or extensive burns, we are safe in pronouncing them simple hemorrhages into the retina, but in most other cases we have to look very sharply if we wish to exclude a retinitis. The hemorrhagic form is met with in rare cases of almost if not quite every disease capable of exciting a retinitis, but more commonly it occurs when there is serious disease of the heart, or of the blood vessels, and then, as a rule, it is unilateral. Schmidt-Rimpler says that he has seen it produced by tumors of the orbit, and it is the most prominent symptom in venous thrombosis. In young people we have to think of nephritis, diabetes, pernicious anemia, an acute infectious disease like influenza or mumps, chlorosis, and changes in the composition of the blood. It is said to have been caused by obstruction to the venous circulation, by poisoning with phosphorus, and by vicarious menstruation, but it is certainly possible that in some of the cases reported the hemorrhages were without an accompanying retinitis.
THROMBOSIS OF THE CENTRAL VEIN When one eye suddenly becomes blind, or nearly so, without known cause, and we find in the fundus a large number of dark red, lumpy, and striated hemorrhages, some small, others large, often with little round ones intermixed, all grouped about and over very dark, broad, and tortuous veins, one of which may be seen perhaps to end in a pool of blood, while the arteries are small, and the margins of the papilla are hidden, partly by an oedematous swelling of the retina and partly by the hemorrhages, we diagnose a thrombosis of the central vein of the retina, or of one of its branches. If the hemorrhages are scattered about in all directions the thrombus is in the central artery itself, but if they are confined to a certain area it is the branch that drains this region that is occluded. Grayish patches are to be seen between the hemorrhages.
<Callout type="warning" title="Be Cautious">This condition can mimic retinitis and requires careful examination.</Callout>
Convolutions of vascular loops are visible on the papilla, perhaps in the surrounding retina, which are anastomoses formed to relieve the engorgement. This condition may be difficult to differentiate from a hemorrhagic retinitis, except through the extreme engorgement and tortuosity of the veins, which are quite disproportionate to the other signs of inflammation.
The only other condition for which it can be mistaken is the possible, though rare, partial occlusion of the central artery attended by great retinal hemorrhages, and this cannot be differentiated with certainty. Thrombosis of the veins is met with usually in elderly people who are suffering from sclerosis of the retinal and other vessels, heart disease, or some lesion that obstructs the venous circulation, but it may occur in orbital cellulitis, and has been ascribed to the various causes of hemorrhagic retinitis. Once in a while we meet with a case in which the cause defies detection.
<Callout type="important" title="Detailed Examination">A thorough examination is crucial for accurate diagnosis.</Callout>
PIGMENTARY DEGENERATION OF THE RETINA When a person does not see as well as he should in reduced light, as during a cloudy day, or in the evening, the sensitiveness of his retina to light is subnormal and we say that he has hemeralopia. Such a condition may be met with in persons who are extremely debilitated by starvation or any exhausting disease, or have suffered from such an infectious disease as malaria, and sometimes it is present in myopes, at least to a minor degree, but when these causes have been excluded we have to search for some pathological condition of the retina that has impaired its sensitiveness.
THE RETINA AND CHOROID 381 Concentric contraction of the visual field of a hemeralopic patient, whether the central vision is reduced much or not, suggests pigmentary degeneration of the retina, or retinitis pigmentosa, as it is commonly though erroneously called. This diagnosis is positive if the fundus is found to have a bluish gray tint, and to present in its periphery a network of jagged, irregular black spots, many of them shaped like bone corpuscles, some stellate, others looking like granules, which tend to follow the courses of the vessels and to overlay them in places, while the vessels themselves are very small, and the papilla has a yellowish tone and slightly hazy margins. As the disease progresses the pigmentation draws closer and closer to the macula and the papilla, until finally it covers the former and surrounds the latter.
