Transcript 11 Ocular Manifestations Of Systemic Dieasesx

Dr mahmood fauzi
ASSIST PROF OPHTHALMOLOGY
AL MAAREFA COLLEGE
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To review the normal features of the human eye and the
ocular fundus.
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To record the common systemic diseases affecting the eye.
To describe the ocular signs and symptoms associated
with selected systemic diseases and their serious ocular
squeal.
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To compare and contrast the important features of diabetic
retinopathy types and the current screening guidelines.
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To review the important ocular features of hypertension,
thyroid disease, sarcoidosis and inflammatory conditions,
malignancy and acquired immunodeficiency syndrome.
Infectious
Non-infectious
 Toxoplasmosis
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 Toxocariasis
 TB
 Syphilis
 Leprosy
 HIV
 CMV
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Endocrine – diabetes, thyroid
Connective tissue disease –
RA/ SLE/ Wegeners/ PAN/
Systemic sclerosis
Vasculitides (GCA)
Sarcoidosis
Behcet’s Disease
Vogt Koyanagi Harada
syndrome
Phakomatoses
GRADE 1
GRADE 3
GRADE 2
GRADE 4
diabetes mellitus. Cataract formation can occur as a complication of diabetes
Diagnosis:
Clinical history
Stat ESR &/or CRP
Temporal artery biopsy
Treatment:
High-dose systemic steroids (do not defer until after biopsy)
THYROID EYE DISEASE
May occur with hyper-, hypo-, or euthyroid states
Hyperthyroidism: goiter, tremor, pretibial myxedema,
atrial fibrillation, etc.
PATHOPHYSIOLOGYAutoimmune process with cross-reaction against orbital
and
periorbital soft tissues
Ocular Findings:
Proptosis (exophthalmos)
Lid Retraction -- Thyroid stare
Corneal exposure (dry eye, corneal ulcer)
Diplopia (due to eye muscle restriction)
Optic Nerve compression (optic neuropathy) – 5%
Thyroid stare
Soft tissue involvement
Periorbital and lid swelling
Chemosis
Conjunctival hyperaemia
Superior limbic
keratoconjunctivitis
Signs of eyelid retraction
Occurs in about 50%
• Bilateral lid retraction
• No associated proptosis
• Bilateral lid retraction
• Bilateral proptosis
• Unilateral lid retraction
• Unilateral proptosis
• Lid lag in downgaze
Proptosis
• Occurs in about 50%
• Uninfluenced by treatment of hyperthyroidism
Axial and permanent in about 70%
May be associated with choroidal folds
Treatment options
• Systemic steroids
• Radiotherapy
• Surgical decompression
Optic neuropathy
• Occurs in about 5%
• Early defective colour vision
• Usually normal disc appearance
Caused by optic nerve compression
at orbital apex by enlarged recti
Often occurs in absence of significant
proptosis
Restrictive myopathy
• Occurs in about 40%
• Due to fibrotic contracture
Elevation defect - most common Abduction defect - less common
Depression defect - uncommon
Adduction defect - rare
CT scan orbits or Orbital Ultrasound
Look for--- enlargement of eye muscles
 Restrictive myopathy
= Double Vision
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Treatment Considerations:
Artificial tears & lubrication
 Systemic steroids & external beam radiation (if vision
threatening)
 Surgery:
 Orbital decompression
 Eye muscle surgery
 Eyelid Surgery
 Stop Smoking
 Orbital Decompression
(For TED-related
Optic Neuropathy)
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AROUND THE EYE
• Molluscum contagiosum
• Herpes Zoster
Ophthalmicus
• Kaposi’s Sarcoma
• Conjunctival Squamous
Cell Carcinoma
• Trichomegaly
FRONT OF THE EYE
• Dry Eye
• Anterior Uveitis
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BACK OF THE EYE
• Retinal Microvasculopathy
• CMV Retinitis
• Acute Retinal Necrosis
• Progressive Outer Retinal
Necrosis
• Toxoplasmosis
Retinochoroiditis
• Syphilis Retinitis
• Candida albicans
endophthalmitis
NEURO-OPHTHALMIC
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Purplish red to bright red highly vascular lesions with
surrounding telangiectatic vessels
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Associated with Human Herpes Virus-8 (HHV-8)
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20-24% of AIDS-related Kaposi sarcoma will involve
eye
Eyelid & Conjunctiva
Mostly local mass effects – pain, poor eyelid closure, etc
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Treatment: chemotherapy, surgical (if large to debulk)
The typical lesion of Kaposi's
sarcoma on the conjunctiva.
