Optic Nerve Head Swelling Research Articles

Clinical characteristics of retinal venous occlusions occurring at different sites. Beaumont PE, Kang HK. Br J Ophthalmol 2002;86:572–580.

The authors sought to identify the contributory factors associated with different sites of retinal vein occlusion (RVO) and the presence or absence of optic nerve head swelling (ONHS). There were 874 cases of RVO prospectively examined and classified according to three defined sites of occlusion: arteriovenous crossing RVO (AV-RVO); optic cup RVO (OC-RVO); and optic nerve-sited RVO. Optic nerve-sited RVOs were further divided on the basis of presence (ONHS-RVO) and absence (NONHS-RVO) of ONHS. RVOs not occurring at any of the defined sites were grouped as no-site RVO (NS-RVO). Important clinical parameters were compared among four of the five subgroups by multivariate analysis of variance and χ2 test (NS-RVO excluded). The overall multivariate analysis of variance for differences in the mean age, systolic and diastolic blood pressure, body mass index, and intraocular pressure (IOP) among the four subgroups were highly significant. The F ratios indicated that the differences in the mean age and IOP accounted for this statistical trend. The mean age was statistically significantly lower in the ONHS-RVO group compared with the rest of the groups. The mean age was significantly higher in OC-RVO compared with the AV-RVO group. The mean IOP was significantly higher in OC-RVO than in the rest of the groups, while it was also higher in the NONHS-RVO group compared with the ONHS-RVO and AV-RVO groups. The prevalence of primary open-angle glaucoma (POAG), sex, laterality, involvement of the fellow eye, smoking and hypertension were compared by χ2 tests. POAG was significantly more prevalent in the OC-RVO group than in the rest of the groups, while it was also significantly more prevalent in the NONHS-RVO group compared with AV-RVO or ONHS-RVO groups. Smoking was significantly more prevalent in AV-RVO than in the rest of the groups. The proportion of male sex was significantly higher in ONHS-RVO compared with the AV-RVO group. Hypertension was significantly more prevalent in the AV-RVO than in the ONHS-RVO or NONHS-RVO groups. The authors conclude that the higher prevalence of hypertension and smoking in AV-RVO suggests a particular importance of cardiovascular risk factors in this group. The association of POAG with CRVO has been confirmed, but only for those cases without ONHS. A distinctive relation between raised IOP and OC-RVO has been demonstrated, suggesting a causal association. RVOs with ONHS tend to occur in younger people, with a higher proportion of males and a lower prevalence of hypertension and POAG, suggesting that other causal factors may be important in this group. The authors feel that their classification schemes resolve the confusion in the literature regarding classification of RVO and has diagnostic, causal, prognostic, and therapeutic implications.—Thomas J. Liesegang

Cup-to-disc ratio, intraocular pressure, and primary open-angle glaucoma in retinal venous occlusion

