Saturday, 1 June 2019

DIFFERENTIAL DIAGNOSIS OF OCULAR SIGNS

TRICHIASIS (INWARD TURNING LASHES)


*1. Inflammation/infection
 A. Chronic blepharitis
 B. Herpes simplex or zoster
 C. Trachoma
2. Lid Tumors
 A. Basal cell carcinoma
 B. Capillary hemangioma
 C. Conjunctiva amyloidosis
3. Medications
 A. Epinephrine
 B. Idoxuridine
 C. Phospholine iodide
 D. Pilocarpine
 E. Practolol
 F. Trifluridine
 G. Vidarabine
4. Systemic/Immunologic Disorders
 A. Erythema multiforme
 B. Ocular cicatricial pemphigoid
 C. Stevens-Johnson syndrome
 D. Toxic epidermal necrolysis
 E. Vernal kertoconjunctivitis
5. Trauma
 A. Chemical injury (lye)
 B. Mechanical injury or repair of injury
  (1) Lower lid transconjunctival approach for floor fracture repair or           blepharoplasty
  (2) After enucleation
  (3) After ectropion repair
  (4) Thermal bums to face/lids
 C. Surgery


 PRESEPTAL CELLULITIS OF EYELID


1. Eczema
*2. Hordeolum
3. Neonatal conjunctivitis
4. Otitis media
5. Sinusitis
6. S. aureus
7. Toxic shock syndrome
8. Trauma
9. Upper respiratory tract
10. Varicella


PSEUDOPTOSIS

Pseudoptosis includes conditions that simulate ptosis, but lid droop is not the result of levator malfunction, and ptosis is usually corrected when the causative factors are cleared up or removed.
1. Due to globe displacement
 A. Anophthalmia including poorly fitting prosthesis
 *B. Enophthalmos such as that resulting from blowout fracture of the floor of the  orbit or atrophy of orbital fat
 *C. Microphthalmia
 *D. Phthisis bulbi
 E. Hypotony and inward collapse of eye
 F. Cornea plana
 G. Hypotropia of that eye or hypertropia of the other eye
2. Due to mechanical displacement of the lid
 A. Inflammation
  (1) Trachoma-thick, heavy lid
  *(2) Chalazion or hordeolum
  (3) Elephantiasis
  (4) Chronic conjunctivitis-conjunctival thickening
  (5) Traumatic or infectious edema involving the lid
  *(6) Blepharitis
  (7) Corneal foreign body
  (8) Contact lens
  (9) Sinusitis, cellulitis
 B. Tumors, especially fibromas, lipomas, or hemangiomas
 C. Scar tissue due to burns, physical trauma, and lacerations that can bind the lid  down
 D. Tumors of lacrimal gland-S-shaped lid
*3. Dermatochalasis (ptosis adiposa, baggy lids, "puffs" -senile atrophy of the lid skin)
4. Blepharochalasis-a rare condition occurring in young persons, characterized by recurrent bouts of inflammatory lid edema with subsequent stretching of the skin
5. The oriental lid-the palpebral fissure is narrower than normal and the upper lid rarely has a furrow; hence, the fold usually hangs down to or over the lid margin.
6. Dissociated vertical deviation (DVD)
7. Duane syndrome (retraction syndrome)
8. Blepharospasm-eyebrow lower than normal, hemifacial spasm
9. Contralateral widening of the lid fissure as pseudoproptosis (see p. 4), exophthalmos, or lid retraction (see p. 62)
10. Vertical strabismus

BILATERAL MACULAR LESIONS

1. Development defects (colobomas)
2. Drugs, including the following:
 
allopurinol (?)
amodiaquine
broxyquinoline
chloroquine
chlorpromazine
clonidine (?)
griseofulvin (?)
hydroxychloroquine
ibuprofen (?)
indomethacin (?)
iodochlorhydroxyquin
iodoquinol
quinine
thioridazine
3. Infectious entities
 A. Herpes simplex
 *B. Cytomegalic retinitis
 C. Candidiasis and nocardiosis
 D. T. canis (visceral larva migrans syndrome)
 E. Congenital syphilis
 *F. Tuberculosis
 *G. Ocular histoplasmosis
 H. Congenital toxoplasmosis
4. Intrauterine inflammations
5. Noninfectious entities
 A. Best disease
 B. Stargardt disease
 *C. Exudative age-related macular degeneration
6. Presumed inflammatory origin


