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Decompression of the perioptic meninges for intractable chronic papilledema was done in a patient with a right parietal temporal glioblastoma multiforme. The patient died 39 days postoperatively. Histologic study of the optic nerves indicated fistulas in the dura compatible with cerebrospinal fluid (CSF) egress and maintenance of a normal subarachnoid space around the nerve. Two additional patients with unilateral optic nerve decompression producing bilateral resolution of papilledema were studied. We contend that egress of CSF was the principle mode of action in these three cases. How long the dural fistula remains patent is unknown. Reports in the literature show considerable variation in the effects of optic nerve decompression. Anatomic variation of the intracanalicular subarachnoid space together with differences in underlying pathologic condition, surgical technique, and patient response may explain discrepancies among the results reported.

Blind spot enlargement in papilledema has been attributed to either mechanical disruption of the integrity of the peripapillary percipient elements by the swollen optic disk or to the Stiles-Crawford effect. We investigated the possibility that blind spot enlargement in papilledema is caused, at least in part, by a refractive scotoma due to peripapillary hyperopia. We reduced the enlarged blind spot in a patient with focal peripapillary hyperopia, without papilledema, to near normal size by using progressively stronger plus lenses. Similarly, with the addition of plus sphere, we reduced the size of the blind spot in five of six patients with papilledema, but in none of our normal subjects.

An analysis of visual field findings in the material of 200 patients with pseudopapilloedema with and without verified optic disc drusen was performed. Goldmann and/or Friedmann visual fields were examined in 383 eyes. Visual field defects were found in 38.9% of the pseudopapilloedema eyes without verified drusen, but in 73.4% of the drusen eyes. The strongest field defects were found only in the eyes with drusen seen by oblique illumination or with superficial drusen. The eyes with superficial drusen showed visual field defects in 85.8%. Only 7 patients complained of visual field defects. A progression of visual field defects could be documented in 22% of 118 patients with visual field examinations 2 or more times.

A detailed review of optic nerve axoplasm is presented. A number of hypotheses have been postulated for the pathogenesis of papilledema associated with increased intracranial pressure. These hypotheses, mechanical and nonmechanical, are critically evaluated in relation to five essential features of papilledema. Theories, as well as clinical and experimental studies, of axonal transport are reviewed, and a new hypothesis is proposed: Papilledema is primarily a mechanical, nonvascular phenomenon in which an excess amount of extracellular fluid is present in the prelaminar region of the optic disc and the accumulation of that fluid results from the leakage of axoplasm from optic nerve fibers which are compressed posterior to the lamina cribrosa of the optic disc. The authors believe that this is the only existing hypothesis consistent with all the known facts about papilledema. Discussions by Drs. J. Terry Ernest, Thomas R. Hedges, and S. S. Hayreh follow the review.

Intrapapillary refractile bodies were observed in seven of 13 patients with chronic optic disc edema secondary to optic nerve sheath meningioma. The observation of refractile bodies preceded or coincided with decline in vision and disappeared as optic atrophy supervened. Intrapapillary refractile bodies appear to be a nonspecific sign of chronic optic disc edema.

Using Littmann's method for correcting the magnification of central fundus photographs, we determined the absolute size of 35 unselected optic discs with pseudopapilledema. The optic disc area (1.95 +/- 0.33 mm2 (mean and SD)) was significantly (P less than 0.001, Student's t-test ) smaller than standard values of normal unselected optic nerve heads (2.73 +/- 0.76 mm2). There was no cupping in the discs with pseudopapilledema. Coefficients of variation for intraobserver re-evaluation were 0.045, and for interobserver re-evaluation 0.08. The markedly reduced optic disc size coincide with an abnormally small optic nerve scleral canal may inhibit the intraaxoplasmatic flow with secondary swelling of the juxtapapillary optic nerve fibers. This may lead t o prominence and indistinct borders of the optic disc. Pseudopapilledema might be related to optic nerve head drusen, which are also associated with abnormally small optic discs.

We have quantified retinal edema in rat by specific gravity measurements in vitro. Specific gravity corresponds to tissue water content, which is increased in retinal edema. The gravimetric analysis of retinal edema corresponds well with light and transmission electron microscopy and radiolabelled albumen studies. Specific gravity measurements are the standard method to measure brain edema and are also applicable to the measurement of retinal edema.

