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Diagnosis of Pupillary Disorders

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Diagnosis of Pupillary Disorders Chapter 5 Diagnosis of Pupillary Disorders H. Wilhelm and B. Wilhelm Pupillary testing serves two purposes, first, to find disorders of pupillary function itself, and second, to detect disorders of the afferent visual system and the autonomic innerva- tion of the eye. A systematic approach will help significantly with interpretation of the findings. The examination should be done in a logical order, since the pupillary system responds in a logically predictable way. Tests that yield no useful information can only create confusion. Anatomic and Physiologic Fundamentals of the Pupillary System There is a great deal of useful information in the anatomy and physiologic responses of the pupillary system, infor- mation that the clinician can put to immediate good use. Three aspects of pupillary behavior are of particular rele- vance during a clinical examination: 1. The size and speed of pupillary constriction in response to a light stimulus of medium strength are proportional to the logarithm of the luminance intensity of the stim- ulating light. This being the case, pupillary responses can be used as a test of the eye’s sensitivity to light. This is the basis for the interocular comparisons made dur- ing the swinging flashlight test as described in Chap. 2. 2. Normal pupillary reactions are highly variable and are influenced by many variables (status of accommoda- tion, emotions, vigilance, drug effects). This variance is both inter- and intraindividual, which diminishes the value of individual observations and requires multiple repetitions of a test, before a pathological finding can be confirmed. Fig. 5.1. Schematic overview of the anatomy of the pupillary light 3. The pupillomotor centers in the pretectal midbrain – a reflex arc. By way of the optic tract the afferent path (1) of the pupil- region of nuclear centers anteroventral to the quadri- lary system projects to the dorsal midbrain (pretectum, 2). From geminal plate – receives neural signals evoked by retinal there, the signal is carried to the Edinger-Westphal subnucleus in illumination and transmitted by way of the optic tract the nuclear complex of the oculomotor (third) cranial nerve. The (■ Fig. 5.1). Pupillomotor afferents are not separated by oculomotor nerve innervates the ciliary ganglion (3), from which the short posterior ciliary nerves arise and enter the eye to inner- eyes, but rather by visual hemifields. Each side of the vate the pupillary sphincter (4). Not to be overlooked are afferents pretectum in turn sends signals via multisynaptic con- in this reflex system that arise in the visual cortex and project to the nections to both sides of the Edinger-Westphal nuclei. pretectal region, providing another input pathway that partici- Neurons with their somas in these nuclei send their pates in the reflex process 55 Chapter 5 H. Wilhelm, B. Wilhelm 56 Diagnosis ofPupillary Disorders 57 Flow diagram. Diagnostic procedures for pupillary disorders. LR Light reaction, RAPD relative afferent pupillary defect Chapter 5 H. Wilhelm, B. Wilhelm axons by way of the third cranial nerve to the ipsilateral • Pearl ciliary ganglion from where postganglionic parasym- These two steps can be combined. Illuminate both eyes pathetic fibers innervate the eye via the short posterior from a position below the visual axis and then slowly ciliary nerves, terminating at the pupillary sphincter. bring the light source closer to the eyes. Determine This anatomically bilateral sharing of neural input has whether the pupils are symmetrical in size and whether the important consequence that damage to the afferent both constrict equally. visual pathways that lead to the Edinger-Westphal nu- cleus cannot cause an anisocoria. Thus, anisocoria is Third step: Do the swinging flashlight test. This step com- never a sign of an afferent disturbance, but is always a pares the afferent pupillary responses of one eye to the sign of an efferent pupillary disorder. This is the physi- other. (The swinging flashlight test is described in detail in ologic foundation for the tests to be described in the Chap. 2). following sections of this chapter. When a pupil is poorly reactive or does not react to a light stimulus, the swinging flashlight test cannot be done in the usual way since the test requires that both pupils re- What Is Needed For Pupillary Testing? act equally to light. Moreover, if there is an anisocoria, the requirements for this test are somewhat different. Experi- Pupillary testing requires two bright lights (e.g., an indirect ence has found that when there is an interocular difference ophthalmoscope and a bright flashlight), a pocket gauge of 0.5 mm or more in pupillary diameter, the test is best for measuring pupillary diameters, neutral density filters, a done by judging the movements of the pupil that has the