MACULAR PUCKER

Macular pucker involves tiny folds that often are arranged in a stellate manner around macula and usually are associated with a preretinal membrane (preretinal macular fibrosis, preretinal vitreous membrane, surface wrinkling retinopathy, cellophane maculopathy).
1. Associated with proliferative retinopathy
 *A. Diabetes retinopathy
 B. Eales disease
 *C. Hypertension
 D. Sickle cell disease
 E. Vein occlusion
2. Central serous chorioretinopathy
 A. Psychopharmacologic medication use
 B. Corticosteroid use
 C. Hypertension
3. Congenital
4. Following photocoagulation or cryoretinopexy
5. Following traumatic posterior vitreous separation, such as blunt trauma to the eye and whiplash injury (craniocervical syndrome)
6. Loss of formed vitreous at operation
7. Idiopathic (probably related to spontaneous posterior vitreous detachment)
8. Macular detachment
9. Multiple retinal operations
10. Penetrating or blunt injuries
11. Posterior uveitis
* 12. Proliferative vitreoretinopathy following vitreoretinal surgery
13. Retinal detachment
14. Trauma (blunt)
15. Vitreous hemorrhage

HERRY-RED SPOT IN MACULA

1. Cardiac myxomas
2. Cryoglobulinemia
3. Dapsone poisoning
4. Hallervorden-Spatz disease (pigmentary degeneration of globus pallidus)
5. Hollenhorst syndrome (chorioretinal infarction syndrome)
6. Hurler syndrome (MPS I-H)
*7. Hypertension (severe)
8. Intralesional chalazion corticosteroid injection
9. Leber congenital amaurosis
10. Macular hemorrhage
* 11. Macular hole with surrounding retinal detachment
12. ML I (lipomucopolysaccharidosis)
13. Myotonic dystrophy syndrome (Curschmann-Steinert syndrome)
14. Multiple sulfatase deficiency
*15. Occlusion of central retinal artery (see p. 457)
16. Quinine toxicity
17. Sphingolipidoses
 A. Cherry-red spot myoclonus
 B. Farber syndrome (Farber lipogranulomatosis)
 C. Gangliosidosis GM1-type (juvenile gangliosidosis)
 D. Gaucher disease (glucocerebroside storage disease)
 E. Goldberg syndrome
 F. Infantile metachromatic leukodystrophy (van Bogaert-Nyssen disease)
 G. Niemann-Pick disease type A
 H. Niemann-Pick disease type B
 I. Sandhoff disease (gangliosidosis GM2-type 2)
 *J. Tay-Sachs disease (gangliosidosis GM-type I)
18. Steroid injection intranasally
*19. Temporal arteritis (giant cell arteritis)
20. Traumatic retinal edema (commotio retinae; Berlin edema)
21. Vogt-Spielmeyer cerebral degeneration (Batten-Mayou syndrome)

 MACULAR HOLE

Macular hole must be differentiated from macular cyst with Hruby lens or contact lens and slit lamp)
* 1. Idiopathic (most common, may be bilateral)
  2. From the following:
 A. Edema (see p. 439)
  (1) Inflammatory
  (2) Toxic
  (3) Vascular
  (4) Following papilledema
 B. High myopia
 C. Ischemic, such as with retinal detachment or choroidal tumor-the macula is  separated from choriocapillaris
 D. Degenerative conditions of the retina and retinal dystrophy
 E. Trauma
 F. Radiation injury
 G. Glaucoma
 H. Posterior senile retinoschisis
 I. High tension electric shock
 J. Central serous chorioretinopathy
 K. Optic disc coloboma
 L. Posterior retinal detachment associated with optic pits
 M. Industrial laser bums
 N. Lightening-induced
 O. Posterior microphthalmos
 P. Septic embolization
 Q. Subhyaloid hemorrhage
 R. Topical pilocarpine use
 S. YAG laser
3. Dawson disease (subacute sclerosing panencephalitis)
4. Foveomacular retinitis-usually young males
*5. Pseudohole due to epiretinal membrane (may differentiated from true hole by fluorescein angiography)
6. Sickle cell disease





