Severe retinopathy in a child with hypoplastic left heart syndrome

PURPOSE: We present the first reported case of a condition similar to retinopathy of prematurity in a full-term patient with hypoplastic left heart syndrome. DESIGN: Interventional case report. METHODS: We describe the clinical presentation and surgical treatment of a male baby with severe retinopathy and retinal detachments. RESULTS: The clinical course of this baby was similar to retinopathy of prematurity. The patient had areas of avascular retina, significant retinal neovascularization, fibrous proliferation, and tractional detachment. One eye had a totally detached retina; the other had functional vision after the retina was reattached by several vitreoretinal surgeries, including the use of intraocular silicone oil. CONCLUSIONS: This cyanotic condition can cause a retinopathy with chronic retinal neovascularization and retinal detachments. Permanent surgical retinal reattachment is difficult in the presence of neovascular activity that leads to intravitreous cicatrization.     

Anterior hyaloidal fibrovascular proliferation after diabetic vitrectomy

Vitrectomy was performed to treat 74 consecutive eyes for complications of diabetic retinopathy. Eight (13%) of 61 eyes followed up for an average of 12 months developed anterior hyaloidal fibrovascular proliferation. This was the most common postoperative complication, whose features included recurrent hemorrhages into the vitreous cavity or anterior vitreous, or both; vessels or fibrovascular tissue on the posterior lens capsule; anterior extraretinal vascularization extending toward the lens on the anterior hyaloid; traction detachment of the peripheral retina or ciliary body; and hypotony. Patients who developed this complication tended to be young males with severe retinal neovascularization and extensive retinal ischemia; traction retinal detachment as an indication for surgery; placement of a scleral buckle; postoperative rubeosis iridis, recurrent vitreous hemorrhages, and retinal detachment; and multiple surgeries. Four eyes progressed to atrophia bulbi. Early recognition followed by additional surgery in two patients and extensive additional photocoagulation in two other patients was successful in preserving good visual function.     

Late traction detachment in retinopathy of prematurity or ROP-like cases

Five cases of traction retinal detachment occurring later in life as a sequel of cicatricial retinopathy of prematurity or showing the clinical picture of retinopathy of prematurity are reported. They presented with taut membranes in vitreous cavity, causing traction retinal detachment, and often showed preretinal membranes. These membranes were collagen-rich and contained cells with glial characteristics. They seemed to be continuously produced on the surface of the retina from which they detached sometimes in multiple generations. It is likely that chronic exudation from vascular abnormalities is a stimulus for this proliferation. These cases are very similar to other vitreoretinal proliferations in association with vascular abnormalities (Coats' and von Hippel disease, exudative vitreoretinopathy). Supported by: Helena Rubinstein Foundation, N.Y., and Research to Prevent Blindness, N.Y.Presented at the Club Jules Gonin, Vienna, Austria, September 9, 1992     

Retinal vascular development and pathologic retinal angiogenesis are not impaired in matrix metalloproteinase-2 deficient mice

PURPOSE: Earlier studies have suggested a role for metalloproteinase-2 (MMP-2) in retinal angiogenesis. To investigate this further, we have studied retinal vascular development and pathologic ischemia-induced retinal angiogenesis in MMP-2-deficient and wild-type mice. METHODS: Vascular development of the retina was studied in retinal flatmounts, whereas pathologic retinal angiogenesis was analyzed in retinal flatmounts and on histologic sections using a model of ischemia-induced retinopathy. The time course of MMP-2 mRNA expression was determined by in situ hybridization and real-time polymerase chain reaction (PCR). RESULTS: Formation of the retinal vascular plexus was not significantly different in MMP-2-deficient mice as compared to wild-type mice. In ischemia-induced retinopathy, there was an increased formation of extraretinal neovascular tufts in the MMP-2-deficient mice (p < 0.05). MMP-2 mRNA expression did not correlate to either retinal vascular development or to ischemia-induced formation of extraretinal vascular tufts. CONCLUSIONS: The current data suggest that MMP-2 is not essential for either retinal vascular development or pathologic retinal neovascularization in the mouse.     