The sheath of the optic nerve was studied in rhesus monkeys, humans, and rabbits. The sheath (i.e., dura mater and arachnoid mater) was normally found to be loose near the eyeball, with a much bigger space between the optic nerve and the sheath than anywhere else in its course, consequently presenting bulbous appearance just behind the eyeball. The space was narrowest in the region of the optic canal, with thick fibrous bands stretching from the dura mater to the pia mater of the optic nerve-so that the space assumed the character of a trabecular meshwork of closely knit fibers in the canal, with the subarachnoid and subdural spaces reduced to almost a capillary size in this region. Communication between the subarachnoid spaces of the cranial cavity and of the sheath was almost always seen; however, the extent of communication in the optic canal showed wide variation from one specimen to another. The significance of our findings in the interpretation of some clinical and pathological findings and processes is discussed.

A case of posttraumatic unilateral enlargement of the optic nerve sheath associated with a venous obstructive retinopathy and progressive visual loss responded with marked improvement in visual function after nerve sheath decompression. Indirect (closed-head) trauma may be related to the progressive nerve sheath enlargement, venous obstructive retinopathy, and progressive visual loss. It is apparent that a venous stasis retinopathy can result from a posttraumatic optic nerve sheath lesion. Prompt recognition by echographic and/or roentgenographic investigation and subsequent decompression may be curative.

Six months after cataract extraction, a 71-year-old man had cystoid macular edema confirmed by fluorescein angiography. Microscopic examination revealed cystoid spaces in the outer plexiform and outer nuclear layer of the neurosensory retina. Chronic inflammatory cells were scattered throughout the ciliary body. Inflammatory cells were also present in a section of iris that had an adherent strand of vitreous leading to the pars plana. Comparison of these findings with other related clinical entities supported the hypothesis that ciliary body inflammation may play a significant role in postcataract extraction macular edema.

Four unusual patients had bilateral anterior ischemic optic neuropathy (AION). In all four cases, AION developed in the first eye with the classic presentation. The fellow eye had symptomless optic disc edema (ODE) with no subjective or objective visual loss initially, but the classic AION clinical picture developed later on. The findings indicate that symptomless ODE may precede the visual loss in AION and could constitute the earliest sign of this disease. Since ODE in AION is due to axoplasmic flow stasis that, by itself, does not produce visual loss (this is produced by disruption of visual impulse transmission), this would suggest that mild optic nerve head ischemia interferes with axoplasmic flow without disrupting the visual impulse; however, more severe ischemia would disrupt both. Possible effects of various grades of acute optic nerve head ischemia are discussed.

We reviewed the clinical features, natural history and visual prognosis of 26 patients with histologically confirmed sarcoidosis. Compatible chest x-ray changes were found in 75% of patients. Periphlebitis was the commonest fundus feature. Disc changes were seen in a substantial number of patients, and the condition can present as unilateral disc oedema. The disease seems primarily to involve equatorial retinal veins, and occlusions of a hemisphere branch vein or central retinal vein did not occur. Changes in the subretinal pigment epithelium were noted in a substantial number of patients but did not produce visual morbidity, and these patients seemed to have less florid periphlebitis than others. The disease has a low visual morbidity unless neovascularisation develops; the treatment of this remains controversial.

The ocular morphological findings of three methyl alcohol-intoxicated rhesus monkeys with optic disc swelling was investigated with light and electron microscopy in conjunction with intravascular horse radish peroxidase. Alterations observed in the optic nerve head were confined to the axons and consisted of swelling and clustering of the mitochondria, disruption of the neurotubules, the formation of vesicles, and enlargement of the axon segments in the prelaminar region. Swelling of the oligodendroglial cytoplasm in contact with the axons and of the astrocytes was seen in the retrolaminar optic nerve and the intraorbital optic nerve. Alterations were not observed in the retina. It is hypothesized that the alterations in the axons are the result of disrupted axoplasmic flow. Possible mechanisms relating methyl alcohol intoxication to disruption of axoplasmic flow are discussed.

