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Ординатура / Офтальмология / Английские материалы / Ocular Differential Diagnosis 7th edition_Roy_2002

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& Wilkins, 2002.

Periarteritis Retinalis Segmentalis

White or yellow plaques are arranged in segments encircling arteries like a cuff and are localized to one or more arterial branches.

1. *Arteriosclerosis secondary to vein obstruction

2. Herpes zoster

3. Hypercholesterolemia

4. Lupus erythematosus (disseminated lupus erythematosus)

5. Metastatic uveitis

6. Periarteritis nodosa (necrotizing angiitis)

7. *Sarcoidosis syndrome

8. Syphilis (acquired lues)

9. *Temporal arteritis (giant cell arteritis)

10. *Tuberculous retinitis

11. Uveitis, idiopathic

Crouch ER, Goldberg MF. Retinal periarteritis secondary to syphilis. Arch Ophthalmol 1975;93:384â??387.Bibliographic Links

Rask AJ. Peri-arteritis retinalis segmentalis. Acta Ophthalmol 1969;47:234â??237.Bibliographic Links

Frosted-Branch Angitis

In this condition, unusual thick sheathing surrounds all the retinal veins and less often the arteries, making them look like frosted tree branches.

1. Herpes simplex viruses types 1 and 2

2. Acute lymphoblastic leukemia

3. Large cell lymphoma

4. Crohn disease

5. Systemic lupus erythematosus

6. Acquired immune deficiency syndrome

Kim TS, et al. Retinal angiopathy resembling unilateral frosted branch angiitis in a patient with relapsing acute lymphoblastic leukemia. Am J Ophthalmol 1994;117,6:806â??808.

Ridley ME, et al. Retinal manifestations of ocular lymphoma. J Ophthalmol

1992;99,7:1153â??1161.

Sheathing of Retinal Veins

In this condition, white or gray envelopes are around veins; retinal or vitreous hemorrhage and exudates may be present.

1. Disc onlyâ??developmental

2. Disc and retinaâ??papillitis or papilledema P.465

3. Peripheral sheathing

A.*Acute retinal necrosis

B.Amebiasis

C.Behçet disease (dermatostomatoophthalmic syndrome)

D.Brucellosisâ??rare, tortuosity and sheathing of veins, vitreous haze, retinal hemorrhages

E.Candidiasis

F.Coccidioidomycosis

G.Eales disease (periphlebitis)

H.Diabetes mellitus

I.Filariasisâ??hemorrhages and exudates

J.Hypertension

K.Infectious mononucleosisâ??peripheral or central perivascular involvement, venous engorgement and sheathing associated with retinal hemorrhages

L.Lupus erythematosus

M.Non-Hodgkin lymphoma

N.Onchocerciasis syndrome (river blindness)

O.Rickettsial infectionsâ??peripheral or central perivascular involvement, venous engorgement and sheathing associated with retinal hemorrhages

P.*Sarcoidosis

Q.Septicemia and bacteremiaâ??rare, venous engorgement, usually with multiple hemorrhages and focal sheathing

R.Sickle cell disease

S.Syphilis (secondary) (acquired lues)

T.Tuberculin or bacille Calmetteâ??Guérin (BCG) vaccinationâ??rare, sectorial, or generalized changes

U.Viral infections, including the following:

1. Cytomegalovirus retinitis

2. Herpes simplex (likely responsible for acute retinal necrosis) 3. Herpes zoster ophthalmicus

4. Influenza

5. Rift Valley fever

4. Peripheral sheathing without secondary retinopathyâ??multiple sclerosis

5. Wide and usually dense sheathing of dilated and tortuous veins, suggestive of myelogenous leukemia

Brown S, et al. Intraocular lymphoma presenting as retinal vasculitis. Surv Ophthalmol 1994;39:138â??140.

George RK, et al. Primary retinal vasculitis. Opthalmology 1996;103:384â??389.

Kohno T, et al. Ocular manifestations of adult T-cell leukemia/lymphoma. Ophthalmology 1993;100:1794â??1799.Bibliographic Links

Roy FH. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2002.

