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The eyes of Woodrow Wilson. Retinal vascular disease and George de Schweinitz.

Woodrow Wilson suffered a retinal hemorrhage in 1906, which is central to a historical controversy over whether medical problems significantly affected his political behavior prior to his stroke of October 1919. In terms of modern knowledge, the most likely cause of the event of 1906 is a retinal vein occlusion. Confidence in this judgment is gained from evidence that George de Schweinitz, one of the prominent ophthalmologists of his day, examined Wilson and showed through writings that he was well aware of the major categories of retinal vascular disease that are recognized today. Wilson was also subject to more ordinary problems, illustrated by anecdotes about his refractive errors, difficulty in learning to read, asthenopic symptoms, and visual problems with golf.

Famous Persons↗

Eye findings in patients with juvenile dermatomyositis.

OBJECTIVE: Reports of eye involvement in juvenile dermatomyositis (JDM), including significant retinopathy with visual loss, have led some to recommend routine formal ophthalmologic assessments for all patients with JDM at diagnosis. Our objective was to document the frequency and spectrum of eye involvement in patients followed in a single clinic caring for children with JDM. METHODS: A chart review was conducted of formal ophthalmologic consultation notes for patients with JDM followed at the Hospital for Sick Children between 1981 and 2002. RESULTS: Ophthalmologic assessments were found for 82 of 108 patients with JDM. The mean age at diagnosis of JDM was 7.0 years and 68.3% were female. Forty-five patients (55.6%) had abnormal eye examinations. Lid manifestations, found in 37 patients (45.7%), were the most common abnormality. Fourteen patients (17.1%) had corticosteroid-induced cataracts. Two patients had retinal abnormalities; one had a small retinal hemorrhage, the other an incidental chorioretinal scar. Neither had impairment of vision. No patient had uveitis. CONCLUSION: Eyelid and lens abnormalities are common in patients with JDM, while retinopathy is rare. As lid lesions and cataracts are easily detected by non-ophthalmologists, and retinal lesions are rare, we feel that JDM patients without visual symptoms do not require routine formal ophthalmologic assessment for disease manifestations.

Adolescent↗

Ocular findings in allogeneic stem cell transplantation without total body irradiation.

OBJECTIVE: To evaluate the ophthalmologic complications in hematologic patients after allogeneic stem cell transplantation (ASCT) without total body irradiation. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: A total of 150 ASCT patients. INTERVENTION: Ophthalmologic examinations of 130/150 patients were made, with particular attention to the occurrence of graft-versus-host disease (GVHD). RESULTS: One hundred thirty patients with a mean age of 35.5 (SD 10.6) years at the time of ASCT were followed up for a mean of 12 months (range 3-60 months). GVHD developed in 73 patients (56.2%). Of 130 patients, 52 (40.0%) had ocular complications, and 29 (22.3%) of those had GVHD. Keratoconjunctivitis sicca was diagnosed in 13 (10.0%) patients, and 12 (9.2%) had different stages of pseudomembranous conjunctivitis. Cataract developed in 3 patients (2.3%) in the observation period, and 7 (5.4%) had keratitis. Six (4.6%) patients had uveitis, and 8 (6.2%) had retinal hemorrhages. No ischemic retinopathy was found. Bilateral optic disc edema developed in 10 (7.7%) patients. CONCLUSIONS: Fewer ocular complications were observed in this series than in earlier studies, and the visual outcome was favorable. Cyclosporine has been linked to the development of optic disc edema and ischemic retinal lesions. The latter condition was not observed in the study patients, and the optic disc edema resolved within 1 year in all patients without any detectable visual impairment, in spite of continuation of the drug. Seven patients had acute GVHD at the time of optic disc edema, which is considered to be another manifestation of acute GVHD.

Adult↗

[Self-mutilation with crystalline lens dislocation in Gilles de la Tourette disease treated with retropupillary "iris claw" lens].

BACKGROUND: Gilles de la Tourette syndrome is a neurological disorder characterized by motor and vocal tics. Extreme forms of this disease manifest themselves with self-mutilating behavior and foul language. HISTORY AND SIGNS: A 59-year-old man, treated for Gilles de la Tourette syndrome, presented with visual loss after an attack during which he punched his right eye with his fist. On examination visual acuity was decreased to finger counting but could be improved to 1.0 with an S + 13D lens. There was vitreous prolaps into the anterior chamber and pigment was noted in the vitreous cavity. The complete dislocated lens could be seen ophthalmoscopically in the inferior fundus of the eye. THERAPY AND OUTCOME: The patient was treated with vitrectomy and phacoemulsification through the pars plana. An additional encircling band was placed due to peripheral retinal tears in the inferior quadrants. Visual rehabilitation was achieved with a secondary implant of a retropupillary iris-claw lens nine months later. DISCUSSION: Self-inflicted injuries are common in Gilles de la Tourette syndrome. Ocular lesions described in the literature comprise isolated cases of orbital hemorrhage, retinal detachment and permanent severe visual loss. Lens luxation has not been described before, and is probably due to weakened zonulae induced by repeated trauma. Treatment with a retropupillary iris-claw lens is the preferred therapy as it reduces the risk of endothelial touch during repeated trauma on the eye.