Pathologic study following vitrectomy for proliferative diabetic retinopathy

This article details the pathologic findings in eyes removed postmortem from a diabetic man with proliferative retinopathy, vitreous hemorrhage, and tractional retinal detachment. Several years before death, to control hemorrhage from extraretinal vasoproliferative lesions, one eye only was treated with argon laser panretinal photocoagulation. Eight months before death the treated eye also was operated for tractional retinal detachment with current vitrectomy methods: membranotomy, partial peeling, and segmentation of preretinal membranes. Despite their atropic clinical appearance, the extraretinal lesions on pathologic study were "active." In the operated and nonoperated eyes the vascular and nonvascular proliferative lesions were of equal severity. The sclerotomy wounds were complicated by intraocular granulation tissue, focal granulomatous inflammation related to suture fragments, and intraocular hemorrhage.     

The pathogenesis of retinopathy of prematurity as it relates to surgical treatment

Our current surgical treatment for threshold retinopathy of prematurity (ROP) is based upon three concepts which emerged from morphologic and biochemical study of 250 pairs of whole eye donations obtained over a ten year period. 1) Spindle cells normally migrate and canalize to form inner retinal vessels, but when stressed, spindle cells secrete angiogenic factors. The clinical implication is that transretinal cryotherapy to the avascular retina is efficacious because it obliterates spindle cells. The number and timing of cryosessions are determined by the migration and kinetics of spindle cells. 2) Myofibroblasts originate from the shunt, are the major cellular component of extraretinal fibrovascular proliferation (EFP), and contract to produce retinal distortion and detachment. The clinical implication is that a second transretinal cryotherapy session should obliterate the shunt and the EFP, and should eliminate the source of retinal traction. 3) Anterior ocular growth occurs exponentially during the period when ROP develops and is treated. The clinical implication is that a prophylactic scleral buckle supports the fixed surface area of the developing retina while the choroid and sclera enlarge anteriorly. Retinal distortion produces misaligned photoreceptors, and retinal detachment results in rapid retinal death.     

Acoustic studies in proliferative diabetic retinopathy

Based on analysis of published reports and his own experience of acoustic studies (B-method) in more than 500 patients with proliferative diabetic retinopathy, the author concludes that preretinal fibrovascular membranes (posterior detachment of the hyaloid membrane with neovascularization) are often mistaken for retinal detachment. Acoustically detected solitary or multiple adhesions between fibrovascular and eye membranes are an important differential diagnostic sign indicating the absence of retinal detachment. These adhesions result from gradual staged "creeping" posterior vitreal detachment and indicate the sites of previous contact of the hyaloid membrane with the retina. In subtotal detachment of the retina there are no adhesions with ocular membranes and the retina acquires the configuration of a rigid fibrovascular membrane.     

Tractional retinoschisis in proliferative diabetic retinopathy: a histopathological study

· Background: Proliferative diabetic retinopathy (PDR) often results in a tractional elevation of the retina. This study investigates the anatomical and histopathological structures of the vitreous body and the elevated retina in PDR. · Methods: Four globes (three patients) with PDR and known retinal elevation were analysed histologically. The tissues were embedded in celloidin and a special thick section technique (250 μm) was used for analysis under a stereomicroscope. · Results: All four cases showed adherence of the vitreous body with the retina and a tractional elevation of the retina at that site. In two cases retinoschisis was combined with retinal detachment and in the other two cases the retinal elevation was a pure retinal schisis. · Conclusion: Our histopathological investigations of PDR show that traction of the retina by shrinking of the vitreous body may result in a combination of retinoschisis and retinal detachment at the same area. These cases are clinically often diagnosed as retinal detachment, but because of the additional retinal schisis they are of poor functional prognosis. Received: 10 May 1999 Revised version received: 1 July 1999 Accepted: 22 July 1999     

Echography of optic disc related pathology in diabetic retinopathy

Combined A- and B-scan examination of eyes with diabetic retinopathy and opaque media provides information about the presence and density of vitreous hemorrhages, can detect membranes inserting into the optic disc or the retina and differentiate them from retinal detachment. Absolute and relative indications for echographic examination are listed and some echograms concerning vitreous hemorrhage, proliferating membranes and traction retinal detachment are presented. The value of both A-scan standardized echography and contact B-scan technique for previtrectomy evaluation is stressed.     