At the same time white spots appear among the black, perhaps with little shining dots here and there, the patient becomes nearly or quite blind, and the appearance of the fundus closely resembles that produced by a very extensive old choroiditis, but the differentiation is not so very difficult when we consider the slight details. A creamy yellow, waxy color of the papilla, and retinal vessels which are so small that the veins cannot be distinguished from the arteries and are covered in places by the black spots, establish the diagnosis of pigmentary degeneration of the retina, while a similar yellowish tone of the papilla with vessels that are larger, though smaller than normal, and the passage of the latter across the black spots, are indicative of choroiditis.
So long as the vision remains sufficiently good to enable us to make a perimetric examination of the field the marked concentric contraction is a great aid in the differentiation of this disease. As a rule this contraction is quite considerable before the central vision begins to fail, is much more regular and uniform than in choroiditis, and sometimes is so great that the patient seems to see through a tube.
<Callout type="tip" title="Monitor Visual Field">Regular visual field tests can help detect early signs of pigmentary degeneration.</Callout>
The picture of pigmentary degeneration of the retina is not always as typical as the above. One variation is the total absence of pigmentation, when the patient has all of the other symptoms, hemeralopia, concentric contraction of the field, small vessels, and finally a yellowish papilla, and many intermediate cases between the two extremes of typical and of no pigmentation are to be met with.
Another variation is that a baby may be born blind with no visible lesion in his eyes to explain the amaurosis, but within a year we may see pigmentation develop, the vessels become small, and a yellow optic atrophy supervene. 7 A few cases have been reported in which the ordinary signs of pigmentary degeneration were complicated by the presence of discrete, round, white spots scattered over the fundus, which enlarged and blended while atrophy of the choroid became evident. The white spots may be separated by strips of normal choroid, or nearly the entire fundus may show the picture of choroidal atrophy.
This combination of retinal and choroidal atrophy has received the name of gyrate atrophy of the choroid and retina. It seems to be a variety of pigmentary degeneration in which the atrophy of the choroid becomes unusually prominent.
In rare cases numerous bright white spots of variable size take the place of the pigmentation in the periphery, and present the same appearance as the retinitis punctata albescens of Mooren, by which name the picture often is called. Our knowledge concerning this class of cases is quite limited, but this punctate condition of the periphery seems to occur in at least two distinct diseases.
In Mooren’s case there was no hemeralopia, the peripheral vision was normal, and central vision was reduced. Other cases have been accompanied by hemeralopia, concentric contraction of the field, and late failure of central vision. Roemer states that he saw typical pigmentation develop in a case of this nature.
When we add that the disease seems to be congenital and hereditary, the probability is increased that such cases are atypical forms of pigmentary degeneration of the retina. . The cause of this degeneration is not known. The disease is apt to be hereditary, and to occur in several members of the same family, but isolated cases are not uncommon.
Usually it is congenital, or develops in early childhood, so that its victims have hemeralopia from the first, but rare cases are on record in which the onset took place in adult life. Consanguinity of the parents and syphilis have been quoted as causes, but at best they are questionable.
Other congenital defects often are present. Males seem to be more subject to the degeneration than females. Almost invariably both eyes are affected about equally, though the disease may be confined to one eye in rare cases. The progress is slow, and sometimes seems to be stationary for a long period of time, but no arrest can be ascribed fairly to any form of treatment.
The prognosis is bad, and blindness supervenes during middle life, as a rule. A few cases become complicated with glaucoma, and posterior polar cataract may develop, though this does not happen as frequently as in choroiditis.
AMAUROTIC FAMILY IDIOCY Occasionally we meet with a Hebrew child not many months old who is becoming increasingly sluggish, and whose muscles are becoming paretic gradually. When we touch him he is apt to struggle so hard that an examination is a matter of difficulty, yet he relapses into a sort of stupor as soon as we cease our efforts. We find his pupils dilated and sluggish in their response to light, perhaps completely irresponsive, and he may have nystagmus.
Examination with the ophthalmoscope reveals a cherry red spot at the fovea surrounded by a broad whitish area that does not extend to the papilla, a picture that reminds us of that of an occlusion of the central artery, but this condition is excluded by the practically normal vessels and the absence of any opacity about the papilla. The disk is pale and atrophic.
