Download MBBS Ophthalmology PPT 34 Ocular Manifestations of Systemic Diseases Lecture Notes

Ocular Manifestations of Systemic Diseases

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Objectives of learning

? Why some systemic disorders can involve

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eyes?

? What are commoner disorders you can

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encounter during your clinical postings in

other specialities?

? Learn a multi-speciality , judicious cross

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referral.

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The eye is linked with rest of the body by-

? Development

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? Blood supply

? Meninges & nerve
fibres of brain

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Common Systemic Diseases affecting the Eye

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? Autoimmune disorders
? Hematological abnormalities
? Infections
? Endocrine disorders
? Muscular disorders

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? Inherited disorders

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Auto-immune disorders

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Rheumatoid Arthritis

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a)Kerato Conjunctivitis Sicca

(Dry eye)- 25% patients

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b) Ulcerative Keratitis

c) Scleritis

K C S

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Scleritis

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Systemic Lupus Erythematosus

a) Kerato Conjunctivitis Sicca

(Dry Eye)- 25% patients

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b) Scleritis

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Raynaud's phenomenon

c) Optic Neuropathy

Madarosis

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Peripheral ulcerative Keratitis

Retinal Vasculitis

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SARCOIDOSIS

?25-30% patients have ocular signs

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Mutton fat kps

Iris nodule

Phlebitis

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Sjogren's Syndrome

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Dry Eye

in

30% Patients

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Dry fissured tongue

Dental caries

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Parotid enlargement

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Bulbar conjunctiva

congestion

in Interpalpebral

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area

Schirmer test

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? 5mm end of 5 minutes

SIGNIFY DRY EYE
( less than 10 mm at the

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end of 5 min is

suggestive of

abnormality)*

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Corneal Staining

? Fluorescein Staining

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? Punctate epithelial

staining present in

lower half of cornea

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(interpalpebral region)

TEAR FILM BREAK UP TIME

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? Less than 5 seconds ( SIGNIFY DRY EYES)
? ( less than 10 seconds is considered

abnormal)*

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Ankylosing Spondylitis

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?Acute anterior uveitis

(prevalence of 0.2?

1.2%) ,

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? HLAB27 is +ve in

75% cases

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?

?Bilateral sclerosis

Scleritis

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?Erosion of s-i joint

?Bony fusion of

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spine

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Reiter Syndrome

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Urethritis

Triad Arthritis

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Conjunctivits

Eye Signs:

? Conjunctivitis

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? Ac. anterior uveitis

? Numular Keratitis

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? Episcleritis

? Scleritis

? Papillitis

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? Retinal vasculitis

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Behcet's Disease

Triad of oral aphthous ulcers, genital ulcers, and uveitis

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Eye Signs:
a) Hypopyon
b) Uveitis / Iritis

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Inflammatory Bowel Disease- Ulcerative Colitis

Crohn's Disease

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Ophthalmic

manifestations:
a) Acute anterior uveitis

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b) Episcleritis
c) Conjunctivitis

Acute anterior uveitis

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d) Peripheral corneal infiltrates
e) Scleritis
f) Papillitis
g) Retinal Vasculitis

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b) Peripheral corneal

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infiltrates

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Giant Cell Arteritis

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Predilection for superficial temporal A, ophthalmic A, posterior

Ciliary & proximal vertebral A

a) AION (Arteritic)

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b) TIA , Cilio-retinal Occlusion

c) Central retinal artery occlusion

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Giant cells & small round cells

Superficial temporal arteritis

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Vasculitis

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?Wegener granulomatosis

?Relapsing Polyarteritis

nodosa

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?Polychondritis

Dermal infarcts

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Ocular features-



a) Necrotising scleritis

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b) Peripheral ulcerative Keratitis

c) Occlusive retinal periarteritis

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Peripheral ulcerative Keratitis

d) Peripheral ulcerative Keratitis

e) Dacryocystitis

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f) Acute anterior uveitis

Dacryocystitis

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Haematological diseases

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Haematological diseases

1) Anaemia
2) Leukaemia
3) Lymphomas

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4) Sickle cell Anaemia

Ocular Presentation:
Haemorrhagic Retinopathy
"ROTHS SPOT"

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Hypertensive retinopathy

Infectious diseases

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1) Tuberculosis

May involve all Structures of Eye except Lens
a) Uveitis: chronic granulomatous anterior uveitis,