 BULL'S-EYE MACULAR LESION

1. Autosomal-dominant, benign, concentric annular macular dystrophy
2. Ceroid lipofuscinosis
*3. Chloroquine or hydroxychloroquine retinopathy
4. Cone dystrophy
5. Hereditary ataxia
6. NARP syndrome
7. Spielmeyer-Vogt syndrome (Batten-Mayou syndrome)
*8. Stargardt disease (or fundus flavimaculatus)
9. Trauma
10. Unknown

RETINAL ARTERY OCCLUSION

This condition involves a sudden, painless visual loss; on ophthalmoscopic examination, a diffuse retinal pallor and a cherry-red spot in macula are noted.
1. Embolism-cardiac or pulmonary sources
 A. Air emboli following trauma or surgery
 B. Amniotic fluid embolization
 C. Cardiac myxoma
 D. Corticosteroid emboli
 E. Espildora-Luque syndrome (ophthalmic Sylvian syndrome)
 F. Fat emboli following long-bone fractures
 G. Iatrogenic trauma induced by angiography
 *H. In older patients-due to atheroma of carotid artery
 I. In young persons-due to postrheumatic vegetations (rheumatic fever), cardiac  catheterization, or valvotomy
 J. Leudoemboli - vasculitis, Purtscher retinopathy, septic endocarditis
 K. Moyamoya disease (multiple progressive intracranial arterial occlusion)
 L. Nicolau syndrome (emboli of medication inadvertently introduced into artery)
 M. Synthetic material used in cardiac and vascular procedures
 N. Talc emboli-long-term intravenous drug abusers
 O. Tumors-atrial myxoma, mitral valve papillary fibroelastoma
 P. With cerebral infarction after periocular subcutaneous cosmetic silicone  injection
*2. Atherosclerosis of common carotid artery (ophthalmodynamometry employed for diagnosis)
3. Ischemia
 A. Carotid occlusion or dissection
 B. Essential hypotension
 C. Following orbital floor fractures or repair
 D. Following surgery for retinal detachment
 E. Generalized shock
 F. Heart failure (rare)
 G. Kahler disease (multiple myeloma)
 H. Knee-chest position
 I. Massive hemorrhage, such as that occurring in hematemesis. Gastrointestinal  bleeding, or surgical procedures
 J. Migraine
 K. Mosse syndrome (polycythemia-hepatic cirrhosis syndrome)
 L. Orbital hemorrhage following retrobulbar injection
 M. After surgery for scoliosis
 N. Too rapid lowering of blood pressure in hypertensive subjects
4. Inflammation
 A. Abdominal typhus (typhoid fever)
 B. African eye-worm disease (loiasis)
 C. Arteriole vasculitis, such as periarteritis nodosa (Kussmaul disease)
 D. Bacterial endocarditis
 E. Behçet disease (dermatostomatoophthalmic syndrome)
 F. Diphtheria
 G. Familial factor V Leiden polymorphism and positive rheumatoid factor
 H. Giant cell arteritis
 I. Herpes zoster
 J. Metastatic bacterial endophthalmitis
 K. Mucormycosis (phycomycosis)
 L. Pancreatitis
 M. Recurrent toxoplasmic retinochoroiditis
 N. Rocky Mountain spotted fever (spotted fever)
 O. Rubeola (measles)
 P. Subacute bacterial endocarditis
 Q. Systemic lupus erythematosus
 R. Takayasu disease (pulseless disease)
 *S. Temporal arteritis
 T. Toxoplasma retinochoroiditis
 U. Varicella (chickenpox)
5. Blood disease
 A. After platelet transfusion
 B. Following ocular trauma with secondary glaucoma in youths with sickle-trait  hemoglobinopathy
 C. Polycythemia vera (Vaquez-Osler syndrome)

