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Retinopathy in acute leukaemia at initial diagnosis: correlation of fundus lesions and haematological parameters.

PURPOSE: To determine the prevalence of retinal changes in newly diagnosed acute leukaemia patients, and to establish the relationship between retinal lesions and haematological parameters in these patients. METHODS: A total of 127 patients with acute leukaemia (myeloid and lymphoid), of both genders, aged between 13 and 77 years, were examined by an ophthalmologist for retinal changes using direct/indirect ophthalmoscopy within 2 days of diagnosis before starting chemotherapy. RESULTS: Retinal lesions were seen in 62 cases (49%), with intraretinal haemorrhages being the most common lesion (42%). A high white blood cell count was significantly associated with intraretinal haemorrhages (p = 0.04) and white-centred haemorrhages (p = 0.001), while a low platelet count was significantly associated with intraretinal haemorrhages (p = 0.03) in acute myeloid leukaemia patients. CONCLUSIONS: A high white blood cell count may be considered as important as a low platelet count in the pathogenesis of leukaemic retinopathy.

Acute Disease↗

[Thrombotic thrombocytopenic purpura (Moschcowitz's disease)].

BACKGROUND: TTP is a rare haematological disease consisting of thrombocytopenia, haemolytic anaemia, fever, neurological symptoms and renal failure. PATIENT: A 63-year-old female patient presented to our clinic with bilateral progressive loss of visual acuity accompanied by metamorphopsia. Ophthalmoscopy revealed pigmentepithelial choroidal atrophy secondary to the occlusion of chorioidal vessels and disseminated intraretinal and diffuse vitreal haemorrhage. Fluorescein angiography showed an obvious hyperfluorescence in the areas of choroidal atrophy as well as perivascular leakage. The patient had a history of TTP, as diagnosed by internal medicine. CONCLUSION: Vascular damage in TTP is due to disseminated hyalin clots of fibrin platelets which aggregate in the small capillaries, caused by a disorder of the endothelium-thrombocyte interaction. When evaluating patients with occlusion areas of the choriocapillaris, Moschcowitz's disease should be taken into consideration even in elderly patients.

Atrophy↗

[Pigment epithelial detachments in age-related macular degeneration -- classification, differentiation and prognosis].

BACKGROUND: A pigment epithelial detachment (PED) is an exudative manifestation of late age-related macular degeneration (AMD). Its long-term unfavourable prognosis is influenced by the association of a neovascular membrane. Seemingly identical PEDs in ophthalmoscopy can be further differentiated by fluorescein and indocyanine green angiography. The purpose of this prospective follow-up study was to determine the most reliable angiographic classification to distinguish PEDs in AMD and to evaluate their outcome. MATERIAL AND METHODS: 73 eyes of 68 patients with a PED in AMD were examined with simultaneous angiography with fluorescein and indocyanine green. 34 eyes were additionally scanned by retinatomography. Fluorescein angiographic classification comprises 4 types: 1. early hyperfluorescent type, 2. late hyperfluorescent type, 3. drusen type, 4. irregular fluorescent type. Indocyanine green angiographic classification consists of the following three groups: 1. angiographically no vascularisation, 2. associated choroidal neovascularisation, 3. associated polypoidal choroidal vasculopathy. RESULTS: Both classifications show statistically significant correlation with visual acuity (fluorescein classification: P = 0.0112, ICG-classification: P = 0.0004) and area of the PEDs (fluorescein classification: P = 0.0002, ICG-classification: P < 0.0001). ICG-classification additionally is significantly associated with the volume (P = 0.0008). Both classifications correlate (P = 0.0001). Factors influencing visual acuity are: age (P = 0.0044), associated neovascular membrane (P = 0.0004), area (P < 0.0001), volume (P = 0.0077) and height (P < 0.0001). CONCLUSIONS: PEDs in AMD can best be classified by indocyanine green angiography on the basis of an associated prognostically important neovascular membrane, which can only be further distinguished into choroidal neovascularisation or polypoidal choroidal vasculopathy by indocyanine green angiography. Statistical analysis shows qualitatively and quantitatively more significant results for the indocyanine green angiographic classification.

