Band atrophy of the optic disc secondary to ventricular subependymoma compression

CASE REPORT: A 42-year-old man was diagnosed with band or "bow tie" optic atrophy with a right homonymous hemianopia. Computerized tomography (CT) revealed a calcified lesion in the left hippocampus. Craniotomy and tumor resection were performed. The biopsy revealed a subependymoma of the temporal horn of the left ventricular system. DISCUSSION: Optic tract lesions are uncommon clinical entities, in which homonymous hemianopia and contralateral band optic atrophy are characteristic. Subependymomas are infrequent and benign tumors that are typically associated with the ventricular system.     

Optic disc morphology in diabetes mellitus

Background: Diabetes mellitus is a systemic disease affecting multiple tissues throughout the body. This study was performed to evaluate intravitally the diabetic changes of the optic disc Methods: Color photographs of 115 eyes with varying severity of diabetic retinopathy and of 29 normal eyes were morphometrically examined Results: We found that the size and shape of the optic disc, the neuroretinal rim and the parapapillary atrophy did not differ significantly between the diabetic eyes and the normal eyes. These variables were independent of the degree of diabetic retinopathy. There was a tendency toward decreased visibility of the retinal nerve fiber layer and increased optic disc pallor in the diabetic eyes Conclusion: The results indicate that the area and form of the optic disc, the neuroretinal rim and parapapillary atrophy are not altered by diabetes mellitus. This is important for the diagnosis of glaucoma in diabetic patients, since glaucoma leads to a decrease of rim area and an enlargement of parapapillary atrophy. The reduced visibility of the retinal nerve fiber layer, the increased optic disc pallor and the unchanged size of the neuroretinal rim and parapapillary atrophy suggest that diabetes mellitus may be associated with nonglaucomatous optic nerve atrophy.     

Progression of gyrate atrophy measured with ultra-wide-field imaging

The study aims to determine the progression of gyrate atrophy by measuring the area growth of chorioretinal atrophic lesions using ultra-wide-field images (UWFI). A retrospective, observational, and comparative study was conducted and UWFI (200°) were obtained from two patients with gyrate atrophy at baseline and follow-up. Measurements of atrophy were obtained for three types of lesions: Solitary atrophic lesions (SAL), De novo solitary lesions (DNSL), and peripapillary atrophy (PPA). Comparison of baseline and follow-up was done using t tests. Two patients with gyrate atrophy were included. Patient 1 presented 16 SAL, 5 DNSL, and PPA measured for both eyes (BE). Overall area growth (OAG) for SAL (expressed in decimals) presented a mean of 3.41, σ 3.07. DNSL area for BE presented a mean of 1586.08 P ², σ 1069.55. OAG for PPA presented a mean of 1.21, σ 0.17. Patient 2 presented 5 SAL, no DNSL, and PPA was measured for BE. OAG for SAL presented a mean of 1.58, σ 1.05 (range 1.02–3.47). OAG for PPA presented a mean of 1.05, σ 0.001. Gyrate atrophy progression can be determined by measuring the changes in area using UWFI.     

Visual rehabilitation of patients with advanced stages of glaucoma,optic atrophy,myopia or retinitis pigmentosa

Ninety-six patients with advanced stages of glaucoma, optic atrophy, myopia or retinitis pigmentosa (RP) who could not manage with ordinary spectacles or simple magnifying aids were taken care of at the Low Vision Clinic for rehabilitation and followed for an average of 3.6 years (the glaucoma group) up to an average of 6.0 years (the optic atrophy group). They were given high power optical aids and subjected to educational training in the proper use of these aids for best utilization of residual vision. Many of them (for optic atrophy as high a percentage as 40.7) were taught to use extra macular retina by means of eccentric viewing technique. The mean age of the oldest group, the glaucoma patients, was 69.3 years. The three other groups were about 20 to 25 years younger, on an average. (Another 35 patients) were seen for the first series of visits but could not be followed up, the main reasons being death (13 patients) and moving out of the area (9 patients).The mean power of the aids (mainly telescopes) used for distance vision was 2.1 × (RP)-5.3 x (optic atrophy). Increased near addition and hyperocular lenses were the main aids for reading and near vision, the mean power being 17.0 dioptres (glaucorna)–23.5 dioptres (RP)(4.3 × –5.9 x). Aids were also provided for intermediate distance and for spot use. The mean number of series of visits was 3.1 (myopia)–3.5 (glaucoma) and the average number of l h training sessions 2.2 (glaucoma)–2.5 (optic atrophy, RP) per series of visits.With aids and educational training, the mean visual acuity improved on the first series of visits from 0.31 to 0.60 for the glaucoma group, from 0.19 to 0.70 for the optic atrophy group, from 0.12 to 0.68 for the myopes and from 0.35 to 0.52 for the RP group. After the last series of visits acuity was still as good as 0.51, 0.61, 0.73 and 0.45, respectively.The number of individuals able to read newspaper text increased from 16.1% to 100.0% for the glaucoma patients, from 14.8% to 100.0% for the optic atrophy patients, from 75.0% to 100.0% for the myopes, and from 50.0% to 95.5% for the RP patients.The results show clearly that the methods used for rehabilitation of patients with glaucoma, optic atrophy, myopia or RP through optical aids and sessions of educational training are very successful, with substantial improvement of life quality.     

