Anterior ischaemic optic neuropathy in a patient with optic disc drusen

Background: Although visual field defects are well-known complications of optic disc drusen, reduction in visual acuity with this condition is rare. Method/Results: We report on a 68-year-old male with bilateral optic disc drusen who presented with monocular loss of vision in the right eye associated with an inferior altitudinal visual field defect and signs consistent with acute anterior ischaemic optic neuropathy, confirmed on fluorescein angiography. He also had a left inferior nasal step, but no evidence of glaucomatous cupping. The disc drusen were documented clinically and on B scan ultrasound and computed tomography. Conclusions: The diagnosis of acute anterior ischaemic optic neuropathy should be considered in patients with optic disc drusen who present with reduced visual acuity, particularly when the visual loss has been acute and non-progressive and is associated with altitudinal field loss and characteristic fluorescein angiography signs.     

非青光眼性大视杯临床分析

目的 探讨非青光眼性疾病引起视杯扩大的病因以及鉴别要点,为临床识别非青光眼性大视杯提供依据。设计 回顾性病例系列。研究对象 12例（19眼）非青光眼大视杯患者。方法 分析比较这些患者的病因、视盘形态学特征以及相关影像资料。主要指标 病因、视盘形态特征以及视功能改变。结果 12例患者中,4例为视神经炎,1例视神经脊髓炎,1例Leber遗传性视神经病变,2例垂体瘤,1例基底节脑出血,1例睫状视网膜动脉阻塞合并视网膜中央静脉阻塞,1例视网膜中央动脉阻塞,1例视神经损伤。所有患者视杯呈弥漫性或局限性扩大,盘沿苍白。视野表现为与原发病相应的缺损。结论 各种视神经疾病和视网膜疾病均有可能导致大视杯,它与青光眼性大视杯的鉴别点在于盘沿色泽、有无盘沿局限性缺失以及视功能异常和视盘改变的相关性。（眼科, 2012, 21: 306-309）     

Measurements of Reversibility of Optic Disc Cupping and Pallor in Ocular Hypertension and Glaucoma

Four cases are presented of young and elderly glaucoma patients who had both surgical and medical therapy and showed reversal of cupping and pallor of the optic disc. The cupping was measured by photogrammetry and the pallor by computerized image analysis from photographs of the optic disc. Two patients showed regression of visual field loss. The optic disc and visual field changes corresponded to the changes in ocular pressures, generally showing worsening with an increase in ocular pressure. With a decrease in ocular pressure there was regression of visual field loss and a decrease in cupping; pallor did not decrease consistently. The changes in cupping and pallor in some patients followed similar courses but in others behaved in an independent manner. It is proposed that these new sensitive and reproducible techniques for measuring changes in the optic disc may allow the detection of disc changes early in the disease, prior to visual field loss. If treatment is begun at this time, reversal of optic disc changes may occur.     

Clinical Feature Analysis of Congenital Optic Nerve Abnormalities

Purpose

We investigated the clinical characteristics and associated ocular and systemic anomalies in young children with congenital optic nerve abnormalities, and evaluated the therapeutic results of treatment for strabismus and amblyopia.

Methods

A retrospective study was conducted using the medical records of patients who were diagnosed with congenital optic nerve abnormalities in our hospital between 1995 and 2004.

Results

This study involved 72 eyes of 51 young children (M?:?F = 25?:?26); 21 of these patients (41.2%) had bilateral abnormalities. The types and percentages of each abnormality were as follows: optic nerve hypoplasia, 47%; optic disc coloboma, 31.4%; morning glory syndrome, 11.8%; and myelinated nerve fiber, 9.8%. The first clinical manifestations noticed by parents were strabismus and decreased visual acuity. Occlusion therapy was carried out in 16 patients, and the visual acuities of two patients were improved by more than two lines of the Early Treatment Diabetic Retinopathy Study visual acuity chart. Accompanying strabismus was found in 70.6% of the patients; 14 patients underwent strabismic surgery, and 10 of these patients (41.2%) maintained a stable angle.

Conclusions

The most common congenital optic nerve abnormality was optic nerve hypoplasia, and the associated systemic abnormalities found were optic nerve hypoplasia and disc coloboma. Patients with congenital optic nerve abnormalities usually have a generally poor visual prognosis, but we emphasize the importance of treatment for associated strabismus and amblyopia.?Jpn J Ophthalmol 2006;50:250–255 © Japanese Ophthalmological Society 2006     

Comparative findings in childhood‐onset versus adult‐onset optic disc pit maculopathy