Kaposi's sarcoma of the conjunctiva
with typical surrounding hemorrhage.
Typically multiple lesions in
HIV or AIDS
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Clinically appears like painless,
small, umbilicated nodules,
which produce a waxy
discharge when pressured.
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Treatment
excision of the lesion, curettage
or cryotherapy
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Multiple eyelid lesions, which are small, round, waxy, whitish,
umbilicated nodules on the eyelid. The affected eye will be
red, with some discharge.
For a few days, this 73-year-old woman had had an itchy, painful rash on the right side of her
face (A). Despite its proximity to her eye, she had no ocular involvement and no blurring of
vision.This was the rash of herpes zoster. The patient’s herpes virus titer was elevated; she
responded well to acyclovir . Also of interest in this case are the 3 lesions on this patient’s
forehead (B). These are classic umbilicated papules, of molluscum contagiosum which are not
to be confused with herpetic vesicles.
(A).
(B).
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Due to the reactivation of a latent
infection by Varicella Zoster Virus
in the dorsal root of trigeminal
nerve ganglion.
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It manifests with a maculo-papulovesicular rash which often is
preceded by pain.
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Usually involves the upper lid and
does not cross the midline
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Treatment
oral Aciclovir
Ocular manifestations such as
anterior uveitis, are treated with
topical steroids and mydriatics.
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Hutchinson’s sign
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Most common intraocular infection with AIDS
Much reduced incidence since HAART (50% to 10% of pts)
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CD4 count typically < 50 cells/mm3
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Retinal necrosis, exudation, & hemorrhage
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Treatment:
IV ganciclovir/foscarnet
 Intravitreal ganciclovir/foscarnet; Ganciclovir intravitreal implant
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CMV Retinitis
CD4<100
Tertiary Syphilis
Need LP
Rx with IV Penicilin G
Uveitis
Choroidal granulomas
Periphlebitis
Granulomas = Choroidal Tubercules
An Ocular clue for Diagnosis of Tuberculosis
Cilio-retinal granuloma in TB
Fundus photography of a 40-year-old male with positive
Mantoux test with choroidal tuberculoma.
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Spondylarthropathy of the axial skeleton
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Typically affects males (4:1)
90% are HLA-B27 (+)
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Presents in early adulthood (15-35 yo)
with pain & stiffness in lower back
Limitation of spinal flexion
Juxta-articular osteoporosis & fusion of
sacro-iliac joints
“Bamboo spine”
Ophthalmic features:
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Anterior uveitis in 30-40%
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Symptoms
Photophobia
Redness
Decreased vision
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Treatment:
 Topical corticosteroids
 Cylcoplegia
Fusion of sacro-iliac joints
Chronic inflammatory back pain onset age
15, chronic acute iritis, chronic fatigue are
some symptoms and I am positive for hlab27. Diagnosis?
 Vasculitis
ulceration
 Oral
leading to chronic inflammation &
aphthaous ulcers
 Genital
ulcers
 Skin
lesions (e.g. erythema nodosum)
 Eye
inflammation (iritis, retinal vasculitis)
Behcet’s Disease
Acute central retinal artery occlusion in Adamantiades-Behçet disease
Fundus photography and fluorescein angiography. Note grossly impaired perfusion, retinal whitening
and relative cilioretinal sparing.
