|Year : 1960 | Volume
| Issue : 4 | Page : 81-86
Vasospastic macular retinopathies
Lalit P Agarwal, S.R.K Malik
Department of Ophthalmology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||5-May-2008|
Lalit P Agarwal
Department of Ophthalmology, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Agarwal LP, Malik S. Vasospastic macular retinopathies. Indian J Ophthalmol 1960;8:81-6
A large number of macular lesions are described where the aetiology and the exact pathological sequences are unknown. Recently Agarwal and Malik (1959) envisaged that the lesions of solar retinitis arc essentially initiated by a vasospasm in an area surrounding the macula. Amongst vasospastic conditions we include central angiospastic retinopathy, Berlin's oedema, Toxic oedema and Solar retinitis.
Central angiospastic retinopathy was well described by V. Graefe (1866). Horniker in 1937 drew attention to the functional angioneurotic nature of the disease which was later supported by Gifford (1939). Fairly large number of cases have been reported by others and no attempt has been made to analyse these cases aetiopathologically. Recently Mitsui and his co-workers (1956) classified macular lesions into three types
(a) circumscribed serous detachment of retina, (b) circumscribed sero-fibrinous detachment of retina, (c) circumscribed retinal oedema.The production of toxic oedema in macular disease is of common occurrence especially in the inflammation of uvea and of the retina. It also occurs subsequent to an extra ocular infection, e.g. sinusitis, acute dental abscess, acute febrile disease, chronic anaemia and cachexia.
Berlin (1873) was the first to describe the occurrence of the opacity in the macular area after a concussion injury. He attributed the lesion to an extravasation of blood between the chorio-capillaries and a consequent transudation associated with an (edematous exudation. This concept has wide acceptance. The oedematous changes in the macula may progress to formation of holes and cysts or pigmentary anomalies.
Haab (1885) described degenerative changes limited to macula which are usually punctate in nature occurring bilaterally and ascribed these changes to sclerosis and obliteration of chorio-capillaries in the central area. His contention has wide pathological support.
A similar aetiological concept and resembling clinical picture has been shown to occur in heredo-macular degeneration though such an assumption lacks pathological support (Klein1953).Elwyn(1955) has propounded an ingenious enzymato-genetic theory as a cause of these degenerations.
We (1959) recently evaulated our series of 56 cases of solar retinitis and we graded our cases according to the degree of macular involvement. We also hypothesised that the solar energy excites a vasospasm and the events that follow are primarily initiated by the degree of vascular involvement which in turn is dependent upon the solar energy absorbed.
Similarity in clinical pictures and some end results have made us think and record some of our cases. No histopathological studies have been made and so the views should be regarded as inconclusive.
| Methods and Material|| |
93 eyes of various types of macular retinopathies have been observed by us:
Central serous retinopathy- 24 eyes of 16 cases
Toxic oedema of macula- 7 eyes of 5 cases
Commotio retinae- 6 eyes of 6 cases
Solar retinitis- 56 eyes
A careful history of these cases has been taken with regard to the manner of onset visual disturbances and the nature of trauma if any. They have also been closely questioned with regard to stress, occuptional or otherwise. The cases have been examined and followed up for at least one year. They have been treated with retrobular Priscol -injections with varied results.
| Central Angiospastic Retinopathy|| |
Two illustrative cases are being described:-
A.S. Male aged 27 years, a member of the armed police, whose company was posted to comb out dacoits in a dacoit infested area, was trailing the desperadoes and had two sleepless nights. The company had an armed encounter with dacoits and in the morning he felt diminution of vision, soon after he began to feel as if he was seeing through a mist with a central back spot. The left eye showed gross diminution of vision, (1/ 60) .
