Familial exudative vitreoretinopathy: surgical intervention and visual acuity outcomes

Background: Familial exudative vitreoretinopathy (FEVR) is a hereditary condition that may lead to vitreous hemorrhage and traction retinal detachment necessitating surgical intervention. In this paper we review the results of surgery on seven such patients (eight eyes). Methods: Seven patients (eight eyes) were followed up after surgery that had been performed because of vitreous hemorrhage and/or traction retinal detachment due to FEVR, in an effort to evaluate outcomes. Parameters that were noted were the current age, gender, age at the time of first surgery, length of follow-up and postoperative retinal status and visual acuity. Results: Seven patients (eight eyes) ranging in age from 6 months to 44 years with a mean of 24.7 and a median of 26 years were followed. There were three females and four males. The lowest age at which surgery was first performed was 6 months and the highest was 28 years, with a mean of 14.7 and a median of 17 years. Six of the 8 eyes were reattached following surgery, although some required multiple procedures. Conclusion: Vitreoretinal surgery may be of benefit in helping to preserve some degree of vision in eyes of patients with FEVR who develop vitreous hemorrhage and/or retinal detachment.Presented at the combined meeting of the Club Jules Gonin and the Retina Society, 3 September 1996     

Unusual retinal pigment epitheliopathy and choroidopathy in carcinomatosis: A rare case of cancer-associated retinopathy

Background: Cancer-associated retinopathy is a syndrome causing ocular symptoms. It is a rare entity and only a few cases have been reported. Methods: A 67-year-old woman with small-cell endometrial carcinoma suffering from deterioration of visual acuity is presented. Results: The patient presented with extensive mottled changes of the retinal pigment epithelium, accompanied by diffuse subretinal fluid in the posterior pole and exudative retinal detachments inferior in both eyes. Conclusion: This patient suffered from a rare variety of cancer-associated retinopathy.     

Pressure sore in a patient who underwent repair of a retinal tear with gas injection

Purpose: To demonstrate a pressure sore following strict head positioning in a patient who underwent encircling band, vitrectomy and gas injection. Methods: A male patient was admitted to the hospital with a large posterior horseshoe tear in the inferior temporal retina with severe vitreous traction and retinal detachment. Encircling band, vitrectomy cryotherapy and gas injection was performed. After surgery the patient was instructed to sit in a facedown position. Results: A pressure sore resulted from prolonged immobility of the right elbow due to face-down positioning following encircling band, vitrectomy and gas injection. Conclusion: A patient injection. Conclusion: A patient may rarely have compulsive personality traits that result in extreme compliance to the physician's recommendations; therefore, general instructions given for head positioning should include permission for a change in position when required, at least for brief periods of time.     

Vitrectomy for traction macular detachment in diabetic retinopathy

Background: A small number of eyes with proliferative diabetic retinopathy develop massive central fibrovascular membranes characterized by vitreoretinal tractions along the arcades and optic disk and retinal traction lines extending through the macula. The aim of our study was first to present the results of vitrectomy for removal of these central membranes and second to determine the correlation between preoperative parameters and postoperative visual outcome. Subjects and methods: We treated 28 eyes with severe central fibrovascular diabetic membranes by a modified bimanual en bloc excision technique during vitrectomy. Preoperative examination included general status, visual acuity, slit-lamp investigation, binocular funduscopy, ultrasound investigation and visual evoked potentials (VEP). Further, we analyzed intraoperative complications and postoperative anatomic and functional outcomes. Results: The retinas of 27 eyes with central traction retinal detachments were reattached by surgery. With a minimum of 6 months' follow-up, the macula remained attached in 24 eyes, while the retinas were completely attached in 22 eyes. Preoperative visual acuity was defective light perception to 0.1; an increase in visual acuity to maximal 0.1 was seen in 50% of the patients postoperatively. Preoperative visual acuity of light perception was associated with no functional improvement. Preoperative ultrasound investigation gave information about the real anatomic situation of the retina, especially if funduscopy was not possible. The other preoperative parameters could not predict correctly the functional outcome of vitrectomy in diabetics with severe central fibrovascular membranes because of the damage of the optic nerve and the retina. Conclusions: The high rate of anatomical rettachment after vitrectomy in diabetic eyes with severe central fibrovascular membranes is associated with a slight improvement of function; only preoperative visual acuity of hand motions or better was associated with an improvement of function.     

