Ocular Disease Training and Management

STUDY GUIDE

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Dear Prospective Colleague: Thank you for requesting our Treatment and Management of Ocular Disease Study Guide. Your personal copy is enclosed. We hope it will be helpful as you prepare for your boards. Good luck! As you approach graduation, we also hope you will consider the practice options available with Luxottica Retails well-known and trusted brands including LensCrafters, Pearle Vision, Sears Optical and Target Optical. We seek to affiliate with Doctors of Optometry who share our mission to be the best at helping the world see. We offer many exciting and diverse opportunities to pursue a dynamic, motivating and rewarding career for optometrists at all stages of their careers, including new graduates. Luxottica Retail has a variety of practice options to consider including leasing arrangements, employment as an Associate OD or Managing OD in select states, and franchising. Many of our practice options require no upfront capital investment and provide an established customer base. With Luxottica Retail, youll enjoy the quality, company support and professional growth opportunities of North Americas leading optical retailer while preserving your independent professional judgment and ability to practice full-scope optometry. We offer both full- and part-time options. Some doctors also practice with us on a part-time basis while completing residency programs or establishing their own private practices. Visit us online at www.LuxRetailDocs.com to learn more! Sincerely,

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OCULAR DISEASE TREATMENT AND MANAGEMENT STUDY GUIDE

EDITED BY: Casey L. Hogan, O.D., F.A.A.O. Assistant Professor of Optometry Illinois College of Optometry Co-Director Advanced Eyecare Professionals P.C. Co-Director Revised :2006
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TMOD: National Board of Examiners in Optometry Note: Please refer to the NBEOs website www.optometry.org for detailed information regarding the TMOD Exam. A supplement of 2006 Generic Drugs & Trade Name Equivalents is provided. Go to www.optometry.org/part_tmod_sample.cfm for sample TMOD test questions. This website is very complete containing information regarding exam content, scoring information, registration, and general information.

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This study guide is designed as an aid in preparing for the TMOD examination given by the National Board of Examiners in Optometry. It should be used as an adjunct to textbooks and lecture notes which cover the material included. Before taking the TMOD, be sure to review the information provided by the National Board of Examiners in Optometry for the content of the TMOD and sample test questions.

TABLE OF CONTENTS THERAPEUTIC MANAGEMENT OF OCULAR DISEASE Page OCULAR DISEASE AND THERAPEUTIC MANAGEMENT Orbital Disease Eyelids and Adnexa Lacrimal Disorders Conjunctival Disease Scleral and Episcieral Diseases Corneal Disease Pupillary Anomalies Uveal Tract Glaucoma Pre-operative and Post-operative Cataract Management Disorders of the Vitreous and the Retina Optic Nerve Disease Visual Pathways Oculomotor Disorders Urgent and Emergent Eye Care Systemic Disease Presentation in the Eye PHARMACEUTICAL AGENTS Sympathomimetics Parasympatholytics Alpha-adrenergic antagonists Topical anesthetics Topical Anti-inflammatory Agents Corticosteroids Antibacterials Antivirals Antifungals Antiglaucoma /Ocular hypertensive Agents Hyperosmotic agents Ocular antiallergy/ decongestants . BIBLIOGRAPHY . 6 15 42 46 61 66 100 104 115 140 155 188 195 199 208 219 237 238 240 240 243 246 248 257 261 261 278 282 288

ORBITAL DISEASE GRAVES OPHTHALMOPATHY SEE SYSTEMIC DISEASE SECTION For Detailed Information Graves disease is the ocular manifestation of thyroid disorders. It may present in patients with euthyroid disease, hyperthyroidism, and hypothyroidism. OCULAR SIGNS: Upper lid retraction, lid lag on downgaze (von Graefes), lid edema, vessel engorgement over the insertion of the EOMs, inflammation and hypertrophy of the EOMs, prolapse of orbital fat, chemosis, palpable lacrimal gland, infiltrative changes in the retrobulbar tissue. Proptosis 3mm or more in excess of upper limit, with and without symptoms (a difference of 2 mm or more between the two eyes with exophthalmometry warrants thyroid testing.) Infiltration of the muscles results in enlargement, loss of elasticity and fibrosis of the muscles. Muscles become injected and full. Optic neuropathy from nerve compression. Resistance on forced duction testing present. Ultrasonography and neuroimaging may be used. SYMPTOMS: Diminished visual acuity Proptosis ( bulging eyes) Pain with eye movement Conjunctival hyperemia ( redness) Diplopia ( double vision) Foreign body sensation and dryness Tearing and photophobia ( light sensitive) MANAGEMENT: Standard testing such as color, visual fields, and pupils should be performed Orbital CT scan MRI-T2 weighted including fat suppression Thyroid Panel >Serum free thyroxine FT4 >Thyroid Stimulating Hormone TSH >Anti-thyroid antibodies >FT3 TREATMENT: Artificial tear supplements Topical NSAIDs Lid edema: Elevating head during sleep, cold compresses Lid retraction: adrenergic blocking agents Oral prednisolone IV-steroids Severe cases with optic neuropathy: surgical orbital decompression with immunosuppresion

ORBITAL BLOW OUT FRACTURE *Blow-out fracture due to blunt trauma compressing globe within orbit *Fracture of the orbital floor ( the superior aspect of the maxillary bone) *Medial Wall ( ethmoid bone) can be affected *Orbital floor fractures can lead to the globe dropping into the maxillary sinus causing enophthalmos and entrapment of the inferior rectus muscle or inferior oblique muscle SYMPTOMS: *Pain on eye movement *Tender to the touch *Diplopia on up and down gaze *Eyelid swelling after nose bleed *History of recent trauma SIGNS: *Restricted eye movement (esp. upgaze and downgaze IR entrapment) *Orbital subcutaneous emphysema (crepitation or air in tissue-orbital crepitus) *Maxillary or ethmoid sinus fracture *Enophthalmos (retraction) *Nosebleed due to communication between the orbital and maxillary sinus *Eyelid edema *Ecchymosis *Hyphema *Ptosis *May have hypoesthesia of the ipsilateral cheek due to entrapment of the infraorbital nerve DIFFERENTIAL DIAGNOSIS: *Orbital edema and hemorrhage without blow out fracture -Ocular movement restriction -Periorbital swelling -Ecchymosis -Resolution over 7-10 days *Cranial nerve palsy -Ocular movement restriction -No restriction on forced duction testing MANAGEMENT: Rule Out ocular injury such as: hyphema, traumatic iritis, and retinal/choroidal damage *Axial and coronal CT scans of orbit ( imaging study of choice) *Floor fracture with associated herniation of orbital contents may require surgical intervention *Prophylactic oral antibiotic therapy if fracture present

