Part 8

On account of the importance of the case, I suggested another examination a year and a half later, on the 22nd December, 1880, which showed precisely the same result as the former one--optic disc, macula lutea, &c., perfectly normal, the ophthalmoscopic determination of the refraction shows H. 2.5 D.

The child's father also possesses in the left eye a slight degree of congenital defective sight, observed for many years, with normal ophthalmoscopic condition; No. 0.5 is read with + 6.5 D. at 10 cm.

CASE 27.--Tina S--, aet. 6. The defective sight of the left eye had been remarked some months previously; report on July 16th, 1878: R. full visual acuteness, L. movements of the hand are scarcely visible. The child cannot count fingers. Normal ophthalmoscopic condition. Eserine and separate use were prescribed. On September 9th, 1878, fingers were counted with the left eye at 1.5 m., single words of No. 4.0 were recognised, No. 3.5 with convex 6.5 D., but always with oscillating fixation. The improvement in the child's statements may be referred to the fact that she had meanwhile learnt to form right conclusions from the very imperfect sensual impressions of her left eye.

CASE 28.--Frank J--, aet. 10. Left eye. V. 10/50 to 10/40, No. 1-1/2 Snellen is read at 4 inches. On the right, nystagmus on fixation, fingers are counted at 5-6 feet. The ophthalmoscopic condition is normal. A sister of the boy squints.

CASE 29.--Ernest G--, aet. 8, has slight nebulae on both corneae. On the left V. 15/40. On the right, fingers are counted at 4 inches with visual axis deviating inwards.

CASE 30.--I operated on Moritz L-- for congenital cataract before he was a year old in 1869 by means of a needle operation. In June, 1877, a thin ophthalmoscopically transparent secondary cataract appeared in both eyes; on the left, with convex 12 D. V. 3/24 to 3/18, with convex 16 D. No. 0.4 is read at 10 cm. On the right, with convex 12 D., fingers are counted with difficulty at about 1 m., with inward deviation of visual axis.

CASE 31 is also worthy of note. Carl H--, aet. 22, shows quite a number of congenital anomalies on the left side of the face, harelip, deformed nostril and a skin defect on the inner corner of the eyelid. There is a congenital dermoid growth of the size of half a pea situated on the inner lower corneal margin. A slight irregularity in the curve of the cornea near the dermoid is detected with the ophthalmoscope; the fundus of the eye is perfectly normal. Fingers are not counted further off than a metre with visual axis deviating inwards. The right eye is emmetropic (perhaps slightly hypermetropic), and has full visual acuteness. There is no squint.

It is customary to "explain" these cases of monocular amblyopia by previously existing squint, and one is quite satisfied if by the examination of patients it is only possible to prove that they have occasionally squinted, although the advocates of the amblyopia ex anopsia disallow the presence of the same under these conditions, that is, in periodic squint. Of course a theory which cannot exist without the assertion that occasional alternation suffices to hinder the development of defective vision caused by disuse, cannot possibly hold periodic squint to be the cause of it. Certainly permanent squint may also disappear, but this much I have been able to determine, that this seldom happens before the twelfth year of life, and one may surely reckon that children in whom permanent squint is developed at the usual early period of life, still squint at the age of ten years. Cases 24 and 26 to 30 can under no circumstances be explained by previous squint, notwithstanding that they represent the extremest degrees of amblyopia, but the question is undoubtedly that of congenital defective vision; moreover I have excluded from the statistics of congenital amblyopia all cases in which the previous presence of squint could even be supposed.

A table of the cases above described with reference to the defective condition is interesting; when a determination of refraction existed for the weak eye I have given it, and when this was not the case I have stated that of the better eye, thus it is seen that among 85 cases in which the refraction was determined, hypermetropia (including hypermetropic astigmatism) was present in 39. Hypermetropia was found then in 47 per cent of all the cases. The percentage would probably be higher, if all weak-sighted eyes had been examined from the beginning as to their state of refraction, but as I only learnt to know the relation between hypermetropia and the higher degrees of congenital amblyopia from my statistics, I did not take notice of this relation when investigating individual cases.

How does congenital amblyopia now stand in relation to that disturbance of vision which we observe in squint? I see no difference; whether squint is present or not, the form of defective vision is precisely the same, and nothing happens in the combination with squint which could not also be proved without it. The relation to hypermetropia, which is proved with congenital amblyopia, also appears in squint.

A collective table of cases of convergent and divergent squint included in the statistics (pp. 19 and 47) shows:

(_a_) In myopia, emmetropia, and doubtful hypermetropia, with convergent and divergent squint together 329 cases. Among them: Visual acuteness to 1/7 239 " less than 1/7 to V. 1/12 19 " " 1/12 to V. 1/36 46 " " 1/36 25 Defective sight of higher degree than 1/7, 27.3 per cent.