Aged↗

[Evulsion of the optic nerve following blunt bulbar trauma. Case report and review of the literature].

BACKGROUND: Evulsion of the optic nerve is a rare form of traumatic optic neuropathy. It is a rupture of the optic nerve at the disc without damage of its sheaths occurring in association with a blunt skull trauma or a blunt bulbar trauma. METHOD: We report on a 13-year old girl with complete evulsion of the optic nerve after blunt bulbar trauma and give a literature review. RESULTS: While swinging on a liana the patient had crashed into a tree. The initial examination 3 hours after the accident revealed at the right eye an amaurosis without any afferent reaction of the pupil to light. Ophthalmoscopy displayed a vitreous haemorrhage at the optic disc, peripapillar white retina and interruption of the blood flow of all visible retinal vessels. Fluorescein angiogram showed no retinal perfusion and a detatched retina above the disc. Echographic examination revealed a vitreous haemorrhage at the disc shaped like a mushroom. From the eighth day, examination of the retina became impossible due to disseminating vitreous haemorrhage. After 8 months vitreous haemorrhage had disappeared revealing a white fibrosis at the area of the optic disc. Since first histological description of an optic nerve evulsion by His in 1856, 42 patients with complete and 23 with partial evulsion of the optic nerve were reported in the literature. In our review different mechanisms of injury are discussed. CONCLUSION: Evulsion of the optic nerve is rarely reported. It is possible that it may occur more frequently, because it cannot be seen in blunt or also penetrating bulbar traumas with severe intraocular cloudiness.

Adolescent↗

Retinal vasculitis and cystoid macular edema after body tattooing: a case report.

BACKGROUND: To report a case of retinal vasculitis occurring after the placement of permanent tattoos. HISTORY AND SIGNS: A 21-year-old male was referred to our department with impairment of visual acuity. Permanent tattoos covered the head, body, arms and legs. The patient was examined with ophthalmoscopy, fluorescein angiography, indocyanine green angiography and optical coherence tomography. Systemic medical and laboratory work-up were performed in order to exclude an infectious agent or an inflammatory disease. He had no history of intravenous drug abuse. THERAPY AND OUTCOME: Our patient presented severe posterior uveitis associated with retinal vasculitis and cystoid macular edema. Laboratory tests ruled out all diseases causing vasculitis. HIV and B, C hepatitis tests were negative. Cystoid macular edema and vasculitis were resolved after immunosuppressive therapy. CONCLUSIONS: This is the first description of a retinal vasculitis associated with cystoid macular edema in a completely healthy individual after the placement of permanent tattoos. A phagocytosis of tattoo pigments leading to their lysis is described in the literature as a mechanism causing vasculitis.

Adult↗

[Bull's-Eye Maculopathy with Deferoxamine Treatment].

BACKGROUND: A bull's-eye maculopathy can be provoked by drugs. CASE REPORT: A 67-year-old man observed transient green-yellow spots with both eyes. The visual acuity was 1.0 in the right eye and 0.9 in the left. Tiny pigmentary irregularities of the macula were observed by ophthalmoscopy. Fluorescein angiography revealed a cockade formation of the macula. The multifocal ERG showed normal latencies in the periphery, however, in the centre the amplitudes were reduced to approximately half of the normal values. An acute myeloid leukaemia was diagnosed in 1997 which arose from a myelodysplasia, after performance of a peripheral allogenic blood stem cell transplantation. Because of a serious anaemia, the patient received numerous multiple transfusions with packed blood red cells from April 1999 to April 2000 (a total of more than 90 transfusions). In consequence, a haemosiderosis occurred with a ferritin value of 4390 ng/ml (November 1999) which made treatment with deferoxamine necessary. Multiple injections of deferoxamine (one to two grams were given from August 2000 until February 2001, afterwards an intermittent application until November 2002 was carried out) which resulted in the maculopathy. CONCLUSION: Retinal changes, in particular, a maculopathy, should be considered even if visual acuity and visual fields are not remarkably changed. Dependent on the extent of the disease, deferoxamine treatment should be discontinued in case of distinct visual deterioration.