IF CREPITUS IS PRESENT: *Avoid blowing nose *Cold compresses/ice packs to orbit first 48 hours *Hot compress for 5-7 days *Oral decongestants as needed *Broad spectrum oral antibiotics Follow-up: *Re-evaluation at 2 to 3 days and 7 to 10 days post trauma to rule out orbital cellulitis as well as diplopia and enophthalmus *Monitor for the development of associated ocular injuries -Orbital cellulitis -Angle recession glaucoma -Retinal Detachment IF CREPITUS IS NOT PRESENT *Avoid blowing nose *Cold compresses/ice packs to orbit first 48 hours *Oral decongestants as needed Follow-up: *Re-evaluation at 3 to 5 days or as needed post trauma to rule out orbital cellulitis and monitor for diplopia and enophthalmus *Monitor for the development of associated ocular injuries -Orbital cellulitis -Angle recession glaucoma -Retinal Detachment *SURGICAL MANAGEMENT Surgical resection of the periosteum and repair of the fracture: If diplopia persists If cosmetically unacceptable enophthalmus If significant fracture exists ORBITAL CELLULITIS *URGENT CASE: orbital cellulitis affects the intracranial region such as the CNS and meninges. CONDITION IS LIFE THREATENING. SYMPTOMS: *Marked swollen eye lids *Marked red eye *Pain (moderate to severe) *Blurred vision *Fever *Headache *Double vision

SIGNS: *Eyelid edema, erythema (deep red or red purple), warmth, and tenderness *Conjunctival chemosis and injection *Proptosis *Restricted EOM with pain upon movement *Decrease sensation of first division of fifth cranial nerve *Decreased VA *APD *Retinal venous congestion *Optic disc edema *Purulent discharge *Fever ETIOLOGY: *Extension from a sinus infection *Focal orbital infection -Dacryoadenitis -Dacryocystitis -Panophthalmitis *Cavernous sinus thrombosis *Neoplasia *Orbital fracture *Dental infection *S/P orbital trauma *S/P eye surgery *Extension from a vascular infection ORGANISMS: *Staphylococcus *Streptococcus *H influenzae - children *Fungal DIFFERENTIAL DIAGNOSIS: *Preseptal cellulitis *Arteriovenous fistula i.e. cavernous sinus fistula *Cranial nerve palsies *Enlarged globes MANAGEMENT: URGENT REFERRAL - CONDITION IS LIFE THREATENING! Refer to primary care physician Consider neurology consult Admit to hospital

*CBC with differential *Blood cultures *Culture any debridement / drainage *CT scan *Lumbar puncture -R/O meningitis Treatment: *IV antibiotics *Hospitalization ORBITAL INFLAMMATORY PSEUDOTUMOR Idiopathic inflammation of the orbit. Inflammatory orbital infiltrate that occupies space and may lead to compression of the orbital tissues including the optic nerve. Diagnosis of exclusion. SYMPTOMS: *Acute, recurrent, or chronic *Painful red eye *Double vision *Decreased vision SIGNS: *Mostly unilateral *Proptosis *Lacrimal gland enlargement/palpable orbital mass *Restriction of ocular motility *Eyelid erythema and edema *Conjunctival chemosis and injection *Uveitis *Elevated IOP *Optic nerve and retinal edema and choroidal folds *Febrile * CT scan: thickened posterior sclera, orbital fat involvement, thickened extraocular muscles DIFFERENTIALS: *Orbital cellulitis *Thyroid ophthalmopathy *Sarcoidosis *Lymphoid tumor *Lymphoangioma *Metastatic carcinoma *DIAGNOSIS OF EXCLUSION

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MANAGEMENT: *Contrast-enhanced MRI with multiple coronal views and fat saturation is recommended *CT scans (axial and Coronal) *Blood tests: -ESR -CBC with differential -ANA -BUN and creatinine -Fasting blood sugar *Systemic steroids *If unresponsive to systemic steroids: low-dose radiation therapy *If unresponsive to systemic steroids and diagnosis is uncertain: Consider undergoing an orbital biopsy ORBITAL TUMORS CAROTID CAVERNOUS FISTULA Normal communications of the arteriole and venous systems in the cavernous sinus region may be altered by trauma or aided by systemic disease. Aberrant connections result in increased venous pressure and resultant reduction of arterial perfusion, ocular hypoxia, and venous congestion. Fistulas are usually initiated through trauma or spontaneous changes in the walls of the carotid vasculature. The major outflow structures of the sinus, the orbital veins, become engorged. Secondary congestion of the periocular tissues follows. These are called highflow lesions. Signs of high flow lesions include lid swelling, orbital pain, pulsating exophthalmos, ocular bruit (patient may hear), diplopia, chemosis, engorged vessels, and increased IOP. Retinal evaluation may reveal disc edema, absence of spontaneous venous pulsation, retinal hemorrhages, venous stasis retinopathy, choroidal effusions, and vein occlusion. In addition, anterior segment ischemia can lead to cell/flare, corneal edema, glaucoma, and cataract. Spontaneous rupture may be seen in patients with atherosclerosis of the internal carotid and hypertension, intracavernous aneurysm, or connective tissue disease. These are typically low flow lesions. Lower pressure, slower flow fistulas, which are usually spontaneous in nature, present with a lesser degree of congestion, IOP elevation, and motility deficit. No bruits are audible. They tend to occur more often in elderly females. Most cases resolve spontaneously. TREATMENT: *Best diagnosed with neuroimaging and angiography. *High flow, high pressure fistulas, usually trauma-induced, may require surgical correction. Those methods used include detachable balloon embolization.

