|Year : 1968 | Volume
| Issue : 1 | Page : 12-18
Vascular pseudoneuritis and atrophy of the optic nerve
Humboldt University Clinic, E:. Berlin (G.D.R.)
|Date of Web Publication||22-Dec-2007|
Humboldt University Clinic, E:. Berlin (G.D.R.)
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Goder G. Vascular pseudoneuritis and atrophy of the optic nerve. Indian J Ophthalmol 1968;16:12-8
The so called vascular pseudoneuritis or pseudopapillitis is going to become more and more common in European countries increasing with rising life-expectation.
PARSONS-SMITH in 1959 found 7 bilatral blind people on account of vascular atrophy among 1 million of the population. In our experience it is thrice as much. The incidence is substantially higher if the cases of unilateral blindness due to it are also accounted.
There is a dispute about the pathological causes. Through KREIBIG  we know that there takes place a noncoagulative necrosis of the optic nerve in a circumscribed field. In most cases it is situated near the lamina cribrosa and surrounded by a collateral oedema. This appears as a papilledema. The more anterior this necrosis lies, the more strongly marked is the papilledema, which may overlap even the retina, causing an ophthalmoscopic picture quite similar or even indistinguishable from the picture of an occlusion of the central retinal artery. (Plate 1)-[Figure - 2]. FRANCOIS, VERRIEST, NEETENS de RAVEK and HANSSEN 
What is the reason for this optic necrosis? FRANCOIS AND NEETENS  and FRANCOIS AND COLETTE  put the blame on an obstruction of the so called central artery of the optic nerve. The existence of this vessel was already postulated in 1879 by KUHNT.  In agreement with SINGH AND DASS  we could find this vessel in only 25% of cases of post mortem studies as a somewhat thicker branch of the ophthalmic artery, about a sixth of the diameter of the central retinal artery. The optic nerve is supplied by capillaries, arising from branches of the ophthalmic artery, perforating the sheaths of the optic nerve. We filled the capillaries with polyethylen, digested the tissue and found that in some older people there was a lack of capillaries just near the lamina cribrosa and in the scteral part of the optic nerve. We could prove this fact also by counting the capillaries in histological serial sections, coloured for example with dialysed iron. (Plates 2 A and B)-[Figure - 3]-(GODER  ).
Only these areas are meeting points of the blood supply, the first between the capillaries from the arch of Zinn and the capillaries entering the optic nerve with the central retinal artery, and the second between the intraorbital and intracranial vessels. Atrophy of such tops of the capillary tree is already well known in the brain and spinal chord. It also takes place in the optic nerve. We call this process according to BURGER  "Wipfeldurre", which means tree top dryness.
This tree top dryness in the optic nerve is the main cause of ischemic necrosis. When the blood supply becomes diminished on account of an arteriosclerotic or inflammatory narrowing of the ophthalmic artery, then there is a lack in reserve capillaries in this part, and necrosis starts. The reserve capillaries open in moments of distress. This necrosers was called opticomalacia KREIBIG , .
What about the practical meaning of this knowledge?
Sometimes it is difficult to diagnose a vascular atrophy of the optic nerve only by clinical means especially in older stages. We carried out cadaver studies in 100 cases comparing the changes of the tree of the ophthalmic artery with other parts of the vascular system and we found that the tightest correlation exists with the superficial temporal artery. In every case of well marked arteriosclerosis of the ophthalmic artery with reduction of the capillaries in the mentioned parts, there exists a strongly marked arteriosclerosis of the temporal artery; but not in every case the opposite is true. The arteriosclerosis proceeds to the periphery. The thickness of the intima in these cases is two or three times that of the normal for that age. [Figure - 1]-(GODRR  )
Most commonly we find a so called stratified sclerosis which means, we find three layers in the intima: in the innermost one the small elastoid fibres predominate, in the middle, mainly collagen fibres can be found and near the media there are muscle cells, which grow through enlarged pores of the elastic membrane Plates 3-[Figure - 4] and Plate 4-[Figure - 5]. The latter is a very important step in the beginning of arteriosclerosis of vessels of the caliber of the ophthalmic artery and the temporal artery as our electron microscopic investigations have shown. The muscle cells play an important role in sclerosis of these vessels. In the capillaries the most important event is the thickening of the basal membranes. In the greater vessels we find changes of the muscular cells together with fibrous material.