Inquiry is apt to reveal that other children in the same family have suffered in the same way. The cause of this disease, which is called amaurotic family idiocy, is not known. The affection seems to be confined to the one race, and only a single case is on record in which the child lived to be more than two years old.
The only conditions with which it is likely to be confounded are occlusion of the central artery, which is readily excluded by a study of the details of the picture, and maculocerebral degeneration, which occurs in other races and at a later age.
MACULOCEREBRAL AND MACULAR DEGENERATION When the history of a more or less idiotic child is that he was ordinarily healthy and intelligent until he reached the age of six or eight, the time of his second dentition, when he began to grow stupid and at the same time to lose his power of vision, it is probable that he is suffering from a degeneration of his retina and of his brain. This probability is increased if we find that other children in the family are suffering in the same way, though we are likely to search in vain for evidence of heredity.
It is not necessary for all of the children in the family to be thus afflicted, on the contrary the rule seems to be that some are spared, so far as we can judge from the small number of cases that have been reported. Oatman made a special study of these cases and divided them into two classes, one in which the onset appeared at the time of the second dentition and both the eyes and the brain were affected, and one in which the onset was at the age of puberty and the degeneration was confined to the retina.
The ophthalmoscopic picture is the same in both. I had the privilege of watching his cases while they were under his observation, and can draw no better sketch than the one he has left us in The Fundus Oculi. The following is his description of the younger of the two children, a boy of eight, in whom a failure of vision and of intellect had been noticed two years before.
<Callout type="important" title="Detailed History">A thorough family history can help diagnose these conditions.</Callout>
“He is in excellent health; no paralysis; no malformation. Vision in right eye 10/200; in left 8/200. Small central scotoma for green and red; peripheral fields normal for white and colors; central fixation; talks well and memory good.
Right eye:—Media clear. The macula is encircled by a transversely oval ring of granular pigment, measuring about one disk diameter in length. The enclosed area has a dirty yellowish cast and contains dark spots that can be resolved into fine black granules. The surrounding retina is covered with dustlike pigment. The optic nerve is white on the temporal side. Retinal vessels are narrowed.
The left eye presents the same picture as the right, except that the macula within the pigment ring is more atrophic and the fundus as a whole is somewhat lighter in color.
Four years later. The disease has steadily advanced. No paralysis. Central scotoma is now absolute. Peripheral field appears normal. Memory poor. Is apathetic and makes little effort to see. Eccentric fixation with eyes upward. Retina is becoming depigmented. Has had five epileptiform convulsions, the first of which occurred three years ago.”
The condition in the eyes of the older child was more advanced and optic atrophy was clearly present when she was first seen, at the age of twelve. Her vision and her intellect had begun to fail when she was seven, and she was completely imbecile before she died at about the age of seventeen. These children were brother and sister.
An intermediate sister was normal at the age of fourteen. In both of these patients the Wassermann and the von Pirquet tests gave negative results.
THE RETINA AND CHOROID 885 This affection of the eyes is always bilateral and may be differen- tiated from amaurotic family idiocy by the fact that it appears at a much later age. This alone might hardly suffice were it not for the fact that so far as we can judge the pathological changes are not the same in the two diseases.
Furthermore, maculocerebral degeneration occurs seldom if ever among Hebrews, who seem to have almost, if not quite, a monopoly of the other disease. In another family we may find children whose vision begins to fail with the development of the same ophthalmoscopic picture at about the age of puberty, but with no indications of cerebral involvement.
This is macular degeneration, which seems to differ from the maculocerebral only in the preservation of the intellect, and the age of onset. We have to differentiate these conditions from congenital defects, inflammations of the retina or of the choroid, and other forms of degeneration of the retina.
A defect in the macula may be congenital, may be present in several members of the same family, and may or may not be bilateral, but it does not make its appearance after the child has enjoyed good vision, it does not progress or induce optic atrophy, and it is not associated with a progressive loss of the intellect.