Purpose To investigate the association of cup-to-disc ratio (CDR), intraocular pressure (IOP), and primary open-angle glaucoma (POAG) with the site of retinal venous occlusion (RVO) and optic nerve head swelling (ONHS). Design Prospective observational case series. Participants Four hundred fifty consecutive cases from a single tertiary referral center. Methods Fundus photography of the retinopathy, 30° stereo photography of the optic disc, and Goldmann applanation tonometry were the main parameters studied. The diagnosis of POAG was obtained from the referring ophthalmologist. Main outcome measures The main outcome measures were the CDR, measured vertically through the center of the optic disc, and the site of RVO, identified as an arteriovenous (AV) crossing, optic cup, or optic nerve. Retinal venous occlusions occurring within the optic nerve were subdivided on the basis of the presence or absence of ONHS. Results There were poor correlations between CDR and IOP ( r = 0.18; P = 0.000209) and CDR and age ( r = 0.21; P = 0.000012). There were 197 AV crossing-sited RVOs (AV-RVOs) (43.8%) and 46 optic cup (OC)-sited RVOs (10.2%). The remainder occurred within the optic nerve; the ONHS group had 80 cases (17.8%) and the nonoptic nerve head swelling group (NONHS) had 127 cases (28.2%). The OC-RVO group tended to be the oldest of the four groups, whereas the ONHS group was the youngest ( P < 0.000001). The mean CDR was significantly higher in the OC-RVO (0.65) compared with the rest of the groups (0.45–0.48). The proportion of cases with CDR ≥ 0.7 was significantly higher in the OC-RVO group (39.1%) compared with the rest of the groups (0–6.3%). There was a trend ( P = 0.000012) for IOP in the OC-RVO group (19.0 mmHg) and NONHS group (17.6 mmHg); the proportion of cases with IOP more than 21 mmHg was also higher in these cases ( P = 0.00033). The prevalence of POAG was highest ( P < 0.000001) in the OC-RVO group (39.1%) followed by the NONHS group (18.1%), ONHS group (8.8%), and AV-RVO (4.1%) group, respectively. Conclusions Optic cup and optic nerve-sited RVO without ONHS are associated with raised IOP and may share a common management strategy aimed at controlling ocular pressure. Glaucomatous optic disc cupping, in contrast, seems to be important in the OC-sited RVO group only. Intraocular pressure, POAG, and glaucomatous optic disc cupping do not significantly seem to contribute to the development of RVO at an AV crossing or when the occlusion occurs within the optic nerve in association with ONHS.

Adenoviral gene therapy with catalase suppresses experimental optic neuritis.

To determine if adenoviral-mediated transfer of the gene for catalase (CAT), the reactive oxygen species scavenger, suppresses experimental optic neuritis. Gene therapy with CAT delivered by an adeno-associated viral vector was previously shown to suppress experimental optic neuritis. Because the transduction of protein expression with recombinant adeno-associated viral vector is relatively slow, taking weeks to reach full levels, we studied the effects of replication-deficient adenovirus containing CAT in suppressing experimental optic neuritis. Transduction with adenovirus occurs within days of inoculation, thus, it may be more applicable for the treatment of patients with acute optic neuritis. Replication-deficient adenovirus containing CAT was injected above the right optic nerve heads of SJL/J mice that were simultaneously sensitized for experimental allergic encephalomyelitis. For controls, the left eyes were injected with the replication-deficient adenovirus without CAT or no virus. The histological effects of CAT on the lesions of experimental allergic encephalomyelitis were measured by computerized analysis of the myelin sheath area (for demyelination), optic disc area (for optic nerve head swelling), the extent of the cellular infiltrate, extravasated serum albumin labeled with immunogold (for disruption of the blood-brain barrier), and the in vivo hydrogen peroxide reaction product. After 1 month, cell-specific catalase activity, evaluated by the quantitation of catalase immunogold, was increased about 2-fold each in endothelia, oligodendroglia, astrocytes, and axons of the CAT-inoculated right optic nerves compared with the control left optic nerves. The increased cellular levels of catalase reduced demyelination by 30%, optic nerve head swelling by 25%, cellular infiltration by 26%, disruption of the blood-brain barrier by 61%, and in vivo levels of hydrogen peroxide by 81%. Adenoviral-mediated gene transfer increased catalase levels in all optic nerve cell types, and it persisted for 1 month after inoculation. The increased cellular levels of catalase suppressed demyelination and blood-brain barrier disruption at the foci in the optic nerve where prior magnetic resonance imaging and histopathologic studies have demonstrated the demyelinating inflammation of experimental and human optic neuritis. Together, they suggest that gene therapy with CAT may be helpful in the treatment of patients with optic neuritis.

Adeno-associated viral-mediated catalase expression suppresses optic neuritis in experimental allergic encephalomyelitis.