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*Observation is recommended for low flow shunts until vision or life threatening situations arise. CAVERNOUS HEMANGIOMA Refers to benign vascular tumors, which arise in the second to fourth decades of life. These lesions are very slow growing. SIGNS: *Progressive proptosis (usually<5mm), lagophthalmos Retropulsion will move the globe backward *Can produce hyperopic shift *Can produce choroidal folds *Diplopia TREATMENT: *A and B-scan ultrasonography and CT scan to confirm diagnosis *May be removed surgically DERMOID CYSTS Developmental tumors may occur congenitally to early adulthood. There are several categories including dermoids, epidermoids and lipodermoids. Epidermoids are found in the skin of the periorbital area and do not contain adnexal structures such as hair. They are subcutaneous masses typically on the lateral portion of the brow. Dermoids are found in the orbit itself usually in the superior temporal quadrant and may extend posteriorly. They may cause proptosis. Dermoids contain hair follicles and sebaceous glands. Lipodermoids are made up of solid fatty tissue and are found below the conjunctiva. MANAGEMENT: *Consider B scans, CT or MRI for diagnostic purposes *Dermoids and epidermoids can be surgically excised for cosmesis reasons *Lipodermoids should be monitored OPTIC NERVE GLIOMA Tumor of the optic nerve which is relatively uncommon. The peak incidence is 2-6 years of age. Females are affected slightly more than males. Over 50% of patients with optic nerve gliomas also have neurofibromatosis. The most common initial complaint is vision loss, not proptosis. It may also initially present as strabismus or nystagmus. Proptosis, when it occurs, is mild and may displace the eye infero-nasally. There is usually no pain. May see optic atrophy or optic swelling. An APD may be present. TREATMENT: *CT scan or x-rays, ultrasonography. *Observation, surgical excision, or radiotherapy.

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MENINGIOMA Intraorbital meningiomas account for 5-7% of all intraocular tumors. Any middle age female presenting with progressive proptosis and loss of vision should be highly suspected of meningioma. Meningiomas may arise at various sites along the route of the optic nerve. Primary meningiomas arise from the intraorbital optic nerve sheath while secondary meningiomas arise at the sphenoid wing and move into the orbit. As in the case of gliomas, vision loss usually precedes proptosis. Meningiomas presenting behind the globe will present as proptosis and vision loss. In addition, invasion into adjacent muscle and nerve tissue can involve the extraocular muscles. Fundus evaluation may show optociliary venous shunt vessels on the optic nerve head which are pathopneumonic signs of optic sheath meningioma. Optic nerve involvement may be present in the form of optic atrophy or papilledema. Visual field loss may accompany the changes. TREATMENT: *Best evaluated with neuro-imaging or radiology. *Always do pupil testing! *Primary meningioma could be excised surgically. *Radiotherapy and surgical intervention by skilled neurosurgeon are the treatments of choice for secondary meningioma. MUCURMYCOSIS or Rhino-orbital PHYCOMYCOSIS Occurs predominately in individuals who are diabetic and are in a state of metabolic acidosis. May present in infants in a state of metabolic acidosis or in patients with leukemia, lymphoma, or renal failure. The clinical presentation includes facial, palatal, or nasal necrosis, proptosis, orbital cellulitis, internal and external ophthalmoplegia, and central nervous system involvement. The syndrome may develop in hours to days. Once started, the syndrome can result in death in 48 hours. The rhinitis gives way to pain, proptosis and chemosis. Gangrene sets into the nasal and facial tissues, which appear as gray-black discoloration. An unpleasant odor accompanies the picture. Orbital cellulitis and orbital apex syndrome follow this. Infection, then infarction of the brain results in death.

MANAGEMENT: CHILDREN: *CT scan *Abnormal lesions are surgically removed. *Chemotherapy and Radiotherapy. *Best managed by a pediatric oncologist. ADULTS:

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Orbital symptoms may appear suddenly such as diplopia, visual loss, lid swelling, and ptosis. Ophthalmoplegia and diplopia present sooner in metastatic tumors than seen in primary orbital tumors. Pain presents early and is persistent. Proptosis usually exceeds that of primary tumors. *Early pain, ptosis, bone destruction, and ophthalmoplegia are signs of metastatic tumor vs. primary tumor. TREATMENT: *Chest X-ray *Biopsy with pathological diagnosis of primary site *Radiotherapy or Chemotherapy *Surgical intervention RHABDOMYOSARCOMA Rhabdomyosarcomas are malignant tumors of children and adolescents. It is the most common primary malignant orbital tumor in childhood. Average age of onset is 7-8 years old. Initial presentation is usually a rapidly moving exophthalmos with lid and conjunctival edema. Most common in the superior nasal region and possesses a bluish tinge. Pain is seen generally in the later stages. Nasal extension of the tumor can cause nasal stuffiness, frequent nosebleeds, and orbital swelling. Metastases are usually limited to lungs, cervical lymph nodes, and on rare occasions, the bone. *Choroidal folds and disc edema may be present. *Need to rule out orbital or preseptal cellulitis. TREATMENT: *IMMEDIATE referral to pediatric oncologist or ophthalmologist *IMMEDIATE BIOPSY! *CT or MRI scan *Radiotherapy or chemotherapy.

