Dr mahmood fauzi ASSIST PROF OPHTHALMOLOGY AL MAAREFA COLLEGE

 To review the normal features of the human eye and the ocular fundus.  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.  To compare and contrast the important features of diabetic retinopathy types and the current screening guidelines.  To review the important ocular features of hypertension, thyroid disease, sarcoidosis and inflammatory conditions, malignancy and acquired immunodeficiency syndrome.

The human eye- appreciating the “NORMAL ”

Infectious  Toxoplasmosis  Toxocariasis  TB  Syphilis  Leprosy  HIV  CMV Non-infectious  Endocrine – diabetes, thyroid  Connective tissue disease – RA/ SLE/ Wegeners/ PAN/ Systemic sclerosis  Vasculitides (GCA)  Sarcoidosis  Behcet’s Disease  Vogt Koyanagi Harada syndrome  Phakomatoses

GRADE 1GRADE 2 GRADE 3 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)

May occur with hyper-, hypo-, or euthyroid states Hyperthyroidism: goiter, tremor, pretibial myxedema, atrial fibrillation, etc. PATHOPHYSIOLOGY- Autoimmune 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 EYE DISEASE

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 Lid lag in downgaze Unilateral lid retraction Unilateral proptosis

Proptosis Treatment options Systemic steroids Radiotherapy Surgical decompression Occurs in about 50% Uninfluenced by treatment of hyperthyroidism Axial and permanent in about 70%May be associated with choroidal folds

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

Occurs in about 40% Due to fibrotic contracture Restrictive myopathy Elevation defect - most common Abduction defect - less common Depression defect - uncommonAdduction defect - rare

Thyroid Eye disease: Management  CT scan orbits or Orbital Ultrasound Look for----  enlargement of eye muscles  Restrictive myopathy = Double Vision  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)

 AROUND THE EYE Molluscum contagiosum Herpes Zoster Ophthalmicus Kaposi’s Sarcoma Conjunctival Squamous Cell Carcinoma Trichomegaly  FRONT OF THE EYE Dry Eye Anterior Uveitis  BACK OF THE EYE Retinal Microvasculopathy CMV Retinitis Acute Retinal Necrosis Progressive Outer Retinal Necrosis Toxoplasmosis Retinochoroiditis Syphilis Retinitis Candida albicans endophthalmitis  NEURO-OPHTHALMIC

 Purplish red to bright red highly vascular lesions with surrounding telangiectatic vessels  Associated with Human Herpes Virus-8 (HHV-8)  20-24% of AIDS-related Kaposi sarcoma will involve eye  Eyelid & Conjunctiva Mostly local mass effects – pain, poor eyelid closure, etc  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  Clinically appears like painless, small, umbilicated nodules, which produce a waxy discharge when pressured.  Treatment  excision of the lesion, curettage or cryotherapy

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).

Herpes Zoster Ophthalmicus  Due to the reactivation of a latent infection by Varicella Zoster Virus in the dorsal root of trigeminal nerve ganglion.  It manifests with a maculo-papulo- vesicular rash which often is preceded by pain.maculo-papulo- vesicular rash  Usually involves the upper lid and does not cross the midline  Treatment  oral Aciclovir Ocular manifestations such as anterior uveitis, are treated with topical steroids and mydriatics. Hutchinson’s sign

 Most common intraocular infection with AIDS  Much reduced incidence since HAART (50% to 10% of pts)  CD4 count typically < 50 cells/mm3  Retinal necrosis, exudation, & hemorrhage  Treatment:  IV ganciclovir/foscarnet  Intravitreal ganciclovir/foscarnet; Ganciclovir intravitreal implant

CMV Retinitis

CD4<100

Tertiary Syphilis Need LP Rx with IV Penicilin G

Uveitis Choroidal granulomas Periphlebitis Granulomas = Choroidal Tubercules Cilio-retinal granuloma in TB An Ocular clue for Diagnosis of Tuberculosis

Fundus photography of a 40-year-old male with positive Mantoux test with choroidal tuberculoma.

 Spondylarthropathy of the axial skeleton  Typically affects males (4:1)  90% are HLA-B27 (+)  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:  Anterior uveitis in 30-40%  Symptoms  Photophobia  Redness  Decreased vision 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 hla- b27. Diagnosis?

 Vasculitis leading to chronic inflammation & ulceration  Oral 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.

 Ulcerative colitis: relapsing, non-transmural, restricted to colon  Crohn’s disease: relapsing, transmural, affects entire GI  tract  Ocular complications in 10%  Uveitis  Episcleritis  Scleritis  Women at higher risk  Associated with HLA-B27

Scleritis.Episcleritis Inflammatory Bowel Disease –ocular manifestations

 25% may have ocular findings  Dry eyes (15-25%)  Episcleritis  Scleritis  Corneal ulcers   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

Multiple Sclerosis: Optic Neuritis  50% of patients with MS will develop Optic Neuritis  20-30% of time will be presenting sign for MS

LOOK FOR EARLY SYMPTOMS OF MS

 Most common intraocular malignancy in adults  May be asymptomatic  May produce decreased or distorted vision  Most common primary: Lung, Breast  10% have unknown primary  No prior history of Cancer in 25% Metastatic Lung Cancer.

 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  Diagnosis: Tensilon test, single-muscle fiber EMG  Treatment: Acetylcholinesterase inhibitor (Mestinon)

 Autosomal Dominant…..Chromosome 17  Diagnostic Criteria -Café au lait spots -Intertrigenous freckle -Neurofibroma -Optic nerve glioma -Lisch nodules -Osseous leasions  Family history in 1st degree relative Neurofibroma tumors

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. S-Shaped Eyelid Neurofibroma Operative photograph demonstrating optic nerve glioma. Note fusiform enlargement of the optic nerve sheath Neurofibromatosis I, enlarged optic foramen

 Sporadically inherited phakomatoses  Glaucoma  Dilated & tortuous episcleral vessels  Seizures  CNS angiomas (Leptomeningeal hemangioma)  Calcification

Sturge-Weber Syndrome: Choroidal Hemangioma Nevus flammeus (Port Wine Stain)

 Benign ocular conditions  Amiodarone – whorl keratopathy  Toxic Retinopathies  Thioridazine, chloroquine, hydroxychloroquine, tamoxifen  Toxic Optic Neuropathies  Ethambutol, isoniazid

 Used for rheumatoid arthritis, SLE, etc  Ocular toxicity rare with usual dose 200 mg bid (5- 7mg/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 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. Plaquenil Toxicity - Bulls Eye Maculopathy

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