(_b_) In hypermetropia 1 to 3 D., including the few cases of hypermetropia with divergent squint, 177 cases. Among them: Visual acuteness to 1/7 121 " less than 1/7 to V. 1/12 17 " " 1/12 to V. 1/36 27 " " 1/36 12 Defective sight then, 31.6 per cent.

(_c_) In hypermetropia 3 D. and more, 70 cases with convergent squint, with: Vision to 1/7 39 V. < 1/7 to V. 1/12 8 V. < 1/12 to V. 1/36 14 V. < 1/36 9 Defective sight then, 44.2 per cent.

This regular increase of defective sight with the increase of the hypermetropia can be no mere accident, and speaks strongly for the identity of defective vision in squint with congenital amblyopia. Were defective vision caused by the squint the various states of refraction would show no difference in the percentage of defective vision.

Further, the circumstance is worthy of remark that among 198 cases of periodic squint (convergent and divergent) which are applicable for the statistics of visual acuteness--

170 possess V. to 1/7. 16 " V. < 1/7 to V. 1/12. 9 " V. < 1/12 to V. 1/36. 3 " V. < 1/36. 14.2 per cent. then of defective vision of considerable degree.

That defective sight on the whole plays an influential part as a cause of squint is doubted by no one, indeed we see blind eyes lapse into squint as soon as the conditions necessary to it are supplied by the muscles. Of all the prevailing causes present defective vision will be the more decisive in proportion as it is of high degree; for the motive which despite the presence of favouring circumstances can prevent the real occurrence of squint, binocular vision, becomes less efficacious as the defective vision becomes more considerable. As binocular fusion takes place frequently in periodic squint, for a time at least, that is as long as proper fixation lasts, one can understand that periodic squint exists chiefly in cases where the visual faculty of both eyes is good. Even the highest degrees of congenital amblyopia are not excluded, for periodic squint appears where the faculty of binocular fusion has been completely lost. Further, that considerable congenital defective sight is more frequent with than without squint, may be accounted for quite simply by the fact that, in extreme degrees of it, binocular fusion cannot be learnt at all, while in the lesser degrees it is more easily forgotten again.

If defective vision is undoubtedly one of the causes of squint, we must seek for the grounds upon which it has been taken to be a consequence of squint, and described as amblyopia ex anopsia. I will not inquire to whom the honour of this invention belongs. I do not want to write a history of mistakes but only to examine the basis of the views now current. The most complete record of the same may be found in the well-known journal on the 'Cure of Eye Diseases,' vol. v, p. 1011. Leber, who does not seem to recognise the existence of congenital amblyopia, has shown quite a special predilection for amblyopia ex anopsia.

Amblyopia from want of use, which formerly included all possible disturbances to vision, great and small, is now only accepted in two cases, for squint and congenital cataract, if the latter is not operated on very early in the first or second year of life.

The fact is simply this, that in congenital cataract even the most successful operation is frequently deceiving as to its issue without ophthalmoscopic report; this is the more disagreeable as the most exact reflection test before the operation fails to prove the existence of this defective sight. But does it follow from this, that congenital cataract has induced defective sight from want of use? We find the same defective vision also in congenital defective development of the transparent lenses (so-called luxation of the lens). On the whole, we often find several congenital defects in the same individual. The very circumstance that the cataract is congenital makes it probable that the defective sight is so also, or are we to take congenital cataract as being a guarantee against congenital amblyopia?

Von Graefe, who first considered this defective sight to be congenital, designated it in his later lectures as originating from want of use, probably in order to advise the earliest possible performance of an operation. There is no mention of his having brought forward evidence for this assertion; that the great master himself said it was enough, and the host of believers felt themselves to be the happy possessors of a new dogma.

A number of children appeared in my practice, in whom congenital cataract was needled by von Graefe in the first or second year of life with recovery of transparent media, who showed, however, the extremest degrees of defective vision when they were sufficiently intelligent to have their vision tested. Whoever is interested in this can find a number of such cases in the Royal Institution for the Blind at Steglitz, which I am accustomed to visit several times a year by request of the committee. On p. 91 I have related a case of monocular congenital defective sight in congenital cataract of both eyes.

Everywhere then the principle holds good, that whoever makes an assertion must be prepared to verify it; amblyopia from non-use is denoted as an inherited trouble, and still not a single observation exists which furnishes proof that an eye of previously ascertained good visual acuteness has become amblyopic in consequence of disuse, a fact I drew attention to ten years ago. Leber replies to this, he remembers "to have seen patients with complete amblyopia in the squinting eye, who stated that its visual faculty had been found to be good during an examination instituted years before." Is this intended as an observation? By that I mean is it a proof of facts, for the trustworthiness of which he holds himself responsible: in the handling of scientific questions I do not place the least reliance on the dim recollections of unnamed individuals. Even in personally conducted examinations we must be on our guard to avoid mistakes, and now we are confronted with mere recollections of tests of vision!