Principles of qualifying retinal detachment for pneumoretinopexy]

The gases SF6 and C2F6 were used in 88 patients (88 eyes) with retinal detachment as endotamponade. Eighty eight patients constituted 3 groups in which the gas was used on the basis of following indications: 1) the eyes with retinal detachment from above and with holes between 9-12-3 or with a macular hole; 2) eyes with retinal detachment "without hole" or with a hole for evaluation for its size and localization, as an intervention preceding an extrascleral invagination; 3) eyes with retinal detachment from above in which after extrascleral indentation in spite of a good localization the retina did not reappose. On the basis of the presented material the authors emphasize the values of the pneumoretinopexy as a solitary procedure, a diagnostic procedure or completing an extrascleral indentation. The perils of this method are presented and the causes of failures discussed.     

松弛性视网膜切开术治疗视网膜脱离合并严重前增殖性玻璃体视网 …

目的 探讨松弛性周边视网膜切开术联合眼内激光治疗视网膜脱离合并严重前增殖性玻璃体视网膜病变的效果。方法 对４４例视网膜脱离合并严重前增殖性玻璃体视网膜病变的患者行松弛性周边视网膜切开术,联合眼内激光和硅油填充治疗。观察术后视网膜解剖复位,视功能及术后眼压变化情况。其中２２例取出了硅油。结果 ４４例全部获得视网膜复位（其中８例合并皱褶）,３８例获得不同程度的视力改善。２２例取出硅油后,有２１例视网膜     

Pigment epithelium-derived factor in the vitreous is low in diabetic retinopathy and high in rhegmatogenous retinal detachment

PURPOSE: To report the levels of pigment epithelium-derived factor in the vitreous of patients with diabetic retinopathy, rhegmatogenous retinal detachment, and idiopathic macular hole. METHODS: Using enzyme-linked immunosorbent assay, we measured the levels of pigment epithelium-derived factor in the vitreous of 34 eyes of 33 patients who underwent vitrectomy for the treatment of diabetic retinopathy (17 eyes of 16 patients), rhegmatogenous retinal detachment (10 eyes), and idiopathic macular hole (seven eyes). RESULTS: The vitreal concentration of pigment epithelium-derived factor was 1.15 +/- 0.23 microg/ml (mean +/- standard error) in eyes with diabetic retinopathy, 3.28 +/- 0.69 microg/ml in rhegmatogenous retinal detachment, and 1.71 +/- 0.39 microg/ml in idiopathic macular hole. The pigment epithelium-derived factor level in rhegmatogenous retinal detachment was significantly higher than that in diabetic retinopathy (P =.0008) and idiopathic macular hole (P =.034). For eyes with diabetic retinopathy, the pigment epithelium-derived factor level was 0.88 +/- 0.21 microg/ml in proliferative diabetic retinopathy and 2.43 +/- 0.37 microg/ml in nonproliferative diabetic retinopathy (P =.0083). Additionally, the pigment epithelium-derived factor level in active diabetic retinopathy (0.70 +/- 0.22 microg/ml) was significantly lower than the level in inactive diabetic retinopathy (1.79 +/- 0.35 microg/ml; P =.018). CONCLUSIONS: These results suggest that pigment epithelium-derived factor inhibits angiogenesis and that lower levels of pigment epithelium-derived factor may be related to the angiogenesis in diabetic retinopathy and result in active proliferative diabetic retinopathy. The results also suggest that higher levels of pigment epithelium-derived factor in the eyes with rhegmatogenous retinal detachment may act as a neuroprotective agent for the detached retina.     

