered. CONCLUSIONS AND IMPORTANCE: A best corrected visual acuity of 20/32 could be achieved and maintained up to 13.5 years after an RPE-choroid graft transplantation, despite an unfavorable preoperative presentation and some early and late complications. This case is a proof of principle that an RPE-choroid graft harvested from the midperiphery can support the macular metabolism up to 13.5 after surgery in a patient with severe exudative AMD. It also represents a rationale for pursuing stem cell derived RPE replacement. Anti-vascular endothelial growth factor injections are nowadays the mainstay of therapy for choroidal neovascularization and/or small hemorrhages and offer good results. Nevertheless, selected patients that cannot benefit from this therapy may profit from an autologous RPE-choroid graft transplantation.

INTRAVITREAL VERSUS SUBRETINAL ADMINISTRATION OF RECOMBINANT TISSUE PLASMINOGEN ACTIVATOR COMBINED WITH GAS FOR ACUTE SUBMACULAR HEMORRHAGES DUE TO AGE-RELATED MACULAR DEGENERATION: An Exploratory Prospective Study.

PURPOSE: Current management of submacular hemorrhage (SMH) favors vitrectomy and gas with subretinal administration of recombinant tissue plasminogen activator (rtPA) over mere intravitreal rtPA injections and gas. In this study, we aimed to compare the effectiveness of both treatment modalities to displace submacular blood. METHODS: Twenty-four patients with SMH secondary to age-related macular degeneration were included. The SMH had to exist ≤14 days at time of surgery and SMH thickness had to be between 250 µm and 1,250 µm. Patients were randomized to either intravitreal injections of rtPA, perfluoropropane (C3F8) gas, and bevacizumab (n = 12) or vitrectomy with subretinal rtPA administration, intravitreal C3F8 gas, and bevacizumab (n = 12). The SMH volume change was measured on spectral domain optical coherence tomography postoperatively within a 2.5-mm cylinder centered at the fovea. RESULTS: Median relative volume reduction of subretinal blood at 6 weeks postoperatively was 97% (95% confidence interval: 91-99%) in the intravitreal rtPA group and 100% (95-100%) in the subretinal rtPA group and did not differ significantly between groups (P = 0.56). CONCLUSION: Both treatment modalities effectively displaced SMH in this exploratory clinical trial. To more definitely study the noninferiority of intravitreal rtPA with gas to subretinal rtPA, vitrectomy with gas, a larger clinical trial would be necessary.

Prospective, Randomized Intervention Study Comparing Retinal Pigment Epithelium-Choroid Graft Surgery and Anti-VEGF Therapy in Patients with Exudative Age-Related Macular Degeneration.

PURPOSE: To investigate whether patients with exudative age-related macular degeneration and a submacular hemorrhage, retinal pigment epithelium (RPE) tear or nonresponders to anti-vascular endothelial growth factor (VEGF) benefit more from a free RPE-choroid graft transplantation surgery than from (continuation of) anti-VEGF treatment. PROCEDURES: A total of 20 patients were included in this prospective, international, multicenter, randomized intervention study. RESULTS: The change in the mean number of Early Treatment of Diabetic Retinopathy Study (ETDRS) letters in the graft group 1 year postoperatively was -15 (range -54 to +26), whilst 2 patients experienced a gain of >10 letters. The median preoperative visual acuity (VA) was 0.75 logMAR (range 0.46-2.8), and the mean postoperative VA was 1.48 logMAR (range 0.14-2.8). The change in the mean number of ETDRS letters in the anti-VEGF group was -8 (range -26 to +6); no patients experienced a >10 letter gain. The median preoperative VA was 1.36 logMAR (range 0.58-1.6), and the median postoperative VA was 1.42 logMAR (range 0.44-1.66). CONCLUSIONS: The included patient group is far too small to draw conclusions. However, both gain and loss of VA may be experienced by patients undergoing either treatment method; more gain might be possible for patients with a graft in the absence of complications.

Direct Blood Flow Measurements in a Free RPE-Choroid Graft with Phase-Resolved Doppler OCT.

PURPOSE: We directly demonstrated the revascularization in a free retinal pigment epithelium (RPE)-choroid graft with direct blood flow detection by experimental phase-resolved Doppler optical coherence tomography (PRD-OCT). METHODS: Seven patients with age-related macular degeneration underwent an RPE-choroid graft translocation in a prospective institutional cohort study. Spectral domain optical coherence tomography (SD-OCT) was used to measure the revascularization stage. With PRD-OCT the presence of flow was imaged postoperatively. RESULTS: The PRD-OCT confirmed flow in three patients when SD-OCT indicated the afferent vessel ingrowth stage, and in all seven patients when the SD-OCT indicated the efferent vessel ingrowth stage. CONCLUSIONS: The PRD-OCT study was able to detect the presence of blood flow in a free RPE-choroid graft. The PRD-OCT findings directly confirmed the revascularization that was otherwise based on the more circumstantial evidence provided by SD-OCT images and angiography. TRANSLATIONAL RELEVANCE: The use of both techniques to monitor the revascularization process in a free graft in patients are an interesting example of replacing more invasive by noninvasive techniques. There is potential future use of PRD-OCT for the visualization of vascularization patterns in other pathologies.