By means of the above observations the theory that "the peculiar variety of monocular amblyopia which is so frequent in monocular squint is hardly observed without squint" is sufficiently disproved.

Leber seeks to enfeeble Alfred Graefe's statement that the presence of extremely defective vision may sometimes be proved at a very early age, in children who have only squinted a short time (the rapid development of amblyopia in consequence of the squint really appears incredible), by the assertion "that just at the earliest age, when the activity of the optic nerve is not yet sufficiently strengthened by use, the conditions for producing amblyopia from non-use are most favorable with complete exclusion of one eye," but complete exclusion of the squinting eye does not take place even in extremely defective sight, as can easily be seen by the mirror test (p. 66) I described fourteen years ago. Which activities of the optic nerve apparatus are strengthened then by use? Perhaps visual acuteness? The physiological conditions of this are only to be sought in the anatomical structure, and the physiological arrangements of the retina or the visual organs, which cannot be changed much by use. What we can learn from the visual act relates solely to the conclusions which we are able to draw from sensual impressions; but visual acuteness, _i. e._ the faculty for the recognition of distinct points, is an anatomical, physiological gift, and not a thing to be acquired.

The opposing observation, that squint, even of monolateral character dating from earliest childhood, continued to the middle and later years of life, can still exist with very good visual faculty, may easily be explained by alternation from time to time. If that is so indeed, if squint begins during the presence of good visual acuteness, and nothing further is necessary to its maintenance than alternation from time to time, why should defective vision from non-use ever be developed? With good visual faculty on both sides alternations also occur from time to time.

Still more convincing are those cases which are numerous where the visual acuteness of the squinting eye only amounts to about 1/7 to 1/12, and where, on this account, there is no alternation. Were this defective sight acquired through non-use it must of necessity be progressive; it must exist in proportion to the duration of the squint. A moderate experience will suffice to show that this is not the case. And further, defective sight must continue progressive even after removal of the squint by operation, for by the operation nothing is changed in the relations of the binocular vision present in squint, which are dismissed with the one word, "suppression," by the advocates of defective vision from non-use.

Moreover, suppression may exist for years without the slightest disadvantage to the visual faculty.

CASE 32.--In November, 1873, I operated on Fritz F-- for a slight divergent squint of the left eye. Slight hypermetropia was present on both sides, and nearly full visual acuteness. In October, 1880, perfectly normal position of the eyes showed itself with the same visual acuity and emmetropia in both eyes; at the same time, however, the boy affirmed that when reading he could never see with his left eye but only with the right; in reality only the right visual field was perceived in the stereoscope.

The second reason brought forward is, that the variety of amblyopia from non-use is quite a peculiar one; "it consists of a functional disturbance of those parts of the retina whose images belong to the common V. F., and are suppressed in squint in order to render vision distinct--the macula and the temporal and only a part of the nasal halves of the retina." Does this hold good for all cases of amblyopia in squint, or do those cases only belong to amblyopia from non-use where excentric fixation takes place with an inward deviating visual axis? It would be difficult to draw the line. I have seen a case in which the squinting eye possessed a visual acuteness of 5/36 together with excentric fixation and nystagmus; however, I attach no value to isolated cases. We frequently find excentric fixation with a visual acuteness of 1/12 to 1/36. Further, those cases cannot possibly be regarded as results of squint, which possess unsteady oscillating fixation or rapidly trembling nystagmus, which occurs as soon as the squinting eye fixes. But this conclusion is false, even for the excentric fixation with visual axis deviating inwards; if it were right the angle at which the eye deviates inwards on fixation in convergent squint would always be greater than the squint angle. Those cases are, of course, more remarkable where this is not the case; however, on close investigation those cases are more frequent where the angle of deviation is about the same size or smaller than the squint angle, and is fixed with a part of the retina which undoubtedly belongs to the common visual field.

On p. 91 I have described two cases of excentric fixation in children who had never squinted, and it is only necessary to take a little trouble to repeat the mirror test which I described, to be convinced that squinting eyes have not lost the power "of using those parts of the retina," even if they are amblyopic to an extreme degree; without the slightest doubt the reflection is perceived as soon as it falls on the retina.