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multifocal choroiditis


b) Eyelids: lupus vulgaris

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c) Orbit: dacryoadenitis,dacryocystitis,

osteomyelitis

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d) Conjunctiva: phlyctenular conjunctivitis
e) Cornea: phlyctenular keratoconjunctivitis,

interstitial keratitis

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f) Sclera: episcleritis, nodular scleritis
g) Retina: exudative retinitis, Vasculitis
h) Optic nerve:- papilloedema, ON

2) Leprosy

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a) Facial palsy
b) Madarosis
c) Granulomatous iritis
d) Episleritis/scleritis
e) Secondary Glaucoma

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f) Cataract

3) Syphilis
Congenital: Acute interstitial keratitis, Diffusely opaque cornea,
salt & pepper fundus

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Secondary : Iritis, choroiditis, and/or exudates around
disc + vessels
Tertiary: Chorioretinitis and/or diffuse neuro-retinitis and
vascular sheathing

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Viral Infections associated with Ocular Diseases

? Herpes zoster
? Herpes simplex
? CMV

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? Rubella- cataract
? Measles
? AIDS

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AIDS

Kaposi's sarcoma-

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AIDS retinopathy- 50 to 70%

most common tumour

CMV retinitis-most common

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Anterior Uveitis

opportunistic infection

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? Fungal

Candidiasis- Fluffy white-yellow

superficial retinal infiltrate, vitritis

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? Parasitic
? Toxoplasmosis : Macular scarring, retinochoriditis,
vitritis, VR Granuloma
? Toxocariasis

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? Cysticercosis Cyst

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Al India Institute of Medical Sciences,

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Rishikesh

Ocular Manifestations of Systemic Diseases

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[Lecture 2] Dated 10-10-2017



Dr Sanjeev Kumar Mittal

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Professor & Head, Ophthalmology

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Endocrine & Metabolic disorders

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Endocrine & Metabolic disorders

? Diabetes Mellitus
? Muco-polysaccharidoses

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? Wilson disease
? Homocystinuria
? Hyperthyroidism
? Hypothyroidism

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Metabolic Cataract

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Diabetes Mellitus-

? Pre-senile cataract
? Snowflake cataract
[Sorbitol accumulation in

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lens due to aldose
reductase pathway

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Metabolic Cataract

? Galactosemic Cataract

Deficiency of GPUT- Oil droplet cataract

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Deficiency of Galactokinase-Lamellar Cataract
Metabolic Cataract

? Wilsons disease
Hepatolenticular degeneration

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Sun flower Cataract
Kayser-Fleischer Ring

Metabolic Cataract

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? Lowe's syndrome [oculo-cerebral-renal

syndrome]

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inborn error of amini acid metabolism
Congenital cataract, microphakia
Posterior lenticonus, glaucoma
Metabolic Cataract

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? Mannosidosis
Deficiency of alpha-mannosidase mannose rich

oligosaccharides in tissues spokes like

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posterior capsular cataract

Metabolic Cataract

? Fabry's disease

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Deficiency of alpha galactosidose spoke like

cataract & vortex keratopathy
? Hypocalcemia-
Decreased parathyroid activity leads to cataract

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Diabetes Mellitus

? Diabetic Retinopathy
? Diabetic Maculopathy

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? Corneal erosions
? Optic nerve Papillopathy
? Transient Myopia

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FUNDUS

Retinopathy

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Maculopathy

THYROID EYE DISEASE

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Unilateral lid retraction & proptosis

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OCULAR MOVEMENTS

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Restrictive myopathy

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Dalrymple Sign

Lid Retraction

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Normal

Naffziger test

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Exophthalmos

MRI

? Thickening of Muscle bel y

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? Tendon sparing

? Radiological y both eyes

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are involved


Slight straightening
of optic nerve in Right

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eye

Muscular disorders

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Muscular disorders

? Myasthenia gravis

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? Muscular dystrophy

Ocular Manifestations:
a) Ptosis,
b) diplopia,

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c) exophthalmoplegia,
d) cataract(Christmas-

tree cataract)

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Inherited disorders

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Marfan Syndrome

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a) Ectopia lentis BE

[superior Lenticular
subluxation]

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b) Microspherophakia
c) Retinal Detachment
d) Myopia
e) Keratoconus