Absent Venous Pulsations (Spontaneous Venous Pulsations Absent at Venules on the Disc)

1. Normal individuals

2. *Impending central vein occlusion (see p. 468) 3. *Papilledema (see p. 593â??601)

Ballantyne AJ, Michaelson IC. Textbook of the fundus of the eye, 3rd ed. Baltimore: Williams & Wilkins, 1981.

Newell FW. Ophthalmology: principles and practice, 7th ed. St. Louis: CV Mosby, 1991.

Diagnostic table

Central retinal vein occlusion

View Table

P.466

Dilated Retinal Veins

Normally, the arterioleâ??venule ratio is 2:3; with an increase in this ratio, the retinal veins may be dilated.

1. Congenital

A.Congenital tortuosity of retinal vesselsâ??rare, sometimes associated with coarctation of aorta

B.Fabry disease (hereditary dystrophic lipidosis)

C.Hemangioma

D.Longfellowâ??Graether syndrome

E.Ocular fundi in newborns

F.Racemose (arteriovenous) aneurysmâ??rare, arteriovenous anastomoses localized to sector of retina

G.*Retinopathy of prematurity with plus disease

H.von Hippelâ??Lindau disease (angiomatosis)â??familial in 20% of cases, bilateral in 50%

2. Trauma and inflammation

A.Anterior uveitisâ??dilatation of veins, often slight hyperemia of disc

B.Carotid-cavernous fistulaâ??fracture of base of skull, progressive exophthalmos, bruit

C.Cavernous sinus thrombosisâ??rare, proptosis and orbital edema

D.*Impending obstruction of the central retinal vein

E. Periphlebitisâ??sheathing of vessels

3. Cardiovascular diseaseâ??dilatation may be present but rarely dominates the fundus picture

A.Arteriosclerosis

B.Involutionary sclerosis (later stages)

C.Secondary to defective arterial flow, such as in the following:

1. Aortic arch syndrome (pulseless disease)

2. Cardiac insufficiency

3. Congenital heart disease

4. Iatrogenic (lowering of blood pressure)

5. Severe blood loss

6. *Stenosis or occlusion of common carotid

7. *Temporal arteritis

8. Venous stasis (hypotensive retinopathy of Kearns and Hollenhorst)

D.Heritable thrombophilia and hypofibrinolysis

4. Respiratory diseaseâ??venous dilatation may occur with purplish hue of whole retina; obstruction of venous return from the head, such as in the following:

A.Congenital septal defect (Fallot tetralogy)

B.Emphysema

C.Hammanâ??Rich syndrome (diffuse pulmonary fibrosis syndrome)

D.Heart failure of any type

E.Kartagener syndrome (sinusitisâ??bronchiectasisâ??situs inversus syndrome)

F.Mechanical compression of chest

G.Mediastinal tumor obstructing superior vena cava

5. Diseases of the central nervous system

A.*Carotid-cavernous fistulaâ??fractured base of skull; rupture of berry aneurysm, arteriosclerosis

P.467

B.Hemangioma of posterior fossaâ??rare, papilledema, often grossly dilated veins

C.Optic nerve lesionâ??rare, secondary to orbital space-occupying lesion

D.*Papilledema (see p. 593â??601)

E.Retrolenticular syndrome (Dejerineâ??Roussy syndrome)

F.*Subarachnoid hemorrhageâ??head injury; subhyaloid hemorrhages near disc, dilated veins, sometimes papilledema

6. Blood diseases

A.Aplastic anemiaâ??hemorrhage is the most striking sign, often spreading around the disc

B.Cryoglobulinemiaâ??rare, may occur with multiple myeloma, veins dilated, tortuous, and sometimes beaded

C.Gansslen syndrome (familial hemolytic icterus)

D.Lymphatic leukemia

E.Macrocytic anemia of all typesâ??common, retinopathy absent unless hemoglobin below 50%; pale fundus, superficial hemorrhages, cotton-wool spots

1. Pernicious anemia

2. Steatorrhea

3. Celiac disease

4. Carcinoma of stomach

F.Macroglobulinemiaâ??rare; veins dilated tortuous and sometimes beaded, hemorrhages and occasionally microaneurysms

