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Background: Primary vitreous floaters can be highly bothersome in some patients. In the case of persistently bothersome floaters, pars plana vitrectomy may be the most effective treatment. The aim of this study is to evaluate the incidence of complications, and patient satisfaction, after pars plana vitrectomy for disabling primary vitreous opacities., Methods: We included a total of 110 eyes that underwent pars plana vitrectomy between February 1998 and August 2010. Fifty-seven eyes (51.8%) underwent 20-gauge vitrectomy, whereas 53 eyes (48.2%) underwent 23-gauge vitrectomy. In a retrospective manner, we assessed intraoperative and postoperative complications. There was a considerable range of time between surgery and questionnaire (range: 4-136 months). Patient satisfaction was assessed by a questionnaire based on a modified NEI VFQ-25 questionnaire., Results: A retinal detachment occurred in 10.9% of cases, and the incidence did not differ significantly between the 20-gauge and 23-gauge vitrectomy groups. In 4.5% of the eyes, a retinal detachment developed within the first 3 months, and 6.4% occurred later in the postoperative period. Cystoid macular edema occurred in 5.5%, and an epiretinal membrane was seen postoperatively in 3.6% of cases. Development of glaucoma requiring glaucoma surgery, a macular hole, and postoperative scotoma, each occurred in 0.9% of cases. No cases of endophthalmitis occurred. Eighty-five percent of patients were satisfied or very satisfied with the results of the vitrectomy. Eighty-four percent of all patients were completely cured from their troublesome vitreous floaters, and an additional 9.3% of patients were less troubled by vitreous floaters. Ten patients (9.3%) were dissatisfied, and six of these patients (5.6%) had a serious complication that resulted in permanent visual loss., Conclusions: Pars plana vitrectomy is an effective approach to treat primary vitreous floaters, resulting in a high rate of patient satisfaction. Postoperative complications may be more frequent than previously reported, so patients should be well-informed about the complication rate before reaching informed consent about this surgical intervention. Additional preventive measures should be considered to reduce this complication rate.

Wnt signaling is a crucial component of the cell machinery orchestrating a series of physiological processes such as cell survival, proliferation, and migration. Among the plethora of roles that Wnt signaling plays, its canonical branch regulates eye organogenesis and angiogenesis. Mutations in the genes encoding the low density lipoprotein receptor protein 5 (LRP5) and frizzled 4 (FZD4), acting as coreceptors for Wnt ligands, cause familial exudative vitreoretinopathy (FEVR). Moreover, mutations in the gene encoding NDP, a ligand for these Wnt receptors, cause Norrie disease and FEVR. Both FEVR and Norrie disease share similar phenotypic characteristics, including abnormal vascularization of the peripheral retina and formation of fibrovascular masses in the eye that can lead to blindness. In this mutation update, we report 21 novel variants for FZD4, LRP5, and NDP, and discuss the putative functional consequences of missense mutations. In addition, we provide a comprehensive overview of all previously published variants in the aforementioned genes and summarize the phenotypic characteristics in mouse models carrying mutations in the orthologous genes. The increasing molecular understanding of Wnt signaling, related to ocular development and blood supply, offers more tools for accurate disease diagnosis that may be important in the development of therapeutic interventions.