Anterior chamber inflammation after transconjunctival cryosurgery

Background: Inflammation caused by transconjunctival cryotherapy for prophylactic retinal detachment surgery was measured in various conditions. Methods: Thirty-four eyes of 28 patients with peripheral retinal lesions predisposing to retinal detachment were studied by laser flare cell meter before and after treatment. Results: The mean flare value for 34 eyes was 4.06 ± 1.45 photon counts/ms before surgery and 5.72 ± 2.52 pc/ms after surgery (p < 0.05). Flare value was elevated at 1, 2, and 3 weeks after treatment, peaking at 2 weeks (p < 0.05), and normal again at 4 weeks. There were no significant differences in flare increase between eyes with and without retinal breaks, eyes with and without limited retinal detachment, eyes with myopia more and less than – 8.0 D, and eyes with a treatment area limited to one quadrant and extending over more than one quadrant. Conclusion: Transconjunctival cryosurgery caused mild inflammation in the anterior chamber of the eye for 3 weeks. The inflammation was not affected by the presence of retinal break or limited retinal detachment, the degree of myopia, or the extent of the treatment area.     

Preoperative vitreous hemorrhage associated with rhegmatogenous retinal detachment: a risk factor for postoperative proliferative vitreoretinopathy?

Background: We conducted a prospective clinical study to elucidate the role of preoperative vitreous hemorrhage in the development of postoperative proliferative vitreoretinopathy (PVR) in primary rhegmatogenous retinal detachment. Materials and methods: We prospectively evaluated 409 eyes of 390 patients affected by primary rhegmatogenous retinal detachment referred before any failed attempt to reattach the retina. Single and multiple logistic regression analysis were used to test 14 categories of variables. Results: Postoperative PVR occurred in 48 (11.7% of 409 eyes). Postoperative PVR developed in 41 (11.8%) of the 347 eyes with no preoperative vitreous hemorrhage, and 7 (11.3%) of the 62 eyes with preoperative vitreous hemorrhage (P=0.90). The results of multiple logistic regression analysis showed that only four variables were significant factors which had independently and jointly an effect on the risk of postoperative PVR: (1) 90° or greater circumferential extent of the retinal tears; (2) preoperative PVR grade B; (3) preoperative PVR grade C-D; and (4) the use of cyrotreatment as the method of retinopexy. Conclusion: With the surgical techniques currently used, mild preoperative vitreous hemorrhage is not an independent risk factor for postoperative PVR in primary rhegmatogenous retinal detachment. The role of moderate and severe vitreous hemorrhage remains to be fully evaluated in a larger series of eyes.     

The effect of chemical stability and purification of perfluorocarbon liquids in experimental extended-term vitreous substitution

Background: To determine the importance of chemical stability and purification of perfluorocarbon liquids (PFCLs) in experimental retinal tolerance, we tested four different substances as long-term vitreous tamponade: purified and nonpurified perfluorodecalin (PFD) and perfluoro-octylbromide (PFOB) Method: After mechanical vitrectomy we replaced the vitreous of 65 rabbit eyes. Five groups were formed; four of them received the four PFCLs, while one served as control and received Ringer solution. The eyes were observed clinically every week and examined histologically after 1, 2, 4 and 8 weeks Results: After 1 week we observed foam cells and intraretinal macrophages in all eyes with PFCLs. Purified PFD caused retinal lesions in the photoreceptor, ganglion cell and outer nuclear layers after only 2 weeks in the lower part of the eyes. In eyes filled with purified PFOB we observed more pronounced damage of the same nature. Unpurified substances caused severe inflammation and retinal detachment Conclusion: Our study demonstrates that purification and chemical stability are important factors in retinal tolerance of PFCLs for vitreous replacement. Although purified PFD was tolerated by the rabbit eyes for 1 week, we cannot recommend this substance for short-term clinical use as a vitreous substitute.     