 D. Sickle cell disease
6. Syphilis (acquired lues)
7. Associated factors
8. Diathermy of persistent hyaloid
 A. Drusen of optic nerve (see p. 559-560)
 B. Giant cell arteritis
 C. Papilledema (see p. 593-601)
 D. Subdural cerebral hemorrhage
 E. Arteriosclerosis of central retinal artery
 F. Chronic simple glaucoma
9. After dye, yellow photocoagulation
10. Complication of retrobulbar block
11. Degos syndrome (malignant atrophic papulosis)
12. Disseminated lupus erythematosus
13. Fabry-Anderson syndrome (glycosphingolipid lipidosis)
14. Goldenhar-Gorlin syndrome (oculoauriculovertebral dysplasia)
15. Homocystinuria syndrome
16. Hyperhomocystinemia
17. Lyme disease
18. Neoplastic angioendotheliomatosis
19. Polymyalgia rheumatica
20. Protein S deficiency
21. Relapsing polychondritis
22. Sneddon syndrome (livedo reticularis, neurologic abnormalities, and labile hypertension)
23. Use of tranexamic acid therapy

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
  (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. Carotid-cavernous sinus fistula
 *H. Syphilis
 I. With immunoglobulin G (IgG) lambda monoclonal gammopathy
 J. Coil embolization of carotid-ophthalmic aneurysms
 K. Oral contraceptive

RETINAL NEOVASCULARIZATION

1. Anemia
2. Behçet syndrome (dermatostomatoophthalmic syndrome)
*3. Central retinal vein occlusion (see p. 468)
*4. Diabetes mellitus
5. Eales disease (periphlebitis)
6. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
7. Hypertension (malignant and essential)
8. Leukemia
9. Lupus erythematosus
10. Macroglobulinemia (Waldenstrom syndrome)
11. Retinal detachment with hemorrhage
*12. Sickle cell disease
13. Syphilis (acquired lues)
14. Trauma
15. von Hippel-Lindau syndrome (retinocerebral angiomatosis)
16. Werlhof disease (hemophilia and thrombocytopenic purpura)





RETINAL DETACHMENT

1. Equator
 A. Myopic type-equatorial horseshoe tear
 B. Equatorial type associated with lattice degeneration
2. Ora serrata
 A. Aphakic, with multiple small breaks often in nasal periphery
 B. Dialysis in young, lower temporal quadrant, often bilateral
 C. Giant dialysis, often bilateral
3. Posterior pole
 A. Macular breaks, rare
 B. Other breaks at posterior pole, from cellular proliferation in inner retinal  surface

COTTON-WOOL SPOTS

These spots are soft exudates (fluffy, white, focal infarcts in the nerve fiber layer).
1. Acute pancreatitis
2. Amniotic fluid embolization
3. Anemic conditions
 A. Cirrhosis of the liver
 B. Following cardiac surgery
 C. Gastric ulcer syndrome
 D. Hypotensive retinopathy
 E. Ligation of the carotid artery
 F. Severe primary and secondary anemias
 G. Severe systemic blood loss
4. Blood disease
 A. Aplastic anemia
 B. Dysproteinemia
 C. Leukemia
 D. Multiple myeloma (myelomatosis)
 E. Pernicious anemia (vitamin B12 deficiency)
 F. Waldenström syndrome (macroglobulinemia syndrome)
5. Carbon monoxide poisoning
6. Carcinomatous cachexia
7. Collagen diseases
 A. Dermatomyositis (polymyositis dermatomyositis)
 B. Diffuse scleroderma
 C. Disseminated lupus erythematosus (systemic lupus erythematosus)
 D. Polyarteritis nodosa (necrotizing angiitis)
 E. Rheumatoid arthritis with scleromalacia perforans or polymyalgia                           rheumatic a
*8. Diabetic retinopathy
9. Hodgkin disease
10. Infective conditions
 *A. HIV
 B. Pneumonia
 C. Rheumatic fever
 D. Rift Valley fever
 E. Rocky mountain spotted fever (spotted fever)
 F. Roth septic retinitis
 G. Subacute bacterial endocarditis
11. Microemboli following cardiac operation
12. Primary amyloidosis (idiopathic amyloidosis)
13. Primary open-angle glaucoma
14. Protein C and protein S deficiency
15. Purtscher retinopathy (fat embolism syndrome)
16. Renal disease
17. Serum disease
18. Suprarenal-sympathetic syndrome (pheochromocytoma syndrome)
19. Takayasu syndrome (aortic arch syndrome)
20. Toxemic retinopathy of pregnancy
*21. Untreated malignant hypertension