Suppression of oxidative injury by viral-mediated transfer of the human catalase gene was tested in the optic nerves of animals with experimental allergic encephalomyelitis (EAE). EAE is an inflammatory autoimmune disorder of primary central nervous system demyelination that has been frequently used as an animal model for the human disease multiple sclerosis (MS). The optic nerve is a frequent site of involvement common to both EAE and MS. Recombinant adeno-associated virus containing the human gene for catalase was injected over the right optic nerve heads of SJL/J mice that were simultaneously sensitized for EAE. After 1 month, cell-specific catalase activity, evaluated by quantitation of catalase immunogold, was increased approximately 2-fold each in endothelia, oligodendroglia, astrocytes, and axons of the optic nerve. Effects of catalase on the histologic lesions of EAE were measured by computerized analysis of the myelin sheath area (for demyelination), optic disc area (for optic nerve head swelling), extent of the cellular infiltrate, extravasated serum albumin labeled by immunogold (for blood-brain barrier disruption), and in vivo H2O2 reaction product. Relative to control, contralateral optic nerves injected with the recombinant virus without a therapeutic gene, catalase gene inoculation reduced demyelination by 38%, optic nerve head swelling by 29%, cellular infiltration by 34%, disruption of the blood-brain barrier by 64%, and in vivo levels of H2O2 by 61%. Because the efficacy of potential treatments for MS are usually initially tested in the EAE animal model, this study suggests that catalase gene delivery by using viral vectors may be a therapeutic strategy for suppression of MS.

Optic Nerve Head Swelling in the Hadju-Cheney Syndrome

The Hadju-Cheney syndrome is one of the idiopathic acroosteolyses. Associated neurologic abnormalities are often a result of progressive basilar invagination. A 48-year-old man with the Hadju-Cheney syndrome developed progressive bilateral visual loss. On examination, he had hyperopia, choroidal folds, optic nerve head swelling, and mild optic neuropathy. Computed tomographic scans showed massive enlargement of both intraorbital optic nerve sheaths. Improvement occurred after optic nerve sheath fenestration. Visual loss due to optic nerve meningocele can occur in the Hadju-Cheney syndrome. Optic nerve sheath fenestration can result in visual improvement. It is unclear whether the occurrence of optic nerve meningocele is causally or fortuitously related to the Hadju-Cheney syndrome.

Favorable Visual Outcome in Cryptococcal Meningitis

To describe an unusual, favorable visual outcome in a patient with lupus erythematosus and cryptococcal meningitis and to present bilateral superior oblique muscle paresis. Case report. A 15-year-old girl with lupus erythematosus and cryptococcal meningitis had bilateral superior oblique paresis, bilateral optic nerve head swelling, and increased intracranial pressure. She developed a visual acuity of no light perception in the right eye. Treatment with oral fluconazole, acetazolamide, and dexamethasone, as well as repeated lumbar punctures to reduce intracranial pressure, was followed by recovery to a visual acuity of 20/20 in both eyes and normal ocular motility. With appropriate treatment, visual loss associated with cryptococcal meningitis may have a favorable outcome.

Ophthalmic Manifestations of Rochalimaea Species

Rochalimaea henselae and R. quintana belong to the order Rickettsiales and are thought to be responsible for trench fever, bacillary angiomatosis, and cat scratch disease. We recently examined four patients with intraocular inflammation of unknown origin. Each patient had either unilateral or bilateral moderate loss of visual acuity ranging from 20/25 to counting fingers. Bilateral intraocular inflammation manifested by anterior and posterior segment cells, retinal lesions, macular exudate, and optic nerve head swelling was present to varying degrees. The R. henselae to R. quintana antibody titers were greater than or equal to 1:256 in each case. Marked improvement in vision occurred after treatment with either oral ciprofloxacin hydrochloride and prednisone or doxycycline hyclate. Rochalimaea species should be considered in the differential diagnosis of intraocular inflammation and inflammatory optic neuropathy. Appropriate treatment may result in marked improvement in visual acuity.

Optic Nerve Granuloma as First Sign of Systemic Sarcoidosis

We report a rare casse of optic nerve involvement as the first sign of systemic sarcoidosis. A 43-year-old white woman presented with visual loss in her right eye, and the clinical appearance of optic nerve head swelling. Visual acuity was reduced to 20/200, and the only clinical signs were the presence of an enlarged optic nerve head, with engorged retinal veins and flame-shaped haemorrhages. Computed tomography scans showed an enlarged orbital portion of the right optic nerve, which was confirmed by magnetic resonance imaging. With a diagnosis of optic nerve tumour (possibly glioma) a surgical resection of the tumour was performed. Histopathology showed multiple nodular granulomas with epithelioid cells, consisted with the diagnosis of sarcoidosis. This was confirmed by systemic involvement of the parotids and liver. We review the literature and consider all possible involvement of the optic nerve in sarcoidosis. The rarity of systemic sarcoidosis with a first manifestation as papilloedema is discussed.

