|Year : 1970 | Volume
| Issue : 1 | Page : 1-9
Papilloedema and its diagnosis
Armed Forces Medical College, Poona-1, India
R C Sharma
Armed Forces Medical College, Poona-1
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sharma R C. Papilloedema and its diagnosis. Indian J Ophthalmol 1970;18:1-9
Papilloedema, the ominous ophthalmic sign of increased intra-cranial pressure and possible space occupying lesion, carries with it a heavy responsibility as its presence commits the patient to certain investigative procedures that may themselves be hazardous. Hence an accurate diagnosis of this serious ocular sign is mandatory.
The present day nomenclature is not quite satisfactory. The term papilloedema was intended to apply to a condition resulting from increased intracranial pressure in which the degree of swelling was atleast 2 dioptres. This excludes most cases of true papillitis, and also cases suffering from increased intracranial pressure in which the oedema is in an early stage. Moreover, oedema of the disc is also present in papillitis. There is, therefore perhaps a logical tendency to apply the term "papilloedema" to oedema of the nerve head from whatever cause of whatever degree, and to use the term "plerocephalic oedema" as recommended by Traquair to describe oedema of the papilla due to elevation of intracranial pressure. For other conditions oedema of the disc should be described as "disc oedema with optic neuritis" or "disc oedema with hypertension".
In this paper a general outline of aetiological factors has been given and various pit-falls in arriving at a diagnosis of disc oedema have been discussed in seven cases which were erroneously diagnosed as cases of papilloedema.
| Case Reports|| |
A 18 years old patient (DKK) was admitted for investigation as he had an epileptic fit. General and systemic examination did not reveal any positive findings. The patient was diagnosed as having papilloedema both eyes and was referred to the Ophthalmic department prior to carotid angiography.
Both eyes quiet. Vision - right eye 6/60; left eye 6/12; Ophthalmoscopy - media clear; both discs were pale in colour and showed evidence of situs inversus of the optic nerve head which gave an appearance of blurred disc margin at the upper pole. Veins were not engorged. Perimetry and scotometry were normal.
The disc appearance was due to myopia and situs inversus of the optic nerve head and not due to papilloedema.
A 43 years old sepoy (SAN) was admitted in January '67 complaining of loss of vision right eye and pain right orbit of 3 days duration. He was diagnosed as a case of papilloedema right eye and advised carotid angiography. The patient was seen in the Ophthalmic department before carotid angiography was done.
Eye Examination (23-1-67)
Right eye was quiet and white with Vision 1/60 no improvement. The fundus had oedema of disc with a few haemorrhages over the disc and along the superior nasal vessels and a few exudates. Perimetry : showed nasal constriction of the peripheral field by 5-10°. Left eye Vision was 6/6, fundus and Visual field were normal. Carotid angiography done on 28-1-67 was normal.
On 30-1-67 the patient complained of defective vision in his left eye. On examination, the left eye had normal pupillary reacions. The tension was normal but the vision was merely perception of tight with doubtful projection. The fundus was normal. The right eye condition was the same as on 23-1-67.
9-2-67 the findings were as under:
Both eyes were quiet, pupillary reactions were sluggish; tension was normal. Vision right eye was finger counting at 2 metres, left eye -at /metre which could not be improved.
Ophthalmoscopy : Right fundus showed a pale atrophic disc; a burnt out patch of choroiditis near the disc in the superior temporal quadrant with retinal scarring and cytoid bodies.
Left fundus showed a pale atrophic disc. Blood vessels showed nipping and a few cytoid bodies were present in the fundus.
Comments : The patient was a case of optic neuritis R E and carotid angiography was not indicated at that stage. The disc changes in the left eye were probably the result of complication of carotid angiography.
CASE - III
A 44 years old patient (RV) was admitted to the hospital after, an accident resulting in head injury and fracture of the left femur. On routine eye examination both discmargins appeared blurred; so a diagnosis of papilloedema was made.
Eye Examination : Eyes were quiet and white; Pupil - normal; Visual acuity-B E 6/6; field of vision-normal. Fundus examination-both media were hazy due to vitreous opacities. Discs and blood-vessels, were normal. No exudates and no haemorrhages.
Comments : The appearance of blurred disc margin was due to opacities in the media.
A 25 years old cook (KB) was admitted for tiredness, excessive perspiration and laboured breathing. He gave a history of having been diagnosed as a case of epilepsy (grand mal) in 1965. On ophthalmoscopic examination both fundi presented blurred disc margins. All investigations including C S F examination and angiography were normal.