Aged↗

[Bilateral visual field defects with optic disc drusen and secondary open angle glaucoma with PEX--clinical correlation with the HRA].

BACKGROUND: Arcuate visual field defects are a typical sign of glaucomatous damage. Elevated intraocular pressure in combination with pseudoexfoliation syndrome (PSX) manifests the diagnosis glaucoma. Beyond this state, in microdiscs with optic disc drusen, the exact classification of the visual field defects is crucial. CASE REPORT: A 57-year-old male with pseudoexfoliation glaucoma was referred because of progressive glaucomatous visual field defects. The visual acuity was right 20/40 and left 20/25. Maximum intraocular pressure was 36 mm Hg. A simple optic nerve atrophy was diagnosed superonasally. The optic disc size was OD 2.24 mm(2) and OS 1.89 mm(2) (HRT I). An Ultrasound B-mode scan demonstrated the diagnosis of optic disc drusen. Over a follow-up of 1 year, a growth tendency was observed, especially in the superonasal quadrant. The mulberry-shaped surface of the drusen was visualized with infrared reflection images (HRA II, 830 nm). Confocal scanning laser ophthalmoscopy (HRA II, excitation 488 nm, 500 nm notch filter) showed an increased intrapapapillary autofluorescence (> 50 % papillary area: OD 1.67 mm(2), OS 1.26 mm(2)). This technique could detect drusen in areas that looked normal in classical retinoscopy. CONCLUSION: The differential diagnosis of arcuate scotomas includes simple optic nerve atrophy and glaucomatous optic nerve atrophy. Optic disc drusen in glaucoma eyes can obscure the main cause of progressive visual field loss. Superficial optic disc drusen can be measured planimetrically over the years. An adequate reduction of intraocular pressure should be realized in these eyes.

Diagnosis, Differential↗

[Choroidal neovascularization followed in a patient with "Multiple Evanescent White Dot Syndrome" (MEWDS) -- a case report].

BACKGROUND: Since the description of the "multiple evanescent white dot syndrome" (MEWDS) by Jampol et al, choroiditis has been in the focus of interest. But the classical type of MEWDS was an exceptional case in clinical routine. CASE REPORT: A 48-year-old female presented to our hospital with a sudden unilateral visual acuity decrease and an extension of the blind spot. Ophthalmoscopy and fluorescein angiography revealed typical multiple grey-white chorioretinal patches of the same stage with lesion areas of about 100 - 200 microm compatible with the diagnose of MEWDS. Although visual acuity increased continuously the patient developed a classical choroidal neovascularization within 4 weeks. She was treated with PDT and visual acuity as well as the ophthalmoscopic diagnosis remained stable. CONCLUSION: In spite of visual improvement in MEWDS, regular control is recommended. In addition we propose to consider the diagnosis of MEWDS if an enlargement of the blind spot and CNV without lesions of the retinal pigment epithelium are diagnosed.

Choroid↗

[Variations in intraocular pressure (IOP) and necessity for paracentesis following intravitreal triamcinolone injection].