The simultaneous presentation of retinopathy of prematurity and central serous chorioretinopathy

A case report of central serous chorioretinopathy (CSC) and retinopathy of prematurity (ROP) in the same patient is presented. CSC is a disease of the outer retinal layers, specifically the retinal pigment epithelium, which results in serous detachment of the neurosensory retina. ROP is a disease of the anterior retinal layers, affecting the retinal vasculature. Ocular manifestations may range from the peripheral neovascularization observed in the milder states to retinal detachment and secondary angle closure glaucoma that is often found in the later stages. Therefore it would appear that there is no direct relationship between the presence of ROP and the presence of CSC in a patient who presented with complaints of a unilateral relative central scotoma. To our knowledge this represents a heretofore unreported association.     

玻璃体积血的形态结构与玻璃体后脱离的图像特征

目的探讨玻璃体积血的形态结构和治疗特点.方法对74例(79只眼)增生性糖尿病视网膜病变、视网膜血管炎、视网膜静脉阻塞所致玻璃体积血的临床资料进行比较分析.术前超声检查、术中手术显微镜观察患者的玻璃体形态特点,分析玻璃体与视网膜的关系.结果所有患者均有不同程度的玻璃体后脱离,根据图像的形态特征可归纳为完全后脱离和部分后脱离两种.部分后脱离又分为"V"型、"L"型及后部玻璃体劈裂型,劈裂型多见于视网膜缺血性疾病的增生期.结论了解和掌握玻璃体后脱离及玻璃体劈裂的形态特点,可提高手术治疗的成功率并改善其预后.     

Incidence of rhegmatogenous retinal detachment after vitrectomy in eyes of diabetic patients

PURPOSE: To assess the incidence of rhegmatogenous retinal detachment (RRD) after pars plana vitrectomy (PPV) among diabetic patients with complications of proliferative diabetic retinopathy. METHODS: Ninety-three eyes of diabetic patients-who underwent PPV with or without intraocular gas tamponade for complications of proliferative diabetic retinopathy-were reviewed retrospectively. Indication for vitrectomy was vitreous hemorrhage in 80 patients (86.1%), tractional retinal detachment in 3 (3.2%), and vitreous hemorrhage associated with tractional retinal detachment in 10 (10.7%). RESULTS: Four (4.3%) of 93 eyes developed an RRD after vitrectomy. The primary reason for vitrectomy was recurrent or nonresolving vitreous hemorrhage. The retina was attached with one additional surgical procedure in two of these eyes; the other two had to undergo a third operation before attachment was achieved. CONCLUSION: RRD occurs in a small percentage of patients after PPV with or without gas tamponade for vitreous hemorrhage or tractional retinal detachment caused by proliferative diabetic retinopathy. Thorough postoperative follow-up is important to make early diagnosis and intervention possible.     

Retina-specific expression of PDGF-B versus PDGF-A: vascular versus nonvascular proliferative retinopathy

PURPOSE: Platelet-derived growth factor (PDGF) has been implicated in vascular proliferative retinopathies, such as diabetic retinopathy, and in nonvascular retinopathies, such as proliferative vitreoretinopathy. Traction retinal detachment is a central feature of both types of disease. Hemizygous rhodopsin promoter/PDGF-B (rho/PDGF-B) transgenic mice exhibit proliferation of vascular cells, glia, and retinal pigmented epithelial (RPE) cells, resulting in traction retinal detachment. Hemizygous rho/PDGF-A transgenic mice show mild proliferation of glial cells and no traction retinal detachments. This study was undertaken to determine whether higher levels of endogenously produced PDGF-A in the retinas of mice result in retinal detachment. METHODS: To achieve high-level expression of PDGF-A in the retina, homozygous rho/PDGF-A (rho/PDGF-AA) mice were generated. The phenotype of these mice was compared with that of homozygous rho/PDGF-B (rho/PDGF-BB) mice and double hemizygous rho/PDGF-B-rho/PDGF-A (rho/PDGF-AB) mice. RESULTS: Rho/PDGF-BB and rho/PDGF-AB mice showed a phenotype similar to that previously described in rho/PDGF-B mice. There was extensive proliferation of glial and vascular cells, resulting in fibrovascular membranes that detached the retina. PDGF-AA mice showed extensive proliferation of glial cells and traction retinal detachment. CONCLUSIONS: High retinal expression of PDGF-A results in extensive proliferation of glial cells and traction retinal detachment without vascular cell involvement, similar to proliferative vitreoretinopathy in humans. High retinal expression of PDGF-B results in traction retinal detachment from proliferation of both vascular and nonvascular cells, similar to diabetic retinopathy in humans.     