Optic Nerve Sheath Decompression for Glaucomatous Optic Neuropathy With Normal Intraocular Pressure

To report our therapeutic experience with optic nerve sheath decompression in patients with normal-pressure glaucoma. A case series of seven eyes from six patients with glaucoma and normal intraocular pressures who continued to have progressive visual field loss despite conventional therapy. A hospital-based, referral glaucoma service. Three men (67, 67, and 72 years of age) and three women (58, 61, and 70 years of age). Optic nerve sheath decompression. Visual field data and visual acuity measurements were obtained at regular intervals during the postoperative periods (range, 3 to 18 months). Two of seven eyes from six patients appear to have enjoyed an initial significant improvement in their visual fields with improved visual acuity in one eye of one patient. The visual fields, however, appear to have deteriorated 18 months after the initial procedure in these two patients. In the remaining four patients, no further improvement or deterioration was observed within a limited follow-up period. The transient improvement in the visual fields of one eye from each of two patients documents an initial successful use of optic nerve sheath decompression in patients with nerve fiber bundle damage in the absence of optic nerve head swelling. However, the long-term potential of optic nerve sheath decompression in these patients may be of limited value.

Optic Nerve Head Swelling and Optic Atrophy in the Systemic Mucopolysaccharides

The ocular findings in 108 patients representative of all types of mucopolysaccharidoses (MPS) were reviewed. Attention was focused on optic nerve head appearance. Optic nerve head swelling was observed in 8/14 eyes of MPS I-Hurler (MPS I-H); 9/21 MPS I-Hurler-Scheie (MPS I-H-S); 0/4 MPS I-S; 13/66 MPS II; 1/22 MPS III; 0/58 MPS IV; 5/12 MPS VI; and 2/2 MPS VII eyes. In three patients, optic nerve head swelling was present in one eye and optic atrophy in the fellow eye; in four patients, optic atrophy followed disc elevation; three patients presented with bilateral optic atrophy. It was concluded that optic nerve head swelling precedes the development of optic atrophy in the systemic MPS.

Spatial contrast sensitivity impairment in optic nerve head swelling

Papilledema uncomplicated by ischemic or atrophic changes, or by a macular edema, produces only enlarged blind spots and no other changes in standard clinical tests. Thirty-three patients with simple papilledema (two with obstructive hydrocephalus, 31 with pseudotumor cerebri) were tested for spatial contrast sensitivity. Seventy percent showed sensitivity losses only at low spatial frequencies. Five patients with unilateral optic glioma (asymptomatic) and disc edema produced the same loss pattern.Findings suggest that edema of the optic nerve compromises ganglion cells with large caliber axons in the retina (large receptive fields), sparing other axons whose function is revealed by standard clinical tests. Spatial contrast sensitivity testing detects silent, asymptomatic visual loss in optic nerve edema and compression.

Bilateral Optic Neuropathy and Osteolytic Sinusitis

COCAINE is a naturally occurring alkaloid of the coca plant Erythroxylon coca . Its psychopharmacological properties are due to stimulation of the central and sympathetic nervous systems by augmentation of the normal effects of the neurotransmitter norepinephrine. The Incas chewed coca leaves prior to 1000ADto satisfy the hunger, provide the weary and fainting with new vigor and cause the unhappy to forget their miseries. 1 Recent increases in the production, trafficking, and use of cocaine have brought concomitant increases in associated medical problems. 2,3 The usual routes of illicit cocaine use are intranasal, intravenous, and inhalational. Each has unique medical hazards. 4,5 We describe a patient with unusual ophthalmic and otolaryngologic complications related to long-term intranasal abuse of cocaine. Report of a Case A 43-year-old man was referred to the Neuro-ophthalmology Service with bilateral decreased visual acuity and asymmetric optic nerve head swelling. The patient had noticed progressive