HARD EXUDATES

1. Circinate retinopathy
2. Coats disease (retinal telangiectasia)
*3. Diabetes mellitus
*4. Exudative age-related macular degeneration
*5. Hypertensive disease
6. Radiation induced
7. Retinal arterial macroaneurysm

 RETINAL EXUDATE AND HEMORRHAGE

1. Capillary telangiectasis of retina (Reese)
2. Coats disease (retinal telangiectasia)
*3. Diabetes mellitus
4. Eales disease (periphlebitis)
5. Multiple retinal aneurysms (Leber syndrome)
6. Racemose hemangioma of the retina
7. von Hippel-Lindau, with absence of visible angioma (retinocerebral angiomatosis)
RETINITIS OR PSEUDORETINITIS PIGMENTOSA

Pigment may be bone corpuscular dots or heaped-up masses; salt and pepper fundus
1. Retinitis pigmentosa
 A. Abetalipoproteinemia (Bassen-Kornzweig syndrome)
 B. Alström disease (cataract and retinitis pigmentosa)
 C. Cockayne syndrome (dwarfism with retinal atrophy and deafness)
 D. Dialinas-Amalric syndrome (deaf-mutism-retinal degeneration syndrome)
 E. Hallgren syndrome (retinitis pigmentosa-deafness-ataxia syndrome)
 F. Hypotrichosis, syndactyly and retinitis pigmentosa-autosomal recessive
 G. Hunter syndrome (MPS II)
 H. Hurler syndrome (MPS I)
 I. Infantile phytanic acid storage disease
 J. Jeune syndrome
 *K. Kearns-Sayre syndrome (ophthalmoplegic retinal degeneration syndrome)
 L. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly- adiposogenital syndrome)
 M. Metaphyseal chondrodysplasia with retinitis pigmentosa-autosomal recessive
 N. Microcephaly with chorioretinopathy
 O. Multiple sulfatase deficiency
 P. Muscular atrophy, ataxia, retinitis pigmentosa, diabetes mellitus-autosomal  dominant
 Q. Olivopontocerebellar atrophy, type III
 R. NARP syndrome
 S. Pallidal degeneration, progressive with retinitis pigmentosa-autosomal  recessive
 *T. Refsum syndrome (phytanic acid storage disease)
 U. Retinitis pigmentosa alone (usually autosomal recessive but may be autosomal  dominant or sex linked)
 V. Retinitis pigmentosa associated with myopia, keratoconus, or glaucoma
 W. Retinitis pigmentosa, congenital deafness-sex linked
 X. Retinitis pigmentosa inversa (predominant-pigmentation around the disc and  macula) and deafness-autosomal recessive
 Y. Retinitis pigmentosa, nerve deafness, mental retardation, and hypogonadism -  autosomal recessive
 Z. Retinitis pigmentosa, PPRPE type (with preserved para-arteriole retinal  pigment epithelium) - autosomal recessive
 AA. Retinitis pigmentosa, spastic quadriplegia, and mental retardation-autosomal  recessive
 BB. Rud syndrome
 CC. Sanfilippo disease (MPS III)
 DD. Scheie disease (MPS IS)
 EE. Spielmeyer-Vogt syndrome (cerebroretinal degeneration)
 FF. Usher syndrome (hereditary retinitis pigmentosa-deafness syndrome)
2. Senile changes-degenerative pigmentation
3. Vascular lesion, such as occlusion of arteriole
4. Inflammatory
 A. Behçet disease (oculobuccogenital syndrome)
 B. Chickenpox virus
 C. Cytomegalic inclusion disease
 D. Dawson disease (inclusion-body encephalitis)
 E. Fetal varicella effects
 F. Focal dermal hypoplasia (Goltz syndrome)
 G. Harada disease (Vogt-Koyanagi-Harada syndrome)
 H. Hypomelanosis of Ito
 I. Influenza virus
 J. Nematode endophthalmitis (visceral larva migrans syndrome)
 K. Onchocerciasis (river blindness)
 L. Polyarteritis nodosa (Kussmaul disease)
 M. Rubella (German measles)
 N. Rubeola (measles)
 *O. Syphilis
 *P. Toxoplasmosis
 Q. Typhoid fever (enteric fever)
 R. Vaccinia
5. Toxic
 A. Accidental intraocular injection of depot corticosteroids
 B. Chloroquine and atabrine
 C. Diaminodiphenoxyalkanes-possible drug for treatment of schistosomiasis
 D. Indomethacin
 E. Phenothiazine
  (1) Chlorpromazine
  (2) Thioridazine (Mellaril)
 F. Pregl solution (Septojod, formerly used for treatment of puerperal sepsis)
 G. Quinine
 H. Sparsomycin
6. Acute lymphocytic leukemia
7. Alagille syndrome
8. Alport syndrome
9. Bardet-Biedl syndrome
10. Battens disease
11. Cryogenic "pigmentary fallout" - following use of cryosurgery for retinal detachment
12. Cystinosis syndrome (Lignac-Fanconi syndrome)
13. External ophthalmoplegias
*14. Gardner syndrome (congenital hypertrophy of the retinal pigment epithelium and familial intestinal polyposis)
15. Hagberg-Santavuori syndrome (neuronal ceroid lipofuscinosis)
16. Hallervorden-Spatz syndrome (pigmentary degeneration of globus pallidus)
17. Hereditary ataxias (Friedrich and Marie)
18. Leber congenital amaurosis
19. Lens dislocated into vitreous
20. MERRF syndrome
21. Mucolipidoses IV (ML IV)
22. Myotonic dystrophy syndrome (Curschmann-Steinert syndrome)
23. Neuronal ceroid lipofuscinosis
             A. Infantile form
 B. Late infantile form (Jansky-Bielschowsky)
 C. Adult form (Kufs syndrome)
24. Pelizaeus-Merzbacher syndrome (aplasia axialis extracorticalis congenita)
25. Pellagra (ariboflavinosis)
26. Progressive cone-rod degeneration
27. Renal disorders, including familial juvenile nephronophthisis (medullary cystic disease)
28. Rud syndrome (hypophyseal deficiency)
29. Sjögren-Larsson syndrome (oligophrenia-ichthyosis-spastic diplegia syndrome)
30. Tapetal-like reflex syndrome
31. Trauma, including blunt, penetrating, obstetric, and radiotherapy, Frenkel syndrome (ocular contusion syndrome)
32. Waardenburg syndrome (interoculoiridodermatoauditive dysplasia)
33. Zellweger syndrome and pseudo-Zellweger syndrome