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Ulcerative colitis: relapsing, non-transmural, restricted to
colon
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Crohn’s disease: relapsing, transmural, affects entire GI
tract
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Ocular complications in 10%
Uveitis
Episcleritis
Scleritis
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Women at higher risk
Associated with HLA-B27
Episcleritis
Scleritis.
Inflammatory Bowel Disease –ocular manifestations
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25% may have ocular
findings
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Dry eyes (15-25%)
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Episcleritis
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Scleritis
Corneal ulcers
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Uveitis
The Schirmer's test is used to assess the
function of the lacrimal glands- Dry eye in RA
Peripheral Ulcerative Keratitis
Ocular Manifestations In
Juvenile Rheumatoid Arthritis
50% of patients with MS will
develop Optic Neuritis
 20-30% of time will be
presenting sign for MS
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LOOK FOR EARLY SYMPTOMS OF MS
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Most common intraocular
malignancy in adults
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May be asymptomatic
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May produce decreased or
distorted vision
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Most common primary: Lung,
Breast
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10% have unknown primary
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No prior history of Cancer in 25%
Metastatic Lung Cancer.
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Autoimmune neuromuscular
disorder leading to fluctuating
muscle weakness & fatigability.
Pathophysiology-Circulating
antibodies block Ach receptors at
post-synaptic NM junction, inhibiting
stimulative effect of neurotransmitter
Ach.
Symptoms
Ocular- Ptosis, double vision
Other- problems chewing, talking, and
swallowing
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Diagnosis:
Tensilon test, single-muscle fiber EMG
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Treatment:
Acetylcholinesterase inhibitor
(Mestinon)
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Autosomal Dominant…..Chromosome 17
Diagnostic Criteria
-Café au lait spots
-Intertrigenous freckle
-Neurofibroma
-Optic nerve glioma
-Lisch nodules
-Osseous leasions
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Neurofibroma tumors
Family history in 1st degree relative
Lorette, a 25 year-old young woman from Panama, was
diagnosed at the age of one with neurofibromatosis
Lisch nodules- neurofibromatosis type 1. More than
95% of individuals with neurofibromatosis type 1 older than 10
years of age exhibit this finding.
Neurofibromatosis I, enlarged
optic foramen
Operative photograph demonstrating optic nerve glioma.
Note fusiform enlargement of the optic nerve sheath
S-Shaped Eyelid Neurofibroma
 Sporadically
inherited phakomatoses
 Glaucoma
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Dilated & tortuous episcleral vessels
 Seizures
 CNS
angiomas (Leptomeningeal
hemangioma)
 Calcification
Sturge-Weber Syndrome:
Choroidal Hemangioma
Nevus flammeus (Port Wine Stain)
 Benign
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ocular conditions
Amiodarone – whorl keratopathy
 Toxic
Retinopathies
 Thioridazine, chloroquine, hydroxychloroquine,
tamoxifen
 Toxic
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Optic Neuropathies
Ethambutol, isoniazid
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Used for rheumatoid arthritis,
SLE, etc
Ocular toxicity rare with usual
dose 200 mg bid (57mg/kg/day)
Toxicity related to cumulative
dose (>460 g) & duration of use
Ocular findings: bulls-eye
retinopathy
Recommended screening:
Baseline exam & Central VF
testing
Annual examination & repeat
central VF for pts using
medicine > 5 yrs
Plaquenil Toxicity - Bulls Eye Maculopathy
70-year-old woman with systemic Lupus erythematosus and clotting
problems. She was on the Plaquenil for about eight years and then off
the Plaquenil for the last eight years because she developed macular
toxicity. Although her vision was hazy, it was stable. Recent deceased
vision left eye.
 http://www.aao.org/theeyeshaveit/acquir
ed/
 http://www.aafp.org/afp/2002/0915/p99
1.html
 http://www.ncbi.nlm.nih.gov/pubmed/1
1926152
 http://ocularmanifestofsystemicdisease.
weebly.com/quiz.html
 http://www.easynotecards.com/quiz/622
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