Eye Injuries↗

Vitreous, retinal and subretinal hemorrhages associated with von Willebrand's syndrome.

Two patients with von Willebrand's syndrome had intraocular hemorrhages without trauma or neovascularization. A 13-year-old Japanese girl complained of sudden spotted vision in her left eye. Vitreous, peripapillary retinal, and subretinal hemorrhages were seen by ophthalmoscopy in the affected eye. In addition, a 19-year-old Japanese man had repeated vitreous hemorrhage and epistaxis. Also noted was gyrate atrophy of the choroid and retina. Analysis of the coagulation-fibrinolysis system revealed decreased activities of Factor VIII and von Willebrand's factor in both patients. The amount of von Willebrand's factor antigen also was decreased. These results suggested that the patients had von Willebrand's syndrome. The bleeding tendency in the disorder appeared to be responsible for the pathogenesis of the intraocular hemorrhages seen in these patients.

Adolescent↗

Manifestations of graft-versus-host disease following allogenic bone marrow transplantation.

Sixty-seven patients undergoing allogenic bone marrow transplantation (BMT) were examined before and at regular intervals for up to 87 months (1-87 months, mean 18) after transplantation. Within a period of 1-39 months, 14 of these patients died (11 male, 3 female; age at BMT 16-46y). Five of these patients died within the first 100 days. They showed no eye involvement; three patients had intraretinal hemorrhage, in one case of squamous blepharitis and filiform keratitis developed during chronic graft-versus-host disease (GVHD). In contrast, 22 of 53 (41.5%) surviving patients (30 male, 23 female; age at BMT 1-47y) were found to have ocular involvement. Before BMT only two cases of retinal hemorrhage and central chorioretinal scars each were detected. During the stage of acute GVHD (up to day 100), nine patients were free of ocular manifestations. However, 16 of the 20 patients with chronic GVHD showed ocular involvement; 14 (70%) had reduced tearflow, ten had severe keratoconjunctivitis sicca, four suffered from sterile corneal ulcerations. Bilateral cataracts were detected in 11 patients, nine of whom only had minimal posterior subcapsular opacification, possibly resulting from highdose steroid medication. One additional case presented with bilateral multifocal recurrent chorioretinitis and panuveitis. The fundus lesions appeared some months after BMT (before cyclosporin-A treatment started) and recurred during systemic treatment. All patients undergoing allogenic BMT, especially when treated for severe chronic GVHD, require regular ocular observation to avoid complications such as keratoconjunctivitis sicca at an early stage, as late complications are often severe and hardly amenable to conservative or surgical treatment.

Acute Disease↗

Characteristics of diabetic retinopathy in SDT rats.

BACKGROUND: We previously reported a new diabetic strain of the Sprague-Dawley rat, named the Spontaneously Diabetic Torii (SDT) rat. The purpose of the present study was to report the histologic and ultrastructural characteristics of diabetic retinopathy (DR) in a new animal model, the SDT rat. METHODS: Fifty-three eyes of 43 SDT rats of various ages (35-82 weeks) were examined, of which 33 underwent histopathologic examination, 15 eyes fluorescein-dextran microscopy, and five eyes the trypsin digestion method. RESULTS: Of the 33 eyes examined histopathologically, DR was identified in 20 eyes (61%). Large retinal folds mimicking diabetic tractional retinal detachment were observed in 20 eyes (61%). Retinal hemorrhages were seen in four eyes (12%). A neovascular fibrous membrane around the iris developed in five eyes (15%), of which two eyes had a massive anterior chamber hemorrhage. Of the 15 eyes examined by fluorescein-dextran microscopy, an area of nonperfusion and/or extensive hyperfluorescence was observed in 12 eyes (80%). Of the five eyes examined using the trypsin digestion method, acellular capillaries and pericyte loss were observed in four eyes (80%). Of the 53 eyes, the previously mentioned retinal changes of DR were observed in 36 eyes (68%). The rats with DR (49-82 weeks; mean, 60 weeks) were older than the rats without DR (35-55 weeks; mean, 40 weeks) (p < 0.001). CONCLUSION: Large retinal folds mimicking tractional retinal detachment with extensive leakage of fluorescein around the optic disk was the most prominent finding of DR in SDT rats.

Animals↗

The relationship between retinal receptor orientation and photoreceptor optics.