On examination there was edema in the macular area characterized by indistinctness as compared with the normal eve. The macular area showed some elevation. A few yellowish spots were seen over the swollen macula. Perimacular arteries were thinned and tortuous. He was put on retrobulbar priscol therapy, 1cc on alternate days and 2 tablets of priscol three times a day, the vision improved to 6/9 after 8 injections. The macular oedema was still present though less and some pigmentary spots were appearing. Six months later the oedema had disappeared and there was macular stippling with 6/6 vision,
Z.A.Male 27 years, A mill worker was being continuously harassed by his superiors and pestered at home
due to poor economic conditions. He came to us with sudden diminution of vision in both the eves. On examination his vision was 4/60 in RE and 5/60 in LE. The funds were normal. He was allowed to go with the diagnosis of functional amblyopia. A week later he carne back complaining of further deterioration of vision and seeing as if through a mist with a central black spot. The vision in both eyes was now 1/6o. On fundus examination it was observed that the macular area was indistinct and slightly raised from the surface. The central foveal reflex was dull and a few yellowish dots were seen over the swollen macula. The whole oedematous area was surrounded by a ring shaped light reflex and the perimacular capillaries were thinned.
Out of 24 eyes of central serous retinopathy that were observed by us there were 12 eyes in which the fund us appearance on the first visit was normal.
| Toxic Oedema of Macula|| |
Illustrative case :
A female patient aged 22 years had an attack of cold and corvza. .4 days later she developed pain in moving the eye up and in with inability to see the central part of the object clearly. There was a feeling that the objects were being seen through smoke or mist. On examination there was tenderness of the globe and the fu ldus looked normal. The vision in RE was 6/6 and in the LE it was 6/36 . A relative central scotoma was detected. A provisional diagnosis of retrobulbar neuritis was made. She -as advised to have injections of vitamin Hi and B12 but as no local treatment was prescribed she did not use any drugs. 7 clays later she noticed further deterioration of vision and came to us once again. On examination the vision was 4/6o and at this time a dull foveal reflex was present. The macular area looked oedematous red and congested. A few scattered black pigmented spots were seen.
Retrobulbar priscol injections were given, 1cc on alternate days over a period of 14 (lays (8 injections). The oedema disappeared leaving a few pigmentarv spots. The vision improved to 6/6/6/9 and no central scotoma was detected.
[Table - 3]
| Commotio Retinae|| |
A male aged 27 years was hit by a shuttlecock in his left eve. The patient complained of immediate dimness of vision. On examination the eye was apparently normal except for slight conjunctival congestion. The vision was 6/36 and the macula was normal. Three (lays later there was further deterioration of vision (6/60) and the patient complained of seeing a central black spot and of seeing the objects as if through a mist. On fundus examination the macula was found to be dull and congested. The zone of congestion was surrounded by macular oedema. Two white lines were seen radiating from the macula towards the nasal side. The patient was put on 1 cc retrobulbar priscol injections on alternate days and after six injections the macula became normal and the vision restored to 6/6.
| Solar Retinitis|| |
A man aged 20 years complained of photophobia, glare and diminution of vision after looking at a partial eclipse. The macula was apparently normal and he was advised to take treatment for the eclipse blindness to which he paid no heed. Four days later the macular area was congested and red and Was surrounded by an oedematous area of the retina. A month later in the macular area there was a greyish white patch surrounding the fovea which, in turn, is surrounded by a black pigmentary ring. He slid not report for further observation.
In such cases retrobulbar priscol therapy was of no help and the defect became permanent. These observations have already been reported elsewhere (Agarwal and Malik).
The various macular lesions in this series have been classified into various grades
Grade I : The macula is apparently normal, the patient only complains of diminution of vision.
Grade II : The macula is congested and is surrounded by an area of oedematous retina. In this grade the patients usually complained of seeing through a. mist or smoke in the central area besides other symptoms.
Grade III : A greyish white patch surrounded the oedematous retina which is surrounded in turn by a black pigmentary ring. The patient has gross diminution of vision and has a positive central scotoma.
Grade IV : Macular cyst, macular hole and gross pigmentary changes. The patient complains of gross diminution of vision and a negative, relative or absolute central scotoma.
Our observations indicate the break up of our cases as given in [Table - 4].
| Discussion|| |
The cases included in this term of vasospastic maculopathy are of some clinical interest as they tend to show that some of these lesions which are differently classified, probably have a fundamental basic pathology of vasospasm. The whole pathological process is started by a vasospastic event in the capillaries of the macular area. This leads to a temporary ischaemia and consequent exudation. The constriction results in the dilatation of the peripheral capillaries creating a state of stasis and peristasis with the transudation of fluid into the surrounding tissue which ultimately leads to organic changes resulting in gross pigmentation, macular degeneration and hole or cyst formation. Due to prestasis and peristasis haemorrhages in the macular area are not uncommonly seen especially after a traumatic lesion. A close analysis of our cases of maculopathy described above reveals a considerable degree of similarity in the evolution of a clinical picture which has prompted us to classify them under a common pathogenetic genera of vasospastic maculopathy.
