Kayser Fleischer Ring

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of the sclera with possible involvement of the cornea, uveal tract (i.e., iris, choroid, and ciliary body), or retina. Photophobia is common. Scleritis is usually related to a systemic disorder. It occurs much more commonly in patients with connective tissue disorders. It may be diffuse or localized and nodular. Nodular scleritis is marked by dark localized blue patches in the anterior portion of the sclera;these are seen because the choroid is visible through the translucent sclera. The condition may resolve spontaneously. Figure 10-50 shows nodular scleritis.

Scleromalacia perforans is an uncommon, painless scleral condition characterized by the appearance of one or more dehiscences in the sclera in the absence of inflammatory changes. This necrotizing scleritis without inflammation is classically seen in patients with long-standing rheumatoid arthritis. The underlying uvea is often visible, and it may bulge out, as shown in Figure 10-51. Another example of scleromalacia perforans is shown in Figure 10-52. Notice the thinning of the sclera and the underlying dark uvea, as well as the irregular border of the iris. Anterior synechiae are present, holding the iris bound down to the lens and causing the scalloped appearance of the iris. There is corneal disease as well.

Inspect the Cornea

The cornea should be clear and without cloudiness, ulceration, or opacities.

A whitish ring at the perimeter of the cornea is probably an arcus senilis. In patients older than 40 years, this finding is usually a normal phenomenon. Although there are many false-positive findings, patients younger than 40 years may have hypercholesterolemia. An arcus senilis is seen in Figures 10-53 and 10-54.

An abnormal greenish-yellow ring near the limbus, most evident superiorly and inferiorly, is a Kayser-Fleischer ring. This ring is a specific and sensitive sign of Wilson's disease, which is hepatolenticular degeneration as a result of an inherited disorder of copper metabolism.

Figure 10-46 Conjunctival

Figure 10-46 Conjunctival

Kayser Fleischer RingsNormal Eye Kayser Fleischer Ring
Kayser Fleischer Arcus Senilis
Figure 10-47 Dermolipoma of the conjunctiva. A, Bilateral view. B, A close-up view of the lesion in the right eye.
Kayser Fleischer Arcus Senilis

The Kayser-Fleischer ring is caused by deposition of copper in Descemet's membrane of the peripheral cornea. Figure 10-55 shows a Kayser-Fleischer ring. Notice that the ring is most prominent in the vertical meridian.

Corneal ulcers are extremely painful lesions caused by loss of substance from the cornea by progressive erosion and necrosis of tissue. These ulcers may be caused by a variety of agents, including bacteria, viruses, fungi, and hypersensitivity reactions. Pneumococcus organisms are common bacteria associated with corneal ulceration. Pseudomonas infection is less common but is associated with a rapid spread and corneal perforation. Herpes simplex virus (HSV) is another common cause of corneal ulceration and is the most common cause of corneal-related blindness. It is almost always unilateral and may affect any age group. The keratitis (inflammation of the cornea) is commonly accompanied by conjunctival hyperemia, tearing, and photophobia.

Blauwe Sclerae
Figure 10-48 Blue sclerae secondary to osteogenesis imperfecta.

Figure 10-49 Episcleritis.

Kayser Fleischer Rings

Figure 10-50 Nodular scleritis.

Episcleritis Treatment

Figure 10-51 Scleromalacia perforans.

Syphilis Penile Lesion Images
Figure 10-52 Scleromalacia perforans. Note the anterior synechiae.
Arcus SenilisKayser Fleischer Ring
Figure 10-55 Kayser-Fleischer ring.

Recurrent attacks may be less painful to painless as generalized corneal anesthesia develops. Patients with AIDS or other immunosuppressive conditions are very susceptible to this recurring infection. Figure 10-56 shows a corneal ulceration secondary to HSV infection. Marked blepharospasm is common with corneal ulceration. The most common characteristic finding of HSV-related keratitis is the dendritic ulcer on the cornea. This ulcer is the result of active viral replication in the corneal epithelial cells. Figure 10-57 shows HSV-related keratitis. The eye has been stained with rose bengal. The devitalized, swollen cells laden with the replicating virus stain brightly with this substance. Figure 10-58 shows corneal scarring in another patient as a result of a previous herpes zoster infection. Note the discrete areas of infiltrates in the cornea, as well as the darkening of the skin on the ipsilateral side from the nose to the forehead.

Keratoconus is an acquired abnormality of the shape of the cornea. It has a gradual onset. It is usually bilateral but asymmetric. It is estimated to occur in 1 per 20,000 individuals. The cornea protrudes as a cone, with the apex becoming thin and scarred. Affected patients experience slow visual deterioration. When the patient is asked to look downward, the cone can become quite obvious, as seen in Figure 10-59. This is known as Munson's sign.

Patients with keratoconus or corneal scarring from other causes may require corneal transplantation. A recent corneal transplant is shown in Figure 10-60. Note the sutures and the mild edema.

Figure 10-61 shows a dermolipoma of the corneal limbus. It is a smoothly rounded, yellowish, benign growth (see also Fig. 10-47).

Inspect the Pupils

The pupils should be equal in size, round, and reactive to light and accommodation. In about 5% of normal individuals, pupillary size is not equal;this is called anisocoria. Anisocoria may be an indication of neurologic disease. Pupillary enlargement, or mydriasis, is associated with ingestion of sympathomimetic agents or with administration of dilating drops. A sluggish,

Figure 10-56 Corneal ulceration. Note the pus in the anterior chamber.

Figure 10-56 Corneal ulceration. Note the pus in the anterior chamber.

Arcus Senilis VesselHerpes Scar

Figure 10-58 Corneal scarring secondary to previous herpes zoster infection.

Arcus Kayser Fleischer Rings
Figure 10-60 Corneal transplant.
Scabies Chambers

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