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EYELIDS AND ADNEXA IRRITATIONS AND INJURIES CHEMICAL BURNS *Topic discussed in detail under ocular emergency CYANOCCRYLATE TARSORRHAPHY *Inadvertent/accidental instillation of cyanoacrylate adhesives can induce tarsorrhaphy or total ankyloblepharon *Chemically inert and should not cause permanent ocular injury MANAGEMENT: 1) patient reassurance 2) cut involved lashes to remove irritative glue (allows lids to be gently pried apart) 3) tap water or mineral oil soaks moisten eyepads with either tap water or mineral oil soak eyelids and lashes for at least 24 hours allows lids to be easily separated 4) antibiotic ointment treatment similar to the soaks, apply to the site of adhesion and conjunctival sac cover and gently patch for at least 24 hours allows glue to be removed with forceps 5) oral analgesics for discomfort or pain 6) residual epithelial keratopathy or corneal abrasion 1) cycloplege 2) antibiotic ointment 3) pressure patch 4) RTC 24 hours THERMAL BURNS ETIOLOGY: *Caused by flame, flash, or scald burns DIAGNOSIS: *Careful examination of the globe for ocular damage *Rule out penetrating injury or thermal burns of the corneal and conjunctiva MANAGEMENT: First or second degree burns of the lids may result in 1) loss of lashes 2) trichiasis 3) lid edema 4) corneal abrasion secondary to trichiasis

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Therapy should include: 1. Clean surface and debride loose tissue 2. lid and lash hygiene comprising of cold compresses or iced-saline gauze reduce crusting and lid edema 3. antibiotic ointment such as bacitracin or erythromycin (BID or QID) prophylaxis for secondary bacterial infection 4. epilation of offending lashes to protect cornea 5. soft contact lenses or scleral shells additional protection of the corneal and globe 6. if marked periorbital edema perform tonometry 7. 1% Hydrocortisone cream or ointment to reduce inflammation Third degree burns of the lids Therapy should include: 1. artificial tears 2. bland lubricating ointment 3. antibiotic coverage 4. oculoplastic consultation NONINFLAMMATORY CONDITIONS ANOMALIES OF LID POSITION ECTROPION *Usually affects lower lid but sometimes involve the upper lid as well *Becomes everted exposing conjunctiva and cornea causing irritation and secondary infection ETIOLOGY: 1) involutional most common caused by horizontal laxity of eyelid tissue composition gravity acts to evert lower lid to expose conjunctiva and inferior cornea 2) cicatricial: scarring of the skin of lids caused by chemical/thermal burns chronic dermatoses trauma from previous surgery 3) paralytic caused by prolonged paralysis of the orbicularis muscle of the lower lid i.e. Bells palsy DIAGNOSIS: 1. involutional mild cases lid margin in normal apposition when pulled away, returns slowly rather than briskly advanced cases leads to tearing or epiphora because lower punctum not in apposition

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2. cicatricial palpable scar tissue accentuation of ectropion upon opening mouth widely upper lid cicatricial ectropion lid lag on down gaze lid retraction lagophthalmos MANAGEMENT: Mild to moderate lubricants for corneal protection artificial tears during the day as needed bland ointment HS Severe Bandage contact lens alone or in conjunction with a lateral tarsorrhaphy Treating epiphora: lid-taping at the medial canthus to bring lower lid in apposition with the globe surgical correction if conservative methods fail If keratinization occurs no treatment other than copious lubrication Note: Ectropion secondary to Bells palsy typically improves spontaneously with the resolution of the palsy. ENTROPION is a condition in which the lower lid or upper lid is everted toward the globe may lead to complications such as trichiasis and ultimately to irritation or corneal ulceration ETIOLOGY: 1. congenital - Epiblepharon (medial lid infolding) 2. involutional/senile reduction in tone of orbicularis muscle elongation of lid structures relaxation of tarsus weakness of inferior lid retractors lower tarsal border moves outward while the upper tarsal border movies inward 3. cicatricial occurs as a result of conjunctival scarring, e.g. trachoma 4. spastic

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occurs in cases of severe conjunctival and corneal diseases response on forced lid closure more common in the elderly

DIAGNOSIS: Patient complains of foreign body sensation, hyperlacrimation or lid spasms direct observation of the inverted lid border with/without trichiasis observation after tight lid closure where pressure from the upper lid is exerted on the lower lid *inferior foreign body tracking on cornea rule out infectious/ inflammatory disorder (blepharitis) MANAGEMENT: o Epilation of in-turned lashes often repeated every few weeks or months o Electrolysis, cryotherapy or laser ablation of cilia o Lid taping o Manage secondary corneal complications Lubricating ointment or artificial tears Bandage contact lens Prescribe prophylactic antibiotic ointment in severe cases o Surgical procedures: OCULOPLASTICS CONSULT FLOPPY EYELID SYNDROME soft, rubbery, floppy, and easily everted upper lid which spontaneously everts during sleep chronic papillary conjunctivitis along with a punctate keratitis chronic, thick, mucoid discharge bilateral, tends to be worse on the side which patient sleeps mechanism: loss of tarsal integrity cause eyelid eversion causing superior palpebral conjunctiva to rub against pillow or mattress DIAGNOSIS: Patient complains of irritation, foreign body sensation and increased mucus upon waking careful history observation of rubbery upper lid that easily everts on elevation presentation of mucoid discharge with papillary conjunctivitis MANGEMENT: lid taping or use of a shield at bedtime have patients refrain from sleeping face down definitive treatment is surgical lid shortening procedure

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LAGOPHTHALMOS *inability to fully close the eyelids 1. physiologic or nocturnal occurs during sleep symptoms occur more often in adults than in children because of tear compromise/reduction in former group 2. orbital associated with severe proptosis 3. mechanical scarring of lid muscles or lids preventing closure 4. paralytic associated with 7th CN palsy (Bells Palsy) DIAGNOSIS: ocular irritation especially on awakening punctate keratopathy of inferior cornea often in shape of horizontal band if severe melting of inferior cornea conjunctival injection affected portion of cornea may be hypoesthetic physical examination 1. instruct patient to gently close eyes 2. patient may have to be in supine position 3. use penlight to demonstrate incomplete closure 4. query spouse or parents or family member if patient sleeps with eyes open MANAGEMENT: 1. mild corneal exposure bland lubricant ointment HS artificial tears Q 2-4 hrs p.m. during day taping eyelids HS apply tape to cheek first pull lower lid upward attach free end of tape onto forehead 2. severe cases antibiotic ointment-if indicated CW bandage CL with antibiotic or lubricant treatment surgical procedure if long-standing or if conservative methods fail Note: In cases of complete palsy due to Bells consider po steroids: 60mg QD x 4 or 5 days then taper over next 5 days