slit lamp, a topical solution of 5% cocaine, occasionally a better light reaction, comparing its direct and consensual 1% solution of hydroxyamphetamine (where available), responses. This method is more completely described in pilocarpine 0.1% and 1.0%, and phenylephrine 2.5% eye Chap. 2. drops. Anisocorias of 0.3 mm or less are not usually visible. If normal responses have been found up to this point, the test Pearl • is concluded. It should be recorded as, “Pupils sizes and For presbyopic examiners, a generous near correction light responses are equal; there is no relative afferent pupil- is needed for adequate study of pupillary sizes, shapes, lary defect.” and movements. Pupillary examinations are usually Fourth step: Examination of pathological findings. After best done in a dimly illuminated, nearly dark room (see completion of the first three steps, the following pathologi- below), which makes proper correction of the examin- cal states are possible: er’s refractive errors particularly important. 1. A relative afferent pupillary defect (RAPD) 2. An anisocoria with normal responses to light in both eyes Systematic Examination of the Pupils 3. A monocular or bilateral deficit in light responses First step: Confirm that the pupils respond to light. Only in the dark can the pupils really show how well they can react, Further Testing When Pupillary Signs so be sure that the room light is as dim as convenience will Are Abnormal allow. The patient should be asked to look into the distance, to limit intrusion by the miosis of the near reflex. Using a Relative Afferent Pupillary Defect strong light source (such as an indirect ophthalmoscope) When an RAPD is found, the cause must be identified. This stimulate both eyes simultaneously. If both pupils visibly process is described in some detail in Chap. 2. If the cause and symmetrically constrict, go to the second step. cannot be found, perimetric examination of both eyes is If one or both pupils do not appear to react, a patho- necessary. logical state in need further examination has been found (go to the fourth step). Anisocoria with Bilaterally Normal Pupillary Second step: Compare the pupillary diameters to one Reactions to Light ­another. Examination and interocular comparison of the Dilation Test Video 5.1 Video pupillary diameters determines whether the autonomic The dilation test uses a comparison of the speed of pupil- (efferent) innervation of the eye is intact. If there is an an- lary dilation of both eyes after extinguishing a bright light isocoria, repeat testing of both pupils’ responses to a strong, stimulus. It determines whether there is evidence of a prob- binocular light stimulus. lem with sympathetic innervation of the pupil(s). A prob- 58 Diagnosis of Pupillary Disorders Fig. 5.2. Typical responses of the pupils in the normal state and in ­afferent defect; 3, 4 light reactions for the interocular comparisons the classical pupillary disorders during routine examination. a Test- during the swinging flashlight test with normal responses and no ing of anisocoria and the pupillary light response. b Responses to relative afferent pupillary defect (RAPD); 5, 6 left RAPD; 7–12: pupil- the swinging flashlight test with normal afferent function. c Re- lary light responses in the case of a widely dilated pupil in the right sponses found in monocular disorders of the afferent portion of eye that does not respond to light; 9, 10 no RAPD; 11, 12 in the pres- the reflex arc. 1 Inspection in the dark; 2 inspection with lights on: ence of an RAPD on the right no anisocoria, and with either normal responses or signs of an 59 Chapter 5 H. Wilhelm, B. Wilhelm lem is that the test is done in a darkened room. Ideally, one eye, the drop should be immediately repeated. When test- should use an infrared video system to examine pupillary ing infants and small children, use of a 2.5% solution of movements in the dark, i.e., after turning the light stimulus cocaine is recommended. The diameters of both pupils are off. A practical alternative is to use a separate, weak light measured before and 1 h after instillation of the drops, us- source to illuminate both eyes at a tangential angle from ing the same levels of illumination for both measures. It is below, so that both pupils are visible and a minimum area usually sufficient to measure the pupils’ diameters with a of retina is being illuminated in each eye. It is best not to pocket card that has semicircles of various diameters ar- look at the eye with the brighter stimulus, since this causes rayed along one margin. If greater precision is desired, the light adaptation of the examiner’s eyes, making it difficult measurements should be done with photography. to see the dimly illuminated pupil. Pearl When the pupils dilate well and with no speed differ- • ence between them, the anisocoria is likely to be physio­ If 1 h after cocaine instillation there remains a differ- logic.
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