A central retinitis is excluded by the pigmentation of the retina, while in central choroiditis the black and white spots in the macula are not enclosed in a defined oblong, and are not likely to be associated with a dusky pigmentation of the surrounding retina, such as is common in this disease, or these diseases.
When the choroidal markings are visible within the affected area we have to deal probably with a choroiditis, or a sclerosis of the choroid. The history of previously good vision, and the absence of symptoms of hereditary syphilis, suffice to show that the dusky pigmentation of the retina is not the same as that of the snuff colored fundus.
Pigmentary degeneration of the retina is excluded by the absence of hemeralopia, the early failure of central with preservation of peripheral vision, instead of the reverse, the early appearance of a central scotoma, and the white rather than yellowish color of the papilla after optic atrophy has set in.
We can often trace the hereditary nature of the trouble in pigmentary degeneration, but this has not yet been done in either the maculocerebral or the macular. When idiocy attends pigmentary degeneration it is congenital, but in maculocerebral it seems to develop with equal steps along with the failure of vision.
The picture presented in circinate degeneration 386 DIAGNOSIS FROM OCULAR SYMPTOMS is quite different and this condition seldom is met with in childhood. CIRCINATE DEGENERATION Occasionally we see in or about the macula of an elderly patient, whose central vision has been rendered imperfect by the presence of a relative, or of an absolute scotoma, an elliptical, usually incomplete zone of irregular round spots, over which the retinal vessels pass.
Sometimes these spots are found to have coalesced so as to form an irregular, lobulated design which resembles a yellowish white exudate. Hemorrhages are present, as a rule, but there is no optic neuritis. This condition commonly is called circinate retinitis, but
Key Takeaways
- Identify retinal hemorrhages and thrombosis of the central vein to diagnose eye injuries.
- Recognize pigmentary degeneration through visual field contraction and fundus examination.
- Distinguish maculocerebral and macular degeneration based on age of onset, family history, and ophthalmoscopic findings.
Practical Tips
- Regularly monitor the visual fields of individuals who may be at risk for pigmentary degeneration to catch early signs.
- Use a thorough family history to aid in diagnosing conditions like amaurotic family idiocy and maculocerebral degeneration.
- Be cautious when differentiating between retinal hemorrhages, thrombosis, and retinitis; careful examination is crucial.
Warnings & Risks
- Misdiagnosis of retinal conditions can lead to improper treatment or lack thereof, potentially causing permanent vision loss.
- Failure to recognize the early signs of pigmentary degeneration may result in delayed intervention and worsening of the condition.
- Incorrect differentiation between maculocerebral and macular degeneration could lead to mismanagement of patient care.
Modern Application
While the techniques described in this chapter are historical, they provide a foundation for understanding retinal conditions. Modern technology has improved diagnostic tools and treatments, but the principles of careful examination and thorough history remain critical in emergency triage scenarios.
Frequently Asked Questions
Q: How can one differentiate between retinal hemorrhages and retinitis during an eye injury assessment?
Retinal hemorrhages are often associated with underlying systemic conditions such as heart disease or blood vessel issues, while retinitis is typically characterized by signs of inflammation like edema, exudates, and changes in the appearance of the vessels. Careful examination for these distinguishing features is essential.
Q: What are some common causes of retinal hemorrhages that a modern reader should be aware of?
Common causes include cardiovascular diseases, blood disorders, and certain systemic infections. It’s important to consider the patient's medical history and current health status when assessing potential causes.
Q: How can one identify pigmentary degeneration in its early stages?
Early signs of pigmentary degeneration include hemeralopia (difficulty seeing in low light) and a gradual contraction of the visual field. Regular monitoring with perimetry tests can help detect these changes before significant vision loss occurs.
Q: What are the key differences between maculocerebral and macular degeneration?
Maculocerebral degeneration affects both the retina and brain, often appearing in early childhood with a decline in intelligence. Macular degeneration primarily impacts vision without significant cognitive decline, usually developing later in life.
**Q: Why is it important to differenti