Indocyanine green angiography and age-related serous pigment epithelial detachment

Background: Fundus fluorescein angiography has shown that pigment epithelial detachment in age-related macular degeneration is often associated with choroidal neovascularisation (CNV). Indocyanine green angiography (ICG-A) provides a better visualisation of choroidal circulation and of CNV than fluorescein angiography (FA). Methods: We studied the ICG angiograms of 58 eyes presenting age-related pigment epithelial detachment, either with signs of occult CNV (48 eyes) or without signs of CNV (10 eyes) on FA. In selected cases the neovascular complex defined on the ICG angiogram was photocoagulated. Results: ICGA-revealed hyperfluorescence interpreted as CNV in 46 of 48 eyes with fluorescein angiographic signs of occult choroidal neovascularisation. The neovascular complex seen on the ICG angiogram was well delineated in 29 eyes and ill defined in 17 eyes. ICG-A revealed CNV in 2 of 10 eyes without signs of CNV on FA. In these two cases the neovascular complex was ill defined. Photocoagulation in selected cases resulted in stabilisation or even improvement of visual acuity and flattening of the pigment epithelial detachment in 9 of 18 cases. Conclusion: ICG-A may offer a better definition of the neovascular complex associated with pigment epithelial detachment in age-related macular disease and be helpful in guiding laser treatment. In some cases FA still outlines more clearly the lesions to be treated. FA and ICG-A should thus be used concurrently to determine treatment strategy.     

The retinal depression sign in diabetic retinopathy

Background: The retinal depression sign, an abnormal light reflex due to ischemia-induced focal inner retinal atrophy, was initially described in patients with sickle cell retinopathy. We undertook this study to characterize the finding in diabetic retinopathy. Methods: The fundus photographs of 97 consecutive patients with diabetic retinopathy seen in the Penn State University Ophthalmology Department were reviewed. We recorded age, sex, type of retinopathy, visual acuity and photographic details, including the presence or absence of the retinal depression halo. Results: The retinal depression sign was present in 9 of 22 patients age 45 years or under, versus 1 of 75 patients age 46 or older (P<0.0001, two-tailed Fisher's exact test). The presence or absence of retinal depression sign did not predict the type of retinopathy in a given patient. Eleven (70%) of the affected eyes had visual acuity 20/40. Conclusion: The retinal depression sign is common in younger patients with diabetes and should be distinguished from other macular lesions associated with diabetic retinopathy.     

Surgical risk factors for severe postoperative proliferative vitreoretinopathy (PVR) in retinal detachment with grade B PVR

Background: Previous studies have shown that grade B proliferative vitreoretinopathy (PVR) is a considerable risk factor for the development of severe postoperative PVR. We conducted a prospective study to elucidate which surgical procedures used in retinal detachment management may stimulate the PVR process in such eyes. Materials and methods: The study included 156 eyes of 152 consecutive patients with rhegmatogenous retinal detachment complicated by grade B PVR referred before any failed surgery and operated on between 1983 and 1993. The parameters evaluated by multivariate statistical analysis included the cumulative circumferential extent of the retinal tears, the extent of the scleral buckle, gas injection, vitrectomy, the method used for retinopexy, and the time of surgical management during the period of the study. Results: The incidence of severe postoperative PVR was 25.8% in eyes managed with cryotreatment versus 2.2% in eyes managed with argon laser photocoagulation (P=0.001). The rate of severe postoperative PVR was not influenced by the other surgical variables. Conclusion: We conclude that cryotherapy may be a risk factor for the development of severe postoperative PVR in retinal detachments associated with grade B PVR.     

Retinal pigment epithelial cells: autocrine and paracrine stimulation of extracellular matrix contraction