LESIONS CONFUSED WITH RETINOBLASTOMA

1. Anomalous optic disc
2. Anteriorly dislocated lens with secondary glaucoma
*3. Coats disease (retinal telangiectasia)
4. Coloboma of choroid and optic disc (see p. 555-556)
5. Congenital corneal opacity
6. Congenital rubella syndrome (Gregg syndrome)
7. Cysts in a remnant of the hyaloid artery
8. Developmental retinal cyst
9. Glioma of the retina
10. Hematoma under retinal pigment epithelium
11. High myopia with advanced chorioretinal degeneration
12. Juvenile (x-linked) retinoschisis
13. Juvenile xanthogranuloma (nevoxanthoendothelioma)
* 14. Larval granulomatosis (T. canis)
15. Medullation of nerve fiber layer
16. Metastatic endophthalmitis
17. Norrie disease (atrophia oculi congenital
18. Oligodendroglioma of the retina
19. Organization of intraocular hemorrhage
*20. Persistent hyperplastic primary vitreous
21. Retinal detachment due to choroidal or vitreous hemorrhage
22. Retinal dysplasia (massive retinal fibrosis)
*23. Retinopathy of prematurity
24. Retrolental membrane associated with Bloch-Sulzberger syndrome (incontinentia pigmenti)
25. Rhegmatogenous and falciform retinal detachment
26. Secondary glaucoma
27. Sex-linked microphthalmia
28. Tapetoretinal degeneration
29. Trisomy 13 (Patau syndrome)
*30. Toxoplasmosis (ocular toxoplasmosis)
31. Traumatic chorioretinitis
*32. Tumors other than retinoblastoma
33. Uveitis in secondary retinal detachment
34. "White-with-pressure" sign