Purpose: To compare the clinical characteristics of eyes with childhood‐onset to those with adult‐onset optic disc pit maculopathy. Methods: Twenty‐seven eyes of 25 patients with optic disc pit maculopathy were reviewed. The clinical characteristics, clinical history including a history of blunt trauma, ophthalmoscopic evaluations and intraoperative findings in the childhood‐onset (age <15 years, four eyes of four patients) cases were compared with those in the adult‐onset (≥15 years, 23 eyes of 21 patients) cases of optic disc pit maculopathy. The strength of the vitreous adhesions was graded by what was required to create a posterior vitreous detachment (PVD); grade 1 = with vitreous cutter, grade 2 = by microhook or forceps and grade 3 = by forceps with removal of remnants of Cloquet’s canal. Results: The incidence of visual impairments following blunt trauma was significantly higher in childhood‐onset (three of four eyes) than that of adult‐onset (0 of 23 eyes, p = 0.0014). Vitreous surgery with creation of a PVD was performed in the four childhood‐onset eyes and 18 adult‐onset eyes after an absence of a spontaneous resolution. The grade of the vitreous adhesions was significantly higher in childhood‐onset than in adult‐onset eyes (p = 0.0096). Conclusions: An ocular trauma may provide an opportunity to detect optic disc pits. However, childhood‐onset optic disc pit maculopathy was noted most commonly following blunt ocular trauma in eyes with a strong vitreous adhesion to the optic disc margin.     

Bilateral optic neuropathy due to amiodarone with recurrence

Patients on amiodarone have an increased incidence of optic neuro­pathy. The unusual case is reported of a 67‐year‐old man who developed bilateral optic neuropathy 4 weeks after commencing amiodarone and then had unilateral recurrence of segmental disc swelling despite cessation. The case highlights amiodarone‐induced optic neuropathy as a different clinical entity to anterior ischaemic optic neuropathy. It is characterized by insidious onset, bilateral disease and protracted disc swelling progressing to optic atrophy. Patients that experience any visual disturbance while taking amio­darone should seek urgent ophthalmic review.     

Comparison of clinical optic disc assessment with tests of early visual field loss

Purpose: Clinical optic disc assessment may identify glaucomatous optic neuropathy prior to a patient developing visual field abnormalities on achromatic automated peri­metry (AAP). Tests targeting axons that are selectively damaged or whose redundancy is low, such as short wavelength automated perimetry (SWAP) and frequency doubling perimetry (FDP), may detect visual field loss before it is seen on AAP. This study investigated whether patients in whom characteristic glaucomatous optic disc damage was present without AAP abnormalities had visual field abnormalities with SWAP and FDP. Methods: A sample of patients (n = 50) with ocular hypertension (normal AAP) were selected, who had SWAP, FDP and stereofundus photography performed. The photographs were then analysed by two glaucoma subspecialists who were masked to the assessments of the other and to the patients’ SWAP and FDP results. A categorization of the optic discs was made as either normal or abnormal and this was compared with their SWAP and FDP findings. Results: On comparing SWAP and FDP with clinical optic disc assessment as the ‘gold standard’, the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were 33%, 92%, 57% and 81%, respectively, for SWAP and 25%, 89%, 49% and 79%, respectively, for FDP. Conclusion: In glaucoma suspects, the study suggests that SWAP and FDP identify subjects with early glaucomatous optic neuropathy missed by AAP.     

Optic disc progression and rates of visual field change in treated glaucoma

Purpose: To investigate the relationship between optic disc progression and rates of visual field (VF) change in patients with treated glaucoma. Methods: Glaucoma patients with repeatable VF loss, ≥8 SITA‐Standard 24‐2 VF tests and good quality optic disc stereophotographs evaluated over a 10‐year period were included. Optic disc photographs were reviewed for signs of glaucoma progression (neuroretinal rim change, widening of retinal nerve fibre layer defect, disc haemorrhage and enlargement of beta‐zone parapapillary atrophy) by two glaucoma specialists masked to their temporal sequence. Disagreements were adjudicated by a third grader. VF progression was evaluated using automated pointwise linear regression (PLR) and defined as at least two adjacent test points progressing >1.0 dB/year at p < 0.01. VF progression outcomes were compared with photograph review results. Results: Three‐hundred and eighty nine eyes (389 patients; mean age 64.9 ± 13.0 years; mean baseline MD, ?7.1 ± 5.1 dB) were included. Most patients had primary open angle glaucoma (54%). Eighty‐two eyes (21%) had confirmed optic disc progression and 115 eyes (29%) met the VF PLR criteria. Eyes with documented optic disc progression had more rapid rates of VF change (?0.66 ± 0.7 versus ?0.36 ± 0.7 dB/year, p < 0.01) and met the VF PLR endpoint more often (univariate OR = 1.85, p = 0.02; multivariate OR = 1.78, p = 0.03) than eyes without optic disc progression. There was moderate spatial consistency between the location of the optic disc progression and the hemifield with more rapid progression (81%, kappa = 0.40). Conclusions: Treated glaucomatous eyes with documented optic disc progression are at increased risk of diminished visual function over time and may require more aggressive therapy to prevent future vision loss. Among the indicators of structural progression, disc haemorrhage was the single most significant predictor for VF deterioration.     