Background: This study was carried out to examine the biological activity of contraction promoters produced by dedifferentiating retinal pigment epithelial cells (RPE) and to evaluate the importance of autocrine and paracrine effects within a semi-closed environment like the vitreal cavity. Methods: RPE at different stages of dedifferentiation in culture were examined for their ability (a) to generate tractional forces in vitro, with and without serum stimulation, and (b) to produce and release contraction-stimulating proteins. Autocrine versus paracrine effects of cell-secreted promoters were tested by using RPE or human dermal fibroblasts (HDF) as target cells. The contraction-stimulating activity of the cell-secreted promoters was partially characterized and compared to the activity of defined promoters. Results: Our study confirmed that RPE can synthesize and secrete cell-contraction-promoting factor(s) active in stimulating the development of tractional forces by RPE as well as HDF. The quantity of biological activity secreted per cell decreases with progressive dedifferentiation, yet the responsiveness of the cell to contraction promoters increases. The contraction promoter(s) synthesized by RPE is partially distinct from the promote rs in serum, TGF-1 and 2, IGF-1, ET-1 and PDGF. The contraction-promoting effects of the RPE product(s) can be completely blocked by staurosporine. Conclusion: Dedifferentiation of RPE is characterized by increasing capacity to generate tractional forces and decreasing synthetic capacity. RPE within a semi-closed system like the vitreal cavity can, theoretically, act both as promoting and active component of traction-related events (tractional retinal detachment).     

Communication between the subretinal space and the vitreous cavity in the morning glory syndrome

Background: The aim was to describe a pathogenic mechanism for a rhegmatogenous retinal detachment in a 69-year-old man with the morning glory syndrome. Methods: During vitreous surgery for a retinal detachment, a membrane was removed that covered the optic disc anomaly and produced traction on the peripapillary retina. A retinal hole was found in tissue lying within the optic cup, and the hole was sealed using a autologous plasma —thrombin mixture. Silicone oil was used for retinal tamponade. Results: A retinal hole in tissue lying within the optic cup provided a fluid pathway between the vitreous cavity and the subretinal space. Following vitrectomy surgery, bubbles of silicone oil passed through the retinal hole into the subretinal space of the macula. Conclusion: This case demonstrates that a retinal hole in tissue lying within the optic disc anomaly of the morning glory syndrome provides a communication for fluid between the subretinal space and the vitreous cavity, resulting in a rhegmatogenous retinal detachment. Vitreous replacement with silicone oil resulted in the migration of silicone bubbles into the subretinal space.     

Indirect scatter laser photocoagulation to subfoveal choroidal neovascularization in age-related macular degeneration

Background: Occult choroidal neovascularization (CNV), poorly defined on fluorescein angiography, is present in the majority of patients with exudative complications of age-related macular degeneration. For patients who present with this type of subfoveal CNV but who have useful visual acuity, no form of treatment is of proven benefit. Accordingly, a pilot randomized trial of indirect laser treatment was performed. The rationale of this treatment was to inhibit the CNV through laser-induced effects on the retinal pigment epithelium. Methods: Patients with occult subfoveal CNV without retinal pigment epithelial detachment and with visual acuity of 20/200 or better were randomized to treatment or control groups. A grid of laser burns was applied to the macula beyond the area of serous retinal detachment and of angiographically defined occult CNV Results: After an average follow-up of 38 months, there was no difference in mean final visual acuity (0.12 treated, 0.14 control) or clinical outcome between treated and untreated groups. Fluorescein angiography showed gradual enlargement in the occult CNV in 58% of eyes in both groups. A decrease in visual acuity to worse than 20/200 (54% of treated, 50% of control eyes) was associated with ingrowth of well-delineated CNV (6 treated, 7 control eyes) or progression to a fibroglial or atrophic scar (11 treated, 8 control eyes). Conclusions: No benefit was demonstrated for scatter photocoagulation of the macula in patients with age-related macular degeneration and occult subfoveal CNV with initially good visual acuity. There were, however, no complications related to treatment.     

Experimental microendoscopic photoablative laser goniotomy as a surgical model for the treatment of dysgenetic glaucoma

Purpose: The aim of this study was to investigate the feasibility of photoablative Er:YAG laser goniotomy under microendoscopic control in a surgical cloudy corneal model of primary infantile glaucoma. Methods: Pectinate ligaments of 12 freshly enucleated cadaver porcine eyes were treated by ab interno single-pulse (5 mJ, 200 s) Er:YAG laser (2.94 m) photoablation. Through a clear corneal incision near the limbus an ophthalmic microendoscope (18 and 20 gauge) was inserted into the anterior chamber. Internal structures were observed and photoablative laser goniotomy was conducted under video guidance. Following treatment all eyes were prepared for light and scanning electron microscopy. Results: Anterior chamber angle structures and tissue photoablation were clearly visualized on the videoscreen using ophthalmic microendoscopy. Energy settings of 5 mJ per pulse proved to be sufficient for reproducible photoablation of pectinate ligaments, accompanied by the root of the iris falling back and exposing trabecular meshwork. This was confirmed histopathologically. Scatter thermal damage was less than 30 m. Conclusion: This new therapeutic modality, which combines endoscopic visualization of the internal structures with photoablative laser goniotomy, can be effective in the management of dysgenetic glaucoma in the presence of a cloudy cornea. High reproducibility of contact laser photoablation enabled sufficient control of incision depth and was not accompanied by inadvertent tissue damage to adjacent intraocular structures.     