PERIPHERAL FUNDUS LESIONS

1. Pale raised lesions
A. Vitreous opacities-white fluffy or discrete opacities, associated with pars planitis or sarcoid uveitis
B. Retinopathy of prematurity-retinal edema and dense white lesions with  neovascularization
C. Toxocariasis (nematode ophthalmia syndrome)-vitreous opacities with  peripheral granuloma
D. Leprosy (Hansen disease)-peripheral exudates with anterior uveal involvement
E. Vitreoretinal dystrophies-bands in vitreous with retinoschisis or retinal  detachment
F. Angiomatosis-retinal tumor with enlarged, feeding vessels
*G. Retinoblastoma-raised, creamy-white, fluffy lesion without inflammatory  signs
2. Flat lesions
A. Coloboma-pale area with pigmented edge in region of fetal cleft
B. Chorioretinitis
           (1) Disseminated, congenital syphilis-pepper-and-salt or larger confluent  lesions
           (2) Toxoplasmosis-pigmented scars of old lesions
           (3) Cytomegalic inclusion disease-localized chorioretinitis or general peripheral                           infiltration
         *(4) Histoplasmosis-peripheral punched-out lesions with or without  pigmentation
C. Peripheral degenerations
         (1) Senile changes-of eyes older than years of age, depigmented areas with           pigmented margins (cobblestone degeneration).
            (2) Secondary pigmentary degeneration-peripheral pigmentary changes similar to             senile type or to retinitis pigmentosa
            (3) Cystoid degeneration-multiple cystic spaces and thin areas in  peripheral         retina
            *(4) Lattice degeneration-lace work of white lines with depigmented and     pigmented patches
            (5) Cystinosis (cystine storage aminoaciduria dwarfism syndrome)-granular rings of pigment in periphery of fundus, similar to cobblestone degeneration
D. Equatorial linear pigment disturbance
            (1) Ophthalmomyiasis internal
            (2) Histoplasmosis syndrome
E. Retinitis
            *(1) Acute retinal necrosis
            *(2) Cytomegalovirus retinitis

3. Dark raised lesions
A. Choroidal detachment (see p. 532-535)
            (1) Spontaneous-slowly progressive detachment, no inflammatory signs
            *(2) Postoperative-intraocular operation; particularly for cataract and glaucoma;    shallow anterior chamber; leaking wound
            (3) Exudative
                        a. Inflammatory-shallow anterior chamber, myopia, and peripheral                                                   detachment
                        b. Vascular-nephritis, hypertension, toxemia of pregnancy, polyarteritis                                           nodosa, leukemia
                        *c. Tumors-intraocular tumors; tumors of orbit and lacrimal gland
                        d. Traumatic-contusion injuries, perforating wounds, hypotony, anterior                                           chamber may be shallow or deep if perforation occurs posteriorly
B. Exudative retinal detachment (see p. 487)
*           (1) Secondary to general disease with retinopathy-hypertension, toxemia of           pregnancy, leukemia, dysproteinemia, polyarteritis nodosa, rickettsial arteritis,   venous congestion, talc and cornstarch emboli
            (2) Secondary to local disease of the eye-inflammatory signs with exudative             detachment. Harada disease, sympathetic ophthalmitis, scleritis, tenonitis,             choroidal tumor, and ophthalmomyiasis
C. Simple detachment-myopia in two thirds of patients, trauma, may follow cataract         extraction or discission for congenital cataract
D. Cysts
            (1) Ciliary body-larger cysts usually push iris forward; rarely, cyst extends backward to be seen ophthalmoscopically
            (2) Pars plana-may enlarge and appear as a multilocular reddish-brown  cyst
E. Scleral indentation-retinal detachment operation
F. Neoplasms of ciliary body
            (1) Benign epithelioma - brown spot to 2 mm in diameter on surface of  ciliary                               body
            *(2) Other tumors - diktyoma, leiomyoma, reticuloses, neurofibroma, malignant                             melanoma, rare, usually present as a mass protruding through the root of                          iris; may cause glaucoma; dark bulge seen ophthalmoscopically; lens                            changes adjacent to tumor
G. Neoplasms of choroid
            (1) Congenital melanosis-cat's-paw patches of pigment in one sector of  fundus
            (2) Choroidal nevus-flat, bluish gray or black lesion
            *(3) Malignant melanoma-raised, pigmented lesion with secondary  detachment
            (4) Secondary metastatic-rare, primary lesion in breast, lung, and so on
4. Vascular lesions
A. Periphlebitis (Eales disease) common; young adults; sheathing of peripheral veins; hemorrhages in new vessels and later retinal detachment (see p. 487)
*B. Perivasculitis secondary to uveitis - perivascular infiltration, particularly in pars planitis, sarcoidosis, Behçet disease, and toxoplasmosis
C. Systemic diseases
            (1) Rickettsia-engorgement of veins, retinal edema, hemorrhages, and  exudates
            (2) Multiple sclerosis (disseminated sclerosis) - sheathing of veins (see p.  468)
            (3) Polyarteritis nodosa (necrotizing angiitis) - hemorrhages, exudates, and           serous detachment of retina (see p. 488)
            (4) Tuberculin or BCG inoculation-rare, sheathing of peripheral veins with hemorrhages
            *(5) Sickle cell retinopathy (Herrick syndrome) - dilatation of peripheral veins,             hemorrhages, and connective tissue sheets in periphery; new vessel formation

