DSA10 - Pathology of the Eye

Proptosis

Information for Symptom:

bulging or protruding of one or both eyes from the orbit due to forward displacement

Thyroid Ophthalmopathy (Graves Disease)

Information for Condition:

Autoimmune disease which can affect the thyroid --> enlarged rectus muscles due to Hyaluronic acid deposition, the orbit and the skin; eye is forced to protrude forward (exophthalmos)

May not regress w/ Tx for Hyperthyroidism

Lacrimal Gland Neoplasm

Information for Condition:

Mass on major lacrimal gland (which sits superior and lateral to eye globe) that pushes eye inferior and medial (towards the nose) --> proptosis

Idiopathic Orbital Inflammation

Information for Condition:

Inflammation of orbit (may lead to Proptosis) due to inflammatoy pseudotumor, IgG4 disease, or infectious mass.

Commonly bilateral with episodic recurrence

Sx: Decrease vision, diplopia, red eye, headache painful presentation in children - resembles orbital cellulitis. can have constitutional symptoms

Inflammatory pseudotumor:

-Mixed inflammatory cell infiltrate with fibrosis - can cause Idiopathic Orbital Inflammation

*may be idiopathic or IgG4 disease

What is shown here?

Sjogren syndrome

Information for Condition:

Autoimmune lymphocytic attack on lacrimal and salivary glands --> can lead to Proptosis; may progress to lymphoma

Basal Cell Carcinoma

Information for Condition:

Common

Involves sun-exposed areas

Usually involves lower eyelid rim and medial canthus

Blepharitis

Information for Condition:

Eyelid inflammation

Chalazion

Information for Condition:

Ducts for sebaceous glands under the palpebral conjunctive become blocked --> lipogranulomas form due to gland rupture

Sebaceous carcinoma

Information for Condition:

Aggressive condition

May show Pagetoid spreading of carcinoma in-situ cells (Atypical epithelium w/ cytoplasmic lipid) to overlying mucosa

May develop lymph node metastases

(esp intraparotid & submandibular LNs esp)

*Mortality Rate = 22%*

neutrophils; lymphocytes

Bacterial conjunctivitis involves a surge of (neutrophils/lymphocytes), while viral and allergic conjunctivitis involve a surge of (neutrophils/lymphocytes)

Chlamydia trachomatis (trachoma)

Information for Condition:

-Gram-negative bacteria; intracellular cause

-Highly contagious, endemic in low-income counteries

-Scarring inverts upper eyelids --> lashes damage cornea = most common cause of blindness worldwide

-Caustic alkali injury

-Cicatricial pemphigoid

What other conditions can cause Conjunctival Scarring?

Pinguecula

Information for Condition:

-Raised yellowish white mass within the bulbar conjunctiva, adjacent to the cornea

-See solar elastosis & neovascularizaiton

Pterygium

Information for Condition:

-Fleshy triangular growth of bulbar conjunctiva

-See solar elastosis & neovascularizaiton

-Extends onto cornea from limbus, and may be biopsied to evaluate for Squamous Carcinoma

-Squamous papilloma (HPV 16 & 18-associated)

-Squamous carcinoma and carcinoma-in-situ

-Nevi (common, bulbar conjunct, with cysts)

-Melanomas (from primary acquired melanosis with atypia (like lentigo maligna), BRAF in 40%, 25% aggressive)

What neoplasms are associated with the Conjunctiva?

Nevus in Conjunctiva

Information for Condition:

Circumscribed growth in conjunctiva

Small cells w/ cystic glands

Melanoma in Conjunctiva

Information for Condition:

Irregular growth in conjunctiva

Large & atypical cells that invade stroma

Herpes (simplex and zoster)

Information for Condition:

Virus that can cause keratitis (granulomatous inflammation centered on Descement membrane) and corneal ulcers:

-Will see scarring, neovascularization & deep granulomas

Band keratopathy

Information for Condition:

Calcium deposits in Bowman layer; metastatic calcification (may be due to hypercalcemia)

Keratoconus

Information for Condition:

Cone-shaped cornea due to Bowman layer defect --> causes discontinuity in Bowman layer --> Stroma protrudes through + deforms basement membrane

Fuchs dystrophy (bullous keratopathy)

Information for Condition:

Common endothelial dysfunction in Cornea - endothelium produces deposits (guttae) on Descemet membrane --> stromal edema --> subepithelial bullae on cornea

Cataracts

Information for Condition:

Lens opacity (congenital or acquired)

Pseudophakic (artificial lens) bullous keratopathy

Information for Condition:

Corneal decomposition following cataract surgery of lens

Anterior Segment Inflammation

Information for Condition:

Inflammation causing synechia (adhesion) of iris to back of cornea; Often associated with vision loss

Glaucoma

Information for Condition:

-Caused by increased intraocular pressure (leads to optic nerve damage and visual field changes)

-Aqueous humor produced by ciliary body goes from posterior to anterior chamber

-Open Angle Vs Angle Closure

Can damage optic nerve (see increased cupping of disc, atrophy of ganglion cell layer of retina, aka "Glaucomatous Retinal Degeneration" & atrophy of nerve)

-Virus

-Pneumocystis

-TB

What can cause Infectious Uveitis?

Sarcoid Uveitis or Sympathetic Ophthalmia

What Uvea condition(s) would also show granulmoas in the conjunctiva?

Sympathetic Ophthalmia

Information on Condition:

Characterized by immune mediated damage (uveitis) against one eye after a penetrating injury to the OTHER eye.

It is due to an immunologic mechanism involving the recognition of "hidden" antigens

May see granulomas

Nevi in Uvea

Information on Condition:

Growth in common in choroid of uvea

More circumscribed

Melanoma in Uvea

Information on Condition:

Growth in the uvea; NOT related to UV exposure

"Mushroom shape/polygonal shape, epithelioid tumor cells"

Genetics = G-protein activation (not BRAF), also loss of chromosome 3 (BAP1 loss)

Prognosis = related to size, mitotic rate, type (Spindle cell is BETTER, while epithelioid is WORSE)

Can spread to liver (hematogenously), but not via lymphatics

Mortality = 20% at 5 y, 40% at 10 y, then 1% per year

Retinal detachment

Information of Condition

two layers of the retina separate from each other - can lead to vision loss; If traumatic, it's called "rhegmatogenous"

Can cause blood vessels in retina to lose lumen thickness, while their walls become hypertrophic --> vein lumen can become nicked --> Nerve Fiber Layer Infarct downstream ==> Gliosis (scarring - "cotton wool spot")

Describe how Hypertension can negatively affect Retinal Function

Damaged capillaries leak blood---> lipids, fluid seep into retina---> hemorrhage, macular edema, exudates & microangiopathy

Describe Nonproliferative Diabetic Retinopathy

Previous occlusion of retinal blood vessels leads to growth of new fragile retinal blood vessels (neovascularization) that bleed easily (microaneurysms) and obscure vision; occurs on vitreous layer & iris

Describe Proliferative Diabetic Retinopathy

Retinal infarct & cherry red fovea

Retinal arterial occlusion can lead to what issues?

Deposits of material within macula (Bruch membrane deposits), causing possible atrophy of retina/RPE and gradual loss of central vision

Define Dry Age-related macular degeneration

Proliferation of abnormal blood vessels that leak blood and fluid/exudates into macula causing central & more severe vision loss

Progresses more quickly than dry AMD

Define Wet Age-related macular degeneration

Retinitis pigmentosa

Information for Disorder Group:

-Non-inflammatory

-Genetic condition

-Due to mutations affecting photoreceptor cells (rods & cones) or retinal pigment epithelium (RPE) - usually apoptotic loss of rods & cones

-Loss of rods = night blindness & constricted visual fields

-Loss of cones = less central visual acuity

-Pigment from damaged RPE accumulates at vessels

Retinoblastoma

Information for Condition:

-Hereditary & sporadic forms of cancer (40%) = mutation of RB tumor suppressor gene (normally brake on cell cycle & responsible for primitive neuronal differentiation)

-Tumor = Lobulated retinal tumor

-See small blue cells, rosettes, necrosis & calcifications

Anterior Ischemic optic neuropathy

Information for Condition:

Optic nerve ischemia due to overcrowding of nerve fibers and compromised vascular supply at the nerve head

Can be caused by Arteritis (Temporal Arteritis), embolus or thrombus

Papilledema

Information for Symptom:

Swelling and inflammation of the optic nerve at the point of entrance into the eye through the optic disk

-May be due to nerve compression (unilateral), high CSF pressure (bilateral), compromised vein outflow, or axoplasmic transport

*NOT associated with visual loss*

Optic neuritis

Information for Condition:

Inflammation of the optic nerve, causing a decrease in visual acuity and changes in color perception

-May be due to Multiple Sclerosis

Phthisis bulbi

Information for Condition:

Diseased/damaged/atrophied eyeball that has lost function and shrunk; Pt also has blindness in eye

Can be from any cause