Optic disc pit combining with an optic nerve cyst — A case report

Herein, we report a case presenting with the optic disc pit and optic nerve cyst coexistence. A 54-year-old female presented to our office with progressive blurred vision for up to 3 months in her left eye. Upon examination, the best corrected visual acuity in the right eye was 20/20 and in the left eye was 20/100. Fundoscopic examination revealed a black pit at the temporal margin of left optic disc without obvious macular edema. Findings were confirmed later with optical coherence tomography (OCT) examination. Further magnetic resonance imaging (MRI) disclosed a well defined 6 mm x 6mm cystic lesion located at temporal aspect of left retro-bulbar optic nerve within the optic nerve sheath. Compression of left optic nerve was prominent. Visual evoked potential (VEP) test verified dysfunction of left optic nerve. She kept following up for 6 months. Neither maculopathy nor retinal edema occurred during this period. The visual acuity maintained 20/100 in her left eye. The size and location of optic disc pit remained as well. To sum up, in a patient with visual impairment combing with optic disc pit, further imaging surveys should be considered to exclude the possible coexistence of other optic nerve abnormalities.     

Role for surgery as adjuvant therapy in optic nerve sheath meningioma

PURPOSE: To describe a role for optic nerve decompression as adjuvant surgical therapy in the management of optic nerve sheath meningioma in patients with severe, progressive visual loss and optic disc edema before or after radiation therapy. METHODS: Interventional case report. RESULTS: Two patients with unilateral optic nerve sheath meningioma had progressive visual loss (20/200 and no light perception) and disc edema. The first had previously undergone fractionated stereotactic radiotherapy and the second subsequently was treated with fractionated stereotactic radiotherapy after decompression surgery. After excision of a dural window and biopsy of the tumor from the nerve sheath, visual acuity improved to 20/25 and 20/200, respectively, both coinciding with resolution of disc edema. CONCLUSIONS: In patients with optic nerve sheath meningioma with severe disc edema and rapid vision loss, surgery may serve an important but restricted, adjuvant role to radiation therapy under special conditions.     

Consequence of perforation during peribulbar anesthesia in an only eye

A patient with a blind fellow eye had cataract surgery in the right eye; anesthesia comprised an intraocular injection of lidocaine and bupivacaine. Forty-eight hours after surgery, visual acuity in the right eye was light perception (LP). Three days later, fundus examination showed inferotemporal hemorrhage, retinal whitening consistent with needle tracking, and a diffusely pale optic disc in the operated eye. Computed tomography showed an intact optic nerve in both eyes and high-density vitreal lesions in the right eye. Laser photocoagulation was applied to the retinal break. We believe that a jet stream of anesthetic agent may have transiently increased intraocular volume enough to occlude the central retinal artery. Although the retina remained attached, visual acuity failed to improve beyond LP.     

Diagnosis of optic nerve sheath meningioma during optic nerve sheath decompression

Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.     

Unilateral papilledema revealing an intracanalicular optic nerve sheath meningioma

A 43-year-old female is referred to our clinic for evaluation of an incidentally discovered unilateral papilledema in her right eye of unknown cause. She had no past ocular or medical history. Examination revealed a corrected-distance visual acuity of 20/20 in both eyes. Posterior segment examination showed an elevated right optic nerve head with blurred edges. Ultrasonography failed to reveal optic disc drusen. Visual field testing showed enlargement of the blind spot in the right eye. Magnetic resonance imaging of the brain and orbit was done and revealed enlargement of the intracanalicular and prechiasmatic part of the right optic nerve and a diagnosis of a presumed right intracanalicular optic nerve sheath meningioma was made. She was managed conservatively and several years later remains asymptomatic.     

Bilateral nonarteritic anterior ischemic neuropathy following acute angle-closure glaucoma in a patient with iridoschisis: case report

A 55-year-old woman was referred to our clinic because of a one-week history of visual loss and raised intraocular pressure in the left eye followed 4 days later by visual loss in the right eye. Slit-lamp examination showed bilateral conjunctival hyperemia, slight diffuse corneal edema, shallow anterior chamber and fixed and dilated pupil in both eyes. Splitting of the anterior layers of the iris with fibrillar degeneration extending for approximately one quadrant inferiorly was presented in each eye. Fundus examination showed optic disc edema with no vascular tortuosity and no cup in both eyes. The condition was treated as bilateral acute angle-closure glaucoma in a patient with irisdoschisis. After medical treatment and improvement of visual acuity, perimetry revealed a significant visual field defect especially in left eye; this case represents a rare concurrence of acute angle-closure glaucoma and bilateral nonarteritic ischemic optic neuropathy. Although most cases of elevated intraocular pressure, including acute angle-closure glaucoma, do not result in optic disc edema and irreversible vision loss, variations in the vascular supply of the nerve optic head along with others ocular systemic risk factors, may predispose certain individuals to nonarteritic ischemic optic neuropathy during periods of elevated intraocular pressure.     