Ocular blood flow velocity reduction after buckling surgery

Background: It has been reported that scleral buckling reduces the blood flow velocity in retinal vessels. Blood flow changes may also appear in other ocular and extraocular vessels. This study describes the blood flow velocity changes in the ophthalmic artery (OA) after performing this procedure. Methods: The study was carried out in 12 patients (12 eyes) with rhegmatogenous retinal detachment. Color Doppler imaging was used to measure the peak and average blood flow velocity in the OA. Measurements were taken 1 day before and 2 days after scleral buckling surgery was performed. Intraocular pressure (10P) was measured prior to each ultrasound study. Results: We found that statistically significant reductions in the peak flow velocity (33%) and average flow velocity (31%) occur in the OA after scleral buckling. All patients showed an increase in IOP after surgery. Conclusion: Buckling surgery reduces the blood flow velocity in the OA. Since the OA is the origin of the arterial branches that supply blood to the eye, our results suggest that scleral buckling may decrease not only retinal but also choroidal blood perfusion. Some extraocular structures might also be affected.     

Epidemiologic characteristics of rhegmatogenous retinal detachment in Kumamoto,Japan

Background: The epidemiology of rhegmatogenous retinal detachment in Asians is not well known. We studied the epidemiologic characteristics of rhegmatogenous retinal detachment in Kumamoto, Japan. Methods: The study was based on a retrospective chart review of hospital patients who were treated for primary rhegmatogenous retinal detachment in 1990. The data were collected from seven hospitals in the Kumamoto area. Results: From a population of 1 840000, 192 residents developed retinal detachment. The annual incidence was therefore 10.4 per 100000 population (9.6 for males, 11.2 for females). The incidences of three types of detachment — nontraumatic phakic, aphakic, and blunt trauma — were 9.8, 0.5, and 0.2 per 100000 population, respectively. In 109 of 180 patients (60.6%) with nontraumatic phakic detachment, retinal breaks were associated with lattice degeneration. In females, 14 of 106 nontraumatic phakic cases (13.2%) were secondary to macular holes. Conclusion: Compared with previously published studies from other countries, the incidence of detachments associated with lattice degeneration and macular hole was higher, while the incidences of aphakic detachment and detachment due to blunt trauma were lower in Japan. Racial factors and living habits may affect the development of retinal detachment.     

The effect of high-dose methylprednisolone on laser-induced retinal injury in primates: an electron microscopic study

Background: Previously we reported an ameliorative effect of high-dose methylprednisolone in laser injury to monkey retinas. The ultrastructural modification by methylprednisolone has not been examined. Methods: Cynomolgus monkeys were given severe kgrade III) retinal laser burns and treated with an intravenous megadose of methylprednisolone. Pathologic features of the retinal lesions with or without methylprednisolone treatment were evaluated by light and electron microscopy. Results: Ultrastructurally, the treated lesions showed rapid recanalization of choriocapillaris; proliferation of retinal pigment epithelium to replace the necrotic and damaged cells, resulting in rapid re-establishment of blood retinal barrier; mild macrophagic activity; and rapid reformation of the outer limiting membrane by Mueller cells. Conclusion: A high dose of methylprednisolone affected the responses of the choriocapillaris, retinal pigment epithelium, photoreceptor cells and Mueller cells to laser injury, showing an overall beneficial effect. These modifications might be ascribed to methylprednisclone's anti-inflammatory action, protection of the microcirculation and anti-lipid peroxidation effect. Proprietary interest: none     

Assessment of visual function in choroidal neovascularization with scanning laser microperimetry and simultaneous indocyanine green angiography

