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Ростовский Государственный Медицинский Университет

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Oxford American Handbook of Ophthalmology_Tsai, Denniston, Murray_2011

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666 CHAPTER 19 Aids to diagnosis

Corneal iron lines

These are best seen on slit lamp with cobalt blue light.

Table 19.5 Ophthalmic signs—corneal iron lines

Sign	Causes
Ferry’s	Trabeculectomy
Stocker’s	Pterygium
Hudson-Stahli	Idiopathic with age (horizontal inferior 1/3 of cornea)
Fleischer	Keratoconus (base of cone)

Cornea (other)

Table 19.6 Ophthalmic signs—cornea (other)

Sign	Causes
Shape
Thinning	•	Central: keratoconus, keratoglobus, posterior
		keratoconus, microbial keratitis
	•	Peripheral: peripheral ulcerative keratitis, marginal
		keratitis, microbial keratitis, Mooren’s ulcer, pellucid
		marginal degeneration, Terrien’s marginal degeneration,
		chronic exposure keratopathy, neurotrophic keratopathy.

Epithelial

Punctate epithelial erosions

Punctate epithelial keratitis

Epithelial edema

•Superior: vernal keratoconjunctivitis, superior limbic keratitis, ﬂoppy eyelid syndrome, poor contact lens ﬁt

•Interpalpebral: keratoconjunctivitis sicca, ultraviolet exposure, corneal anesthesia

•Inferior: blepharitis, exposure keratopathy, ectropion, poor Bell’s phenomenon, rosacea, drop toxicity

•Viral keratitis (adenovirus, HSV, molluscum contagiosum)

•Thygeson’s superﬁcial punctate keratitis

•iIOP, postoperative, aphakic/pseudophakic bullous keratopathy, Fuchs’ endothelial dystrophy, trauma, acute hydrops, herpetic keratitis, contact lens over wear, congenital corneal clouding

Corneal	•	Keratoconjunctivitis sicca, recurrent erosion syndrome,
ﬁlaments		corneal anesthesia, exposure keratopathy, HZO
Stromal
Pannus	•	Trachoma, tight contact lens, phlyctenule, herpetic
		keratitis, rosacea keratitis, chemical keratopathy, marginal
		keratitis, atopic/vernal keratoconjunctivitis, superior limbal
		keratoconjunctivitis, chronic keratoconjunctivitis of any cause

OPHTHALMIC SIGNS: ANTERIOR SEGMENT (1) 667

Table 19.6 (Contd.)

Sign	Causes
Stromal inﬁltrate	•	Sterile: marginal keratitis, contact lens related
	•	Infective: bacteria, fungi, viruses, protozoa
Stromal edema	•	Postoperative, keratoconus, Fuchs’ endothelial dystrophy,
		disciform keratitis
Stromal deposits	•	Corneal dystrophies: macular, granular, lattice, Avellino
	•	Systemic: mucopolysaccharidoses (some), amyloidosis
Vogt’s striae	•	Keratoconus
Ghost vessels	•	Interstitial keratitis (e.g., congenital syphilis, Cogan
		syndrome), other stromal keratitis (e.g., viral, parasitic)
Endothelial
Descemet’s folds	• Postoperative, dIOP, disciform keratitis, congenital syphilis
Descemet’s	•	Birth trauma, keratoconus/kerataglobus (hydrops), infantile
breaks		glaucoma (Haab’s striae)
Guttata	•	Peripheral: Hassell–Henle bodies (physiological in the
		elderly)
	•	Central: Fuch’s endothelial dystrophy
Pigment on	•	Pigment dispersion syndrome (Krukenberg spindle),
endothelium		postoperative, trauma
Keratic	•	Anterior uveitis: e.g., idiopathic, HLA-B27, Fuchs’
precipitates		heterochromic cyclitis, sarcoidosis, associated with
		keratitis (e.g., herpetic disciform, microbial, marginal)

668 CHAPTER 19 Aids to diagnosis

Ophthalmic signs: anterior segment (2)