Eye Examination : The eyes were quiet and white; pupils-normal : visual acuity - B E 6/6; field of visionnormal.
Ophthalmoscopy : Media of both the eyes were clear. Fundi showed blurring of disc margins due to excessive neuroglial tissue (congenital) which was seen as a folded mass over the lower half of the left disc. Blood - vessels were normal. No exudates and no haemorrhages could be seen.
Comments : The blurred disc margins were due to excessive neuroglial tissue on the disc.
CASE - V
A 22 years old patient (PLB) reporteed to our hospital in December '67 complaining of headache and pain in both eyes of three months duration. The patient was diagnosed as a case of early papilloedema both eyes and referred to the neuro - surgeon. Neurological examination revealed no abnormality. All investigations including pneumoencephalogram, CSF studies, X-Ray skull and haematological examinations gave negative results.
Eye Examination : Early nasal pterygium v; as present in both eyes. Eyes were quiet. Pupils normal. Visual acuity - B E 6/6; Fundus examination B E Media clear. Fundi showed small discs with blurred margins which were more marked in the left eye. Blood vessels were normal. No exudates and no haemorrhages were seen. Field of vision : central as well as peripheral was normal.
Comments : The appearances of blurred disc margins were due to micropapilla.
A 20 year old medical student (MS) came to the eye department complaining of headache in September '67. On routine examination his nasal disc margins in the left eye were found blurred and early papilloedema was suspected. This blurring of disc margin was due to situs inversus, and the crowding of blood-vessels on the nasal disc margin had produced blurring. Neurological examination and field studies have been normal. He was advised change of glasses and since then his headache has disappeared. The Case has been followed for over a year and the disc appearances are the same.
Comments : The disc appearances were due to situs inversus.
CASE - VII
A 18 years old cadet (VKS) was admitted to the psychiatry ward for abnormal behaviour and occasional attacks of headache. He gave history of similar episodes in the past for which he was admitted and treated by E C T and various drugs. His disc appearances were abnormal so the case was refered for excluding any space occupying lesion.
Eye Examination : The eyes were quiet and white; pupils normal, Visual acuity-right eye 6/6; left eye 6/6.
Ophihabnoscopy : B E media clear. Fundi showed discs with ill defined margins and a conus. Physiological cups were not clearly visible. No exudates and no haemorrhages. Blood vessels were normal.
Field of vision : Central as well as peripheral were normal.
Comments : The disc appearances are due to excessive neuroulial tissue on the disc.
When a patient presents a picture of swelling of the optic nerve head, one has to decide if it is a true oedema of the disc or only an illusion.
| Conditions Likely to be Confused with Papilloedema|| |
a) High refractive error : As in high hypermetropia and astigmatic errors. Ophthalmoscopic appearance of swelling and blurred disc margins is largely due to reflexes.
b) Opacities in the media : Particularly when diffuse. Diffuse corneal oedema as in cases of glaucoma, Fuch's dystrophy, lens sclerosis, fine vitreous haze as in posterior uveitis.
c) Pseudoneuritis : Pseudneuritis is a congenital anomaly found more in hyperopic eyes. The borders of the disc are blurred by glial overgrowth; sometimes there also is an elevation of the disc. The anomaly may be confused with papilloedema, but veins are not engorged and the blind spot will be normal.
d) Bergnieister's papilla : It is a cone-shaped mass of glial tissue at the centre of the disc which normally disappears leaving behind a physiological cup. Sometimes filial tissue does not disappear and remains as a thin veil overlying the optic nerve head and if well defined it is apparent as a blurred disc.
e) Opaque nerve fibres : In the trunk of the optic nerve the individual fibres are provided with a medullary sheath of white opaque appearances. When they reach the lamina cribrosa, the sheaths disappear, so that in the papilla and retina the nerve fibres are transparent. Sometimes medullation does not cease at the lamina but persists to a greater or lesser extent over the optic disc or in the retina and becomes visible as an opaque white patch with "feathery" edges which characteristically tends to obscure partially the retinal vessels because of the superficial position of optic nerve fibres.
f) Congenital tortuosity of vessels In this condition, arteries and veins are very tortuous. The vessels run in curves and bends, but almost exclusively in the plane of the nerve fibre layer, not perpendicular to it. The vessels have normal caliber and their colour is homogeneous; often tortuosity of vessels is combined with pseudoneuritis and such a combination may easily induce the erroneous diagnosis of papilloedema.
g) Drusen of the disc : These are described as "creamy coloured nuts" on the nerve head. These may be buried in the nerve which may raise up the nerve head and appear as papilloedema.