Purpose: To evaluate the clinical characteristics, bacterial culture, and visual outcome of patients with acute endophthalmitis after cataract surgery., Design: Retrospective consecutive interventional case series., Methods: Clinical notes from patients treated for acute endophthalmitis after cataract surgery in a single center from 1996 to 2006 were reviewed. Patients with less than 1 month of follow-up and missing bacterial cultures were excluded. Vitreous biopsy or primary vitrectomy followed by intravitreal injection of vancomycin and ceftazidime (+/- prednisolone) was performed. Main outcome measures were bacterial culture and final visual acuity., Results: Bacterial cultures (total 250 cases) showed bacterial growth in 166 cases (66.4%). From these 166 cultures, 89 (53.6%) revealed gram-positive coagulase-negative, 63 (38.0%) other gram-positive, 10 (6.0%) gram-negative, and 4 (2.4%) polymicrobial cultures. Vitreous biopsy with intravitreal antibiotics injection was performed in 225 (90.0%) of cases. Primary vitrectomy with intravitreal antibiotics was performed in 25 eyes (10.0%). Final visual acuity >/=0.5 was achieved in 129 (51.6%) of all cases, 54 (60.7%) of the 89 gram-positive coagulase-negative cultures, 20 (31.7%) of the 63 other gram-positive cultures, 5 (50.0%) of the 10 gram-negative cultures, and 9 (45.0%) of the 20 Staphylococcus aureus cultures. There was no additional effect for treatment by primary vitrectomy or intravitreal prednisolone., Conclusions: Treatment outcome after endophthalmitis is highly dependent on the causative organism. Treatment outcomes for gram-negative bacteria and S. aureus may be better than previously reported. Prompt treatment of endophthalmitis remains essential and the role of complete primary vitrectomy remains subject to debate., ((c) 2010 Elsevier Inc. All rights reserved.)

Familial exudative vitreoretinopathy (FEVR) is a genetically heterogeneous retinal disorder characterized by abnormal vascularisation of the peripheral retina, often accompanied by retinal detachment. To date, mutations in three genes (FZD4, LRP5, and NDP) have been shown to be causative for FEVR. In two large Dutch pedigrees segregating autosomal-dominant FEVR, genome-wide SNP analysis identified an FEVR locus of approximately 40 Mb on chromosome 7. Microsatellite marker analysis suggested similar at risk haplotypes in patients of both families. To identify the causative gene, we applied next-generation sequencing in the proband of one of the families, by analyzing all exons and intron-exon boundaries of 338 genes, in addition to microRNAs, noncoding RNAs, and other highly conserved genomic regions in the 40 Mb linkage interval. After detailed bioinformatic analysis of the sequence data, prioritization of all detected sequence variants led to three candidates to be considered as the causative genetic defect in this family. One of these variants was an alanine-to-proline substitution in the transmembrane 4 superfamily member 12 protein, encoded by TSPAN12. This protein has very recently been implicated in regulating the development of retinal vasculature, together with the proteins encoded by FZD4, LRP5, and NDP. Sequence analysis of TSPAN12 revealed two mutations segregating in five of 11 FEVR families, indicating that mutations in TSPAN12 are a relatively frequent cause of FEVR. Furthermore, we demonstrate the power of targeted next-generation sequencing technology to identify disease genes in linkage intervals., (Copyright (c) 2010 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe the ophthalmic characteristics and to identify the molecular cause of FEVR in a cohort of Dutch probands and their family members., Methods: Twenty families with familial exudative vitreoretinopathy (FEVR) comprising 83 affected and nonaffected individuals were studied. Based on the presence of an avascular zone, the clinical diagnosis was made and biometric data of the posterior pole of 57 patients and family members were obtained by the analysis of fundus photographs and compared with the data of 40 controls. The FZD4, LRP5, and NDP genes were screened for mutations in one affected individual per family. The segregation of the gene variants was studied in the corresponding families., Results: Forty of 83 individuals showed an avascular zone, the most evident clinical sign of FEVR, five showed major signs of FEVR, and 38 persons were not clinically affected. Compared with the control subjects the patients with FEVR had a significantly larger disc-to-macula distance and a significantly smaller optic disc. In 8 of 20 families, a FZD4 mutation was identified, in 2 a mutation in the LRP5 gene, and in 2 a mutation in the NDP gene. Three known and five novel mutations were identified. Nonpenetrance was observed in 26% of the mutation carriers., Conclusions: Significant anatomic differences were identified between the eyes of patients with FEVR with an avascular zone, when compared with those of the control subjects. In patients with an avascular zone, the optic disc was smaller and the disc-to-macula distance larger than in the control subjects. In 60% of the probands, mutations were identified in one of the three known FEVR genes.

