Chapter 29: The Eye

Question 1

Describe the Graves orbitopathy associated with Thyroid disease

Answer 1

Enlargement of EOMs with nongranulomatous inflammation, increased glycosaminoglycans and endomysial fibrosis

Note that the tendons of extraocular muscles and adipose are not inflamed

Question 2

Systemic disease associated with granulomatous uveitis and sympathetic ophthalmia

Answer 2

Sarcoid

Question 3

Most common malignant tumor of periocular skin

Answer 3

Basal cell carcinoma

Question 4

A unilateral keratoconjunctivitis unresponsive to therapy and stained positive with Oil Red O on frozen tissue may indicate what type of malignancy?

Answer 4

Sebaceous carcinoma

Question 5

Most common intraocular tumor in adults due to metastasis to eye

Answer 5

Melanoma

[uveal melanoma is most common primary intraocular tumor in adults]

Question 6

Oncogenes associated with uveal melanoma

Answer 6

GNAQ

GNA11

Question 7

Uveal melanoma tends to first metastasize to what organ?

Answer 7

Liver

Question 8

Difference between open-angle and closed-angle glaucoma

Answer 8

Open: complete open access to trabecular meshwork; increased resistance to aqueous outflow —> increased IOP

Closed: peripheral zone of iris adheres to trabecular meshwork and physical impedes outflow of fluid

Question 9

Gene mutations associated with primary vs. secondary open-angle glaucoma

Answer 9

Primary = MYOC in adults, some adults with OPTN

Secondary = LOX1 gene

Question 10

Consequences of diabetic retinopathy — nonproliferative vs. proliferative type

Answer 10

Nonproliferative = hemorrhages and macular edema

Proliferative = traction on retina

Question 11

Condition with age of onset around 75; may stem from excess complement activity and/or environmental factors such as smoking or intense light exposure

Answer 11

Macular degeneration

Question 12

Inherited retinal degeneration characterized by painless progressive vision loss

Answer 12

Retinitis pigmentosa

Question 13

Submucosal elevations in the conjunctiva as a result of actinic damage in sun exposed regions

Answer 13

Pterygium (migrates onto cornea)

Pinguecula (does not invade cornea)

Question 14

Immune complex depositions within sclera may occur in ________ causing necrotizing scleritis

Answer 14

Rheumatoid arthritis

Question 15

Most common form of glaucoma

Answer 15

Primary open angle glaucoma

Question 16

Most common type of secondary open-angle glaucoma

Answer 16

Pseudoexfoliation glaucoma

Question 17

In intraocular inflammation, vessels in the ciliary body and iris become leaky, allowing cells and exudate to accumulate in the anterior chamber, causing what condition(s)?

Answer 17

Endopthalmitis and panophthalmitis

Question 18

A _________ can extend onto the cornea but does not cross the pupillary axis; it may induce mild astigmatism to affect vision

Answer 18

Pterygium

Question 19

A _______ does not impinge upon the corean, but it does affect tear distribution - potentially causing focal dehydration and corneal depression (delle)

Answer 19

Pinguecula

Question 20

Most common cause of corneal dendritic ulcers

Answer 20

Herpes simplex virus infection

Question 21

Patients with ______ are typically not candidates for LASIK surgery because the marked corneal thinning with breaks in the Bowman layer gives their corneas a conical shape

Answer 21

Keratoconus

Question 22

______ deficiency can lead to keratomalacia and eventual blindness if not treated

Answer 22

Vitamin A

Question 23

Inherited corneal stromal dystrophy has several forms, and most are autosomal dominant. However, the most severe form is _____ _____, which is autosomal recessive and is essentially a form of mucopolysaccharidosis confined to the cornea in which keratan sulfate is deposited

Answer 23

Macular dystrophy

Question 24

What ocular complication is most likely to develop in the setting of chronic glucocorticoid therapy?

Answer 24

Cataracts

Question 25

In some older individuals with ______, the iris is displaced forward to narrow the angle at the anterior chamber, obstructing flow of aqueous humor, so-called primary angle-closure glaucoma

Answer 25

Hyperopia

Question 26

Mutations in the ____ and ____ genes may account for some cases of primary open-angle glaucoma

Answer 26

MYOC; OPTN

Question 27

Increased resistance to outflow of aquous humor into the _____ canal is typical of primary open-angle glaucoma, which occurs in individuals with _____

Answer 27

Schlemm; myopia

Question 28

Unusual but devastating form of uveitis that can complicate penetrating ocular trauma; results from release of sequestered Ag from one eye that causes an immune response with inflammatory reaction in the opposite eye

Answer 28

Sympathetic ophthalmia

Question 29

Granulomatous uveitis is often seen in the context of what condition?

Answer 29

Sarcoidosis

Question 30

Condition of the eye characterized by capillary micro-aneurysms, cotton-wool spots, arteriolar hyalinization, and more severe changes of proliferative retinopathy with neovascularization

Answer 30

Diabetic retinopathy

Question 31

Condition made up of a variety of inherited abnormalities that produce a waxy pallor of the optic disc

Answer 31

Retinitis pigmentosa

Question 32

Complication of premature birth that results from oxygen toxicity to the immature retinal vasculature, leading to neovascularization of the retina with growth into the vitreous

Answer 32

Retrolental fibroplasia

Question 33

What complication is often seen in the eyes of those with sickle cell anemia?

Answer 33

Preretinal, intraretinal, and subretinal hemorrhages

Question 34

Fundoscopic exam shows a cherry-red appearance of the foveola, whereas the remaining retina remains pale. What might cause this (other than Tay sachs), particularly in someone with heart disease?

Answer 34

Thromboembolization from diseased heart to central retinal artery — causing diffuse retinal infarct that obscures underlying vascular choroid, except where the retina is thinner in the fovea, so it appears red compared to surrounding pale retina

Question 35

Tay sachs is an autosomal recessive IEM due to deficiency of what enzyme?

Answer 35

Hexosaminidase A (one ofthe GM2 gangliosidoses)

Leads to lysosomal storage of intermediate metabolites in retinal ganglion cells and neocortical neruons

Question 36

A majority of age related macular degeneration cases may be related to inherited mutations in the ____ gene, made worse through environmental exposures like cigarette smoke

Answer 36

CFH (complement factor H)

Question 37

What condition is described by the following:

Night blindness caused by loss of rod photoreceptors is early symptom. Later, the cone photoreceptors also begin to degenerate, producing blindness

Answer 37

Retinitis pigmentosa

Question 38

Histologically characterized by clustering of small basophilic cuboidal or short columnar cells around a central lumen, sometimes called Flexner-Wintersteiner rosettes

Answer 38

Retinoblastoma

Question 39

_____ is associated with increased IOP, but instead of papilledema it shows the opposite: optic cup excavation

Answer 39

Glaucoma

[the increased IOP with glaucoma is thought to cause the loss of nerve fibers, resulting in the characteristic cupped excavation of the optic disc]