There is no relation between the occurrence of proliferative vitreoretinopathy and the location of the donor site after transplantation of a free autologous retinal pigment epithelium-choroid graft.

PURPOSE: A free autologous retinal pigment epithelium (RPE)-choroid graft can be harvested during transplantation surgery from a 6 or 12 o'clock site in the midperiphery. This study evaluated whether proliferative vitreoretinopathy (PVR) occurs more frequently in patients with an inferior donor site retinotomy, which is not closed by the tamponade and is in contact with the hydrophilic, pro-inflammatory and fibrotic environment, than in patients with a superior donor site retinotomy. METHODS: Retrospective analysis of a prospective cohort of 246 patients with exudative age-related macular degeneration treated with an RPE-choroid graft transplantation and a lighter-than-water, 5000 centistoke silicone oil endotamponade. The location of the donor site, the presence or absence of PVR development and the location of PVR were noted. The two-tailed Fisher's exact test was used for statistical analysis. RESULTS: Thirty-nine of 246 (15.9%) patients developed PVR, of whom 35 had a superior donor site and four an inferior donor site. Of the 209 patients without PVR, 155 had a superior donor site and 25 had an inferior one. For 27 patients, no donor site location was explicitly documented in the patient files. We found no difference between the groups with a superior or inferior donor site and the occurrence of PVR (p=0.8). CONCLUSION: Shifting the inflammatory aqueous milieu away from the graft donor site does not prevent the occurrence of PVR.

Recombinant tissue plasminogen activator, vitrectomy, and gas for recent submacular hemorrhage displacement due to retinal macroaneurysm.

BACKGROUND: The visual prognosis of submacular hemorrhages caused by a retinal arterial macroaneurysm (RAM) is poor if left untreated. The use of recombinant tissue plasminogen activator (rtPA) has frequently been reported to displace submacular hemorrhages from the foveal area in patients with age-related macular degeneration. This study aims to investigate the results of displacement of recent-onset submacular hemorrhages due to RAM. METHODS: Institutional retrospective interventional case series of 12 patients with macular hemorrhage due to RAM, who underwent pars plana vitrectomy (PPV); followed in 11 by submacular injection of rtPA and gas tamponade. The main outcome measures were displacement of the hemorrhage, complication rate, and visual acuity at 1 month after surgery and at the last follow-up visit. RESULTS: One month after surgery, the hemorrhage had been successfully displaced in ten out of 11 patients. In these ten patients, visual acuity (VA) increased by a mean of 1.2 logMAR at 1 month after surgery. At the last follow-up visit, the mean increase was 1.5 logMAR. Complications consisted of a vitreous hemorrhage and hyphema, retinal detachment, a new submacular hemorrhage, and vitreous hemorrhage after argon laser retinal photocoagulation of the RAM. CONCLUSIONS: PPV with submacular rtPA and gas injection may successfully displace a recently developed submacular hemorrhage in patients with RAM, with a marked improvement in VA that is likely to be greater than if left untreated.

Literature review of recombinant tissue plasminogen activator used for recent-onset submacular hemorrhage displacement in age-related macular degeneration.

AIMS: To review and discuss the literature on recombinant tissue plasminogen activator (rtPA) for the treatment of a recent-onset submacular hemorrhage in patients with age-related macular degeneration. METHODS: The administration technique of rtPA, the use of additional gas and vascular endothelial growth factor inhibitors (anti-VEGF), and the displacement rate of submacular hemorrhage and complications were noted from published reports, and a case series from the Rotterdam Eye Hospital (REH). RESULTS: 38 studies with a total of 1,185 patients (1,176 eyes), and 28 patients from the REH were analyzed. Several methods for rtPA administration are available, which can be divided into two groups: submacular rtPA administration with vitrectomy; or intravitreal rtPA administration without vitrectomy. In both groups, the administration of gas and/or anti-VEGF agents could be additional. There appears to be no clear difference in complete displacement or complication rate between the more or the less invasive treatment groups. CONCLUSION: Although intravitreal injection of rtPA and gas only was reported to be as effective as subretinal rtPA with vitrectomy and gas, recent studies tend to use vitrectomy. These data underscore the need for a randomized controlled trial to choose the most effective and safe method of rtPA administration.