G.Monocytic leukemia

H.Myelogenous leukemia

I.Multiple myeloma (Kahler disease)

J.Polycythemia rubra vera (primary; Vaquez disease)â??common in males; hemorrhages; papilledema may be marked and venous thrombosis may occur

K.Secondary polycythemiaâ??common; hemorrhages, papilledema and venous thrombosis may occur

L.Sickle cell diseaseâ??dilatation of peripheral veins with retinal, subhyaloid, and vitreous hemorrhages

M.Thrombocytopenic purpuraâ??retinal and subhyaloid hemorrhages near disc, moderate venous dilatation

7. Acute febrile illnessesâ??rare, occasional dilatation of retinal veins with a few hemorrhages and mild edema of disc

A.Infectious mononucleosis

B.Influenza

C.Rickettsial infections

D.Septicemia

8. Metabolic diseases

A.Cystic fibrosis syndrome (fibrocystic disease of pancreas)â??venous congestion often swelling of disc

B.Plasma lecithin

C.*Diabetic retinopathyâ??larger veins affected, often beaded

9. Collagen diseases

A.Polyarteritis nodosaâ??among other fundus lesions, dilated veins may occur

B.Sclerosis, progressive systemic (scleroderma)

P.468

C.Systemic lupus erythematosusâ??cotton-wool spots, occasional hemorrhages, and moderate dilatation of veins

10. Toxic conditions, such as methyl alcohol ingestion

Glueck CJ, et al. Heritable thrombophilia and hypofibrinolysisâ??possible causes of retinal vein occlusion. Arch Ophthalmol 1999;117:43â??49.Bibliographic Links

Newell FW. Ophthalmology: principles and practice, 7th ed. St. Louis: CV Mosby, 1991.

Roy FH. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2002.

Tortuosity of Retinal Veins and Hypoplasia of Optic Nerves

1. Endocrinopathy, especially pituitary deficiency

2. Alcohol abuse; fetal alcohol syndrome

3. Migraine disturbances

4. Agenesis of the corpus callosum associated with mutations

5. Preterm birth also associated with retinal artery/visceral tortuosity

Hellstrom A, et al. Optic nerve hypoplasia with isolated tortuosity of the retinal veins. Arch Ophthalmol 1999;117:880â??884.Bibliographic Links

Central Retinal Vein Occlusion

This condition is characterized by massive hemorrhage into the posterior portion of the eye and dilated retinal veins.

1. External compression of the vein

A.Atherosclerosis of central retinal artery

B.Connective tissue strand within the floor of the physiologic excavation

C.Multiple crossings of the same artery and vein or congenital venous loops or twists in the retinal surface

D.Pseudotumor cerebri

2. Degenerative or inflammatory venous disease, causing detachment, proliferation, and hydrops

A.Acquired immunodeficiency syndrome (AIDS; HIV retinopathy)

B.Arterial hypertension

C.Arteriovenous malformations of retina

D.Cardiac decompensation

E.Closed-head trauma

F.*Diabetes mellitus (Willis disease)

G.Ipsilateral internal carotid artery stenosis

H.Lyme disease

I.Optic disc drusen

J.Optic nerve inflammation

K.Sarcoidosis

L.Serpiginous choroiditis

M.Systemic granulomatous disease, particularly tuberculosis

3. Thrombosis from venous stagnation

A.Spasm of corresponding retinal arterioles

B.Blood dyscrasias

1. Cryoglobulinemia

2. Emphysema with secondary erythrocytosis P.469

3. Deficiencies in endemic pathway (factor V R506Q mutation) 4. Homocystinemia

5. Increased platelet aggregation

6. Leukemias

7. Lymphoma

8. *Multiple myeloma

9. *Polycythemia vera

10. Sickle cell disease

C.Increased viscosity of the blood

1. Cystic fibrosis of pancreas

2. Following peritoneal dialysis

3. Hyperproteinemia

4. *Macroglobulinemia

5. Use of tranexamic acid

D.Sudden reduction of systemic blood pressure because of cardiac decompensation, surgical or traumatic shock, or therapy for arterial hypertension