BACKGROUND: Intravitreal triamcinolone injection (IVT) has become a treatment option for macular edema of heterogeneous etiology and neovascular retinal diseases including AMD. Besides the risk for a steroid-induced secondary open-angle glaucoma, the acute rise in intravitreal volume induces IOP elevations immediately after injection. To decrease the intravitreal volume a paracentesis is advocated by many surgeons. The aim of this study was to determine variations in IOP at different time points immediately after IVT in order to assess the necessity for routine paracentesis. METHODS: The IOP was recorded by Goldmann applanation tonometry preoperatively, 10 minutes, 1, 3 and 24 hours after intravitreal injection of 0.1 mL (4 mg) triamcinolone. A consecutive series of 32 eyes of 32 patients with diabetic macular edema, diffuse edema after central vein occlusion or occult subfoveal choroidal neovascularization due to age-related macular degeneration was included. Statistical analysis was performed with ANOVA test and Bonferroni correction. RESULTS: Compared to baseline (15.24 +/- 0.52 mm Hg) IOP was significantly elevated 10 min postoperatively (22.28 +/- 1.4 mmHg; p < 0.05). One hour after injection IOP decreased to 15.58 +/- 0.69 mmHg (p < 0.05). Three and 24 h after injection mean IOP was not significantly different from preoperative baseline levels. Immediately after IVT light perception was tested and retinal perfusion was evaluated by indirect ophthalmoscopy. In none of the patients was a paracentesis necessary. CONCLUSION: Intravitreal injection of 0.1 mL triamcinolone led to a moderate transient rise in IOP. Based on these results, a routinely performed paracentesis immediately before or after IVT is not required. As paracentesis bears an additional risk including endophthalmitis it should only be considered if functional testing following injection indicates a relevant impairment of retinal perfusion.

Aged↗

[Entoptic phenomena and potential visual acuity].

REVIEW OF PREOPERATIVE EVALUATION OF VISUAL FUNCTION: In the presence of media opacities, the prevision of the potential postoperative visual acuity constitutes often a complicate diagnostic problem. Its solution requires the experience of the ophthalmologist, who should take into account various elements from the patient's visual aggravation progress, the dissociation between far and near vision under mesopic and scotopic conditions, the actual grating acuity and the eventual hand movement and light-perception, as well as, elements from the patient's objective examination, i.e., from the biomicroscopy with direct and indirect illumination through the still transparent media and from the binocular ophthalmoscopy of the fundus. METHODS REVIEWED: Purkinje figure and luminous darting points visualization. POSSIBLE PREDICTIONS: Light induced entoptic imagery, as Purkinje figure and luminous darting points, visualization, helps as a supplementary diagnostic procedure, for the prevision of a low, but socially useful postoperative visual acuity.

Humans↗

[High altitude retinopathy after Himalayan ascent].

BACKGROUND: Visual disturbances after high altitude exposure were first reported in 1969. Later, the term "High Altitude Retinal Hemorrhage-HARH" has been used for the ensuing retinal hemorrhages and vascular engorgement. CASE REPORT: A 31-year-old Caucasian male presented to our outpatient department 1 week after climbing Mt. Gosainthan in the Himalayas. He had spent 25 days without oxygen supply above 5000 meters, with a maximum of 8046 meters. He now complained of glare and decreased vision in twilight. Visual acuity was 20/25 OD and 20/20 OS. Ophthalmoscopy revealed intraretinal hemorrhages and tortuosity of dilated arterioles and venoles. After 6 weeks of gradual improvement, visual acuity was 20/20 OD and 20/16 OS with normal visual fields. DISCUSSION: The hypoxia at high altitude causes increased retinal blood flow and blood volume possibly via autoregulatory mechanisms. Furthermore, retinal venous pressure can be increased by extreme physical exertion and Valsalva maneuvers during mountain climbing. A hypoxic retinal capillary bed exposed to increased retinal venous pressure predisposes to intraretinal hemorrhage. Retinal changes include marked increase of retinal vessel diameter with tortuosity of arterioles and venoles and hyperemia or edema of the optic disc. The intra- or preretinal hemorrhages often spare the macular area. These patients do not experience debilitating symptoms unless vitreous hemorrhage occurs. This may be potentially hazardous when the patient is still in the high mountains. Clinically, all these retinal changes are reversible within weeks. To prevent high altitude retinopathy, ascending slowly and use of supplemental oxygen is recommended.