Clinical and computed tomographic findings in the Foster Kennedy syndrome

A 52-year-old man had progressive loss of visual acuity in his left eye associated with anosmia of five years duration. Clinical findings included papilledema in the right eye and optic atrophy in the left eye. A diagnosis of Foster Kennedy syndrome was made. Careful attention to the ophthalmoscopic appearance of the left eye disclosed optic disk swelling in regions without significant atrophy as well as dilated retinal veins. Both of these signs suggested increased intracranial pressure, rather than a primary anterior ischemic optic neuropathy. High resolution computed tomographic scanning confirmed the presence of a large subfrontal meningioma and an expanded right optic nerve sheath, consistent with the papilledema noted clinically.     

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.     

A case of optic nerve atrophy with severe disc cupping after methanol poisoning

We report a rare case of optic nerve atrophy with severe disc cupping resulting from methanol poisoning. A 30-year-old man presented to the hospital complaining of decreased visual acuity in both eyes a day after drinking alcohol containing methanol. His initial visual acuity allowed for only visualizing hand motion and not corrected in either eye. Initial intraocular pressure was within normal limits in both eyes. Initial fundus examination showed optic disc swelling in both eyes. Four years later, he visited our hospital for an eye evaluation. Visual acuity in both eyes still only allowed for visualizing hand motion. No nystagmus was observed in either eye during the optokinetic nystagmus test, and no waves were found in a visual evoked potential test. No specific change was noted on brain magnetic resonance imaging. On fundus examination, there was disc pallor in both eyes and disc cupping with a high cup/disc (C/D) ratio above 0.9 in the left eye. C/D ratio of the right eye was 0.5. Methanol poisoning may induce glaucomatous disc cupping in the late stage as well as optic atrophy. One possible mechanism of disc cupping is ganglion cell loss due to acute demyelination of the retrobulbar optic nerve. This report is the first photographic evidence of methanol induced optic disc cupping in Korea.     

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.     

Enlargement of the optic disk in childhood optic nerve tumors

Two children, a 9-year-old girl and an 8-month-old girl, were first examined because of unilateral proptosis. Both children were found to have unilateral optic nerve glioma, accompanied in the second case by neurofibromatosis. Two years after Patient 1 was first examined, the initial findings of thin vertical retinal striae and a gray opacification of the peripapillary retina had resolved. The enlarged left optic disk had an average diameter of 2 mm while that of the normal right optic disk was 1.6 mm. The disk-arteriolar ratio was R.E.:16:1 and L.E.: 20:1. Four years later, the patient's visual acuity was still 20/20 in both eyes and the only visual field abnormality was an enlarged blind spot corresponding to the enlarged left optic nerve head. Fundus photographs, orbital echography, and computed tomography showed no change in the size or location of the optic nerve glioma during the six-year follow-up period. In Case 2, the initial intraocular pressure was higher in the proptotic right eye than in the left eye (25 vs 19 mm Hg), but subsequent intraocular pressures were within normal limits and approximately equal in the two eyes. The disk-arteriolar ratio was R.E.:21:1 and L.E.: 18:1 and the cup-disk ratio was R.E.: 0.7 and L.E.: 0.3. Computed tomography disclosed an enlarged right retrobulbar optic nerve, a superior orbital fissure that was much larger on the right than on the left, and a larger cavernous sinus on the right. The right sphenoid bone was partially absent. Despite mild developmental delay, the patient's visual acuity apparently remained normal and the ophthalmologic findings did not change significantly during the 18-month follow-up period.     