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LID RETRACTION *associated with thyroid disease *please refer to the Orbit section PTOSIS Acquired ptosis 1. Result of an abnormality of the levator muscle or its innervation Traumatic (Secondary to surgery) neurogenic (e.g. Horner Syndrome, Third nerve palsy, Aberrant regeneration) myogenic (e.g. Myasthenia Gravis, Muscular Dystrophy) drugs (e.g. steroid) mechanical (e.g. neurofibroma) involutional (senile) ptosis (Blepharochalasis): caused by disinsertion of the levator aponeurosis ( noncontractile portion of the levator as it approaches insertion into the skin of the upper lid)\ 2. Cicatricial: secondary to trauma, surgery or severe inflammation Congenital ptosis Can be due to myogenic, neurogenic or aponeurotic causes Varies in magnitude Autosomal dominant Examples: Marcus Gunn (jaw winking) syndrome Misdirected third nerve palsy

DIAGNOSIS: Evaluation of ptosis o measure lid position measure from corneal light reflex to upper lid and corneal light reflex to lower lid compare ptotic eye to the non-affected, fellow eye o assess action of the levator muscle immobilize frontalis muscle with practitioners hand measure the levator excursion from upgaze to downgaze assess levator function by looking for a lid fold at the upper margin of the tarsal plate Note the lid folds presence and location The presence of a lid fold suggests there is some levator function. Absent lid fold typically indicates absent levator function (except in Asians)

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A lid fold present but higher than normal and a deeper than normal sulcus is signs of levator aponeurosis disinsertion, which is a common cause of acquired ptosis. congenital ptosis can be differentiated from acquired ptosis by looking at the lid position in full down gaze congenital ptosis inelasticity of the lid results in lid lag acquired myogenic ptosis affected lid assumes a lower position on down gaze assess the orbicularis muscle function by evaluating forced lid closure Palpitate lids and orbit for presence of a mass

*if history is positive for muscular weakness, RULE OUT: Myasthenia gravis perform the Tensilon test (10 mg of edrophonium chloride) Positive diagnosis of myasthenia gravis can be made if there is an immediate elevation of the ptotic lid *if Horners syndrome is suspected See Pupillary Section MANAGEMENT: Congenital ptosis Monitor if mild Surgical correction for congenital ptosis indicated when: ptosis interferes with vision to the degree of possibly inducing amblyopia, ptosis causes abnormal head posture, ptosis causes furrowed brow Acquired ptosis dependent upon the cause 1) surgical correction for residual ptosis OR 2) ptosis crutch mounted to spectacle frame ptosis is minimal surgery contraindicated surgery refused important to prevent exposure keratitis by allowing periodic lid closure 3) ocular prosthesis similar to a ptosis crutch but prevents corneal exposure and allows full lid closure BENIGN TUMORS OF THE EYELID SUDORIFEROUS CYSTS DIAGNOSIS: o small, round, translucent, elevated mass o caused by blockage of ducts of gland of Moll o observed on eyelid margins 1 or more lesions 1 to 2mm diameter

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painless irritation (mild) tend to reform following puncture rarely reappear if excised

MANAGEMENT: 1. cleanse eyelid margin with antiseptic (benzalkonium chloride) 2. anesthetize surface with local anesthetic 3. overlying membrane should be (dissected) with sterile tip of a 25 or 27 gauge needle 4. expression of material with 2 sterile cotton-tipped applicators and remove anterior half of cyst with forceps and curve tipped scissors 5. apply antibiotic ointment to prevent infection as needed XANTHOMA PALPEBRARUM (XANTHELASMA, XANTHOMA) DIAGNOSIS: o Plaque-like elevated, yellowish discoloration o Occurs in older patients women > men o Bilateral, symmetric, medial aspect of upper eyelids o Family history of xanthalasma o Can occur with positive contributory history atherosclerosis hypercholesteremia high risk cardiovascular history (obese, hypertension, family history) MANAGEMENT: R/O hyperlipidemic syndromes, arteriosclerosis, cardiovascular problems refer/obtain general medical and serum lipid evaluation surgical removal for cosmesis discouraged due to reoccurrence R/O syringomas in younger females Differentiate from large sebaceous cysts STURGE-WEBER SYNDROME (ENCEPHALOFACIAL CAVERNOUS HEMANGIOMATOSIS) SEE SYSTEMIC DISEASE PRESENTATION IN THE EYE Section VERRUCAS (Viral Wart) Nonsecreting papillomatous wart viral origin Various shapes Smooth surface with petal or cauliflower-like dentate projections Gray-brown to yellow Contagious nature INDICATION FOR MANGEMENT: None indicated unless causing cosmetic or ocular problems

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Advise on contagious nature Management 1. topical application of chemical agents 2. excision 3. laser removal 4. cryo removal LID COLOBOMA congenital not genetic characterized by absence of a portion of the eyelid Typically a full thickness triangular notch in the lid margin. unilateral or bilateral most common location/position involved: 90% of upper eyelid medial, middle 1/3 of the eyelid associated abnormalities - orbital - facial - ocular (limbal dermoids, strabismus, corneal opacitites) exposure keratopathy - occurs when 30% or more of upper eyelids is absent

MANAGEMENT: If large portion of lid is missing, surgical correction should be performed to prevent corneal exposure and scarring. Exposure keratitis treated with - topical lubricant (ointment) - topical antibiotics (ointment) DISTICHIASIS o characterized by an accessory row of eyelashes posterior to the normal lashes o arises from the meibomian gland orifices o may be autosomal dominant o associated findings: o corneal epithelial staining MANAGEMENT: o if patient asymptomatic and no corneal involvement-no treatment o few to several lashes-epilate or electrolysis o severe-surgical correction or cryotherapy