Comparison of ability of time-domain and spectral-domain optical coherence tomography to detect diffuse retinal nerve fiber layer atrophy

Purpose

Our aim was to evaluate and compare diagnostic capabilities of time-domain (Stratus) and spectral-domain (Cirrus) optical coherence tomography (OCT) to detect diffuse retinal nerve fiber layer (RNFL) atrophy.

Methods

This study assessed 101 eyes from 101 glaucoma patients with diffuse RNFL atrophy and 101 eyes from 101 age-matched healthy individuals. Two experienced glaucoma specialists graded red-free RNFL photographs of eyes with diffuse RNFL atrophy using a four-level grading system. The area under the receiver operating characteristic curves (AUC) of normal eyes was compared with that of eyes with diffuse atrophy. Sensitivity and specificity of each OCT device were calculated on the basis of its internal normative database.

Results

The largest AUC for Stratus and Cirrus were obtained for average RNFL thicknesses (0.96 and 0.94, respectively). Comparison of the AUC with different RNFL atrophy grades revealed no significant difference between the two OCT devices. Using an internal normative database at a <5 % level, the overall sensitivity of Stratus ranged from 58.0 to 84.0 %, whereas that of Cirrus ranged from 75.0 to 87.0 %. According to the normative database, the highest Stratus sensitivity was obtained with the temporal–superior–nasal–inferior–temporal (TSNIT) thickness graph, and the highest Cirrus sensitivity with the TSNIT thickness graph and the deviation map.

Conclusions

The AUC obtained from Cirrus were comparable with those from Stratus. On the basis of their normative databases, these devices had similar diagnostic accuracy. Our results suggest that the diagnostic capabilities of the two instruments to detect diffuse RNFL atrophy are similar.     

Role of iris changes as a cause of blindness in lepromatous leprosy.

Clinical and pathological investigations in a group of 113 patients with leprosy of long duration demonstrated the importance of iris changes as a cause of blindness. In lepromatous leprosy the so called "chronic iritis" produces iris atrophy with small nonreacting pupils which exaggerate the visual impairment created by developing lens changes and corneal opacities. The cause of this "chronic iritis" is believed to be neuroparalytic from early involvement of the small nerves of the iris, particularly the autonomic supply. Clinical and pharmacological evidence for this theory is supported by the histological changes observed in 18 specimens of iris removed during the course of cataract surgery, with progressive atrophy of the iris preferentially affecting the dilator muscle and leading to a nonreacting miosed pupil. Further pharmacological and histological studies are to be undertaken on lepromatous patients with iris involvement with a view to establishing possible methods of prevention.     

Parry-Romberg progressive facial hemiatrophy and localized scleroderma. Nosologic and pathogenic problems

A case of a 65-year-old caucasian woman with progressive hemifacial atrophy (Parry-Romberg syndrome) is reported. The atrophy had begun ten years before. At time of presentation, she was suffering from facial pain and diplopia. She presented with severe enophthalmos associated with eyelid atrophy, loss of cilia and mild blepharoptosis. There was a patch of alopecia and atropic areas of skin, recognised as scleroderma "en coup de sabre". Ophthalmic examination showed miosis. Horner's syndrome and ocular motility disturbance. Biomicroscopic examination of anterior and posterior segments was normal; there was no heterochromia and no disc atrophy. Aesthetic surgical treatment was proposed. The etiology of hemifacial atrophy remains controversial, and its relations with scleroderma and autonomic nervous system disorders are discussed.     