Optic disc pit with peripapillary retinoschisis presenting as a localized retinal nerve fiber layer defect

A 59-year-old woman was referred to our clinic for a glaucoma evaluation. The visual acuity and intraocular pressure were normal in both eyes. However, red-free fundus photography in the left eye showed a superotemporal wedge-shaped retinal nerve fiber layer defect, and visual field testing showed a corresponding partial arcuate scotoma. In an optical coherence tomography examination, the macula was flat, but an arcuate-shaped peripapillary retinoschisis was found. Further, the retinoschisis seemed to be connected with a superotemporal optic pit shown in a disc photograph. After 3 months of a topical prostaglandin analogue medication, the intraocular pressure in the retinoschisis eye was lowered from 14 to 10 mmHg and the peripapillary retinoschisis was almost resolved. We report a rare case of an optic disc pit with peripapillary retinoschisis presenting as a localized retinal nerve fiber layer defect.     

A case of bilateral uveitis and optic disc swelling with Chiari I malformation

We report a case of bilateral uveitis and optic disc swelling with Chiari I malformation. A 16-year-old girl was admitted to our clinic due to conjunctival hyperaemia and blurred vision in her right eye. Ophthalmologic and systemic examinations were performed. Visual acuity was 0.7 (OD) and 1.0 (OS). Bilateral optic disc swelling was observed. Fluorescein angiography demonstrated bilateral retinal vasculitis as well as optic disc hyperflourescence due to leakage. Laboratory examinations were within normal limits. Cranial magnetic resonance venography imaging revealed neither cranial mass nor cerebral venous thrombosis but a Chiari I malformation. The patient was started oral cetazolamid, topical and oral corticosteroids. After six months follow-up, bilateral optic disc swelling was resolved completely and visual acuity was 1.0 in both eyes. Optic disc swelling may be associated with intraocular inflammation; however, patients with bilateral optic disc swelling should be suspected of having an accompanying intracranial pathology.     

Suprasellar germinoma and late perioptic seeding

PURPOSE: To report an unusual case of optic nerve seeding 12 years following treatment of a suprasellar germinoma. METHODS: Observational case report. RESULTS: A 34-year-old woman presented with a 3-week history of subjective right eye visual loss. She had been diagnosed with a suprasellar germinoma at 22 years of age, which had been partially excised and radiated (5400 cGy). The tumor had shown complete radiographic regression without neurologic sequelae. Pertinent findings on current examination included right eye visual acuity of 20/150, right relative afferent papillary defect, and optic nerve pallor in the right eye. In addition, partial left facial paralysis was noted. Examination was otherwise unremarkable including 20/20 acuity in the left eye. Magnetic resonance imaging demonstrated abnormal enhancement and thickening of both optic nerves and along the course of the left V and VII cranial nerves. Serum levels of alpha-fetoprotein and beta-HCG were abnormal, consistent with metastatic germinoma. Following two cycles of chemotherapy (VIP-etoposide, ifosfamide, and cisplatin), visual acuity returned to 20/20 bilaterally, with corresponding radiographic improvement and normal cerebrospinal fluid cytology. CONCLUSIONS: Perioptic subarachnoid seeding may occur over a decade after presumed successful treatment of germinomas, suggesting the importance of lifelong observation.     

Lack of Correspondence between Early Optic Disc Oedema and Macular Ganglion Cell Loss in Non-arteritic Anterior Ischaemic Optic Neuropathy

A 73-year-old Asian male presented with a 2-week history of acute visual disturbance in his right eye. Visual acuity in his right eye was hand movement and fundus examination showed right optic disc oedema and splinter haemorrhages on the disc margin. Each time the patient visited our clinic, spectral domain optical coherence tomography (OCT) was performed. He was diagnosed to have non-arteritic anterior ischaemic optic neuropathy. Optical coherence tomography showed disagreement between the areas of early peripapillary retinal nerve fibre layer thickening and macular ganglion cell layer thickness reduction.     

Bilateral Glaucomatous Optic Neuropathy Caused by Eye Rubbing

In this report, we describe a particular condition of a 52-year-old man who showed advanced bilateral glaucomatous-like optic disc damage, even though the intraocular pressure resulted normal during all examinations performed. Visual field test, steady-state pattern electroretinogram, retinal nerve fiber layer and retinal tomographic evaluations were performed to evaluate the optic disc damage. Over a 4-year observational period, his visual acuity decreased to 12/20 in the right eye and counting fingers in the left eye. Visual fields were severely compromised, and intraocular pressure values were not superior to 14 mm Hg during routine examinations. An accurate anamnesis and the suspicion of this disease represent a crucial aspect to establish the correct diagnosis. In fact, our patient strongly rubbed his eyes for more than 10 h per day. Recurrent and continuous eye rubbing can induce progressive optic neuropathy, causing severe visual field damage similar to the pathology of advanced glaucoma.Key Words: Advanced glaucoma, Eye rubbing, Optic neuropathy     