At this time, based on still-restricted studies, it would seem that a fundamental property of vertebrate receptor optics is anterior pointing by receptors. The anterior pointing locus is most probably a point approximating the center of the exit pupil of the eye. There is evidence for the recovery of orientation when that orientation is disturbed. One or more mechanisms mediate that orientation, and we must seek to define and understand their functional properties. We must better define how disturbance in orientation influences vision. Briefly stated, substantially disturbed receptors have reduced light-guiding capability (and hence reduced sensitivity), reduced contrast sensitivity (caused by increased cross talk and so forth), and reduced resolution capability. These combine to creat a detectable (but not necessarily common) form of amblyopia [30]. In a sense, these cases of poor acuity would seem to represent a failure of the photoreceptor alignment system. An interesting set of theories has evolved in relation to receptor alignment in the neonate. There is considerable mechanical hydraulic stress on the newborn ocular vascular system at the time of delivery. Retinal hemorrhages and transient edema of the optic papilla following intracranial pressure rise and rapid decompression later during delivery of the head commonly occur. These can readily causd disturbances in receptor alignment. In some instances the macula is involved. While hemorrhages are apparently rapidly absorbed, if foveal receptors remain misaligned during the critical period (not yet really defined) for the development of vision, central fine-resolution capability may fail to develop. That is, subsequent elaboration of the visual system is dependent upon the quality of the optically transmitted and neurally transformed retinal image during the critical period for visual development. If the capability for realignment exists and occurs after part or all of the critical period, then signs of a prior receptor disarray may no longer be present in later years, but reduced resolution capability can persist. There is interesting literature on the subject [63-68]. K. Simons is currently reviewing this literature as part of his dissertation. Simons points out that the rupture of vessels may in fact partially serve as a safety valve protective of the infant in the presence of these substantive forces, i.e., fine-vessel rupture may limit the potential for damage to the eye. At this stage of investigation it is dangerous to carry speclation too far. Thus not only must we consider the mechanism for orientation, the nature of a possible error signal, and the mechaniisms for maintaning orientation and relating the retina to its substrate, but we must also analyze the consequences of disturbances in these mechanisms in relation to the individual's visual capability. Of equal importance is the need to understand the mechanisms and processes leading to failure of recovery of proper photoreceptor orientation...

Animals↗

Retinal detachment treated by argon laser photocoagulation.

Argon laser photocoagulation was applied 2-3 weeks after implantation of episcleral silicone sponges as a second-step operation in 130 cases of retinal detachment. The chorioretinal scar for the occlusion of the tears was obtained exclusively with this procedure. Argon Laser used in cases with pronounced retinal elevation, i.e. where cryoapplication had proved inadequate or where a choroidal or retinal hemorrhage could be expected following cryoapplication. The advantages and disadvantages of the technique are evaluated with respect to the experience with cryoapplication.

Argon↗

External buckling without drainage.

Scleral buckling without drainage of subretinal fluid is a technique suitable to a broad range of detachment problems including aphakia, vitreous traction, multiple tears, high myopia, and large breaks, as well as old or extensive detachment. Retinal detachments operated on by modification to the Custodis procedure reattach in a spontaneous and predictable manner determined by the patient's age, the duration of the detachment, and its extent. Ninety percent of cases are completely reattached four days following surgery. Clues to successful surgery when subretinal fluid persists are retinal tear closure, documented daily fluid loss, macular reattachment, and dependent shifting of subretinal fluid. When uncertainty still exists, the final objective evaluation is the observation that fluid fails to accumulate beneath the retina when the patient is unpatched and mobilized. The operation is extraocular and avoids the problems of drainage-choroidal hemorrhage, retinal incarceration, vitreous loss, and restoration of hypotony. The technique offers the modern surgeon a simple, direct, and rational approach applicable to 75 percent of retinal detachments, and it also assures a smooth, uncomplicated recovery.

Drainage↗

[Retinopathy in premature infants. Present value of surveillance of premature and newborn infants at risk].

Systematic ophthalmologic screening of infants in a Neonatal Intensive Care Unit is time consuming and sometimes difficult. In this retrospective study of 1200 infants examined in the neonatal unit of Toulouse Regional Hospital from January 88 through December 89, we tried to summarize our findings and assess the value of systematic screening. We found 118 infants with abnormal examination: 58 preterms had retinopathy of prematurity (ROP) (5.16%) with 36 stage I, 15 stage II, 7 stage III (2 stage III+ had cryotherapy), no stage IV. Risk factors are discussed. 40 preretinal hemorrhages, all found in the first week of life. Only 2 of them persisted beyond the 2nd month (0.2%) and we found vascular anomalies that were cryotreated. 20 had various diseases. Diseases requiring early treatment were found in a general context with obvious need of ophthalmologic examination. Our study shows that 4 infants: 2 ROP III+ and 2 retinal hemorrhages or 0.4% of all infants obtained an effective benefit from this systematic screening. We confirmed the value of this screening: all infants in the neonatal care unit have at least one ophthalmologic exam, before they leave the hospital. Preterm less than 1600 g or less than 2000 g with initial resuscitation are checked during first and second month. Preterm less than 1200 g or less than 1600 g who had more than one month of oxygen therapy are checked every month until the 6th month.