Adult↗

[Clinical measurement of intraocular gas].

BACKGROUND: Increased use of gases in retinal/vitreous surgery requires a good clinical estimate of intraocular gases. This is needed, if a supplement of gas is intended, its disappearance to be predicted (e.g. to 10% to permit travelling by plane) or the amount to be calculated to cover a break. Clinical estimation of gas in the pupil is limited by its size. Accuracy of pupil measurements will be tested and compared with experimental results obtained by estimating the level of gas meniscus at the retina. MATERIALS AND METHODS: Ten cadaver eyes (5 phakic, 4 aphakic, 1 pseudophakic) were fixed in a plastic mold, vitrectomized and injected with multiple randomized volumes of air. Two observers measured the level of meniscus in disc diameters above or below the superior margin of disc and then anteriorly in the pupil in millimeters from the superior limbus. Measurements were converted to milliliters through tables constructed on mathematical models of the phakic and aphakic eye. RESULTS: Observations at the retina and in the pupil were equally accurate for intermediate gas volumes, for which the meniscus fell within the pupil (P = 0.372). Measurements at the retina proved less vulnerable to error induced by misalignment of the observer's eye. Above the pupil observations at the retina were still reliable for small bubbles up to 4 dd above the disc (error < 5% in the aphakic, < 10% in the phakic eye). Below the pupil observations at the retina yielded a result increasing less than the injected volume because of refraction at the anterior segment gas interface (error > 10% of the ocular volume). CONCLUSION: Observations of the level of gas meniscus at retina with indirect ophthalmoscopy is an accurate method for estimating intraocular gas volumes. It extends the range of measurement significantly beyond the pupil. Tables based on a mathematical model convert disc diameters to milliliters.

Gases↗

[Wyburn-Mason syndrome].

BACKGROUND: There are various malformations of retinal vessels. Some of them are associated with cerebral vascular anomalies. MATERIALS AND METHODS: This report is given on a girl 6 years of age with a retinal racemose angioma. RESULTS: The clinical examination showed a unilateral racemose angioma combined with retinachorioidal anastomosis and a hemifacial vascular malformation. We found teleangiectasia in this area. The ophthalmoscopy demonstrated a blurred border of optic disc on the right eye with forward protrusion of the disc about + 2.0 dioptres. Large racemose lesions may be localized to the optic nerve and may involve segmental areas of the macula. The ultrasound showed a papillary stasis with highly reflective structures. The ultrasonic patterns are interpreted as a cholesterosis or fibrovascular tissue. This findings may belong to the rare Wyburn-Mason syndrome. Acute neurological symptoms by similar lesions the midbrain and hemorrhages during extraction of teeth are reported in literature.

Child↗

[Ocular microangiopathy syndrome in patients with AIDS is associated with increased plasma levels of the vasoconstrictor endothelin-1].

OBJECTIVE: Ocular microangiopathic syndrome is the most frequent ophthalmic finding in patients suffering from the acquired immunodeficiency syndrome (AIDS). Ocular microvascular changes including cotton-wool spots are closely associated with neuroretinal and cognitive deficits in patients infected with the human immunodeficiency virus type 1 (HIV-1). Endothelin-1 is a recently identified cytokine with potent vasoconstrictor activity which is associated with various diseases involving vascular structures. METHODS: We studied 29 patients with AIDS by indirect ophthalmoscopy and by slit lamp biomicroscopy, and endothelin-1 immunoreactivity (IR) was measured in plasma by radioimmunoassay. Cotton-wool spots were counted as indicator of retinal microvasculopathy. Conjunctival bloodflow sludging in conjunctival vessels was measured by a standardized rating scale as indicator of conjunctival microvasculopathy. Parameters of immunosystemic damage were determined as covariates. RESULTS: Endothelin-1 IR was closely associated with the number of cotton-wool spots (Spearman r = 0.53, p < 0.01) and with the extent of conjunctival blood-flow sludging (r = 0.61, p < 0.001). The level of significance became stronger applying analysis of covariance with Endothelin-1 IR as dependent variable and number of cotton-wool spots (p < 0.0001) or extent of conjunctival blood-flow sludging (p < 0.0001) as independent variables. CONCLUSION: Our data are consistent with the hypothesis that the vasoconstrictor endothelin-1 has an important role in the pathogenesis of HIV-1-related ocular microangiopathic syndrome. As HIV-1-related ocular microangiopathic syndrome is associated with neuroretinal and cognitive deficits in patients with HIV-1 disease the therapeutic use of endothelin antagonists currently under investigation should be discussed.