Immune intraocular inflammation was induced in primates with homologous retinal outer segments. Perivascular retinitis, uveitis, and edema of the optic nerve head were the prominent acute clinical features. Acute and chronic inflammation was seen in the central and peripheral retinal vessels and granulomatous infiltration was present in the choroid of the uvea. Selective degeneration of the outer segments of the photoreceptor cells of the retina with sparing of the inner segments and of the adjacent pigment epithelium was confirmed by light and electron microscopy. This study strongly suggests that photoreceptor outer segments are highly and specifically immunogenic. The inciting antigen has yet to be identified. Implication of rhodopsin will have to await further studies since it exists as the essential protein in the outer segment.

Twenty-eight patients with pseudotumor cerebri underwent 40 optic nerve sheath fenestrations for relief of visual loss or to preserve vision. Twenty women and eight men underwent 16 unilateral fenestrations and 12 bilateral operations. Papilledema disappeared or was strikingly reduced in 24 of 28 patients. The other four patients had gliotic discs (two patients) or were followed up for only a short time. Visual acuity improved in 12 of 40 eyes and remained the same in 22 of 40 eyes. Seventeen eyes had preoperative visual acuity of 20/30 or better. In six eyes visual acuity decreased. Of eight eyes operated on that had visual acuity of 20/200 or worse, only three showed improvement. Visual fields improved in 21 of 40 eyes and remained the same in ten eyes. Five of the ten eyes that did not change had poor vision before surgery. Eight eyes in five patients continued to lose acuity postoperatively. Each of these eight eyes had a concomitant loss of visual field. An additional two eyes developed visual field loss with preserved visual acuity. The indications for surgery are early evidence of progressive loss of visual field or acuity in a patient with pseudotumor cerebri. Severe vision loss presents little opportunity for improvement but fenestration may be used in a last effort to preserve or restore vision.

We examined 160 patients with pseudopapilledema caused by deep and hidden drusen. The advantages of ophthalmochromoscopy versus fluorescein angiography in diagnosing deep drusen are demonstrated. The role of fluorescein examination in differentiating pseudopapilledema from genuine papilledema is pointed out. After many years of observation of patients suffering from drusen and of their relatives, and by studying the histologic data, the dynamics of the process were revealed. A classification of drusen according to depth of location is suggested. Hidden, deep and amputated drusen are distinguished. The conclusion is that deep and hidden drusen are the most frequent cause of pseudopapilledema.

A clinical analysis of 200 patients (307 eyes with optic disc drusen, 40 eyes with pseudopapilloedema without verified drusen, 53 normal fellow-eyes) was performed to document the characteristic findings in pseudopapilloedema with and without verified drusen. In 4 eyes optic disc drusen accounted for diminished visual acuity. Papillary or peripapillary haemorrhages were found in 6.1% of 347 eyes. Peripapillary pigment epithelial derangement was present in 29.7% of the eyes, mostly with superficial drusen. Subjective symptoms caused by drusen were very rare. Changes in the appearance of the optic discs with drusen during the years could be documented in 15% of 153 patients. There were 5 patients with pigmentary retinopathy. The most usual of coincident disorders was migraine in 21 patients. The visual field findings are analysed in a companion report.

Two adult diabetic patients with chronic asymptomatic optic neuropathy attributed to an ischemic etiology are reported. In one case the typical syndrome of ischemic optic neuropathy occurred in one eye, while the fellow eye had asymptomatic hyperemic optic disc edema that persisted for 6 months without optic atrophy. A minor visual field defect initially detected in that eye resolved spontaneously in 1 month. In the second case, a recent onset, middle-aged diabetic developed bilateral optic neuropathy and optic disc edema that persisted for 12 months, with minimal signs of visual dysfunction. Axoplasmic transport blockage from low-grade ischemia to the optic nerve may cause acute or chronic optic disc edema with minimal or no visual symptoms.

An animal model has been developed in this study to produce commotio retinae (Berlin's edema) by means of standardized, non-penetrating B.B. pistol injury to the cornea. Parameters have been established to ensure uniform blunt injury, enabling study of the retina and blood-retinal barrier by light- and electron microscopy, including use of horseradish peroxidase as a vascular tracer. Retinal whitening and swelling were found in the peripapillary and central regions, as occurs in the human. The earliest damage involved breakage of the connecting cilia of rods and cones, with rapid disorganization of the outer segments. Later changes included swelling of photoreceptor inner segments and breakdown of the outer blood-retinal barrier at the level of the retinal pigment epithelium. The outer blood-retinal barrier was re-established between 7 and 14 days, and at the longest survival (56 days), incompletely regenerated outer segments were present. This model for commotio retinae provides a new approach for the study of outer-segment regeneration in rods and cones after mechanical injury, as well as mechanisms that underlie re-establishment of the blood-retinal barrier following non-penetrating trauma to the eye.