A free retinal pigment epithelium-choroid graft in patients with exudative age-related macular degeneration: results up to 7 years.

PURPOSE: To report and analyze long-term best-corrected visual acuity (BCVA) outcomes following a free autologous retinal pigment epithelium (RPE)-choroid graft translocation in patients with exudative age-related macular degeneration (AMD). DESIGN: Prospective cohort study. SETTING: Institutional. STUDY POPULATION: One hundred and thirty consecutive patients (133 eyes) with AMD underwent RPE-choroid graft translocation between October 2001 and February 2006. All patients had a subfoveal choroidal neovascular membrane with or without hemorrhage and/or an RPE tear. All were either ineligible for or nonresponsive to photodynamic therapy, the standard treatment at the time of surgery. OBSERVATION PROCEDURES: Data collection included preoperative and postoperative visual acuity measurements, fundus photography, fluorescein and indocyanine green angiography, and microperimetry. MAIN OUTCOME MEASURES: Postoperative BCVA. RESULTS: The mean preoperative BCVA was 20/250. Four years after surgery, 15% of the eyes had a BCVA of >20/200, and 5% had a BCVA of ≥20/40. One patient achieved a BCVA of 20/32, which was maintained at 7 years after surgery. Complications consisted of proliferative vitreoretinopathy (n = 13), recurrent neovascularization (n = 13), and hypotony (n = 2). CONCLUSIONS: RPE-choroid graft transplantation may maintain macular function for up to 7 years after surgery, with relatively low complication and recurrence rates. Retinal sensitivity, BCVA data, and fixation on the graft suggest that the graft, rather than simply the removal of submacular hemorrhage and/or choroidal neovascular membrane, was responsible for the preservation of macular function. This surgery may be an alternative for patients with AMD who cannot undergo other standard treatment.

Single-sided and small-scaled grasping of delicate tissues: effectiveness of indirect heat-induced attachment and detachment.

INTRODUCTION: Indirect heat-induced attachment and detachment (iHIAD) is a promising concept for gripping delicate tissues in microsurgery. However, the optimal settings of iHIAD are unknown. This study evaluates the effects of the instrument heating properties and initial contact force on the adhesion force, detachment success and thermal damage. MATERIAL AND METHODS: An instrument prototype was developed to test attachment and detachment for different combinations of generated energy (3.5-20.0 mJ) and pulse length (0.25-2.50 ms). The tissues tested on were kidney and eye from the pig. Thermal tissue damage was estimated with a histological analysis. RESULTS: The adhesion force F(a) depended strongly on the amount of generated energy (F(a) = 0.03-2.5 mN) and contact force (F(a) = 0.25-1.0 mN). Pulse length played a minor role. Detachment success (0-100%) was determined by generated energy (3-16 mJ). Histological analysis showed minimal damage. CONCLUSION: Adhesion forces increased with increasing contact forces. iHIAD proved sensitive to heating characteristics. Detachment success increased with generated energy. Thermal damage was minimal. iHIAD creates a potential to build better performing tissue manipulators.

Early perfusion of a free RPE-choroid graft in patients with exudative macular degeneration can be imaged with spectral domain-OCT.

PURPOSE: To study early flow and revascularization in a free, autologous, retinal pigment epithelium (RPE)-choroid graft. METHODS: This prospective cohort study used spectral domain-optical coherence tomography (SD-OCT) after RPE-choroid graft surgery in 12 patients. This SD-OCT was combined with fluorescein angiography (FA) and indocyanine green angiography (ICGA) in 5 patients. RESULTS: SD-OCT revealed that vessel diameter, number of vessels, and graft thickness increased in 10 of 12 patients, starting between 3 and 10 days after surgery. A subsequent decrease in thickness was found in all 10 patients, beginning as early as 8 days after surgery. Initially, the graft vessels were optically clearer than the underlying choroidal recipient vessels. Between 8 and 30 days after surgery, the optically clear vessels became gray, similar to the recipient choroid. FA and ICGA revealed perfusion in 4 of 5 patients between postoperative days 6 and 15. Between postoperative days 12 and 60, the entire choroidal structure of the graft was visible on ICGA. CONCLUSIONS; These data suggest that enlargement of vessel diameter, increase in the number of choroidal vessels, and graft thickening visualized by SD-OCT correspond with the ingrowth of afferent vessels, as demonstrated by ICGA. The subsequent establishment of efferent vessels results in flow, imaged as a change in color of the graft's vessels from optically clear to gray, graft thinning on SD-OCT, and complete revascularization on ICGA. SD-OCT, a noninvasive examination, can be used to demonstrate early graft perfusion in patients (trialregister.nl/trialreg/admin/rctview.asp number, NTR1768).