E.*Glaucoma (preexisting)

F.Increased risk of thrombosis 1. Hereditary

a.Antithrombin III deficiency

b.Protein C deficiency or protein S deficiency

c.Rare disorders of fibrinogen and fibrinolysis

i.Certain dysfibrinogenemias

ii.Abnormal plasminogen

2. Acquired

a.Carcinoma

b.Hematologic proliferative disorders

i.Acute promyelocytic leukemia

ii.Myeloproliferative disorders (polycythemia, essential thrombocythemia)

c.Behçet syndrome

d.Lupus anticoagulant

e.Nephrosis

f. Complications of therapy

i.Oral contraceptives

ii.Infusion of prothrombin complex concentrates

iii.Heparin-induced thrombocytopenia

G.Carotidâ??cavernous sinus fistula

H.*Syphilis

I.With immunoglobulin G (IgG) lambda monoclonal gammopathy

J.Coil embolization of carotidâ??ophthalmic aneurysms

K.Oral contraceptive

Castillo B, et al. Retinal artery occlusion following coil embolization of carotidâ??ophthalmic aneurysms. Arch Ophthalmol 2000;118:851â??852.Bibliographic Links

Enzenauer RW, et al. Central retinal vein occlusion in a patient with IgG lambda monoclonal gammopathy. Arch Ophthalmol 1999;117:134â??135.Bibliographic Links

Greiner K, et al. Retinal vascular occlusion and deficiencies in the protein C pathway. Am J Opthalmol 1999;128:69â??74.Bibliographic Links

P.470

Hayreh SS, et al. Systemic diseases associated with various types of retinal vein occlusion. Am J Ophthalmol 2001;131:61â??78.Bibliographic Links

Rath EZ, et al. Risk factors for retinal vein occlusions: a case-control study. Ophthalmology 1992;99:509â??514.Bibliographic Links

Sagripanti A, et al. Thrombinâ??antithrombin III complex in acute retinal vein occlusion. Am J Ophthalmol 1999;128,1:124.

Wenzler EM, et al. Hyperhomocystinemia in retinal artery and retinal vein occlusion. Am J Ophthalmol 1993;115:162â??167.Bibliographic Links

P.471

P.472

P.473

Dilated Retinal Veins and Retinal Hemorrhages

1. Carotidâ??cavernous fistula

2. *Cavernous sinus fistula syndrome (carotid artery syndrome)

3. Cavernous sinus thrombosis (hypophyseal-sphenoidal syndrome) 4. *Central retinal vein occlusion (see p. 468)

5. Cervical tuberculosis

6. Choroidal melanoma remote to the neovascularization 7. Congenital tortuosity and dilatation of the retinal vessels 8. Cryoglobulinemia

9. *Diabetes mellitus

10. Intravitreal myiasis

11. Leukemia

12. Lymphomas

13. Macroglobulinemia (Waldenström syndrome)

14. Multiple myeloma (myelomatosis)

15. Ophthalmic vein thrombosis

16. Pappataci fever (phlebotomus fever)

17. Paraproteinemias and dysproteinemias

18. Polycythemia vera

19. Retinal arteritis

20. *Sickle cell disease

21. *Syphilis (acquired lues)

Kalina RE, Kaiser M. Familial retinal hemorrhages. Am J Ophthalmol 1972;74:252â??255.Bibliographic Links

Roy FH. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2002.

Retinal Hemorrhages

Retinal hemorrhages include bleeding that may be intraretinal or preretinal hemorrhages into the vitreous or subretinal hemorrhages.

1. Congestion of the head and neck, such as in newborns, in hanging, or during choking 2. Trauma, including electrical injury, hypothermal injury, and child abuse

3. Vascular obstruction, such as cardiorespiratory obesity syndrome, cystic fibrosis syndrome, negative acceleration syndrome, (hydrostatic pressure syndrome), ophthalmoplegic migraine syndrome, papilledema (see p. 593â??601), subarachnoid hemorrhages, superior vena cava syndrome, Symonds syndrome (benign intracranial hypertension), thrombocytopenia, thrombosis, and Wernicke syndrome (avitaminosis B1)

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