先天性视盘占位病变继发非动脉炎性前部缺血性视神经病变的临床特征分析

目的 探讨先天性视盘占位病变相关的非动脉炎性前部缺血性视神经病变（NAION）的临床特征。设计 回顾性病例系列。研究对象 2010年1月-2019年6月北京同仁医院先天性视盘占位病变继发NAION患者9例（11眼）。方法 回顾及分析患者的眼科及头颅神经影像学资料。所有患者均经半年以上随访。主要指标 性别、年龄、发病眼别、视盘病变特征及与视野改变关系、头颅神经影像学表现。结果 9例继发NAION的先天性视盘占位病变中4例视盘黑色素细胞瘤,3例视盘内玻璃膜疣,2例视盘区残存有髓纤维。年龄26~65岁（中位数39岁）。7例单眼发生NAION,2例视盘内玻璃膜疣患者双眼先后发生NAION。发生NAION时患眼矫正视力0.05~1.0。可行视野检查的4例患者表现为水平下半缺损及向心性缩小。最后随访时均无视功能进行性损伤和病情复发。结论 先天性视盘占位性病变可继发NAION而出现视功能损伤。（眼科, 2020, 29： 141-146）     

Pathophysiology of the progressive optic neuropathy of glaucoma

Glaucoma is the most common chronic optic neuropathy. Although traditionally considered a disease of elevated intraocular pressure, it is now clear that glaucoma is primarily a distinctive optic neuropathy. This article discusses the distinctive features of glaucoma: disk morphology, visual field patterns, and disease progression. The primary goal is to distinguish glaucoma from other optic neuropathies and, in so doing, to suggest some hypotheses for its etiology.     

Etanercept associated optic neuropathy

We report the case of a 14-year-old boy who developed optic neuropathy subsequent to the use of etanercept. There have been 15 reported cases of anti-TNF-alpha-associated optic neuropathy to date and their characteristics are reviewed in this report, as well as possible pathophysiologic mechanisms behind such phenomenon. Such cases demonstrate the importance of prompt ophthalmologic evaluation of visual changes in patients being treated with anti-TNF-alpha antagonists.     

Ischemic optic neuropathy associated with retinal embolism

Ischemic optic neuropathy occurred in three patients (a 55-year-old man and two women, 64 and 68 years old). Visual loss followed coronary bypass surgery in two patients who also had diffuse atherosclerotic disease. Retinal emboli were present in both eyes of each. The third patient developed ischemic optic neuropathy with evidence of ipsilateral retinal emboli shortly after cardiac catheterization. Her only other risk factor for ischemic optic neuropathy was systemic hypertension. Although highly unusual, ischemic optic neuropathy may be associated with and possibly caused by a shower of emboli to the eye.     

Anterior ischemic optic neuropathy associated with udenafil

We report a case of anterior ischemic optic neuropathy associated with udenafil. A 54-year-old male presented with an acute onset visual field defect of the right eye after udenafil use. Examination revealed a relative afferent pupillary defect and a swollen disc. Automated visual fields revealed an enlarged blind spot and a narrowed visual field. Fluorescein angiography revealed both an inferior choroidal filling delay and an inferior sector filling delay of the optic disc in the arteriovenous phase as well as diffuse leakage of the optic disc in the late phase. Optical coherent tomography revealed increased thickness of the retinal nerve fiber layer, especially in the area of the inferior disc. The patient was counseled to discontinue the use of udenafil and to monitor his blood pressure regularly. The disc swelling was resolved with residual optic atrophy one month after discontinuing the use of udenafil.     

Bilateral optic neuropathy associated with amiodarone therapy

Amiodarone, an antiarrhythmic agent, has been associated with mild visual loss secondary to papillopathy and papilledema. We report a patient who developed bilateral optic neuropathy 4 weeks after initiation of amiodarone therapy. Nine months later, his vision was 20/50 in the right eye and 20/200 in the left. This report provides additional evidence that amiodarone may cause toxic optic neuropathy.     

Asymptomatic optic neuropathy associated with Churg-Strauss syndrome

OBJECTIVE: To report asymptomatic optic disc edema in a patient with Churg-Strauss syndrome. DESIGN: Retrospective, observational case report. METHODS: Neuro-ophthalmic examination, sural nerve biopsy, and laboratory evaluation. MAIN OUTCOME MEASURES: Visual acuity, funduscopy, automated perimetry, and histopathology of sural nerve biopsy. CONCLUSIONS: Churg-Strauss syndrome may be associated with optic neuropathy. Careful ophthalmic evaluation may reveal signs of optic neuropathy before the development of symptomatic vision loss.     

Ischemic optic neuropathy associated with Crohn's disease

A 24-year-old man with Crohn's disease suffered bilateral ischemic optic neuropathy following the resolution of an episode of "uveitis." Additional findings of corneal infiltrates, vitritis, retinovascular sheathing, and arthritis suggest an inflammatory basis for his ischemic optic neuropathy. Vasculitis as an extraintestinal manifestation of inflammatory bowel disease is a possible etiology. To our knowledge, the association of ischemic optic neuropathy and inflammatory bowel disease has not been previously reported.     

Stickler's syndrome associated with congenital glaucoma

A case report of Stickler's syndrome associated with congenital glaucoma is presented. Stickler's syndrome is an autosomal dominant disorder characterised by progressive arthropathy, midfacial flattening, Pierre Robin anomaly or cleft palate, sensorineural hearing loss, progressive myopia, vitreoretinal degeneration, and retinal detachment. Congenital glaucoma and Stickler's syndrome are two diagnoses frequently considered in high myopia in infancy. The case report described presents a case of Stickler's syndrome in association with congenital glaucoma. This association is unusual, but important to recognise in the neonatal period. The possibility of coexistence of these clinical entities should be considered in the future.     

Oculocutaneous albinism associated with congenital glaucoma

An infant with oculocutaneous albinism and congenital glaucoma is described. The association of these two uncommon congenital disorders might suggest a common defective factor. Theoretically this patient broadens the spectrum of disorders of neural crest cell migration associated with albinism.