Congenital elevated anomalies of the optic disc that superficially resemble papilloedema are a serious pitfall in neurological diagnosis and once these anomalies have been wrongly diagnosed as papilloedema, suspicion of space occupying lesion becomes difficult to dispel. Misdiagnosis of these elevated anomalies is seldom justifiable since they have peculiar characteristic features that distinguish them from papilloedema.
Once it has been established that the patient has true oedema, the aetiological basis of the condition should be established and differentiated.
Aetiology of true oedema of the disc.
(a) Plerocephalic oedema
(b) Optic neuritis
(c) Associated with generalized retinal oedema
(d) Associated with localized retinal oedema
(e) Ocular hypotension
(f) Orbital causes
(g) Unusual types.
Normally intracranial pressure is lower than intraocular pressure. So theoretically if lamina cribrosa was freely movable, which is not so, under normal conditions or higher degree of intraocular pressure lamina cribrosa will be pushed backward or forward depending upon the intraocular pressure on one side and intracranial pressure on the other (in the subarchnoid). Since the lamina is not freely movable, such a displacement of the disc tissue can occur only to a very restricted degree and gradually after a long duration.
Another part of the picture of papilloedema may be atributed to the impeded flow of central retinal vein. The venous system within the skull, including ophthalmic vein is situated outside the dura hence not directly exposed to the subarchnoid pressure. But the central retinal vein coming from the eye, has to traverse the subarchnoid space of the optic nerve. Here the vein is exposed to increased intracranial pressure and may be partly compressed. This results in engorgement of the retinal veins, haemorrhages etc. Dependence of the occurrence of papilloedma on the increased venous pressure has been proved experimentally.
The signs that constitute the picture of papilloedema may therefore be classified into two groups.
(i) The clevation of disc and the loose appearance of the tissue may be ascribed to increased intracranial pressure behind the disc.
(ii) Dilatation of veins and haemorrhages may be ascribed to impeded outflow of the central vein.
The two groups of signs in the symptom complex of papilloedema are found absent in other conditions. In sudden decrease of intraocular pressure below the level of intracranial pressure, elevations of disc may appear without severe engorgement of veins. In contrast, conditions in which venous flow is impeded for other reasons, such as thrombosis of the central vein, thrombosis of cavernous sinus, aneurysm of cartoid artery in cavernous sinus and pulmonary stenosis, show severe engorgement of veins and haemorrhage but no or only slight elevation of the disc.
Early Ophthalmoscopic appearance
1) Hyperaemia : is due to dilatation of capillaries within the substance of the disc. This hyperaemia affects only part of the disc surrounding the physiological cup, it increases as capillary dilatation increases until it closely matches the deep red colour of the surrounding retina. Incipient papilloedema cannot be recognised on the basis of hyperaemia alone.
2) Venous distention: is an early sign of papilloedema but is significant only if accompanied by increased hyperaemia and blurring of disc. margins. Moderate widening and tortuority of veins often occurs with anomalies of the optic disc including pseudopapilloedema.
3) Absence of venous pulsations: are suggestive but not a pathognomonic sign of early papilloedema. Therefore, the presence of venous pulsation militates against a diagnosis of papilloedema.
Some investigators claim that pressure in the central retinal artery rises in advance of appearance of papilloedema, so they suggest measurement of pressure in ophthalmic artery. But this is not universally accepted.
4) Blurring and elevation of disc margin : following the hyperaemic phase, the disc margins are obscured first and most noticeably near its upper and lower poles, then nasally and last temporally.
5) Elevation of the optic disc : the optic disc is normally a `flat' structure level with the adjacent retina. By the time papilloedema has increased, so much so that the disc margins can be measured, diagnosis is obvious not by virture of disc protrusion but because of associated changes, such as haemorrhages, exudates and vascular congestion. Elevation of the disc may be congenital and totally unrelated to papilloedema. Disc elevation, in itself, does not mean papilloedema.
Disc border haemorrhage : These haemorrhages usually appear as radial linear streaks in the nerve fibre layer and in the presence of a hyperearnic and blurred disc are a definite sign of early papilloedema. Even a single splinter haemorrhage is significant. These are caused by ruptures of distended segments of intra-neural capillary plexuses overlying the disc margin.
The only certain means to diagnose early papilloedema is repeated examination. When all the above signs are present and found to be increasing, diagnosis is no problem. In the absence of these ophthalmic signs further neurologic studies depending upon the general neurologic condition of the patient should be done without taking the appearances of his optic discs into consideration.