Amniotic membrane transplantation (AMT) is an effective treatment for ocular surface reconstruction; however, the mechanisms through which amniotic membrane (AM) exerts its effects as well as its fate after transplantation have not been entirely elucidated and have been investigated only in part. We evaluate the integration of AM in the host cornea in five patients who underwent AMT as the result of Bowen's disease, band keratopathy, radio- or cryotherapy-induced keratopathy, chemical burn or post-herpetic deep corneal ulcer with descemetocele. Due to persistent opacification in four cases and a progressing tumor in one case, penetrating keratoplasty (PK) and enucleation were performed as early as 2 months and up to 20 months after AMT. The corneas were analyzed histopathologically. To evaluate AM remnants, corneas were stained with periodic acid Schiff's reaction (PAS), Alcian blue, and Gomory and Masson trichrome; immunostaining including collagens III and IV antibodies was also performed. None of the corneas showed remnants of AM. In all cases, we observed discontinuity of Bowman's membrane. In three cases, the corneal epithelium was completely restored, ranging from three to six cell layers. In the other two cases, we detected an intense inflammatory reaction with rich neovascularization; the epithelial surface of the central cornea was completely restored, while at the periphery of the cornea goblet mucus-producing cells were present. Although clinically useful in all cases, restoration of a stable corneal epithelium through AMT is limited by the extent and severity of limbal stem cell deficiency (LSCD). The lack of histologically documented AM remnants in our cases seems to explain the efficacy of AMT more through its biological properties than through its mechanical properties., (2004 Wiley-Liss, Inc.)

Purpose: To study the clinical appearance and surgical results of autosomal dominantly inherited rhegmatogenous retinal detachments (RRDs)., Methods: After prospective examination of all but two family members, the medical records of 16 affected patients (21 eyes) of two families from the Netherlands with autosomal dominantly inherited RRD were retrospectively evaluated. Special attention was paid to the age at onset, the ocular morphology and the clinical appearance of the RRD. The type and number of the various surgical procedures were analyzed with respect to preoperative appearance of the RRD, postoperative results and final visual acuity., Results: The mean age at onset of RRD of affected individuals in families A and B was 37 +/- 18 years and 19 +/- 10 years, respectively. The mean ocular axial length in the two families was 24.7 mm and 26.7 mm. The mean number of retinal defects preoperatively found was 2.2 in family A and 7.1 in family B. Round, atrophic retinal holes predominated. Two of 21 affected eyes showed significant preoperative proliferative vitreoretinopathy. Pars plana vitrectomy was the primary procedure in 4 cases; extra ocular buckling was the initial procedure in 15 cases. One eye received scleral folding with diathermy as primary surgery. Redetachment following surgery occurred in 5 of 10 cases in family A and 4 of 10 eyes in family B. Anatomical success could be achieved in 9 of 10 and 8 of 10 eyes in families A and B, respectively., Conclusions: In these families the prevalence of RRD is high. Most patients were affected at a relatively young age compared with non-genetically linked forms of RRD. Because of the low success rate of surgical intervention and, subsequently, the high number of operations necessary to achieve reattachment of the retina, the use of diagnostic genetic techniques to identify individuals at risk would be advisable. In these subjects measures to prevent RRD are an option, even when anatomical substrates of precursors of RRD are absent.

Background: The use of a mixture of silicone oil and partially fluorinated alkanes (high-density silicone oil) has recently been suggested as intraocular tamponade in complicated retinal detachment of the inferior quadrants. We describe a series of patients who developed a clinical picture resembling anterior granulomatous uveitis following endotamponade with high-density silicone oil., Methods: We evaluated 19 eyes of 18 patients who underwent pars plana vitrectomy and intraocular tamponade with high-density silicone oil (Oxane HD). The indication for this type of intraocular tamponade was limited to cases with complicated retinal detachment of the inferior quadrants. Oxane HD was removed after a mean period of 3 months., Results: One to eight weeks following vitrectomy with high-density silicone oil, an intraocular inflammation was observed in 7 of 19 eyes (37%). These eyes presented with keratic precipitates (KP), pigmented clumps in the inferior part of the anterior chamber and increased anterior chamber cellular reaction. This inflammatory response did not react to topical steroids. In addition to KP a considerable amount of cellular precipitation was noted on the surface of the oil bubble. Strikingly, the intraocular inflammatory signs completely resolved following removal of the high-density silicone oil., Conclusions: An inflammatory response, resembling granulomatous uveitis, occurs in a significant number of patients after high-density silicone oil endotamponade. It is likely that this vitreous substitute is an immunogenic agent, given the complete resolution of the inflammation after removal of the high-density silicone oil. The routine use of this newly developed silicone oil should therefore await the outcome of additional controlled clinical trials.