The distinction between true papilledema and pseudopapilledema rests on characteristics of the optic disc when examined ophthalmoscopically. Buried disc drusen frequently simulate papilledema and often result in misdirected diagnostic maneuvers in search of a cause for presumed intracranial hypertension. When an elevated optic disc exhibits an irregular, "lumpy, bumpy" border, a diagnosis of buried drusen of the optic nerve is usually made. We report a case with papilledema secondary to increased intracranial pressure in which the margins of the swollen optic disc presented this lumpy, bumpy border characteristic of buried drusen. The lumpy character of the disc border disappeared with resolution of the papilledema, and ultrasonography demonstrated the absence of any buried drusen. Other characteristics of papilledema, including extension of the disc swelling into the peripapillary nerve fiber layer, telangiectasia of the superficial vessels of the optic disc, and obscuration of the retinal vessels as they crossed the margins of the optic disc, provided strong evidence of true papilledema and remain the most reliable findings allowing a distinction between true papilledema and pseudopapilledema.

Cyclocryotherapy was a reliable method of producing prolonged decreased intraocular pressure and papilledema in the rhesus monkey. Histologic changes in papilledema caused by decreased pressure were indistinguishable from those in papilledema from increased intracranial pressure. Electron microscopic observations in papilledema after cyclocryotherapy suggested that the principal morphologic alteration in the optic disk is axonal swelling. Additionally, many mitochondria accumulated in some swollen axons. These changes, observed at the ultrastructural level, suggest that alternations of axoplasmic transport may play a significant role in the pathogenesis of swelling of the disk.

We examined three patients who developed optic neuropathies while undergoing chronic hemodialysis. One patient developed severe bilateral deterioration of vision, but recovered totally four weeks after discontinuing deferoxamine chelation therapy. Another patient had bilateral visual loss associated with chronic papilledema of idiopathic increased intracranial pressure. A third showed atypically severe consecutive anterior ischemic optic neuropathy. The latter two patients showed little improvement with high-dose intravenous methylprednisolone therapy combined with more vigorous hemodialysis. These cases, in addition to those previously described, underscore the heterogeneity of optic nerve disease in patients with uremia.

Two patients had bilateral papilledema complicating acromegaly. Both patients had enlarged blind spots, but otherwise visual fields were normal. Suprasellar extension of the pituitary tumors was diligently sought with the use of visual field examination, pneumoencephalography, internal carotid arteriography, and computed axial tomography, and tumor extension did not exist. Transphenoidal and transethmoidal routes were used to perform partial hypophysectomies in these patients. The procedure was completely successful in one patient and partially successful in the other patient. After hypophysectomy, papilledema resolved in both patients. This beneficial effect may be the result of anatomical changes, the reduction in growth hormone levels, or both. These observations suggest that the acromegaly may be different from papilledema that occurs secondary to suprasellar expansion of pituitary tumors.

We examined seven patients who had a syndrome of symptomatic monocular blind spot enlargement without optic disc edema. Two patients had previous blind spot enlargement that resolved over several months. The scotoma in each patients was absolute, measured 15 degrees to 20 degrees in diameter, had steep geographic margins, and extended to within 5 degrees to 10 degrees of fixation. Typically, patients had normal visual acuity, color vision, pupillary responses, and ophthalmoscopic findings. Photostress recovery, tested in two patients, was prolonged in the affected eye. Fluorescein angiography showed no abnormalities corresponding to the scotoma. Orbital computed tomographic scans in three patients and visual evoked responses in one patient were normal. Multifocal electroretinography, performed in two patients, showed loss of retinal waveforms in a large region surrounding the optic disc. Our findings suggest that retinal dysfunction produces this big blind spot syndrome, but we do not know its cause.

Papilledema is a rare finding in idiopathic inflammatory polyradiculoneuropathy. A case in which the papilledema arose at the beginning of the disease and had a favorable course is reported and the principal hypotheses regarding its pathogenesis are discussed.