Ophthalmoscopic appearance of fully developed papilloedema.
The progress of papilloedema beyond its early stage depends upon the acuteness of onset and the severity and duration of increased intracranial pressure.
i) Distended tortuous veins and obscured arteries : as swelling increases, the distended retinal veins arch forward over the summit of the protruding disc and appear dark. Retinal arteries maintain their normal caliber but appear narrow when campared with veins. The arteries emerging from the swollen disc may be indistinct because their colour resembles adjacent hyperaemic disc or because they are often buried within opaque oedematous disc tissue. Unlike the veins, the retinal arterioles do not become tortuous.
ii) Increased haemorrhagec changes Haemorrhages become worse, are usually linear and occupy the outer slopes of the swollen nerve head. The number and the shape of haemorrhages vary with the severity of increased intracranial pressure. Occasionally the swollen disc is almost obscured by blotches and pools of blood lying upon and among its nerve fibres.
iii) Retinal folds and striations: These are concentric wrinkles and folds that appear as advancing disc swelling infiltrates which displaces peripapillary retina. The retina in the area becomes gray. Where the corrugations converge at the fovea, strands of fine yellow exudates may form a half fan-shaped figure.
iv) Exudates : Exudative phenomenon may occur as papilloedema advances. They may be soft white exudates on the summit and slopes of the disc indicating local concentration of oedema. Some exudates represent focal ischaemic areas in the nerve fibres similar to cotton wool patches of hypertensive retinopathy. After 4 to 6 weeks they disappear. Other white exudates are the intermediate stages of blood absorption.
v) Optic atrophy : Sustained chronic papilloedema leads to progressive and permanent nerve fibre distruction that in turn causes optic atrophy and blindness.
Visual function with Papilloedema
Papilloedema interferes with vision only when it has become fully developed or chronic. It may cause :
a) transient blurring of vision which lasts only a few seconds and it is closely related to the severity of increased intracranial pressure. They do not signify impending visual failure and cease when the pressure falls.
b) Defect in central vision with distortion of images (Metamorphorsia) although central vision may be normal. This is due to macular changes from peri-papillary oedema. Sometimes a haemorrhage may cover the macula. Blind spot is enlarged. Defective central vision directly associated with papilloedema is correlated with oedema of optic nerve and peripapillary retina and this visual impairment usually disappears as papilloedema resolves.
c) Peripheral contraction of visual field is an ominous sign of nerve fibre damage at the borders of the swollen disc and is usually more severe in the nasal field. Concentric contraction of the visual field may be reversible but usually it becomes worse inspite of neuro-surgical procedures and this visual loss is permanent.
Measuring of the swelling : The patient should be examined repeatedly to see if the swelling is progressing or regressing. Actual measuring it by means of an ophthalmoscope is a dangerous procedure and should be discarded. The swelling should be described as of low, medium or high degree. Ideal is to follow by fundus photography.
II Optic Neuritis
It is an inflammatory condition of the optic nerve in which a process of demyelination is associated with oedema in the surrounding tissues. It is usual to distinguish between two forms papillitis in which the disease occurs in the optic nerve within or near the eye ball and retrobulbar neuritis in which it occurs in some part of the optic nerve away from the eye ball.
Symptoms : Sudden and severe loss of vision which may recover later completely, pain in the eye-ball on moving the eye, Presence of local tenderness to digital pressure to a small area corresponding roughly to the site of attachment of the superior rectus tendon. It is present only in early stages of the disease and disappears in a few days.
Signs : Pupil-Lack of sustained constriction of the pupil to light is of the greatest diagnostic significance. Ophthalmosco . py-disc is at first hyperaemic, margins become blurred, swelling and oedema ensue which spreads into the retina, retinal veins become tortuous, exudates accumulate upon the disc. There are fine vitreous opacities, swelling even in severe cases, rarely, exceeds 2-3 dioptres. Temporal atrophy sets in after a few attacks.
Field of vision shows a central scotoma.
III In association with generalised retinal oedema
It is more marked around the disc where massing of fibres is present.
Early diagnosis is difficult. The things to look for are :
i) Increased reflex - to be distingished from the reflex seen in a hypermetropic fundus normally.
ii) White lines running along the vessels.
iii) Soft exudates in retina.
The conditions presenting this picture are renal retinopathy hypertensive retinopathy, toxaemia of pregnancy, severe anaemia and thrombosis of the central retinal vein.