Purpose: To report a case of bacterial endophthalmitis after otitis media in a healthy adult., Design: Interventional case report., Methods: A healthy 49-year-old Caucasian woman developed acute otitis media of the right ear. Three weeks after the first onset there was a recurrence of the otitis media, with perforation of the tympanic membrane. Two days after this, the woman presented at our clinic with endophthalmitis of the right eye., Results: A culture of vitreous material grew Streptococcus pyogenes (Streptococcus Lancefield group A). The same strain was found in a smear from the perforated ear. Despite aggressive treatment, the affected eye had to be eviscerated., Conclusions: Otitis media can result in a bacteremia. This may, even in a healthy adult, lead to a devastating endogenous bacterial endophthalmitis.

Purpose: To investigate a new type of self-sealing pars plana sclerotomy clinically and by means of ultrasound biomicroscopy and to compare the outcome with conventionally sutured sclerotomies., Design: Prospective, nonrandomized clinical trial., Methods: Twenty-one patients underwent pars plana vitrectomy either with (control group) or without suturing (study group) at the Institute of Ophthalmology, University Medical Centre Nijmegen, The Netherlands, between January 1 and March 31, 2002. One day and 6 to 8 weeks postoperatively, the sclerotomy sites were studied by clinical examination and ultrasound biomicroscopy. Results were graded according to verbal descriptor scales., Results: The study included 21 patients scheduled for pars plana vitrectomy of one eye. Mean follow-up time was 6.5 weeks (range, 6-8). Three patients were lost to follow-up. Of the remaining patients, 12 had self-sealing wedge-sclerotomies and six were conventionally operated. No intraoperative complications occurred in any participant and no visual loss or postoperative hypotony was observed. On the first day after surgery there was significantly more scleral dehiscence in the control group (P =.012). This divergence disappeared during the follow-up period. All other differences noticed were not statistically significant., Conclusions: Our novel technique of sutureless wedge-shaped sclerotomy allows the creation of simple, reliable, and self-sealing pars plana openings. Clinical examination and assessment by ultrasound biomicroscopy showed results to be at least as safe as conventionally sutured sclerotomies. This new method allows stable intraoperative ocular pressure conditions and seems promising.

Background: A massive intraocular hemorrhage in the course of age-related macular degeneration (AMD) is a devastating event. We set out to determine the role of anticoagulant therapy prescribed for vascular or cardiac indications in the development of a massive hemorrhage., Methods: A retrospective case-controlled study was conducted of 50 cases of age-related macular degeneration complicated by massive subretinal and vitreous hemorrhage. The control group consisted of 50 cases of AMD with small subretinal hemorrhage., Results: There was a significant difference in the use of anticoagulant medication (warfarin sodium) between the groups. The difference in the use of antiplatelet medication (aspirin) between the groups was not significant. A patient with a massive intraocular hemorrhage and AMD is 11.6 times more likely to use anticoagulant medication. It appeared that more than 50% of the patients in the massive hemorrhage group were allowed to stop the anticoagulant medication., Conclusion: Anticoagulant medication poses a significant risk in the development of a massive intraocular hemorrhage in patients with exudative AMD. Antiplatelet medication poses a less significant risk. Physicians prescribing anticoagulant medication should be informed about the macular status of the patient. the In case of neovascular AMD, anticoagulant medication should be prescribed only for absolute systemic indications.