Retinopatía MEK. Casos clínicos

Case reports

Three clinical cases are presented of MEK retinopathy associated with the combination of cobimetinib and vemurafenib characterised by alteration of the retinal pigment epithelium and neurosensory detachment. Two of the cases conserved the vision of the unit, and the third developed a large bilateral neurosensory detachment with final visual acuity of 0.6 for the right eye and 0.1 for the left one.

Optical coherence tomography of chronic solar retinopathy

PURPOSE: To describe the optical coherence tomographic findings in eight eyes of four patients with chronic solar retinopathy. All patients had a history of sun gazing months to years prior to presentation. DESIGN: Retrospective case series. METHODS: Patients were examined using biomicroscopy performed by two or more experienced vitreoretinal specialists. All eight eyes had optical coherence tomography performed at the time of initial examination. RESULTS: All patients had a history of sun gazing and decreased vision in both eyes. On biomicroscopy, all eyes had a small, irregularly-shaped lamellar defect in the foveal center. No posterior vitreous detachment was present in any eye. Optical coherence tomography revealed a hyporeflective space at the level of the outer neurosensory retina and retinal pigment epithelium in all eyes. CONCLUSION: Optical coherence tomography demonstrates a characteristic hyporeflective space in the outer retina in patients with chronic solar retinopathy. These findings correlate with the histopathology of this condition and may be a useful clinical tool in confirming its diagnosis.     

RPE细胞发生上皮间充质转化机制及PVR的治疗研究进展

增殖性玻璃体视网膜病变(proliferative vitreoretinopathy,PVR)是眼外伤、糖尿病性视网膜病变、血管性视网膜病变和炎症性视网膜病变等眼部疾病的严重并发症,也是孔源性视网膜脱离手术失败的最重要原因,对视功能的危害较大。大量研究已证明PVR发生的主要危险因素是视网膜损伤后血视网膜屏障受损,视网膜色素上皮(retinal pigment epithelial,RPE)细胞受到玻璃体腔内细胞因子的刺激,RPE细胞发生上皮间充质转化(epithelial-mesenchymal transition,EMT),转分化为成纤维样细胞,细胞的形态发生了变化,细胞间的紧密连接消失,细胞极性丧失,增殖、迁移、侵袭能力增强。在视网膜前表面或视网膜下形成具有收缩性的纤维增殖膜,形成的纤维增殖膜会使视网膜形成皱褶,牵拉视网膜导致视网膜脱离,最终会导致患者视力下降甚至失明。国内外对预防和治疗PVR进行了大量的研究,本文对RPE细胞发生上皮间充质转化相关信号通路及PVR的治疗进行简要综述。     

孔源性视网膜脱离的治疗进展

孔源性视网膜脱离（ RRD）是以玻璃体和视网膜共同参与视网膜裂孔形成为特征,是视网膜神经上皮层（ NSR）及视网膜色素上皮层（ RPE）分离的一类视网膜病变,严重威胁患者的视功能,因此早期的发现预防及选择恰当的治疗方法在临床上显得至关重要。本文将对孔源性视网膜脱离的治疗进展做一综述。