Other Vascular Disorders, Including Carotid Occlusive Disease, Anemias, & Leukemias - Posterior Segment & Ocular Disease Spring 2026

blockage of carotid or ophthalmic artery

What is the cause of hypoperfusion syndrome?

dull ache in or around the eye (ocular pain), TIA symptoms, light induced amaurosis fugax, carotid bruit, decreased carotid pulse

What are the systemic signs of hypoperfusion syndrome?

ophthalmoscopy shows unilateral venous dilation, narrowed arteries, venous beading, non-tortuous vessels, mid-peripheral hemes, retinal emboli

What are the ocular signs of hypoperfusion syndrome?

carotid and heart

Most common origin of retinal emboli?

ocular ischemic syndrome (OIS)

Hypoperfusion syndrome is a precursor to what?

-hypoperfusion

-signs of hypoxia-induced inflammation

**chronic condition

What is ocular ischemic syndrome (OIS) a combo of?

-uveitis

-slugglish pupils

-conj congesiton

-corneal edema

-NVD/NVE/NVI

Hypoxia-induced inflammation can include what conditions?

Internal Carotid Stenosis Before and After a Stent (Pic)

Internal Carotid Stenosis Before and After a Stent (Pic)

carotid stenosis

Ocular Ischemic Syndrome (OIS) is most significant in patients with what?

10; 70

Ocular Ischemic Syndrome (OIS) only occurs in ____% of eyes with ____% or more blocked carotids

-Decrease VA (inflammation in cornea and AC)

-Ocular pain (d/t inflammation & blockage of TM increasing IOP)

-Unilateral red or injected eye

-Corneal Edema with potential folds in Descemets membrane

-Anterior chamber inflammation/uveitis

-Neovascularization

What are the ocular signs of Ocular Ischemic Syndrome (OIS)?

Mid Peripheral Retinal Hemorrhages (Pic)

Mid Peripheral Retinal Hemorrhages (Pic)

Diabetes

What is the #1 differential of this?

80

REVIEW: OIS is _____% unilateral

bilateral

REVIEW: Diabetic Retinopathy is more likely to be (unilateral/bilateral)

-non-tortuous vessels in OIS

-tortuous vessels with diabetic retinopathy

REVIEW: OIS demonstrates what signs in the blood vessels compared to diabetic retinopathy?

mid-peripheral

REVIEW: OIS often shows a preponderance of _______ hemes

anywhere

REVIEW:

Diabetic hemes appear ______

-Systemic vascular workup (FBS, BP, CBC, ESR or CRP, lipid profile)

-Carotid evaluation (Doppler or Duplex ultrasound and/or angiogram)

-Refer to ophthalmology

What is the management of OIS?

It would be difficult to control the development of neovascular glaucoma by ourselves

Why do you refer patients with OIS to ophthalmology?

-increase in size of the foveal avascular zone

-reduced vessel area density (VAD)

What are the signs that may be present on OCT-A with OIS?

inherited disorder with abnormal erythrocyte function -- RBCs are rigid and elongated

What are sickle hemoglobinopathies?

d/t substitution of single amino acids

Why is there abnormal hemoglobin structure with sickle hemoglobinopathies?

distorted Hb decreases RBC O2-transport capability

What is the consequence of abnormal hemoglobin structure with sickle hemoglobinopathies?

-hemolytic anemia

-RBCs are eliminated early because the body recognizes them as foreign

Sickle cell hemoglobinopathies are sometimes considered a ______ anemia. What does this mean?

malaria

Sickle cell hemoglobinopathies are common where _____ is endemic

protects against malarial infections

Why is sickle cell endemic in areas of malaria?