Objective: To describe pattern-reversal visual evoked response (PRVEP) and pattern electroretinogram (PERG) parameters in eyes with macular hole and their value for predicting postoperative visual outcome., Methods: Prospectively we studied 27 eyes (27 patients) with a full-thickness macular hole. Preoperatively the hole and rim were measured and the PRVEP and PERG were recorded. The preoperative parameters were correlated with postoperative visual outcome., Results: The macular hole was closed in 26 of 27 eyes. Sixteen eyes (59%) had an increase in visual acuity (VA) of two lines or more, 10 eyes (37%) remained within one line of preoperative VA and 1 eye (4%) had a decrease in VA of two lines. Duration of symptoms was negatively correlated with preoperative VA (R=-0.547, P=0.0038) and postoperative VA (R=-0.519, P=0.0065) and positively correlated with hole area (R=0.533, P=0.0061) and rim area R=0.633, P=0.0009). Only the PRVEP P100 latency of the 10' check size and the PERG N35 latency were significantly associated with visual outcome (P=0.022 and P=0.042 respectively)., Conclusions: There was no association of either hole or rim size with postoperative visual outcome. Preoperative electrophysiology, however, is useful as a prognostic tool. Utilization is limited to the use of latency parameters of the response and is dependent on the check size of the stimulus.

Background: Surgery for macular gliosis and macular holes has become increasingly successful with regard to anatomical outcome. Assessment of the damage to the receptors by these processes is still difficult, but is important in predicting functional outcome., Methods: Examination with the Nagel II or the Neitz OT anomaloscope was performed in 36 patients with macular gliosis, 23 patients with full-thickness macular holes and 47 patients with central serous choroidopathy. The anomaloscope matches were expressed as the quotient of anomaly., Results: In macular gliosis the mid-matching point is usually 1.0; there is no pseudoprotanomaly. In macular holes the mid-matching point is 1.0 when visual acuity is 0.3 or greater; in eyes with lower visual acuity there may be signs of diminished red sensitivity, but anomaloscope examination becomes difficult. In central serous choroidopathy the mid-matching point is shifted towards red, and pseudoprotanomaly is present, even when visual acuity is normal., Conclusions: Diseases of the inner retina, in early stages, do not alter colour vision substantially, whereas diseases of the outer retina give rise to early colour vision deficiency. In macular gliosis and macular holes, anomaloscope examination enables estimation of macular receptor misalignment.

Background: Prognostically favorable factors for epiretinal membrane removal have been described in the literature by several authors. Little information, however, is available about the objective assessment of the preoperative macular function. This study reports the results of idiopathic epiretinal membrane removal and the prognostic value of preoperative, pattern reversal visual-evoked potentials (PRVEPS) in recovery of visual acuity (VA)., Methods: In 60 patients (60 eyes) with idiopathic epiretinal membrane we performed PRVEP examination preoperatively. All eyes were operated on by standard three-port vitrectomy with membrane removal. Two eyes were excluded because of postoperative complications. Follow-up VA was compared with preoperative VA for the 58 study eyes and correlated with preoperative PRVEP parameters., Results: The mean preoperative VA was 0.2, the mean postoperative VA, 0.4. The PRVEP was recordable in 74%, 67% and 36% of cases for check sizes of 17, 10 and 7 arcmin respectively. Twenty patients (50%) had an increase in VA of two lines or more, in 25 patients (43%) VA remained within one line of the preoperative value, and in 4 patients (7%) VA decreased by two lines or more. The mean preoperative VA was not significantly different between the group with an improved VA and the group that did not benefit from membrane removal. Of the PRVEP parameters, only the N80 latency for the 17' check size was significantly associated with postoperative visual outcome., Conclusion: The PRVEP is applicable as a predictor for visual outcome in cases of epiretinal membrane removal. For the 17' pattern size we found a significant association of the combination of recordability and delayed N80 latency with visual outcome.

Purpose: To investigate whether the pattern reversal visual evoked potential can be useful in the diagnosis and management of macular hole patients., Methods: The pattern reversal visual evoked potential was measured in 66 patients with a macular hole and in 43 healthy control subjects. Check sizes of 34', 17' and 10' were applied., Results: Results showed that, for the check sizes of 34', 17' and 10', eyes with a macular hole had significantly prolonged N80 and P100 latencies and a significantly reduced P100 amplitude as compared to their fellow eyes. Furthermore, for the 10' check size, the fellow eyes appeared to have a significantly reduced P100 amplitude in comparison with the control eyes, whereas N80 and P100 latencies of the fellow eyes of the macular hole patients were not prolonged., Conclusion: Significant pattern reversal visual evoked potential alterations were shown in eyes with macular holes and fellow eyes for small check sizes.