Episclera and sclera

Table 19.7 Ophthalmic signs—episclera and sclera

Sign	Causes
Injection	•	Superﬁcial: episcleritis
	•	Deep: scleritis
Pigmentation	•	True: nevus, melanocytoma, bilirubin (chronic liver disease),
		alkaptonuria, pigment spots (at scleral perforations, e.g.,
		nerve loop of Axenﬁeld)
	•	Pseudo: blue sclera
Blue sclera	• Osteogenesis imperfecta, keratoconus or keratoglobus,
		acquired scleral thinning (e.g., after necrotizing scleritis),

connective tissue disorder (Marfan syndrome, Ehlers–Danlos syndrome, pseudoxanthoma elasticum), other systemic syndromes (Turner’s, Russell–Silver, incontinentia pigmenti)

Anterior chamber

Table 19.8 Ophthalmic signs—anterior chamber

Sign	Causes
iIOP	• Chronic with open angle: primary open angle, normal
		tension, pseudoexfoliation, pigment dispersion, steroid-
		induced, angle-recession, intraocular tumor
	•	Chronic with closed angle: chronic primary angle closure,
		neovascular, inﬂammatory, ICE syndrome, epithelial
		downgrowth, phacomorphic, aqueous misdirection
	•	Acute with open angle: inﬂammatory, steroid-induced,
		Posner–Schlossman, pigment dispersion, red cell, ghost
		cell, phacolytic, lens particle, intraocular tumor
	•	Acute with closed angle: primary angle closure, neovascular,
		inﬂammatory, ICE syndrome, epithelial down-growth,
		phacomorphic, lens dislocation, aqueous misdirection
AC leukocytes	•	Corneal: keratitis, FB, trauma, abrasion, chemical injury
	•	Intraocular: anterior uveitis, endophthalmitis, tumor necrosis
Hypopyon	•	Corneal: severe microbial keratitis
	•	Intraocular: severe anterior uveitis, endophthalmitis,
		tumor necrosis
Hyphema	•	Trauma: blunt or penetrating
	•	Surgery: trabeculectomy, iris manipulation procedures
	•	Spontaneous: iris/angle neovascularization, hematological
		disease, tumor (e.g., juvenile xanthogranuloma), IOL
		erosion of iris, herpetic anterior uveitis
Pigment in AC	• Idiopathic (iwith age), pigment dispersion syndrome,
and angle		pseudoexfoliation syndrome (Sampaolesi pigment line),
		intraocular surgery
Blood in	•	Sturge–Weber syndrome, carotid–cavernous ﬁstula, SVC
Schlemm’s canal		obstruction, hypotony

OPHTHALMIC SIGNS: ANTERIOR SEGMENT (2) 669

Iris/ciliary body

Table 19.9 Ophthalmic signs—iris and ciliary body

Sign	Causes
Iris mass	•	Pigmented: iris melanoma, nevus, ICE syndrome,
		adenoma, ciliary body tumors
	•	Nonpigmented: amelanotic iris melanoma, iris cyst, iris
		granulomata, IOFB, juvenile xanthogranuloma, leiomyoma,
		ciliary body tumors, iris metastasis
Rubeosis	•	Retinal vein occlusion (usually ischemic CRVO),
		proliferative diabetic retinopathy, ocular ischemic
		syndrome, CRAO, posterior segment tumors, long-
		standing retinal detachment, sickle-cell or other ischemic
		retinopathy
Heterochromia	•	Hypochromic: congenital Horner’s syndrome, Fuchs’
		heterochromic cyclitis (the affected eye is bluer), uveitis,
		trauma or surgery, Waardenberg syndrome
	•	Hyperchromic: drugs (e.g., latanaprost), siderosis (e.g.,
		IOFB), oculodermal melanocytosis, diffuse iris nevus or
		melanoma, other intraocular tumors
Transillumination	•	Diffuse: albinism, post–angle closure, Fuchs’
defects		heterochromic cyclitis
	•	Peripupillary: pseudoexfoliation syndrome
	•	Mid-peripheral spoke-like: pigment dispersion syndrome
	•	Sectoral: trauma, post-surgery/laser, herpes simplex or
		zoster, ICE syndrome, iridoschisis
Leukocoria	•	Cataract, retinoblastoma, persistent fetal vasculature
		syndrome, inﬂammatory cyclitic membrane, Coats’
		disease, ROP, Toxocara, incontinentia pigmenti, familial
		exudative vitreoretinopathy, retinal dysplasia (e.g.,
		Norries disease, Patau syndrome, Edward syndrome)
Corectopia	•	Iris melanoma, iris nevus, ciliary body tumor, ICE
		syndrome, posterior polymorphous dystrophy, surgery