Acquired Immunodeficiency Syndrome↗

[Bilateral papilledema and retinochoroiditis ad papillam in a 12-year-old girl].

Ophthalmoscopy in a somnolent girl of 12 years revealed papilloedema of R = 3D and L = 4D. In the right eye there was an additional retinochoroiditis ad papillam. Seroreactions for toxoplasmosis were highly positive. By treatment with Daraprim, Durenat and Leucovorin, the left eye recovered nearly completely, but the right eye developed a severe optic atrophy.

Child↗

[Benign concentric annular macular dystrophy].

In routine eye examination of a 32-year-old male patient an annular macular dystrophy was noted in absence of distinctive visual complaints. Visual acuity was 20/20, the photopic and scotopic electroretinograms were normal. The ring scotoma in perimetry and the macula finding in ophthalmoscopy corresponded to the fluorescein angiographic pattern. Color vision deficiency was only mild without any predominant axis of confusion. Benign concentric annular macular dystrophy is rarely described in literature. Differential diagnosis includes all 'bull's eye' conditions.

Adult↗

[Selection criteria and follow-up of children presenting with retinopathy of prematurity].

The recommendations of the American Academy of Pediatrics for eye examinations of premature infants are based on the following criteria: an age of gestation less than 35 weeks for infants having received oxygen or a birthweight under 1300 g with or without supplemental oxygen. The first examination should take place between the 5th and the 7th week of extra-uterine life, or prior to discharge of the hospital and repeated according to the first observations. It is specified that the examination must be done by a person experienced in neonatal ophthalmology and indirect ophthalmoscopy. In Zurich, following these recommendations, the criteria of the CRYO-ROP study, and our experience, we examine the infants with following features: (1) all premature infants with birthweight less than 1500 g independently of oxygen exposure, (2) neonates who have less than 35 gestational weeks and who were exposed in a FIO2 > 0.4 during more than 24 h. And (3) the first eye examination is done between the 5th and the 6th weeks of extra-uterine life. The follow-up of these children will depend on the primary state. The infants with ROP will be followed by the neonatal ophthalmologist as long as retinal complications can persist. Afterwards these infants will be controlled by the general ophthalmologist for the amblyopie prophylaxis (by anisomyopia or strabismus). Two controls during the first year, then one per year are advised in these cases. For children who had a cryocoagulation or an retinal detachment operation, the follow-up will be assured by the general ophthalmologist for the prevention of the amblyopia and by the neonatal ophthalmologist for the retina.

Amblyopia↗

[Clinical diagnosis of proliferative vitreoretinopathy].

The clinical features of a proliferative vitreoretinopathy (PVR) are systematically described. The patient's history reveals moderate lost of vision and metamorphopsia. Ophthalmoscopy, biomicroscopy of the fundus with the contact lens, and black-and-white photography usually lead to the correct diagnosis. Early stages of PVR are characterized by increased reflectance and a cellophane appearance of the inner retinal surface and tortuosity of both small and larger vessels. Advanced epiretinal tissue formation causes development of surface wrinkling and single or multifocal star-folds. In cases of retinal detachment with PVR, the retina is immobile, the detachment being concave due to traction or bullous if there are secondary breaks. In the final stages of posterior and/or anterior PVR the detached retina forms a narrow or closed funnel.

Fluorescein Angiography↗