Factors associated with the development of chorioretinal atrophy around choroidal neovascularization in pathologic myopia

Purpose To examine the influencing factors on the development of chorioretinal atrophy, which is the main cause of long-term visual decrease in myopic choroidal neovascularization (CNV), in a large series of highly myopic patients.Methods Sixty-five patients (81 eyes) with myopic CNV were studied retrospectively. The influence of the patients age, refractive error, axial length, visual acuity at onset of CNV, size of CNV, and grade of myopic retinopathy on the extent of chorioretinal atrophy more than 3 years after CNV onset was investigated by means of multiple linear regression analysis.Results Seventy-seven of 81 eyes (95.1%) developed chorioretinal atrophy around myopic CNV during the follow-up period. Multiple linear regression revealed that age was the most influencing factor for the development of chorioretinal atrophy in all the subjects. When we divided the subjects into two groups according to their age, however, CNV size was the only factor to influence the development of chorioretinal atrophy in the patients younger than 40 years, whereas age was still the only influencing factor in those older than 40 years.Conclusions The factors influencing the development of chorioretinal atrophy differ according to patient age. Local factors, such as CNV size, determine the tendency to develop chorioretinal atrophy in young patients. Systemic factors, such as patient age, play a greater part in older subjects.Presented in part as a poster at the Association for Research in Vision and Ophthalmology meeting, Fort Lauderdale, Florida, May 2003The authors have no financial interest in any products / drugs discussed in this article     

Papilledema in the setting of x-linked hypophosphatemic rickets with craniosynostosis

Purpose

Introduction to the ophthalmic literature of an unusual cause of papilledema and subsequent optic atrophy: X-linked hypophosphatemic rickets (XLH).

Methods

Case report of a 3-year-old female presenting with papilledema resulting from craniosynostosis secondary to XLH.

Results

Early intervention with craniofacial surgery prevented the development of optic atrophy.

Conclusion

Children with XLH should be screened for ophthalmic evidence of elevated intracranial pressure to aid early intervention and prevention of permanent loss of vision.Key words: Papilledema, Optic atrophy, Rickets, Craniosynostosis     

视神经萎缩的中医药治疗概况

视神经萎缩是一种由于各种病因所致视神经纤维传导功能产生障碍的视神经退行性改变,临床以视力减退和视乳头呈灰白或苍白为特征。本病属于中医学“青盲”的范畴。西医目前对该病治疗尚无理想药物及技术;中医采用中药与针刺等方法可使大多数患者视力有所改善。本文就中医药治疗本病进行综述。     

Optic nerve atrophy and CT scanning: the target sign

Three patients are described who demonstrate the "target-sign" of optic nerve atrophy on CT scanning. This sign is produced by the centrally located atrophic optic nerve surrounded by a patulous perioptic subarachnoid space. An explanation for this phenomenon is proposed.     

The ultrastructure of parapapillary chorioretinal atrophy in eyes with secondary angle closure glaucoma

Background: The present study was performed to investigate the ultrastructure of deep retinal layers and choroid corresponding to the parapapillary chorioretinal atrophy in eyes with secondary angle-closure glaucoma. Methods: The glaucomatous eyes included two eyes enucleated due to iris ring melanoma with high intraocular pressure and one eye with neovascular glaucoma enucleated due to ocular pain. The control eyes included one eye enucleated due to choroidal malignant melanoma with normal intraocular pressure and one eye enucleated during surgery for supramandibular carcinoma. These eyes were studied with light and electron microscopy. Results: In the region of parapapillary chorioretinal atrophy of glaucomatous eyes, the retinal pigment epithelial cells showed degenerative changes, such as loss of basal in foldings and microvilli, degenerated mitochondria, vacuolar degeneration and irregular distribution of melanin granules. The photoreceptors were decreased in number in this area of glaucomatous eyes. The lumen of the choriocapillary vessels adjacent to the optic nerve was collapsed. Conclusion: These results elucidate the fine structures of deep retina and choroid in the region of parapapillary chorioretinal atrophy of glaucomatous eyes, and suggest that the reduced choroidal perfusion might be the pathogenetic mechanism of glaucomatous parapapillary chorioretinal atrophy.     

Makula-Atrophie bei feuchter altersabhängiger Makuladegeneration

Background

Current understanding of the mechanisms that underlie the long-term consequences of anti-VEGF therapy in wet, age-related macular degeneration (AMD) is poor. Here, the impact of this treatment on the development of macular atrophy (MA) is discussed based on our current pathophysiological understanding.

Methods

This review is based on a PubMed literature survey using the MeSH terms “wet AMD” and “macular atrophy” (151 hits) and limited to publications since 2013 (n = 90). Publications focussing on diagnostics and clinical course not in the context of therapy were excluded. Macular atrophy is defined herein as atrophy affecting the functionally relevant complex of photoreceptors, retinal pigmented epithelium (RPE), Bruch’s membrane and choriocapillaris.