EPIRETINAL MEMBRANES-MEMBRANES THAT GROW ON THE INNER SURFACE OF THE RETINA

*1. After retinal photocoagulation, cryotherapy, or reattachment of retina
2. Following blunt or penetrating injuries
*3. Idiopathic
4. Nonproliferative retinal vascular disorders
*5. Proliferative retinopathies
6. Rhegmatogenous retinal detachment (see p. 468)
7. Sickle cell disease, including sickle cell C, sickle cell S, and sickle cell B with thalassemia
8. Vitreous hemorrhage (see p. 424)

 HEADACHE

1. Vascular headache of migraine type
 A. Cephalalgia migraine (migraine equivalent)-migraine aura without headache
 B. Classic migraine-migraine with aura
 C. Common migraine-migraine without aura
 D. Complicated migraine-hemiplegic migraine and ophthalmoplegia migraine
 E. Cluster headache
 F. Lower-half headache

2. Muscle-contraction headache
3. Combined (skeletal vascular)
4. Headache of nasal vasomotor reaction
5. Headache of delusional, conversion, or hypochondriacal states
6. Nonmigraine vascular headaches
 A. Primary or metastatic tumors of meninges, vessels, or brain
 B. Hematomas (epidural, subdural, or parenchymal)
 C. Abscesses (epidural, subdural, or parenchymal)
 D. Post lumbar puncture headache (leakage, headache)
 E. Pseudomotor cerebri and various causes of brain swelling
7. Headache due to overt cranial inflammation
 A. Intracranial disorders
  (1) Mass
  (2) Meningitis
  (3) Subarachnoid hemorrhage
 B. Extracranial disorders (temporal arteritis)
8. Headache because of diseases of ocular structures
9. Headache because of diseases of aural structures
10. Headache because of diseases of the nasal and sinus structures
11. Headache because of diseases of dental structures
12. Headache because of diseases of other cranial and neck structures
13. Cranial neuritides
14. Cranial neuralgia
 A. Glossopharyngeal neuralgia
 B. Trigeminal neuralgia
15. Analgesic/ergotamine rebound headache








PAIN IN AND ABOUT EYE

1. Ocular
 A. Angle-closure glaucoma
 B. Chronic ocular hypoxia, carotid occlusive disease
 C. Dry-eye and tear-deficiency syndrome
 D. Local lid, conjunctival, and anterior segment disease
 E. Ocular inflammation including lyme borreliosis
2. Ophthalmic division
 A. Herpes zoster
 B. Migraine, cluster headache
 C. Painful ophthalmoplegia syndrome
 D. Raeder paratrigeminal neuralgia
 E. Referred (dural) pain, including occipital infarction
 F. Sinusitis
 G. Tic douloureux (infrequent in VI)
3. Mandibular division
 A. Dental disease
 B. Tic douloureux
4. Maxillary division
 A. Dental disease
 B. Nasopharyngeal carcinoma (6th N palsy+infraorbital N)
 C. Sinusitis
 D. Temporomandibular syndrome
 E. Tic douloureux
5. Miscellaneous
 A. Atypical facial neuralgias
 B. Cranial arteritis
 C. Pain with medullary lesions
 D. Trigeminal tumors



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