Candy Cane Syndrome

mid-dilated pupil may be present with acute angle-closure glaucoma. Pupillary constriction, or miosis, occurs with ingestion of parasympathomimetic drugs, with inflammation of the iris, and with drug treatment for glaucoma. Many medications can cause anisocoria. It is therefore important to ascertain whether the patient has used any eye drops or has taken any medications.

Pupillary abnormalities are often markers of neurologic disease. A condition known as Adie's tonic pupil is a pupil dilated 3 to 6 mm that constricts little in response to light and accommodation. This pupil is often associated with diminished to absent deep tendon reflexes in the extremities. It occurs more commonly in women 25 to 45 years of age, and the cause is unknown. There are no serious clinical implications. The Argyll Robertson pupil is a pupil constricted 1 to 2 mm that reacts to accommodation but is nonreactive to light. It occurs in association with neurosyphilis. Horner's syndrome is sympathetic paralysis of the eye that is caused by interruption of the cervical sympathetic chain. In addition to miosis and ptosis, anhidrosis* is also present. Table 10-7 lists features of these significant pupillary abnormalities.

Inspect the Iris

The iris is evaluated for its shape, color, nodules, and vascularity. Normally, iris blood vessels cannot be seen unless the eye is observed with magnification.

An iris coloboma is a notch or gap in the iris. It results from failure of fusion of the embryonic tissue. The typical iris coloboma is usually part of a choroidal coloboma, which is an autosomal dominant trait. It is often bilateral, is usually located inferiorly, and involves the iris, choroid, or overlying retina (see Fig. 10-140). Visual acuity may be normal if the macula and optic nerve head are spared. Figure 10-62 shows a patient with an iris coloboma involving the ciliary body and the choroid.

Inflammation of the iris, iritis or iridocyclitis, is associated with severe pain, photophobia, lacrimation, decreased vision, and circumcorneal congestion. This congestion is caused by injection of the deep episcleral vessels (ciliary flush). The dilation of the iris vessels leads to transudation of protein into the aqueous humor and deposition of inflammatory cells on the corneal endothelium, known as keratic precipitates. As a result of this deposition, the iris becomes blurred and loses its distinctive radial appearance (a ''muddy iris''). There are many causes of iritis, including exogenous infection from perforating injuries;secondary infection from the cornea, sclera, or retina;endogenous infection such as tuberculosis, gonorrhea, syphilis, and viral and mycotic infections; and systemic diseases such as rheumatoid arthritis, systemic lupus erythematosus, Reiter's disease, Behcet's syndrome, and relapsing polychondritis. Figure 10-63 shows the classic features of acute iritis.

As a result of the inflammatory reaction of the iris, the iris may adhere to the cornea, forming anterior synechiae; posterior synechiae are adhesions between the iris and lens. Glaucoma is a well-known sequela of iritis and synechiae formation.

*Absence of sweating in this syndrome is related to interruption of the sympathetic chain. The amount of sweating is assessed by examination of the forehead or the axilla of the affected side.

Candy Cane Syndrome

Table 10-7 Pupillary Abnormalities


Adie's Tonic Pupil

Argyll Robertson Pupil

Horner's Syndrome


Often unilateral



Reaction to light

Minimally reactive




Sluggishly reactive



Pupillary size




Other signs

Absent or diminished tendon reflexes

Absent knee-jerk reflexes

Slight ptosis* Anhidrosis

*The ptosis is slight owing to interruption of the sympathetic chain innervating only the Muller's muscle portion of the levator palpebrae. The rest of the levator palpebrae functions normally; thus, ptosis is not severe.

*The ptosis is slight owing to interruption of the sympathetic chain innervating only the Muller's muscle portion of the levator palpebrae. The rest of the levator palpebrae functions normally; thus, ptosis is not severe.

Candy Cane SyndromeInspect Anterior Chamber

Inspect the Anterior Chamber

Is the anterior chamber clear? If not, is it filled with pus or blood? Figure 10-64A shows pus in the anterior chamber, known as a hypopyon, in a patient with HSV keratitis. Another example of hypopyon, a large hypopyon together with severe conjunctival injection, is shown in Figure 10-64B.

Is there blood in the anterior chamber? This condition, known as a hyphema, is illustrated in Figure 10-65. Note the subconjunctival hemorrhage also in the patient in Figure 10-65A. A hyphema always indicates that the eye has suffered significant trauma to cause bleeding. The bleeding comes from the anterior chamber angle or the iris, and, therefore, pupillary abnormalities are common. Figure 10-66 depicts an example of a ''candy cane'' hyphema resulting from neovascularization of the iris as a consequence of a central retinal vein occlusion. The photograph shows blood breakdown products intermingled with fresh blood.

Assess the depth of the anterior chamber. By shining a light obliquely across the eye, you can estimate the depth of the chamber. If a crescentic shadow on the far portion of the iris is visible, the anterior chamber may be shallow. Shadowing of the anterior chamber refers to the decreased space between the iris and the cornea. The technique for estimating the depth of the anterior chamber is illustrated in Figure 10-67.

The presence of a shallow anterior chamber predisposes an individual to a condition called narrow-angle glaucoma. The term glaucoma refers to a symptom complex that occurs in a variety of disease states. The characteristic finding in all types of glaucoma is an increased intraocular pressure. The Schiotz tonometer, which is a small, portable instrument, is used for the quantitative assessment of intraocular pressure. Palpation of the globe to determine intraocular pressure is a technique of low sensitivity. Palpation, if performed incorrectly, may be

Candy Cane Syndrome

Figure 10-64 A and B, Two examples of hypopyon. Note the conjunctival injection.

Figure 10-64 A and B, Two examples of hypopyon. Note the conjunctival injection.

Candy Cane Syndrome
Figure 10-65 A and B, Two examples of hyphema.

deleterious, especially in an eye that has been subjected to recent surgery, because a retinal detachment may result. Therefore, palpation of the eye should not be performed.

Inspect the Lens

With oblique lighting, inspect the lens. Is the lens clear? Note any opacity that may be visible through the pupil.

The most commonly observed abnormality of the lens is opacification;the most common cause of opacification is aging. Slow, gradual vision loss is the symptom. Other causes include hereditary diseases, such as Down's syndrome and cretinism;ocular diseases, such as high myopia, iritis, and retinal dystrophy;systemic disease, such as diabetes and hypoparathyroid-ism; medications; and trauma, such as a penetrating eye wound.

A cataract is any opacification of the lens, an opacity that causes reduced visual acuity, or an opacity that interferes with the patient's everyday life. Figure 10-68A shows a dense cataract of the left eye. Note the leukokoria related to the cataract. Figure 10-68B shows a dense nuclear, or central, cataract of the left eye in another patient. Figure 10-69 is a close-up view of a dense nuclear cataract with anterior cortical spokes (opacification of the anterior cortex of the lens). The red reflex, described in the following section, Ophthalmoscopic Examination, is absent in all of these patients.

Is the lens in the normal position, or is it dislocated? The lens may be dislocated anteriorly, pressing the iris against the posterior cornea and blocking aqueous humor outflow, or it may be dislocated posteriorly. Secondary glaucoma may result. Marfan's syndrome is an autosomal

Hyphema And Lense Dislocation

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