Results

Experimentally, a primary complete suppression of local VEGF leads to evident changes in the choriocapillaris, whereas its incomplete suppression exacerbates cell death of RPE and photoreceptors. Since pre-existing atrophic changes are already present at diagnosis, the role of anti-VEGF treatment cannot be separated from the spontaneous progression of AMD. The progression of MA appears to be faster under ranibizumab than bevacizumab, and likewise on a monthly rather than as-needed basis. Although MA progresses more rapidly under consequent therapy, visual function remains better. Hence, a functionally relevant progression of atrophy during the first five years of treatment would only be expected in pre-existing advanced MA.

Conclusions

Despite doubts regarding the long-term safety of anti-VEGF therapy, it is the author’s view that this is the only option to stabilise visual function. The impact of therapy-induced damage on the spontaneous progression of AMD and the biological status of the aging individual cannot be unequivocally assessed.

     

老年中心性浆液性脉络膜视网膜病变的光学影像学表现

目的:观察老年中心性浆液性脉络膜视网膜病变(central serouschorioretinopathy,CSC)的光学影像学表现。方法:回顾性分析2008-01/2009-06在我科经眼底荧光血管造影(fundus fluorescein angiography,FFA)、吲哚青绿血管造影(indocyanine green angiography,ICGA)及光相干断层扫描(optical coherence tomography,OCT)共同确诊的17例18眼老年CSC患者的临床资料。结果:老年CSC患者具有与中青年患者相同的FFA,ICGA及OCT改变;也具有一些其独自的特征性改变,主要表现为:(1)FFA检查发现CSC病变所表现的典型RPE渗漏形式,如"炊烟"状或"墨渍"样少见,仅占39%;其他11眼(61%)均表现为扩散性视网膜色素上皮病变;(2)ICGA检查发现其黄斑部还合并一些其他改变,如多灶性浆液性色素上皮脱离(pigment epithelium detachment,PED),息肉样脉络膜血管扩张,脉络膜毛细血管萎缩等;(3)FD-OCT检查发现其黄斑部也合并一些其他改变,如玻璃膜疣引起的RPE结节状隆起,多灶性视网膜萎缩、及IS/OS层的局部缺失等。结论:老年CSC患者的光学影像学表现复杂,容易与老年性黄斑变性相混淆,同步FFA及ICGA检查联合OCT检查有助于确诊。     

Bilateral pigmented paravenous chorioretinal atrophy: a case report

Purpose

To describe a case of bilateral pigmented paravenous chorioretinal atrophy.

Methods

Observational case report.

Results

A 50-year-old female patient complained of right eye pain and decreased near visual acuity. She had a best-corrected visual acuity of 20/20 in both eyes. In the posterior segment, there were atrophic changes along retinal vessels, and bony spicule pigmentation was observed in a paravenous distribution. There were some abnormal changes in the electroretinogram and electrooculogram in both eyes.

Conclusions

A diagnosis of bilateral pigmented paravenous chorioretinal atrophy was made.Key words: Atrophy, Chorioretinal atrophy, Paravenous atrophy     

Congenital high myopia and central macular atrophy: a report of 3 families

Aims

To report the clinical phenotype in a series of four children from three families with the rare association of high myopia, central macular atrophy, and normal full-field electroretinography (ERG).

Methods

Four male patients were ascertained with reduced vision, nystagmus, and atrophy of the macula from early childhood. Patients underwent full ophthalmic examination, electrophysiological testing, and retinal imaging.

Results

Minimum duration of follow-up was 8 years. At last review, visual acuity ranged from 0.22 to 1.20 logMAR (6/9.5–6/95 Snellen) at a mean age of 10.5 years (median 9.5 years, range 9–14 years). Refractive error ranged from a spherical equivalent of −7.40 D to −24.00 D. Three had convergent squint. Fundus examination and imaging demonstrated bilateral macular atrophy in all patients that varied from mild atrophy of the retinal pigment epithelium (RPE) to well-demarcated, punched-out atrophic lesions of retina, RPE, and choroid. Flash ERG was normal under photopic and scotopic conditions in all patients. Pattern ERG, performed in three patients, was consistent with mild to severe macular dysfunction. Progression of the area of atrophy was evident in one patient and of the myopia in two patients but all patients had stable visual acuity.