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 ESSENTIAL BLEPHAROSPASM chronic and progressive bilateral symmetric eyelid closure occurring involuntarily ETIOLOGY: abnormal involuntary contraction of the orbicularis muscle thought to be a dopaminergic imbalance in the CNS DIAGNOSIS females>males fifth or sixth decade severity progresses over 6 months to 3 years usually bilateral can cause functional impairment and interfere with daily routines o patient reassurance* does not occur during sleep emotional stress of fatigue can exacerbate condition physical examination o repetitive forced closure test to elicit blepharospasm o 5 sec cycle repeated 3-10x MANAGEMENT: 1) patient reassurance 2) consider psychologic counseling 3) systemic medications (variable effect, unpredictable, transient) >>>anticholinergics, anticonvulsants, antidopaminergics, antianxiety agents, antipsychotics, and monoamine depletors 4) surgical interventions (variable) 7th CN resection and myectomy 5) biofeedback training (variable) 6) Botulinum A toxin (Botox) Severe cases INCOMPLETE BLINK cause inferior punctate keratopathy o exposure o inadequate tear film MANAGEMENT: blink training artificial tears bland lubricant ointment bandage SCL

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 MOLLUSCUM CONTAGIOSUM Molluscum is an unclassified pox virus found in the human population. It is transmitted through direct contact and is common in communities with noted poor hygiene. Children are more typically affected. It has been associated with sexual contact and has increased in frequency and severity in patients who are HIV positive. SIGNS: Small papular lesions which expand to form excavated lesions with cellular debris inside. The lesion may appear to have cheesy material inside when active. These undergo spontaneous involution. Lesions may present on the lid margin. Virus laden debris from the lesions move into the tear film and trigger a follicular response and keratitis. The keratitis can be in punctate form or as secondary subepithelial infiltrates. Subsequent vascular infiltration may follow. Lesions may be found elsewhere on the body. MANAGEMENT: Clean out central core of the lesions with curetting instrument Alternate methods include excision and cryotherapy Corneal changes should resolve spontaneously along with the conjunctivitis Consider HIV in high risk patients. MYOKYMIA (Blepharospasm) *common occurrence *mild to moderate fasciculation of the orbicularis muscle ETIOLOGY: predisposing factors 1) fatigue 2) stress 3) tension 4) anxiety 5) lack of sleep 6) caffeine consumption 7) irradiated corneal or conjunctival lesions 8) excessive use of alcohol 9) excessive smoking 10) use of topical anticholinesterase agents 11) use of fluphenazine (Permitil and Prolixin) and haloperidol (Haldol) DIAGNOSIS: lower lid more commonly involved unilateral or bilateral symptoms/complaints eye jump, twitches, quivers slit-lamp examination inconclusive Rule out

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Foreign body reaction Raised IOP Neuromuscular disease MS and Myasthenia

MANAGEMENT: *Mild cases patient reassurance most cases resolve spontaneously discontinue offending agents switch to alternative medications with less effects *Persistent or severe cases topical antihistamines may be helpful *for recalcitrant cases >>>quinine reported helpful: ? recommend tonic water TRICHIASIS *inturning lashes of upper or lower lid *if severe, may lead to blindness especially in areas where trachoma is endemic ETIOLOGY: result of aging changes trachoma Stevens-Johnson syndrome ocular pemphigoid trauma DIAGNOSIS: *slit lamp examination inturned lash(es) conjunctival hyperemia corneal epithelial defect MANAGEMENT: *if only a few lashes involved: o epilation of offending lash with tweezers o anesthetize lid margin (optional) o grasp base of eyelash o eyelashes grow back in 2-4 weeks for children 4-6 weeks for adults repeat epilation as needed *electrolysis *laser ablation of the hair follicle

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Treat marginal lid disorders To prevent corneal involvement: - artificial tear substitute - antibiotic prophylaxis if needed ALLERGIC BLEPHARITIS ETIOLOGY: * Type 1 hypersensitivity (anaphylactic, immediate) o blepharoconjunctivitis result from exposure to: o Pollens, molds, animal dander o insect bites/sting *Type IV hypersensitivity (delayed, cell mediated, requires prior exposure) Contact blepharitis results from - poison ivy - nail polish, dyes - cosmetics - topical medications - nickel - preservatives DIAGNOSIS: *history *clinical findings Type 1: bilateral with significant lid edema Type IV: Lids: mildly edematous with scaly eruptions, mild erythema Periorbital skin: erythema, oozing, crusting, scaling Both types typically occur in conjunction with conjunctivitis and serous or mucous discharge. Skin and conjunctival tests intradermal skin test involves applying a minute amount of suspected allergen via a superficial epidermal scratch or injection; positive reaction is characterized by rapid onset (second or minutes) of wheal and flare. Conjunctival sac test involves topically instilling a low concentration of the suspected allergen into the conjunctival sac positive reaction is demonstrated by immediate development of eyelid swelling, chemosis, conjunctival hyperemia, and itching. *the skin and conjunctival test may cause anaphylaxis in highly susceptible individuals and therefore, extreme caution must be maintained throughout performing the test (i.e. low concentration, small doses) MANAGEMENT:

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*prevent exposure / eliminate offending allergen Type 1 reaction 1) Oral antihistamines 2) Cold compresses 3) Mast cell stabilizers may prevent future reactions *desensitization by serial administration Type IV reaction 1. cold compresses 2. topical corticosteriod ointments *desensitization to identified allergen-ineffective ANGULAR BLEPHARITIS ETIOLOGY *most commonly caused by infection with Moraxella and Staphylococcus DIAGNOSIS: Symptoms irritations tenderness near lateral canthus Signs chronic hyperemia desquamation ulceration of the lateral and uncommonly the medial canthal region may have conjunctivitis MANAGEMENT 1) Moraxella organism Zinc sulfate ophthalmic solution 2) Staphylococcal organisms bacitracin or erythromycin ointment CHALAZION ETIOLOGY: *Internal chalazion is a granuloma of the meibomian gland *External chalazion is a granuloma of the Zeis glands *role of demodex brevis in the formation of chalazia *associated with chronic blepharitis *can present after hordeolum DIAGNOSIS: Symptoms: *no pain or tenderness on palpation Signs:

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hard, round, firm mass develops over several weeks ranges in size from 2 mm up to 7 or 8 mm in diameter 25% of chalazia will resolve spontaneously within 6 months of onset; most require some form of intervention

MANAGEMENT: *topical or systemic antibiotics are ineffective because the lesion is sterile 1) hot compresses, 5-10 mins QID for 2 to 4 weeks followed by digital massage to the chalazion 2) intralesional steroid injection, triamcinolone acetonide (Kenalog-10)if chalazia fail to respond to conservative therapy Note: May lead to depigmentation of the injection site 3) surgical excision if chalazia fail to respond to conservative therapy or if chalazion >8-10 mm FUTURE MANAGEMENT: advise patient on possible reoccurrences control meibomian gland dysfunction rule out and/or manage rosacea EXTERNAL HORDEOLUM ETIOLOGY: *acute staphylococcal infection of the glands of Zeis and Moll *usually a localized area of inflammation *may be associated with staphylococcal blepharitis *exacerbated by fatigue poor diet stress *can be recurrent DIAGNOSIS: *Symptoms localized pain of acute onset *Signs localized area of lid margin involvement characterized initially by redness, swelling within a few days of onset yellow pointed head develops in the involved area spontaneous drainage of abscess resolution 2-5 days R/O Blepharitis, dacryocystitis, preseptal cellulitis MANAGEMENT:

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1) hot compresses 5-10 mins @ least QID increase blood flow to affected area and hasten pointing and drainage 2) topical antibiotics (optional) ointment or solution - bacitracin ointment BID to QID 3) can be drained by superficial needling try conservative methods first 4) Surgical excision and oral antibiotics needed for encapsulated infections HERPES SIMPLEX BLEPHAROCONJUNCTIVITIS ETIOLOGY *usually occurs as a primary infection in children *can occur in adults *caused mainly by HSV type 1, but HSV type 2 may be involved *primary infections occur with/without skin involvement in nonimmune host DIAGNOSIS: *two forms of HSV Blepharoconjunctivitis 1. vesicular herpetic lid lesions (classic type) vesicular type o characterized by the presence of vesicles along the base of the eyelashes o vesicles are pinhead in size o have erythematous bases o may involve both lid margins and periocular skin o mechanism vesicles break and ulcerate eyelid margins become edematous dermatitis results 2. erosive-ulcerative type characterized by erosions of the mucocutaneous junction of the lid and by skin ulcers located at the lid margins and/or a combination in the ABSENCE of typical vesicular eruptions Signs: mild lid swelling tender to the touch conjunctival injection follicular conjunctivitis swollen lymph nodes MANAGEMENT: *Lid lesions in primary herpes simplex are self limiting and generally require no treatment. *if needed, can treat skin lesions 1) oral antiviral medication such as Acyclovir 2) topical antibiotic ointment such as bacitracin ( most common treatment) 3) avoid excess handling of affected area-maintain good hygiene

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4) warm saline compresses 5) If lesions near eye can treat with topical antiviral such as Trifluridine 6) steroids: contraindicated *Conjunctivitis can be treated with an antiviral such as trifluridine for 1-2 weeks or until resolved *antiviral therapy of the cornea PLEASE SEE CORNEA SECTION FOR TREATMENT AND MANAGEMENT Advise patient that Herpes simplex can reoccur and be triggered by: ultraviolet light exposure, stress, immunocompromised state, surgery and trauma.

HERPES (VARICELLA) ZOSTER BLEPHAROCONJUNCTIVITIS ETIOLOGY *varicella (chicken pox) and zoster are the same virus *primary infection with varicella-zoster virus results in chickenpox vesicular lesion can occur on the lids during the chickenpox *primary infection is needed prior to reactivation of latent varicella virus resulting in herpes zoster *Recurrent herpes zoster infections typically occur in relatively immunosuppressed patients including those with a malignancy, undergoing chemotherapy and HIV positive/AIDS. *Recurrent infections are often accompanied by a painful neuralgia *for more detailed discussions on the pathophysiology and clinical management refer to the cornea section under Herpes Zoster DIAGNOSIS: Systemic signs/symptoms: 1) headache 2) malaise 3) fever 4) chills *Severe posthepetic neuralgia may develop Ocular signs/symptoms: - Vesicular skin lesions on a erythematous (red) base which develop in a dermatomic distribution - The distribution of vesicles respects the midline; affecting only 1 side of the face - Lesions on the tip of the nose suggest ocular involvement (Hutchinsons sign) - The vesicles form yellow and brown crusts - Vesicles can scar - Other ocular signs include: keratitis, conjunctivitis, scleritis, iridocyclitis, optic neuritis, chorioretinitis, cataracts and secondary glaucoma.

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MANAGEMENT: *refer to the cornea section for a discussion on the management of coincident keratitis o No therapy indicated if little or no pain o *skin and lid lesions are self-limiting and benign o IF mild to no ocular involvement Supportive treatment such as lubricating drops and ointments o For exposure keratitis Broad spectrum antibiotic; prophylaxis against secondary bacterial infections topical steroid or antibiotic steroid combinations o For symptomatic lid lesions oral Acyclovir: can reduce duration and severity of ocular affects if given early in the course of the disease o Oral steroids Treat post herpetic neuralgia INTERNAL HORDEOLUM ETIOLOGY: *localized staphylococcal infection of blocked meibomian gland *usually involves upper lids DIAGNOSIS: Symptoms localized pain of acute onset Signs *localized area of swelling, inflammation, and tenderness within the tarsus *onset and course usually more prolonged than that of external hordeolum R/O Blepharitis, dacryocystitis, preseptal cellulitis MANAGEMENT: Hot compresses qid Topical antibiotic oinment Typically require oral antibiotics for resolution Try conservative methods first Recheck within 48 to 78 hours to rule out preseptal cellulitis