Humans↗

The clinical study of congenital looped/coiled peripapillary retinal vessels.

PURPOSE: To present clinical manifestations of eyes with peculiar looped/coiled peripapillary retinal vessels. METHODS: Seven patients with looped/coiled retinal vessels on or near the optic disc were enrolled. All patients went through detailed ophthalmologic examinations and fluorescein angiography (FAG). RESULTS: There were two men and five women. Patients' age ranged from 15 to 71 years (mean: 39 years). The follow-up period ranged from 3 to 74 months (average: 27 months). One patient had bilateral involvement. Five of the seven patients noticed sudden onset of floaters in one eye. The colour fundus photography revealed looped/coiled retinal vessels on or near the optic disc, and most of the vessels were arteries. Accompanied retinal, preretinal, or vitreous haemorrhage was noted in all five patients who had sudden onset of floaters. Fluorescein angiography showed no leakage from the looped/coiled retinal vessels. No specific underlying diseases were noted in any patients. Follow-up examination revealed reabsorption of haemorrhage, and no change of the abnormal vessel patterns in any eyes. CONCLUSIONS: The peculiar fundus lesion of looped/coiled peripapillary retinal vessels is likely a benign congenital retinal vascular anomaly that does not progress. Floaters secondary to preretinal or vitreous haemorrhage is the most frequent complaint. The prognosis is excellent.

Adolescent↗

Complications of acute posterior vitreous detachment.

Of 201 patients whose presenting symptoms were acute entoptic phenomena or photopsia, or both, 150 patients had posterior vitreous detachment; 69 patients (46%) had retinal breaks; 18 (12%) had a vitreous hemorrhage without detectable retinal breaks; and two (1.3%) had peripheral retinal hemorrhages without retinal breaks or vitreous hemorrhage. Retinal breaks that occur in eyes in conjunction with acute posterior vitreous detachment are potentially dangerous and there is a possibility of delayed break formation.

Acute Disease↗

Optic disc and retinal microvasculopathy after high-dose chemotherapy and autologous hematopoietic progenitor cell support.

The purpose of this study was to prospectively evaluate the retinal and optic nerve changes in patients undergoing high-dose chemotherapy (HDC) followed by autologous hematopoietic progenitor cell support (AHPCS). One hundred and forty patients undergoing HDC and AHPCS underwent extensive pre- and post-transplant ophthalmologic evaluations for development of retinal microvascular complications. One hundred and ten patients received high-dose cyclophosphamide, cisplatin and BCNU; thirty received identical doses of cyclophosphamide and cisplatin, but received paclitaxel instead of BCNU. Thirty-four patients (24%) had retinal findings of either cotton wool spots (CWS) (n = 20) or retinal hemorrhages (n = 18) during follow-up, which ranged from 1 to 12 months. Ten (7%) of these patients, all of whom received BCNU, showed ocular toxicity characterized by CWS 1 to 4 months post transplant (n = 10); optic disc edema (n = 3); and variable vision loss associated with the onset of BCNU-induced pulmonary toxicity. Retinal and optic disc microvascular complications may occur after high-dose chemotherapy followed by AHPCS. The association of ischemic retinal lesions and/or optic disc edema with BCNU-induced pulmonary toxicity and the lack of ocular toxicity in patients that did not receive BCNU may suggest that BCNU is the etiologic agent.

Adult↗

Enzyme-assisted vitrectomy with bacterial collagenase. Pilot human studies.

Clostridiopeptidase A, a bacterial collagenase, was used to assist vitrectomy with membrane stripping in six patients with dense intravitreal fibroproliferative tissue associated with retinopathy of prematurity, diabetic retinopathy, or proliferative vitreoretinopathy. When it was injected intra-operatively, allowed to incubate for 15 minutes, and then removed by irrigation/aspiration, no side effects of lens opacity, lens dislocation, or retinal hemorrhage were observed. Use of this enzyme may facilitate removal of fibroproliferative tissue in certain difficult vitrectomy cases.

Adolescent↗

Retinal disorders of childhood.

Included in this discussion of retinal disorders affecting infants and children are retrolental fibroplasia, diabetic retinopathy, sickle cell retinopathy, retinal detachment, retinal hemorrhages of neonates, macular disease, colobomas, ocular toxocariasis, and TORCH syndrome.

Anemia, Sickle Cell↗