Partly reversible visual failure with methanol toxicity

Methanol is a highly toxic substance which is used as an industrial solvent and in automotive antifreeze. If accidentally ingested blindness or death may result. The case of a young woman who developed sudden onset of visual failure following ingestion of a methanol-fortified beverage is presented. Although she failed to seek immediate medical help visual function improved. Acute changes of bilateral optic disc hyperaemia and venous engorgement were present at initial examination. She subsequently developed optic disc atrophy together with glaucomatous-like cupping of the optic discs. The aetiology of visual failure in methanol poisoning is discussed, as are the current therapeutic guidelines in the management of acute cases.     

先天性视盘凹陷性疾病的研究进展

先天性视盘凹陷性疾病是视盘先天发育异常中的一组疾病,可导致儿童视力低下甚至致盲.属于临床上易误诊的一类疾病.视盘凹陷性疾病的发病机制仍不十分明确.随着近年来对此类疾病的重视和一系列的研究,疾病特征逐渐明朗,诊疗规范也在逐步建立.本文就牵牛花综合征、视盘缺损、盘周葡萄肿、巨大视盘、视盘小凹等先天性视盘凹陷性疾病研究进展进行综述.     

非动脉炎性AION中缺血部位及水肿程度对中心视力的影响

目的 :探讨非动脉炎性前部缺血性视神经病变 (nonarteriticanteriorischemicopticneuropathy ,NAION)中视盘水肿程度、缺血部位对中心视力的影响 ,以利于疗效评定及预后判断。方法 :2 40例NAION患者 (160例接受住院治疗 )的临床资料 ,结合视野和荧光眼底血管造影 (fundusfluoresceinangiography ,FFA)检查进行治疗前中心视力与水肿程度、缺血部位两方面统计分析 ,并分析治疗效果。结果 :2 40例NAION患者发病时中心视力的损害程度 ,依视盘缺血部位、水肿程度之不同而均有显著的差异 (P <0 0 0 5 )。 160例住院患者 ,视盘水肿消退时间最长为 3 5天 ,最短 11天 ,平均时间 2 1 5天 ,FFA复查四项指标改善率 88 1% ,中心视力两行行上者 10 4例 ,占 65 %。结论 :视盘的缺血部位及水肿程度是影响NAION中心视力的重要因素之一 ,积极针对性的治疗对防止NAION致盲有重要意义。     

Adalimumab and Non-Arteritic Anterior Ischaemic Optic Neuropathy: A Case Report

Sequential anterior ischaemic optic neuropathy was observed in a patient treated with a tumour necrosis factor α (TNF) inhibitor, adalimumab, for ankylosing spondylitis. He developed decreased visual acuity in the right eye after 17 months of treatment. Findings showed right optic disc oedema with haemorrhages and visual field defect. Adalimumab was discontinued and vision stabilised. After restarting adalimumab, he developed optic neuropathy in the left eye. Findings showed optic disc oedema, with haemorrhages and visual field changes in the left eye. Adalimumab may be associated with optic neuropathy; providers prescribing TNF inhibitors should be aware of optic neuropathy as a potential complication.     

Congenital anomalies of the optic disc

Although anomalies affecting the optic nerve head are usually clinically innocuous, they can sometimes cause significant symptoms and lead to visual loss. It is important to be able to recognize even the relatively benign lesions in order to differentiate them from other more threatening lesions or disease processes which they may clinically resemble. An awareness of the clinical appearance of disc anomalies is especially important in the differential diagnosis of optic nerve glaucomatous changes. Some anomalies cause various types of visual field loss which, if the actual disc lesion is not recognized, may lead to unnecessary neurologic evaluation or even to intracranial surgery. The optic nerve changes in acquired myopia and in the congenital tilted disc syndrome should be clearly defined and differentiated: high (pathologic) myopia may be highly progressive with many dangerous secondary sequelae, while the latter anomaly is stationary. Finally, there is a group of conditions, collectively termed “elevated anomalies of the disc,” which must be considered in the differential diagnosis of papilledema and potentially dangerous intraocular tumors, particularly retinoblastoma. This review provides a clinicopathologic correlation comparing the characteristics of the normal optic disc to those of the most important congenital anomalies of the disc.     