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Ectopia lentis

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Long spider like fingers

Tall thin stature with

disproportionately long limbs

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compared to trunk

? High arch palate

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Sticklers Syndrome

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(Herditary artho-ophthalmopathy)

?Vitreous syneresis

?Retinal Detachment

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Flat nasal bridge

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3) Pseudoxanthoma elasticum

Prevalence 1/25000

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Angiod streaks

Loose skin folds

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Osteogenesis imperfecta

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1/20000 live births

Blue sclera

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Ehlers - Danlos Syndrome type 6 (Ocular sclerotic)

Keratoconus

Hyperelasticity of skin

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High myopia

RD

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Hypermobility of joints

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PHACOMATOSES - Neurofibromatosus-Type I

(Von Recklinghausen disease)

?Most common phacomatosis

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?Affects 1:4000 individuals

?Presents in childhood

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?Gene localized to chromosome 17q11

Nodular

Facial hemiatrophy-

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Skeletal defects

Caf?-au-lait spots

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3/24/2022

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? C/O mass over right eyelid

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since birth

? Growing with age
? Painless

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Iris-Lisch Nodules

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? LISCH

NODULES/hamartoma

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? Caf? au lait spot and

axil ary freckling were

also present

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Intraocular lesions in NF-1

Lisch nodules

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Choroidal naevi

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Family history

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Father has multiples neurfibromas al over chest and

arm ( 1st degree relative)

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Orbital lesions in NF-1

?Spheno-orbital encephalocele showing

congenital absence of left greater wing

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?Optic nerve glioma(in 15%)

of sphenoid bone

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invading hypothalamus

? Pulsating proptosis without bruit!

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Tuberous sclerosis (Bournevil e disease)

? Autosomal dominant

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? Triad - mental handicap,

epilepsy,

adenoma sebaceum

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Adenoma sebaceum

Shagreen patches

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Ash leaf spots

Retinal astrocytomas

Innocuous tumour pre65

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sent in

50% of patients

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Von-Hippel-Lindau syndrome

?Tumours? renal carcinoma and phaeochromocytoma
?Cysts- kidneys, liver, pancreas, epididymis, ovary and lungs
?Polycythaemia

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?Retinal capil ary haemangioma

Tiny lesion between

Associated dilatation and

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arteriole and venuole

Round orange-red mass

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tortuosity of feeder vessels

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Sturge-Weber syndrome

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Involves face, leptomeninges and the eyes

? Arteriovenous malformations of episclera
? Choroidal haemangioma
? Glaucoma (ipsilateral)

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? Iris heterochromia

Albinism

Hypopigmented skin and hair with eyes

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involvement

? Nystagmus

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? Translucent iris giving rise to a "pink-eyed"

appearance.

? Large choroidal vessels are seen due to lack of

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pigment in fundus.

? Foveal hypoplasia
Malignancy

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Malignancy

? Metastasis

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? From Breast, lung most common.
? Usually localize to choroid but Extra Ocular

Muscles,
? optic nerve and orbit can also be affected

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? Lymphoma, leukemia

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Summary

? Ocular examination plays a significant role in

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diagnosing several systemic illnesses.

? At times, patient present for eye related

problem, which then comes out to be a part of

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systemic disease, requiring inter-departmental

reference of the case to Physician/

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Paediatrician/Neurologist/ Neurosurgeon/

Orthopaedician/Obstetrician.

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Acknowledgement

Photgraphs from Kanski, Parson and Archives Dept of

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Ophthalmology AI Ms Rishikesh
1] Snow-flake cataract is seen in

a)

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Diabetes

b)

Galactosemia

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c)

Trauma

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d) Rubella

2] Cataract associated with atopic dermatitis

a) Sunflower cataract

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b) Coronary cataract

c) Shields cataract

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d) Coralliform cataract

3] Sunflower type cataract is seen in

(a)

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Diabetes

(b)

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Chalcosis

(c)

Argyrosis

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(d)

Tay sach's disease

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4. Most common ocular manifestation of AIDS is:

(a) Cottonwood spots

(b) Cytomegalovirus retinitis

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(c) Kaposi's sarcoma of conjunctiva

(d) Pseudotumors

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5. Which prominent ocular manifestation is

associated with

Marfan's syndrome? :

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(a) Microcornea

(b) Microspherophakia

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(c) Megalocornea

(d) Ectopia lentis

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