10

Sickle cell hemoglobinopathy is present in about ___% of African Americans

normal adult

Sickle Hemoglobinopathies

HbA

normal fetal

Sickle Hemoglobinopathies

HbF

sickled -- substitution of valine for glutamate

Sickle Hemoglobinopathies

HbS

sickled -- substitution of lysine for glutamate

Sickle Hemoglobinopathies

HbC

diminished production of one or two subunits of the hemoglobin molecule

What is thalassemia?

sickle cell anemia (SS)

Inheritance Patterns of Sickle Hemoglobinopathies

HbS + HbS

HbS + HbS

Inheritance Patterns of Sickle Hemoglobinopathies

What is the most severe form?

sickle cell trait (SA)

Inheritance Patterns of Sickle Hemoglobinopathies

HbS + HbA

Asymptomatic; no anemia

Inheritance Patterns of Sickle Hemoglobinopathies

Symptoms of HbS + HbA

sickle cell C disease

Inheritance Patterns of Sickle Hemoglobinopathies

HbS + HbC

moderate severity

Inheritance Patterns of Sickle Hemoglobinopathies

What is the severity of HbS + HbC

Sickle cell thalassemia (SThal)

Inheritance Patterns of Sickle Hemoglobinopathies

HbS + Thal

moderate severity

Inheritance Patterns of Sickle Hemoglobinopathies

What is the severity of HbS + Thal

Hemoglobin C trait

Inheritance Patterns of Sickle Hemoglobinopathies

HbA + HbC

rare systemic problems

Inheritance Patterns of Sickle Hemoglobinopathies

What is the consequence of HbA + HbC

SS (sickle cell anemia), SC (sickle cell disease), and possibly SThal (sickle cell thalassemia)

Sickle cell ocular changes are mainly talking about what forms of sickle cell hemoglobinopathies?

if they have other associated systemic conditions

A patient with SA (sickle cell trait), AC (hemoglobin C trait) could manifest ocular problems is what?

alteration of retinal circulation by sickled hemoglobin coupled with hypoxia particularly in the peripheral retina

Sickle Cell Ocular Changes result from what?

-conj sickling

-iris atrophy

-hyphema

What are the anterior segment changes from sickle cell anemia?

Sickle cell testing should be ordered

If a hyphema appears in a child of African Descent, what do you have to do?

rigid red erythrocytes cannot get through the TM

Any hyphema with sickle cell hemoglobinopathy carries increased risk for severe IOP increase. Why?

-retinal dark without pressure

-salmon patch intraretinal hemorrhages

-black sunburst lesion (RPE hyperplasia in the areas of old hemorrhages)

-Tortuous veins

-Angioid streaks (cracks in Bruch's membrane)

What are the nonproliferative retinal signs of sickle cell anemia?

Conjunctival Sickling -- Comma Sign (Pic)

Conjunctival Sickling -- Comma Sign (Pic)

Retinal Dark w/o Pressure (Pic)

Retinal Dark w/o Pressure (Pic)

No -- most patients with this DO NOT have sickle cell

Is retinal dark w/o pressure an indication to order sickle cell testing?

Salmon Patch Hemorrhages and Black Sunburst (Pic)

Salmon Patch Hemorrhages and Black Sunburst (Pic)

**RBCs deoxygenated in salmon patch

Salmon Patch Hemorrhages -- most of the times the hemes look like dot-blot or flame hemes

What is BY FAR the least common heme in sickle cell disease?

Angioid Streaks (Pic)

Angioid Streaks (Pic)

choroidal neovasc

Whenever you compromise Bruch's membrane, what are you at risk for 70% of the time?

protective lenses (polycarb)

What should you recommend for patients with Angioid streaks?

-PEPSI-HAM

-Pseudoxanthoma elasticum

-Ehlers-Danlos syndrome

-Paget's disease of bone

-Sickle cell disease and other hemoglobinopathies

-Idiopathic

-Homocystinuria

-Acromegaly

-Marfan syndrome

EXAM QUESTION: What are the systemic associations with Angioid streaks?

-sea fan neovascularization -- possibly in the periphery

-fibrotic scaffolding (white)

-vitreous hemorrhage

-traction retinal detachment

What are the proliferative signs of sickle cell retinopathy?

Sea Fan Neovascularization (Pic)

Sea Fan Neovascularization (Pic)

proliferative sickle-cell retinopathy (PSR)

What is the most common cause of vision loss d/t sickle cell retinopathy?