Adalimumab and Non-Arteritic Anterior Ischaemic Optic Neuropathy: A Case Report

Abstract

Sequential anterior ischaemic optic neuropathy was observed in a patient treated with a tumour necrosis factor α (TNF) inhibitor, adalimumab, for ankylosing spondylitis. He developed decreased visual acuity in the right eye after 17 months of treatment. Findings showed right optic disc oedema with haemorrhages and visual field defect. Adalimumab was discontinued and vision stabilised. After restarting adalimumab, he developed optic neuropathy in the left eye. Findings showed optic disc oedema, with haemorrhages and visual field changes in the left eye. Adalimumab may be associated with optic neuropathy; providers prescribing TNF inhibitors should be aware of optic neuropathy as a potential complication.     

Anterior Ischemic Optic Neuropathy in a Patient with Churg-Strauss Syndrome

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.     

Optic nerve sheath meningiomas. Clinical manifestations

A retrospective clinical study of optic nerve sheath meningiomas based on 22 patients showed that symptoms most commonly develop in women between the ages of 35 and 60 years. The most common presenting symptoms were decreased vision and transient visual obscurations. In the earliest stages, many patients presented with normal to mildly impaired acuity (despite subjectively decreased vision), optic disc edema and enlargement of the blind spot. Optic disc edema was frequently associated with refractile bodies indicative of chronic swelling. Optic disc edema preceded the development of optic atrophy. Another group of patients presented with a history of longstanding vision loss, visual acuity of 20/200 or worse and optic atrophy. Optociliary shunt vessels were late findings only seen in five patients. The most consistent visual field abnormality was peripheral constriction. Cecocentral scotomas were uncommon. Intracranial involvement was present in five patients. There were two patients with bilateral optic nerve sheath meningiomas without CT evidence of intracranial involvement. Computerized tomography was found to be indispensable in the diagnosis of optic nerve sheath meningiomas and the detection of intracranial involvement.     

Optic Disc Edema in the Presumed Ocular Histoplasmosis Syndrome

Two patients with presumed ocular histoplasmosis syndrome developed optic disc edema. In both cases the edema resolved without residual impairment of visual function. Although peripapillary changes in ocular histoplasmosis are common, optic disc edema is rare. The pathogenesis of the edema is unknown.     

195例视盘水肿患者的病因分析

目的 分析北京同仁眼科中心神经眼科门诊就诊的视盘水肿患者的病因。设计 回顾性病例系列。研究对象 2018年1月至12月在北京同仁眼科中心神经眼科门诊就诊的195例视盘水肿患者。方法 回顾性分析了195例视盘水肿患者的电子病历资料。对全部、不同年龄组和单双眼视盘水肿的病因分布进行分析,并对视盘水肿排名前两位的病种进行临床特征对比分析。主要指标 病因分布构成比。结果 非动脉炎性前部缺血性视神经病变（NA-AION）是视盘水肿最常见的病因（49.7%）,后面依次是视神经炎（16.4%）、颅内压增高所致视乳头水肿（7.7%）。≤20岁组,视盘水肿最常见的病因是假性视乳头水肿,＞20且≤50岁组和＞50岁组,视盘水肿的最常见病因均是NA-AION。单眼视盘水肿的最常见病因是NA-AION,双眼最常见的是颅内压增高所致视乳头水肿。与视神经炎相比,NA-AION的年龄更大,单眼水肿更常见,主诉视野遮挡更多,较少伴有眼痛。结论 神经眼科门诊最常见的视盘水肿病因是NA-AION,其次是视神经炎。发病年龄和发病眼别（单眼或双眼）对于我们寻找视盘水肿的病因具有一定的提示作用。     

Disulfiram-induced Bilateral Optic Disc Oedema with Disc-Related Visual Field Loss and Sparing of Central Vision

Disulfiram (Antabuse) is a synthetic compound used in the treatment of chronic alcohol addiction. Bilateral retrobulbar optic neuropathy with visual loss has been reported in patients receiving disulfiram. We describe the case of a 37-year-old man with bilateral optic disc oedema occurring after beginning a regimen of disulfiram. There were visual field defects as seen with local disease of the optic disc but not central visual loss as occurs typically in toxic optic neuropathy. Optic disc oedema improved promptly after discontinuation of the drug. After excluding all other causes of bilaterally swollen optic discs, ophthalmologists should be aware of this rare adverse effect of disulfiram in chronic alcoholics who develop bilateral optic disc oedema.     

Focal Superonasal Optic Nerve Hypoplasia

Of 237 patients seen in the Neuro-Ophthalmology Division of the Jules Stein Eye Institute 1986–2009, coded as “congenital optic nerve anomaly”, 13 demonstrated focal superonasal flattening of the optic disc margin and nerve fibre layer thinning, with corresponding inferotemporal arcute visual field defect. In 11 cases, an unusual finding of margination of the visual field defects along the temporal horizontal midline was detected. In only two cases was a history of maternal diabetes positive. Magnetic resonace scans showed no additional brain abnormalities. A syndrome of focal superonasal optic nerve hypoplasia is identified, distinct from the superior segmental optic nerve hypoplasia syndrome.