Conclusions

Patients with congenital high myopia and macular atrophy present in infancy with reduced visual acuity and nystagmus. The macular atrophic lesions vary in size and severity but electrophysiological testing is consistent with dysfunction confined to the macula. There was no deterioration in visual acuity over 8–10 years of monitoring.     

DRUSEN PATTERNS PREDISPOSING TO GEOGRAPHIC ATROPHY OF THE RETINAL PIGMENT EPITHELIUM

Abstract
This report discusses the recognition of drusen referred to as "hard" which appear to predispose the eye to the development of geographic atrophy of the retinal pigment epithelium. When numerous, small hard drusen tend to become arranged in clusters. On the temporal side in particular, this grouping may be so close as to resemble larger confluent drusen within which the small drusen can only be distinguished by fluorescein angiography.
The approximate interval before geographic atrophy involves the fovea can be estimated by noting the distribution of drusen and the state of the pigment epithelium between the drusen. A stage of incipient atrophy can be recognised as an area of diffuse hyperfluorescence in which pigment clumping or reticular pigment figures and fading of drusen occur.     

Gyrate atrophy of the choroid and retina

Gyrate atrophy of the choroid and retina is caused by deficient activity of ornithine ketoacid aminotransferase, a pyridoxal phosphate dependent enzyme. Besides the typical eye findings, abnormalities have been found on muscle biopsy, electro-encephalography, electromyography and electrocardiography, establishing this as a generalized disorder. Ornithine is markedly elevated in plasma and other body fluids. Plasma lysine, glutamate, glutamine and creatine are reduced. The possible contributions of these biochemical disturbances to the pathogenesis of gyrate atrophy are discussed. The disease is one of the few examples of an inherited chorioretinal dystrophy whose underlying biochemical defect is known. It therefore offers a unique opportunity to develop and test rational approaches to therapy. These include lowering of the abnormally high ornithine by dietary restriction of its precursor arginine, facilitation of ornithine excretion by administation of -aminoisobutyric acid, replacement of deficient products such as lysine or creatine, or increasing residual enzyme activity by high levels of cofactor (vitamin B₆). The results of several studies employing such approaches to therapy are presented as well as preliminary indications of possible benefit in a few patients.     

Optic disc morphology in “age-related atrophic glaucoma”

Background: This study was performed in order to evaluate whether, in primary open-angle glaucoma (POAG), patients with a different degree of fundus tessellation vary in optic disc morphology and level of intraocular pressure Methods: Color stereo optic disc photographs of 562 patients with POAG and a myopic refractive error of less than –8 diopters were morphometrically examined. According to the degree of fundus tessellation, the total group was divided into a tessellated subgroup (n = 256) and a nontessellated subgroup (n = 306,) both matched for neuroretinal rim area and refractive error Results: In the tessellated subgroup, as compared to the nontessellated subgroup, the mean maximal intraocular pressure values were significantly lower, the parapapillary atrophy was significantly larger, the optic cup was significantly more shallow, frequency of disc hemorrhages was lower, the mean visual field defect was significantly more marked, and patient age was significantly higher. Within the whole study group, the degree of fundus tessellation increased significantly (P<0.005) with decreasing mean maximal intraocular pressure, decreasing optic cup depth, and increasing degree of parapapillary atrophy. In the subgroups with the highest degree of fundus tessellation, parapapillary atrophy was the greatest and the mean maximal intraocular pressure was the lowest compared to other subgroups Conclusion: At the low-pressure end of POAG, marked fundus tessellation is associated with large parapapillary atrophy, shallow disc cupping, mostly concentric emaciation of the neuroretinal rim, and high patient age. The results suggest a distinct subtype of POAG in older patients with relatively low intraocular pressure leading to a mainly diffuse atrophy of the optic nerve.     

Persistent pupillary dilation in herpes simplex uveitis

Objective: To report the association between herpes simplex virus (HSV) and iris atrophy with pupillary dilation.Design: Retrospective case series.Participants: Patients with a clinical diagnosis of HSV keratouveitis seen between November 1993 and April 1994 in a single university uveitis clinic. There were 6 women and 7 men, aged 17 to 69 years (mean age 44.4 years).Methods: Patient information was collected, including history and medications used, and a complete ophthalmologic evaluation was performed, with careful documentation of iris detail and pupillary size.Results: Thirteen of 13 patients demonstrated some degree of iris atrophy; 9 of 13 had pupillary dilation on the affected side despite not being on dilating drops.Conclusions: Iris atrophy and pupillary dilation in a patient with unexplained anterior uveitis suggests the diagnosis of HSV keratouveitis.