Background: Clinical management and treatment of diseases with choroidal neovascularization (CNV) are mainly based on visual acuity, which may give an incomplete picture of the associated visual dysfunctions. With the advent of new experimental treatment modalities such as alfa-interferon, radiation, or surgical excision of CNV, it is increasingly important to develop better methods for characterizing the associated visual function. Microperimetry with the scanning laser ophthalmoscope (SLO) allows precise point-to-point correlation between visual function and the macular pathology. However, precise delineation of CNV is a prerequisite for accurate correlation of the functional results with the CNV. Methods: A total of 40 eyes with CNV secondary to age-related macular degeneration were evaluated with static manual microperimetry using the SLO to quantitate relative and absolute scotomata within the CNV. For precise delineation of the CNV, indocyanine green (ICG) angiography was simultaneously performed, allowing stimulus presentation at any desired retinal location under visual feedback of the angiogram. Results: A relative scotoma was detected in 19 and an absolute scotoma in 21 out of 40 eyes. The depth of the scotomata was correlated with the duration of symptoms (P<0.01). Eyes with well-defined CNV had significantly deeper scotomas than eyes with occult CNV (P<0.005). Conclusion: Microperimetry using the SLO and simultaneous ICG angiography demonstrated relative and absolute scotoma within the CNV. The depth of the scotoma may guide the ophthalmologist in selecting the adequate treatment.Presented in part at Macula: New Frontier, An International Symposium, Kansas City, Missouri, 1994     

Pars plana lensectomy in cases of cataract with juvenile chronic uveitis

Background: Classical cataract extraction in young patients with secondary cataract following juvenile chronic uveitis often is complicated by serious problems such as severe postoperative uveitis, hypotonia oculi and phthisis bulbi. Lensectomy with partial anterior vitrectomy might be a less inflammatory way of handling these cases. Methods: Over a 15-year period we have treated 10 eyes of 9 young patients (age from 8 to 30 years) with secondary cataract after chronic uveitis by pars plana lensectomy with partial anterior vitrectomy. A classical two-port technique was used with incisions at 4 mm from the limbus. The follow-up period varied from 3 to 12 years with an average of 8 years. There were five patients with chronic iridocyclitis, two with Fuch's heterochromic iridocyclitis, one with sympathetic ophthalmia and one with idiopathic panuveitis. Results: Seven eyes were corrected with contact lenses. During the entire follow-up period six had 20/20 visual acuity and one, 20/40. None of these patients reported tolerance problems with the contact lens. One patient has 20/20 visual acuity with spectacles. One patient who initially had 20/20 visual acuity with a contact lens developed retinal detachment 2 years after surgery, during pregnancy, and now has 20/40 vision after retinal surgery. She prefers not to wear the contact lens any longer because of diplopia. The visual acuity of one patient was no better than hand movement and his aphakia was never corrected. Complications included one vitreous haemorrhage necessitating a second vitrectomy, one retinal detachment during pregnancy and one retinal detachment with proliferative vitreoretinopathy. One patient with sympathetic ophthalmia has 20/20 vision after 9 years' follow-up but still needs systemic steroids and cyclosporine. Conclusion: Pars plana lensectomy, with anterior vitrectomy appears to be a relative safe way to treat secondary cataract in patients during the first 30 years of life.     

Cytochemical localization of guanylate cyclase in photoreceptor cells of the mouse

Background: Light-stimulated excitation causes a decrease of the cGMP concentration in vertebrate photoreceptor cells. The cGMP content is restored by the catalytic action of a guanylate cyclase (EC 4.6.1.2). Methods: The spatial distribution of guanylate cyclase was determined cytochemically in rod visual cells of the mouse. Results: In retinal tissue of the mouse guanylate cyclase was found throughout the photoreceptor cells, in the outer and the inner segments, and was especially prominent in the cilia and in elongations of cilia extending into the outer segments. A reaction product of adenylate cyclase (EC 4.6.1.1) could not be demonstrated in vertebrate rod outer segments. Conclusion: The relatively high amount of guanylate cyclase in the inner segments and the cilia may contribute — at least in part — to the actual concentration and the time course of concentration changes of the cGMP concentration in rod outer segments.