(e.g., complicated cataract surgery, trabeculectomy), anterior segment dysgenesis, coloboma

Ciliary body mass • Pigmented: melanoma, metastasis, adenoma

•Nonpigmented: cyst, uveal effusion syndrome, medulloepithelioma, leiomyoma, metastasis

670 CHAPTER 19 Aids to diagnosis

Ophthalmic signs: anterior segment (3)

Pupil function

Table 19.10 Ophthalmic signs—pupil function

Sign	Causes
RAPD	•	Asymmetric optic nerve disease (e.g., AION, optic neuritis,
		asymmetric glaucoma, compressive optic neuropathy) or
		severe asymmetric retinal disease (e.g., CRAO, CRVO,
		extensive retinal detachment)
Anisocoria	•	Abnormal mydriasis: Adie’s pupil, iris trauma, iris
		inﬂammation, CN III palsy, pharmacological, ischemia
	•	Abnormal miosis: physiological, Horner’s, pharmacological,
		iris inﬂammation
Light-near	•	Unilateral: afferent defect (optic nerve pathology), efferent
dissociation		defect (aberrant regeneration of CN III)
	•	Bilateral: Parinaud syndrome, Argyll–Robertson pupils,
		diabetes, amyloidosis, alcohol, myotonic dystrophy, encephalitis

Lens

Table 19.11 Ophthalmic signs—lens

Sign	Causes
Cataract	•	Sutural: congenital, traumatic, metabolic (Fabry’s disease, manno-
		sidosis), depositional (copper, gold, silver, iron, chlorpromazine)
	•	Nuclear: congenital, age-related
	•	Lamellar: congenital/infantile (inherited, rubella, diabetes,
		galactosemia, hypocalcemia)
	•	Coronary: sporadic, inherited
	•	Cortical: age-related
	• Subcapsular: age-related, diabetes, corticosteroids, uveitis, radiation
	•	Polar: congenital
	•	Diffuse: congenital, age-related
Abnormal	•	Microphakia: Lowe syndrome
size	•	Microspherophakia: familial microspherophakia, Peters anomaly,
		Marfan syndrome, Weill–Marchesani syndrome, hyperlysinemia,
		Alport syndrome, congenital rubella
Abnormal	•	Coloboma, anterior lenticonus (Alport syndrome), posterior
shape		lenticonus (sporadic, familial, Lowe syndrome), lentiglobus
Ectopia	•	Congenital: familial ectopia lentis, Marfan syndrome,
lentis		Weill–Marchesani syndrome, homocystinuria, familial
		microspherophakia, hyperlysinemia, sulﬁte oxidase deﬁciency,
		Stickler syndrome, Sturge–Weber syndrome, Crouzon
		syndrome, Ehlers–Danlos syndrome, aniridia
	•	Acquired: pseudoexfoliation, trauma, high myopia, hypermature
		cataract, buphthalmos, ciliary body tumor
Superﬁcial	•	Pseudoexfoliation, Vossius ring (trauma), glaucomﬂecken
opacities		(subcapsular opacities from acute-angle closure glaucoma)

OPHTHALMIC SIGNS: POSTERIOR SEGMENT (1) 671

Ophthalmic signs: posterior segment (1)

Fundus (chorioretinal)

Table 19.12 Ophthalmic signs—fundus (chorioretinal)