Ophthalmologic Findings in Mucolipidosis III (Pseudo-Hurler Polydystrophy)

Of eight patients with mucolipidosis III, hyperopic astigmatism, and corneal clouding, five had retinal and optic nerve abnormalities. Two patients had surface wrinkling maculopathy, four patients had various degrees of optic nerve head swelling, and two patients had retinal vascular tortuosity. Electrophysiologic studies of the retina gave normal results in the four patients tested. Visual field defects were present in three of the four patients examined. Color vision was normal in three of the patients. Visual acuity remained unchanged in the seven patients clinically followed from one to 11 years (average, six years).

Acute Ischemic Optic Neuropathy

Acute anterior ischemic optic neuropathy (AION) is a common cause of profound visual loss in the elderly and the not-so-elderly and is often associated with systemic disorders. Even so, this condition is not frequently discussed in the medical literature outside ophthalmology specialty journals. For these reasons it is appropriate to call readers' attention to an article that appears in the August issue of Archives OF Ophthalmology in which Guyer et al 1 have analyzed the incidence of cerebrovascular and cardiovascular events in patients with ischemic optic neuropathy. As the name implies, acute anterior ischemic optic neuropathy is defined as an infarct of the optic nerve, usually at the level of the optic disc. This results in abrupt visual loss in one eye and swelling of the optic nerve head. 2 The area of ischemic damage is limited to a small region within the optic nerve, so there is generally no

A DYNAMIC VIEW OF THE RETINAL GANGLION CELL AND ITS TRANSPORT

Material, such as proteins and organelles are supplied continuously from the cell body of the retinal ganglion cell to its axon and nerve terminals under normal conditions. During the last decade a picture of the dynamic transport of proteins and organelles within the retinal ganglion cell has evolved. Due to the dependence of the axon and its terminals on the integrity of these transport systems a complete prolonged transport blockade leads to axon degeneration. The role of axonal transport disturbances in the pathophysiology of axon dysfunction and degeneration in the retina and optic nerve has therefore been studied experimentally in a variety of pathological conditions. This short review discusses recent data and theories concerning the implications of axonal transport studies in ophthalmology. The pattern of axonal transport block at the optic nerve head in experimental intraocular hypertension support the view that mechanical compression at this site is a probable mechanism of optic nerve damage. Transport obstruction within the axons of primarily the slow phase seems to be a major factor in the production of the swollen axon in optic nerve head swelling. Although studies of axonal transport are virtually impossible in man, signs of dysfunction of axonal transport can be observed in the fundus of the human eye by ophthalmoscopy in various diseases.

Periodic Disease

We described 3 types of folds in the retina and a crease in the outer retina associated with papilledema owing to idiopathic intracranial hypertension (IIH) at presentation. We report the change in folds relative to treatment of IIH over the 6 months.In this substudy of a randomized clinical trial, study eyes of subjects assigned to acetazolamide (ACZ, n = 44) or placebo (PLB, n = 43) had spectral-domain optical coherence tomography (SDOCT) images of the optic disc and macula regions at baseline and at 3 and 6 months. Images were evaluated for peripapillary wrinkles (PPW), retinal folds (RF), choroidal folds (CF), and creases using transaxial and en face views. The optic nerve head (ONH) shape, retinal nerve fiber layer (RNFL) thickness, ONH volume, and papilledema grade were measured. Outcome was determination of the presence or absence of PPW, RF, CF, and creases.At presentation, except for an increase of PPW in ACZ eyes (64% vs 28%), both treatment groups were matched for all OCT features. At 6 months, ACZ-treated, but not PLB-treated, eyes had fewer folds of all types (P < .01), with a 57% reduction in frequency of RF. Creases did not resolve. Resolution of RF, but not of PPW and CF, was associated with significant reduction in RNFL thickness, ONH volume, and papilledema grade.The various types of retinal folds associated with papilledema reflect biodynamic processes and show an ACZ treatment effect. Persistence of these folds despite marked improvement in ONH swelling suggests permanent changes in the affected retinal tissues.