In many of our cases the subjective symptoms varied and their severity hardly ever bore any relationship to the objective findings in the macula. The trauma produced varied in our cases. Some exhibited psychosomatic . disorder with certain degree of anxiety neurosis and phenomenon which is commonly associated with a state of sympathicotonia which is responsible for exciting a vasospasm. In some cases a toxic element was evident either in the form of a local retinal lesion or a septic focus. There were some others where concussional injury was responsible for this phenomenon. Lastly solar energy was responsible for exciting vasospasm in a large number of our cases.
In some of the cases there were subjective symptoms like dazzling, micropsia metamorphopsia, photosae and diminution in colour sense and visual acuity while the macular area showed no abnormality clinically. These cases under observation usually developed a macular oedema with further fogging of vision and the feeling of seeing through a mist or smoke (translucscotoma). This has given us :a feeling that even when macula apparently looked normal some pathological change was in being in that area. The phenomenon was seen in cases of so called central angiospastic retinopathy, Berlin's oedema,' Toxic oedema or Solar retinitis. The resemblance was striking. The clinical picture later transformed itself into absolute and positive scotoma with gross diminution of vision along with marked pigmentary changes. In some instances irrespective of the diagnosis of the case a macular cyst or hole may be formed. Another interesting feature of these cases was the resolution of the lesion in most of the cases irrespective of their being traumatic, toxic angiospastic or solar in origin. The late lesions also showed similarity in their response in as much as none of them responded to priscol therapy.
How does priscol help in the stages? We feel that the lesion in grade I is due to vascular spasm which is continued into grade II without any organic changes. This leads to disturbance of nutrition. Priscol relieves this vasospasm and improves the nutrition by improving the circulation. An interesting feature has been seen in some cases where there was considerable visual improvement in spite of continued macular oedema which probably indicates that the visual disturbance is primarily due to ischaemia rather than oedema.
Some interest lies in the cases of trauma where the lesion in some showed haemorrhage in the macular area after an initial diminution in vision with a normal macula. How did this haemorrhage occur ? Was it that continued strong vasospasm led to a loss of nutrition of some capillaries which gave way causing haemorrhage? Similarity may perhaps be found with cases of disciform degeneration of macula where, due to an abiotrophic lesion, there is haemorrhage in the macula. Is it possible that even in disciform degeneration the patient passes through a sub-clinical phase of intense vasospasm leading to a loss of capillary nutrition and bleeding ? The rest of the picture is then easy to explain.
Three patients who had observed the eclipse with both eyes and had one amblyopic eye developed the lesion only in the nonamblyopic eve. Unilateral lesions in other angiospastic maculopathies suggest that the same degree of trauma may not be sufficient to evoke a parallel response in the two eyes. It is quite possible that an amblyopic eye may respond differently from the nonamblyopic, but in what way we do not know. In an earlier report we envisaged a lack of absorption of visual energy due either to the lack of concentration of visual energy from a defective focussing mechanism, or to the lack of some special substance which probably absorbs visual energy present in the normal retina but absent from amblyopic eyes. After all this may not be the answer. The whole picture may be due to unequal responses to the same trauma.
| References|| |
Agarwal, L. P. and Malik S. R. K. (2959) Brit. J. Ophthal. 43, ,366.
Berlin (2873) Kiln, Monats. Aug 11. 42.
Elwyn H (1955) Arch.Ophth. 53,619
Gifford, S. R.et al (1939) Arch. Ophth. 21,211.
Grade, A (1866) Greafe Arch Ophth. 12, 211
Haab (1885) Cb, Pr Aug 9, 383.
Flirchsberg (1875) Berlin KI w. 12, 299.
Horniker, E (1937) Min Mbi Augcnheilk 93, 487.
Klein B. A.(1953) Amer.J. Ophathal 36, 959.
Mistsui, Y and Sakanashi, R. (1956) Arner. J. Ophthal. 41, 105.
[Table - 1], [Table - 2], [Table - 3], [Table - 4], [Table - 5], [Table - 6]