Value is attached to the improvement produced by the separate use of the squinting eye. According to my experience no higher visual acuity can be attained by use of the amblyopic eye, than that which is best detected by the aid of eserine in the first examination, if it is only carried out thoroughly enough. No doubt if we proceed otherwise, and rest content with whatever statements the patient likes to make, without giving ourselves any more trouble, we may expect the most superficial diagnoses to show the most astonishing therapeutic results, as, indeed, often happens. And now, talking of strychnine injections! When two celebrated ophthalmologists occupy themselves simultaneously with the therapeutics of strychnine, one of whom obtains the most astonishing results in atrophic troubles of the optic nerves, but, on the other hand, obtains no real improvement in "amblyopia from non-use," while the other can show brilliant success in the last-named form of defective vision, and, on the other hand, none in atrophy of the optic nerves, we may perhaps conclude that both are right, if even really on the negative side, and that the circumstances are the same in the tests of vision. Again, we must examine more closely some of the cases, in which strychnine injections showed a brilliant result. (Anyone interested in the original work can read up the 'Vienna Weekly Medical News' for the year 1873.)

"1. Wilhelm H--, a strong healthy boy, aet. 12, complains of defective vision. Right eye has nothing abnormal in its outward appearance, and just as little in the fundus. V. 16/100, H. 2.5 D., Snellen IV-I/II; is the smallest type he reads at 3 to 7 inches. With + 10, I-I/II is read at 4 to 6 inches. Left eye V. 16/70. H. 2.75 D. II-I smallest type legible at 3 to 12 inches. With + 4 D. I-I/II is read at 4 to 6 inches. On March 14th, 1872, first injection of strychnine with 0.002 gr. in the temples. An hour later V. of right eye 16/70, left unchanged. On March 23rd, 1872, after one injection daily, V. of each eye is 16/50."

Patient shows then in the right eye visual acuity 16/100, with manifest hypermetropia 2.5 D.; in all probability the total hypermetropia really present was higher, and was scarcely corrected by means of convex 4 D. If the patient now reads No. I-I/II Snellen with + 4 D. at 6 inches, this proves a visual acuity of 1/3 during the first investigation before the strychnine injection, and shows that the estimate of 16/100 was inaccurate. At the close of the treatment, only a visual acuity of 16/50 (almost exactly 1/3) is specified for distance. The result seems to me, then, to be this, that the patient during repeated examinations has gradually learned to make more accurate statements, indeed, with a boy twelve years old one can scarcely expect it to be otherwise.

"4. Paul A--, aet. 18, was operated on ten years ago for internal squint of the right eye, and dismissed with + 2 D. for distance, and + 6.6 D. for near use. He now complains of decrease of his visual acuity. The eyes are normal externally and internally. Hyperopic formation in a high degree. Right eye V. 1/20, with and without convex glasses, without glass only VIII-I/II with difficulty, with + 6 V-I/II the smallest. Left eye appears emmetropic, but is decidedly hyperopic. V. 5/4. Glasses are rejected; I-I/II is read fluently at 6 to 12 inches. After one injection the right eye recognises III-I/II with + 6, after the second II-I/II, after the eighteenth I-I/II with difficulty. The visual acuity, however, remains at 1/20, and is not changed after six months, although latterly patient daily practised with + 3 D."

Visual acuity of 1/20 suffices to read III-I/II at 2.5 inches, II-I/II at 1.5 inches, and I-I/II at about 1 inch; clear, retinal images are then scarcely obtainable, but we know what hypermetropes can do in that case; besides this, if the patient is examined for weeks by Snellen's method, he may get so far as to realise fairly well "the strange fate of that man" of I-I/II, despite larger diffusion circles; in any case vision remained at 1/20, despite strychnine and separate use.

In extremely defective vision little importance should be paid to the fact of slight diversity in the statements, as where visual acuity amounts only to about 1/36, or where fingers are counted at a distance of 1 to 2 metres, it is quite immaterial, as far as the usefulness of the eyes is concerned, whether fingers are counted at a half or a whole metre, and we ought never to forget that all conclusions which we draw from the state of the visual acuity, are unreliable in proportion as the latter is lowered. Indeed, on repeated examination of such cases we frequently find considerable fluctuation in the statements of the patients, therefore we ought not to expect accurate statements for very inexact sensual impressions.

By separate use, even in extremely defective vision, no improvement in visual acuteness is developed, but only a more complete acquirement of the power of deducing right conclusions from imperfect sensual impressions. That which has been most unscientifically designated as "suppression of diffusion circles," depends solely on this method of use. As with indistinct retinal images so with facial impressions which are insufficient, one can never learn to recognise larger objects aright.

We must never forget that vision is a conclusive act acquired by practice; whoever sees well with one eye, and is weak sighted with the other, acquires this end only for the sensual impressions of the better eye, and must first collect experience for the defective eye, before he can use it.