IV In association with localised retinal-oedema
This is seen in the following conditions
a) 'Jenson's choroiditis, occurring in young persons. Oedema is around the disc and macular area. There are other evidences of uveitis, e.g. flare, K. P. and vitreous haze. Field of vision will show sector-shaped defects. Inflammation usually subsides leaving a patch of atrophy but recurrences may take place. Cause is obscure.
b) Neoplasm -any neoplasm near the disc may give rise to macular oedema in addition to changes in the disc, e.g. secondary carcinomas.
V Ocular hypotension
It is not very common but happens after a sudden lowering of intra-ocular tension as seen after perforating wounds of the globe or after filtration operations.
VI Orbital causes
a) Tumour of intra-orbital portion of optic nerve -it may cause venous impediment.
b) Orbital abscess or orbital tumours.
c) Endocrine exophthalmos - Presence of disc oedema is an absolute indication for decompression.
VII Unusual types
a) In emphysema and pulmonary insufficiency usually associated with cardiac failure. The factors producing this are generalised venous congestion, low oxygen tension and high carbon-dioxide tension leading to generalised cerebral oedema and thrombosis of central retinal vein.
Patient complains of headache and becomes drowsy and may be misdiagnosed as a case of space-occupying lesion.
b) Giant-cell arteritis - usually affecting women in later half of life. It has an insidious onset with malaise, persistent headache and may have a tender point on scalp which may establish the diagnosis. There is profound loss of vision with slight swelling of the optic nerve. Disproportion of visual loss with fundus picture is a characteristic feature.
c) Acute bilateral bulbar neuritis in children-affecting children of about 10 years of age. The child gets mild pyrexia, meningeal irritation with cells in CSF followed by abrupt loss of vision in both eyes. Fundus shows slight swelling of the disc. Central field shows a central scotoma whereas the peripheral field is full. Within a few weeks recovery is complete.
The signs and symptoms simulate a space occupying lesion but the degree of visual loss is more important for the diagnosis.
d) Benign intracranial hypertension (Pseudo tumour Cerebri)
Nonne originated the term pseudotumour cerebri for a group of conditions in which there is increased intracranial pressure without an intracranial neoplasm. This is characterized by bilateral papilloedema, a normal or smaller than normal ventricular system, absence of localising neurologic signs and normal crebrospinal fluid except for the increase in pressure. Woodhall in 1939, suggested that certain cases of obstruction of the transverse dural sinuses cause pseudetumour cerebri (benign intracranial hypertension). The increase in cerebrospinal fluid pressure is due to an increase in venous blood within the closed cranium and stasis oedema of the brain. In addition, there is probably a decrease in absorption of cerebrospinal fluid components by the dural sinuses, brain and cerebral capillaries. The obstruction often passes undetected because the evaluation of pseudotumour cerebri rarely includes serial angiography.
The clinical syndrome of pseudotumour cerebri rarely includes serial angiography.
The clinical syndrome of pseudotomour cerebri appears associated in many cases with obstruction of the intracranial venous flow. This may result from otitis media, trauma, tumour involving sinus, foreign body in dural sinus or jugular obstruction. When recanalization occurs or collaterals develop, intracranial pressure returns to normal hence the term benign intracranial hypertension.
Therapy may consist of salt restriction, diuretics, repeated lumbar puncture, anti-coagulants or possibly decompressions. Recanalization of the dural sinuses or the establishment of collateral venous flow appears to be necessary before increased intracranial pressure subsides. Vigorous therapy should be instituted on the onset of transient obscuration.
| Summary|| |
The common conditions which simulate papilloedema have been described and a few cases have been presented which were diagnosed or suspected to be having papilloedema. The mechanism and ophthalmoscopic appearances of papilloedema, have been discussed.
| References|| |
Ballantyne and Michaelson, Text Book of the fundus of the eye E & S Livingitone Ltd. Edinburgh and London p. 440. (1965)
Duke Elder : W.S. Parson's Diseases of the Eye J & A Churchill Ltd. p. 348. (1964)
Gills, J. P., Kapp J.P. and Odom G.L.: Benign Intracranial hypertension. Arch. Ophthal. (Chicago)-78. 1217 (1967)
Hoyt and Beeston,: The ocular fundus in neurological diseases. The C V Mosby Company, St. Louis p.2. (1966)
Sorsby, A: Systemic Ophthalmology Butterworth & Co. Ltd. London, p. 500. (1958)
Traquair W.: Quantitative Perimetry.