In the socalled giant cell arteritis the lumen of the ophthalmic artery is rapidly narrowed and there quickly starts an optical necrosis in cases of tree top dryness. The clinical picture of this disease is well known nowadays. It was rediscovered in the last thirty years but it was already described in the last century by HUTCHINSON, but it must have been known already to the doctors of antiquity, as we learn out of the diary of an eye doctor of the 9th century, the tadhkivat al kahhalin of Ali Ibn Iza of Bagdad.
We see every year 3-4 cases of temporal arteritis with involvement of the eye, because we immediately perform a resection of the temporal artery in every case of suspected disturbance of the blood flow in the optic vessels. There is a large number of cases without the typical clinical symptoms and signs of temporal arteritis which would not be diagnosed without routinely done biopsy of the temporal artery. This is the so called occult form of arteritis temporalis.
The biopsy is not only of diagnostic importance but also of therapeutic value, because it stops a vasovasal reflex, which perhaps causes spastic narrowing of the ophthalmic artery. After establishing the diagnosis, it is necessary to begin immediately a course of cortisone intravenously.
The electron-microscopical picture of the arteritis temporalis we described extensively in a former paper. (GODER  ). It is remarkable that all inflammatory cells develop from muscle cells, but they show changes different than in arteriosclerosis. The first step is a break in the connection of the clastic fibres with the muscle cells. Then the muscle cells undergo variations into macrophages which phagocytose elastic material. Sometimes macrophages are lying so close together that they appear as multinucleated giant cells in the histological picture. (Plate 5)-[Figure - 6] These cells do not show a great phagocytic activity in contrast to the single macrophages. (Plate 6)-[Figure - 7]
| Summary|| |
Pseudo-neuritic with atrophic changes in the optic nerve as a result of retinal vasculities is a clinical manifestation which is increasingly becoming known. Its association with temporal arteritis is of diagnostic and therapeutic value, because biopsy of an excised piece of superficial temporal artery not only shows typical thickening but relieves the condition by breaking the vaso - vasal reflex which causes spastic narrowing of the ophthalmic artery.
| References|| |
BURGER M.: Age and Disease (German) Publ. G. Thieme, Leipzig (1954).
FRANCOIS J. AND NEETENS A.: Central Retinal Artery and Central Optic Nenve Artery. Brit. J. Ophth. 47, 21 (1963)
FRANCOIS J AND COLETTE M.: Ocular Microangiography (French) Ophthalmologica, Basel, 129, 145 (1955).
FRANCOIS J., VERRIEST G., NEETENS A., DE ROUCK A AND HANSSENS M.: Vascular Pseudopapillitis (French) Annal. Ocul. 1. Paris 195, 830 (1962).
GODER G.: The Changcs in Capillaries of the Optic Nerve and their Pathogenic Significance (German) Angiologica 3, 59 (1965).
GODER C.: Vascular Disturbances of the Eyes and Biosy of the Temporal Artery (German), -
Publ G. Thieme. Leipzig (1968).
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GODER G.: David H. and Uerlings. Electron-microscopic Findings in the Vessel Wall in Temporal Arteritis (German) v. Graefes Arch. Ophth. 169, 12 (1966).
On Opticomalacia (German) Ber. Dtsch. Ophth. Ges. 53, 173 (1940).
KREIBIG W.: Opticomalacia, the result of occlusion of the Vessels in the retrobulbar segment of the Optic Nerve. Klin. Mbl. Agenheilk 122, 719 (1953).
Kuhnt: Quoted by Vail in 14.
PARSON-SMITH: G Sudden Blindness in Oranial Arteritis. Brit. J. Ophth. 43, 204 (1959).
SINGH S. AND DASS A.: The Central Artery of the Retina. Brit. J. Ophth. 44, 195 and 280 (1960).
VAIL D.: The Blood Supply of the Optic Nerve and its Clinical Significance. Amer. J. Ophth, 31, 1 (1948).
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]