During the experimentally induced cerebral oedema and intracranial hypertension the eye fundus changes have been evaluated in generally anaesthetized rats. Cerebral oedema was caused either by means of an osmotic or cryogenic method. Dynamics of the observed ocular changes were indirectly correlated with the origin, development and regression of the cerebral oedema caused by both different etiologic factors.

A 35-year-old man with ulcerative colitis who was receiving parenteral feeding with large amounts of glucose, suddenly developed severe optic neuropathy and oculomotor palsy. The visual acuity fell bilaterally to 0. Although it was stated that thiamine has been regularly supplemented in the preceding period, high doses of vitamin B1 were given. Visual acuity promptly returned to 1.0 but large visual field defects persisted. Later on it appeared that erroneously no vitamin B1 has been given before.

Seven cases are presented in which prolonged papilloedema led to the development of acquired optociliary shunt vessels. These vessels may also be found with optic nerve tumours, particularly spheno-orbital meningiomas, optic nerve drusen, glaucoma, and after central retinal vein occlusion. Two patients had intracranial tumours, 4 benign intracranial hypertension, and one Crouzon's disease. Three had marked atrophic changes of the disc. The pathophysiology of the disc changes is discussed. The triad of long-standing poor vision, acquired optociliary shunts, and optic atrophy with blurred disc margins should not be regarded as specific for spheno-orbital meningioma.

A 14-year-old youth had a 2 month history of proptosis of the left eye when he developed pain in the left thigh. Physical examination revealed a mass involving the left orbit and a neurologic deficit suggestive of involvement of the left lumbosacral plexus. The mass, a left frontal epidural tumor, was removed surgically. Subsequently, he developed marked anemia, thrombocytopenia, and acute granulocytic leukemia. He was treated with various anticancer agents but he did not respond well and he expired two months later. Postmortem examination revealed anaplatic granulocytic cells infiltrating numerous tissues including the conjunctiva, choroid, and leptomeninges. Marked degenerative changes of the left retina and bilateral papilledema were evident.

We report the clinical features and results of laboratory testing of seven children with neuroretinitis, diagnosed on the basis of reduced visual acuity, relative afferent pupillary defect, and distinctive appearance of the fundi. Disc swelling was segmental in five patients with unilateral disease and diffuse in two patients with bilateral disease. Retinal infiltrates were transiently present in two patients. Swelling of the optic disc and peripapillary retina, along with the inflammatory vitritis resolved by 2 to 6 weeks but macular exudates persisted for 3 to 12 months. Two patients developed optic atrophy with permanent visual loss and two patients had visual acuity reduction detected only under low contrast conditions. Cat-scratch disease was implicated as the cause of the neuroretinitis in two patients; the etiology of the remaining cases was not established.

This paper describes two young patients who developed subretinal neovascularization (SRNV) of choroidal origin as a complication of chronic uveitis of undetermined etiology. The probable mechanism of SRNV development and the differential diagnosis of the intraocular inflammatory condition are presented.

In summary, we believe that axoplasmic transport alteration is the anatomic substrate for formation of drusen of the optic disc. In familial cases the cause of axoplasmic transport alteration may be related to the presence of a genetically determined, small, crowded optic nerve head. We believe that these congenitally elevated nerve heads evolve over a period of many years through stages of atrophy and drusen formation. It appears that vascular malformations in the familial cases are primarily developmental; however, secondary vascular alterations may occur as the drusen enlarge. In retinitis pigmentosa, the drusen may be cuased by diminished production of axoplasmic material by the ganglion cell. As a general principle, chronic alterations in axonal transport from any cause seem to produce aggregates of swollen nerve fibers which impart a yellow-white appearance to the disc tissue, and account for the yellow filled-in appearance of the disc in patients with drusen, chronic atrophic papilledema and melanocytomas and, in part, for the "waxy yellow" appearance of the disc in retinitis pigmentosa.

Increase in the caliber and course tortuosity of the optic nerve (ON) can be demonstrated by computed tomography (CT) in cases of papilledema due to intracranial tumors, hydrocephalus, pseudotumor cerebri, and other conditions causing increased intracranial pressure. The enlargement of the ON in these conditions is generally bilateral, although one of the nerves, usually of the side of the tumoral pathology, may be thicker. Enlargement on the ON may also be demonstrated by CT in papilledema due to optic neuritis. Occasionally, the thickened ON also display increased attenuation coefficient values.