Background: To report our experience with laser suture lysis (LSL) following trabeculectomy with mitomycin C, its timing, effectiveness and related complications., Methods: We retrospectively examined 38 consecutive eyes of 36 patients that underwent LSL following trabeculectomy with mitomycin C., Results: The mean preLSL intraocular pressure (IOP) was 27.0 mm Hg (SD 6.3, range 16-39 mm Hg) and the postLSL IOP (IOP 1 h after the last session of LSL) was 16.0 mm Hg (SD 7.2, range 3-31 mm Hg). Following the LSL in 7 eyes (7 of 38) hypotony (IOP<6 mm Hg, lasting more than 24 h) developed. Two groups were defined. In group I no hypotony was found after LSL and group II went through a period of hypotony. The time interval between surgery and LSL was significantly shorter in group II (mean 5.7, SD 7.5, range 1-19 days), compared to group I ( mean 14.7, SD 13.0, range 1-44 days) (p=0.041). The mean final IOP (IOP measured at the last visit) was 13.3 mm Hg (SD 3.4, range 6-20 mm Hg) and mean follow-up was 6.1 months (SD 3.9, range 2.0-15.2 months). No hypotony was found at final examination. PostLSL IOP and final IOP were significantly lower in group II (p=0.002 and p=0.024 respectively). IOP reduction by LSL was significantly greater in group II (p=0.046)., Conclusion: LSL is an effective and safe procedure to lower the IOP following trabeculectomy with mitomycin C. Early application of LSL results in lower final IOPs, but has a higher risk of hypotony.

Purpose: To determine the extent of pattern-reversal visual evoked potential parameter alteration by epiretinal membranes and to investigate the use of pattern-reversal visual evoked potential in the estimation of macular function in eyes with epiretinal membrane and in the fellow eyes., Methods: In both eyes of 162 patients with epiretinal membrane, 92 of primary and 70 of secondary origin, pattern-reversal visual evoked potentials were recorded. Check sizes of 17', 10', and 7' (minutes of arc) were used. Parameters investigated were N80 and P100 latencies and P100 amplitude., Results: No significant difference was detected between eyes with epiretinal membrane of primary and secondary origin regarding visual acuity and the pattern-reversal visual evoked potential parameters for the different check sizes. Compared with the fellow eyes, the eyes with epiretinal membrane had a significantly reduced visual acuity, prolonged N80 and P100 latencies, and a reduced P100 amplitude for the different check sizes. Compared with a separate control group (N = 20) with patients 50 to 59 years old, eyes with epiretinal membrane (N = 9) showed the same features as in the total group, but only for the 17' and 10' check sizes. The fellow eyes (N = 9) showed a significant reduction of the P100 amplitude (P < .05) for the pattern sizes of 17' and 10', but no difference in visual acuity or pattern-reversal visual evoked potential latency was found., Conclusions: In eyes with epiretinal membrane, pattern-reversal visual evoked potential latencies are prolonged, and amplitude is reduced. Relationships between clinical parameters and pattern-reversal visual evoked potential parameters require further study.

In the surgical treatment of full-thickness macular holes good results have been published with the combination of vitrectomy, gas and application of Transforming Growth Factor beta 2. Other authors report a 73% success rate in closing a full-thickness macular hole after vitrectomy and gas tamponade alone. We used, in addition to vitrectomy and gas tamponade, a tissue glue to stimulate adhesion of the elevated cuff of neurosensory retina surrounding a full-thickness macular hole and to close to hole itself. A total of 15 eyes of 13 patients (3 men, 10 women), with stage 3 and 4 macular holes were operated. All of the 13 uncomplicated cases showed complete closure of the macular hole. In one case (8%) the visual acuity decreased one line in spite of a funduscopically closed hole, and in two cases (16%) visual acuity remained the same despite closure. Increased visual acuity was seen in ten cases (76%), eight of which improved more than two lines.