Sign	Causes
Choroid
Choroidal	•	Pigmented: e.g., nevus, CHRPE, melanocytoma, metastasis,
mass		BDUMP syndrome
	•	Nonpigmented: choroidal granuloma, choroidal detachment,
		choroidal neovascular membrane, hematoma (subretinal,
		sub-RPE, suprachoroidal), choroidal osteoma, choroidal
		hemangioma, posterior scleritis, metastasis
Choroidal	•	Idiopathic, hypermetropia, retrobulbar mass, posterior
folds		scleritis, uveitis, idiopathic orbital inﬂammatory disease,
		thyroid eye disease, choroidal mass, hypotony, papilledema
Choroidal	•	Effusion: hypotony, extensive PRP, extensive cryotherapy,
detachment		posterior uveitis, uveal effusion syndrome
	•	Hemorrhage: intraoperative, trauma, spontaneous
Retina
Tractional	•	ROP, sickle-cell retinopathy, proliferative diabetic retinopathy,
retinal		proliferative vitreoretinopathy (e.g., trauma or IOFB,
detachment		intraocular surgery, retinal breaks), vitreomacular traction
		syndrome, incontinentia pigmenti, retinal dysplasia
Exudative	•	Congenital: nanophthalmos, uveal effusion syndrome, familial
retinal		exudative vitreoretinopathy, disc coloboma or pit
detachment	•	Vascular: CNV, Coats’ disease, central serous retinopathy,
		vasculitis, accelerated hypertension, pre-eclampsia
	•	Choroidal tumors
	•	Inﬂammatory: posterior uveitis (e.g., VKH), posterior scleritis,
		orbital cellulitis, postoperative inﬂammation, idiopathic orbital
		inﬂammatory disease
General
White dots	•	Idiopathic white dot syndromes: PIC, POHS, MEWDS,
		APMPPE, serpiginous choroidopathy, bird-shot
		retinochoroidopathy, multifocal choroiditis with panuveitis
	•	Infective (chorio)retinitis: syphilis, toxoplasma, tuberculosis,
		candida, HSV
	•	Inﬂammatory (chorio)retinitis: sarcoidosis, sympathetic
		ophthalmia, VKH

672 CHAPTER 19 Aids to diagnosis

Fundus (vascular)

Table 19.13 Ophthalmic signs—fundus (vascular)

Sign	Causes
Hard exudates	•	Diabetic retinopathy, choroidal neovascular membrane,
		macroaneurysm, accelerated hypertension, neuroretinitis,
		retinal telangiectasias
Cotton-wool	•	Diabetic retinopathy, BRVO or CRVO, ocular ischemic
spots		syndrome, hypertension, HIV retinopathy, vasculitis
Retinal	•	Coats’ disease, Leber’s miliary aneurysms, idiopathic
telangiectasias		juxtafoveal telangiectasia, ROP, retinitis pigmentosa, diabetic
		retinopathy, sickle retinopathy, radiation retinopathy,
		hypogammaglobulinemia, Eales disease, CRVO or BRVO
Arterial emboli	•	Carotid artery disease, atrial thrombus, atrial myxoma,
		infective endocarditis, fat embolus (long-bone fracture), talc
		embolus (IV drug abuser), amniotic ﬂuid embolus
Roth’s spots	•	Septic emboli, leukemia, myeloma, HIV retinopathy
Vasculitis	•	Idiopathic retinal vasculitis, intermediate or posterior
		uveitis (idiopathic), sarcoidosis, MS, Behcet’s disease,
		SLE, toxoplasmosis, tuberculosis, HSV, VZV, CMV, ARN,
		Wegener’s granulomatosis, polyarteritis nodosa, Takayasu’s
		arteritis, Whipple’s disease, Lyme disease
Arteritis	•	ARN (HSV, VZV); less commonly in other vasculitides

OPHTHALMIC SIGNS: POSTERIOR SEGMENT (2) 673

Ophthalmic signs: posterior segment (2)

Macula

Table 19.14 Ophthalmic signs—macula

Sign Causes

Cystoid macular edema

Macular hole

•Postoperative: cataract, corneal, or vitreoretinal surgery

•Post-procedure: cryotherapy, peripheral iridotomy, panretinal photocoagulation

•Inﬂammatory: uveitis (posterior > intermediate > anterior), scleritis

•Vascular: retinal vein obstruction, diabetic maculopathy, ocular ischemia, choroidal neovascular membrane, retinal telangiectasia, hypertensive retinopathy, radiation retinopathy