Laser in situ keratomileusis-induced optic neuropathy

OBJECTIVE: To report a case of bilateral optic neuropathy after bilateral laser-assisted in situ keratomileusis (LASIK) surgery. DESIGN: Observational case report. METHODS: Complete eye examination with detailed evaluation of the optic nerve, detailed medical history, stereo disc photographs, GDx Nerve Fiber Analyzer testing, Humphrey 24-2 SITA visual field testing, diurnal intraocular pressure measurement, serologic evaluation, and magnetic resonance imaging of the brain and orbits. MAIN OUTCOME MEASURES: Optic nerve status, visual field status, and visual acuity. RESULTS: A subject with previously healthy optic nerves had bilateral optic neuropathy develop after LASIK surgery. This neuropathy manifested with a subjective decrease in visual field, normal visual acuity, normal color vision, relative afferent pupillary defect, increased cupping of the optic nerve with focal neuroretinal rim defects, decreased nerve fiber layer thickness, and nerve fiber bundle-type visual field defects. The subject had no other risk factors for optic neuropathy. No other cause of neuropathy was identified. CONCLUSIONS: Optic neuropathy is a potential vision-threatening complication of LASIK surgery. This complication may be due to barotrauma or ischemia related to extreme elevation of intraocular pressure by the suction ring. Careful examination of the optic nerve before and after LASIK surgery is warranted.     

Unilateral papilledema after trabeculectomy in a patient with intracranial hypertension

BACKGROUND: Increased intracranial pressure usually leads to bilateral disc swelling. HISTORY AND SIGNS: A patient presented with recurrent visual disturbances following trabeculectomy in the right eye. Intraocular pressure in the right and left eye were 11 and 24 mmHg, respectively. The optic nerve head was swollen in the right, but not in the left eye. Lumbar puncture showed an opening pressure of 32 cmH (2)O. Magnetic resonance imaging, neurological examination and composition of cerebrospinal fluid were normal. According to the modified Dandy criteria, an idiopathic intracranial hypertension was diagnosed. THERAPY AND OUTCOME: Treatment with acetazolamide led to resolution of papilledema in the right eye within six months. CONCLUSION: The intracranial-intraocular pressure gradient in the right eye was markedly higher as compared to that of the left eye. We suggest that this pressure gradient induced the collapse of axoplasmatic transport at the lamina cribrosa with subsequent disc swelling. As no significant pressure gradient was present in the left eye, the optic disc remained normal. Based on analogous calculations in three additional published cases of unilateral papilledema we thus suggest that intraocular pressure should be taken into account when evaluating patients with papilledema.     

Severe Optic Disc Edema Without Hydrocephalus in Neurofibromatosis 2

A 26-year-old man who had neurofibromatosis type-2 with symptoms of unexplained optic disc edema is reported. Magnetic resonance imaging (MRI) revealed bilateral acoustic schwannomas. Obstructive hydrocephalus, however, was not evident in spite of his severe disc edema and visual loss. After partial removal of the right acoustic schwannoma, symptoms of intracranial hypertension, such as vomiting and headache, developed and MRI demonstrated evidence of obstructive hydrocephalus. Placement of a ventricular-peritoneal shunt relieved the symptoms of intracranial hypertension, but visual acuity in his left eye was reduced to hand motion due to secondary optic atrophy. In patients with similar symptoms it is suggested that, in addition to tumor removal, early treatment to decrease intracranial pressure should be considered when visual function is progressively impaired by the symptoms of prolonged papilledema.     

Melanocytoma of the left optic nerve head and right retrobulbar optic neuropathy compressed by a tuberculum sellae meningioma

A 40-year-old woman had a highly pigmented, slightly elevated tumor on the left optic disc. She had no visual disturbance in the left eye. The tumor was stationary for 5 years. At age 45 years, she complained of decreased visual acuity in the right eye. Magnetic resonance imaging showed a right-shifted homogeneous lesion at the tuberculum sellae. Histopathologic study of the excised lesion revealed interlacing bundles of spindle-shaped fibroblast-like cells with whorl formation. We believe that a relationship between melanocytoma of the optic nerve head and a tuberculum sellae meningioma may exist rather than a chance occurrence, as previously suggested by others.