-S thal

-SC

_____ and _____ patients consistently show higher rates of visual impairment from proliferative chanes

true

True or False:

Sickle cell disease can be detected through newborn screening

-Hb studies (HbS)

-CBC

What sickle cell bloodwork needs to be ordered for patients with unaccounted for hemes?

No -- relatively unlikely

Is diagnosis of sickle cell disease common for optometrists?

-sickle cell trait (SA)

-hemoglobin C trait (AC)

In what cases may it be possible to diagnose sickle cell anemia from an eye exam?

1) Decrease in red blood cells (Erythrocytes) OR

2) Decrease in hemoglobin

What are the general definitions of anemia?

Yes

Is it possible to have a normal erythrocyte count and low hemoglobin (iron deficiency)?

hemolysis -- where the erythrocytes are removed from the blood leaving unbound hemoglobin

If the erythrocytes are low and hemoglobin is normal, what is one thing to consider?

-Under production of blood in bone marrow, kidney, diet, absorption, liver

-Hemolytic

-Bleeding

-Anemia of chronic disease (kidney disease, cancer, autoimmune disorders, chronic infections)

What are the 4 major mechanisms of anemias?

iron deficiency anemia

What is the most common anemia in the US?

bleeding

What is the most common etiology for iron deficiency anemia?

hemoglobin

____ decreases as iron deficient anemia progresses

-cells normochromic (normal hemoglobin content)

-Normocytic (normal mean cell volume)

Characteristics of early stage iron deficient anemia

Normochromic and microcytic

Characteristics of middle stage iron deficient anemia

hypochromic and microcytic

Characteristics of final stage iron deficient anemia

-probably lower

-smaller and fewer RBCs

-less blood volume filled by the RBCs

Hematocrit in Iron Deficient anemia

yes

Are there specific tests to run for iron deficiency?

B12 deficiency -- crucial for the synthesis of nucleic acids which are involved in forming blood precursors

What is the etiology of pernicious anemia?

1) Hereditary autoimmune disorder to gastric mucosa secondary to surgery, infection, drugs, and digestive disorders

2) Nutritional deficiency

What are the 2 ways in which you can develop pernicious anemia?

-macrocytic (mean cell volume increased)

-normochromic

Characteristics of pernicious anemia

-elderly of northern European ancestry

-African Americans

-Latin-Americans

Who does pernicious anemia typically present in?

-conj pallor

-anemic retinopathy

-optic nerve head pallor from lack of perfusion

-blue sclera

-subconj hemorrhage

-retinal arterial or venous occlusions

-anterior ischemic optic neuropathy

Ocular findings of pernicious anemia

Not really, in a lot of cases of pernicious anemia this does not present

Is conj pallor useful in dx of pernicious anemia?

Conjunctival Pallor (Pic)

Conjunctival Pallor (Pic)

Iron supplements

What is the "cure" for iron deficient anemia?

yes

If a patient starts to take iron supplements for their iron deficient anemia, will the eye manifestations likely go away?

1) severe anemia

2) thrombocytopenia at the same time as the anemia

Under what 2 conditions would anemic retinopathy present?

Scleral thinning; can see the melanin in the choroid

What is a blue sclera d/t?

No -- there are usually no manifestations

Are retinal arterial or venous occlusions common with anemia?

Not high

EXAM QUESTION: What is the likelihood of ocular manifestations d/t anemia?

bone marrow disorder

What is aplastic anemia?

issues with red blood cells, white blood cells and platelets

What does aplastic anemia d/t?

more

EXAM QUESTION: Systemic and ocular manifestations (retina/optic nerve head involvement) are (more/less) likely with aplastic anemia than with iron deficiency/pernicious anemia

neoplatic disease of the bone marrow characterized by abnormal, uncontrolled proliferation of one or more hematopietic or lymphoid cells

EXAM QUESTION: What is leukemia?

13/100,000

What is the incidence of leukemia?

-Acute v chronic (time course)

-Myelogenous v Lymphocytic (cell type)

What is the classification of leukemia?

acute and rapidly fatal (immature cells are affected -- blasts)

Acute leukemia definition

More prolonged course (involve more differentiated -- mature cells)

Chronic leukemia definition