•Medication: epinephrine, latanoprost

•Other: vitreomacular traction syndrome, retinitis pigmentosa, autosomal dominant CME, tumors of choroid/retina

•Idiopathic, trauma, CME, epiretinal membrane, vitreomacular traction syndrome, retinal detachment (rhegmatogenous), laser injury, myopia, hypertension, proliferative diabetic retinopathy

Epiretinal	•	Idiopathic, retinal detachment surgery, cryotherapy,
membrane		photocoagulation, trauma (blunt or penetrating), posterior
		uveitis, persistent vitreous hemorrhage, retinal vascular
		disease (e.g., BRVO)
Choroidal	•	Degenerative: ARMD, pathological myopia, angioid streaks
neovascular	•	Trauma: choroidal rupture, laser
membrane	•	Inﬂammation: sarcoidosis, toxoplasmosis, POHS, PIC,
	•	Inﬂammation: sarcoidosis, toxoplasmosis, POHS, PIC,
		multifocal choroiditis, serpiginous choroidopathy, bird-shot
		retinochoroidopathy, VKH
	•	Dystrophies: Best’s disease
	•	Other: idiopathic, chorioretinal scar (any cause), tumor
Central serous	•	Central serous retinopathy, optic disc pit, CNV, posterior
detachment		uveitis (e.g., VKH), malignant hypertension; see also
		exudative retinal detachment
Bull’s eye	•	Drug: chloroquine group, clofazamine
maculopathy	•	Macular dystrophies: cone dystrophy, cone-rod dystrophy,
		Stargardt’s
	•	Neurological: Batten’s disease
Cherry-red	•	Systemic: Tay–Sachs disease, Sandhoff disease, GM1
Cherry-red	•	Systemic: Tay–Sachs disease, Sandhoff disease, GM1
spot		gangliosidoses, Niemann–Pick disease, sialidosis,
		metachromatic leucodystrophy
	•	Ocular: CRAO
Foveal schisis	•	X-linked juvenile retinoschisis

674 CHAPTER 19 Aids to diagnosis

Optic disc

Table 19.15 Ophthalmic signs—optic disc

Sign	Causes
Pallor	•	Congenital: Kjer’s, Behr’s, or Wolfram’s optic atrophy
	•	Acquired: compression (optic nerve or chiasm),
		glaucoma, ischemia, toxins, poor nutrition, inﬂammation,
		infection, LHON, trauma, severe retinal disease, post-
		papilledema
Apparent	•	Pseudo: drusen, tilted, hypermetropic, myelinated
swelling	• True: iICP (usually bilateral) or local causes (may
		be unilateral), e.g., inﬂammation, ischemia, LHON,
		inﬁltration, tumor
Pit	•	Congenital
	•	Acquired: glaucoma

OPHTHALMIC SIGNS: VISUAL FIELDS 675

Ophthalmic signs: visual ﬁelds

Normal

Arcuate

Altitudinal

Enlarged blind spot

Central

Centrocecal

Temporal 90°

Inferior 70°

Nasal 60°

Superior 50°

Optic neuropathy 2’ to:

Glaucoma Ischemia (AION) Papilledema (late)

Optic neuropathy 2’ to:

Ischemia (AION) Glaucoma

Vascular

Hemispheric RVO/ RAO

Optic neuropathy

(especially peripapillary axons)

Macular pathology Optic neuropathy

(especially central axons)

Optic neuropathy

(central and peripapillary axons)

Bitemporal		Chemical compression
hemianopia		(respect midline)
		Pseudochiasmal defects
		Pseudochiasmal defects
		(may not respect
		midline):
	Worse superiorly →	Tilted optic discs
	Worse superiorly →	Bilateral retinoschisis
	inferior lesion; worse	Toxicity (chloroquine)
	inferiorly → superior lesion	Sector RP
Junctional
scotoma		Prechiasmal/optic nerve
		Prechiasmal/optic nerve
		lesion, e.g., compressive

Figure 19.1 Visual ﬁeld defects.

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