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MEIBOMIAN GLAND DYSFUNCTION (MEIBOMIANITIS) ETIOLOGY caused by congestion of the meibomian glands which can compromise the meibomian gland component of the tear film can be primary or secondary to rosacea. associated with seborrheic blepharitis DIAGNOSIS: *Symptoms 1) foreign body sensation 2) dry eye 3) chronic burning 4) nonspecific irritation *Signs 1) blocked meibomian glands with plugs over orifices 2) hyperemia and mild papillary hypertrophy of the palpebral conjunctiva 3) foamy or frothy tear film ( saponification) 4) thickened (tylosis) and rounded eyelid margins 5) SPK with fluorescein or rose bengal in the intrapalpebral space of the cornea and conjunctiva 6) Oily tear layer with markedly reduced TBUT 7) Usually bilateral MANAGEMENT: 1) hot compresses/warm soaks mandatory BID to QID 2) digital massage and gland expression excluding general lid hygiene BID or QID 3) oral tetracycline or doxycycline 4) topical antibiotic steroid combination if needed 5) Artificial tears (Alcons Systane clinically proved to extend TBUT time) 6) Off Label use of Restasis in clinical practice ( probably not a TMOD question)

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PHTHIRIASIS /PEDICULOSIS *eyelid infestation by Phthirus pubis (pubic lice); Pediculus corporis (body lice) and less likely Pediculus humanus capitis (head lice). ETIOLOGY: Eyelash, eyebrow and eyelid infection by the louse transmitted by sexual contact, transferred from contaminated bedding, towels and garments, contact with a carrier, and heavy scalp infestation. *inflammatory reaction against the toxic fecal and saliva excretions DIAGNOSIS: *signs/symptoms o chronic itching, irritation usually bilateral o brownish, crusty discharge and matting of the lid margin o presence of nits (eggs) and adults parasites on the eyelashes in severe cases: conjunctivitis, marginal keratitis possible preauricular lymphadenopathy MANAGEMENT: *lid, scalp, body, and pubic areas should be treated with appropriate medications such as: gamma benzene hexachloride (Kwell), pyrethrin based pediculocide (RID) *eliminate lice on the source of infestation *ocular treatment modalities 1) removal of nits and parasites with forceps or cotton-tipped applicator under slip lamp 2) lid medications a) yellow mercuric oxide 1% ophthalmic ointment applied BID for 1 week b) bland petrolatum ointment applied BID for 1 week effective in smothering the parasites c) use carefully on lid margins gamma benzene hexachloride (Kwell) pyrethrin based pediculocide (RID) FOLLOW-UP: 1 week PRESEPTAL CELLULITIS ETIOLOGY *generalized inflammation of the lid structures anterior to the orbital septum *caused by: Traumatic injuries Spread of infection from skin of lids or face

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e.g. from nonresolving internal or external hordeolum, dacryocystitis, sinusitis *common pathogens hemophilus influenzae in children <6 yrs old staphylococcus aureus streptococci anaerobic bacteria *May progress causing orbital cellulitis which affects the intracranial region such as the CNS and meninges. DIAGNOSIS: *Ocular signs/symptoms lid swelling erythema eyelid warm and mildly tender minimal pain no proptosis no limitation in motility NOTE: A dark purple discoloration of the skin of the eyelids and face in a child less than 4 years old is suggestive of preseptal cellulitis caused by H. influenza *Systemic signs/symptoms elevated temperature irritable upper respiratory infection *Preseptal cellulitis occurs most frequently in pediatric population. *Differentially diagnose preseptal or orbital cellulitis from blepharitis or dermatoblepharitis: Tissue distension is typically present in preseptal or orbital cellulitis while it is absent in blepharitis or dermatoblepharitis.

*Differentially diagnose preseptal cellulitis from orbital cellulitis *signs/symptoms of orbital cellulites (SEE ORBIT SECTION) ORBITAL CELLULITIS marked lid swelling erythema chemosis proptosis limitation in motility pain decreased visual acuity elevated body temperature leukocytosis in febrile patients afferent pupillary defect may be present MANAGEMENT of Preseptal Cellulitis Adults

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Oral antibiotic treatment Warm compresses Polysporin ointment for secondary conjunctivitis Follow-up daily until clearly improved Drain abscess Tetanus shot, if penetration injury Children especially less than 5 yrs old Due to concern over infection with H. influenza, which can rapidly affect the CNS, treat more aggressively than adults. 1) Intravenous antibiotics such as ceftriaxone, cefuroxime or cefotaxime followed by oral antibiotics Comanage with ophthalmology or pediatrician ROSACEA *usually affects middle-aged adults (average late 30s) SYMPTOMS: 1) bilateral, chronic ocular irritation 2) redness 3) burning 4) foreign body sensation

SIGNS: check, forehead, and nose involvement o telangiectasias o pustules o papules o erythema o superficial or deep corneal vascularization o rhinophyma of the nose o blepharititis o chalazia o conjunctival injection o superficial punctate keratitis o iritis o corneal perforation (rare)

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WORK-UP: *external and slit-lamp examination TREATMENT: *facial rosacea >>>tetracycline >>>doxycycline *ocular rosacea o tetracycline or erythromycin, if symptomatic: Please note that clinically in practice for 2006: Main therapy includes po doxycycline 100mg bid with taper. For TMOD answer may be po tetracycline 250 mg QID o warm compresses and lid hygiene for blepharitis or meibomianitis o treat chalazia if present o culture and sensitivities and antibiotic therapy, if active infiltrate develops FOLLOW-UP: *mild to moderate: weeks to months *severe (with corneal involvement): frequent days SEBORRHEIC BLEPHARITIS ETIOLOGY *associated with a more generalized seborrheic dermatitis *probably represents a localized from of a more generalized disorder

DIAGNOSIS: *crusting of the lid and lash *crusts are more greasy than that of staphylococcal blepharitis *lid margins are less inflamed *rule out dermatological conditions seborrheic dermatitis rosacea *rule out meibomitis MANAGEMENT: lid hygiene a) soak initially with warm compress to soften and loosen crusts b) clean lid margin with dilute (50:50) baby shampoo solution or eyelid cleansers treatment of associated dermatological condition seborrheic dermatitis hair and scalp should be cleaned vigorously using selenium sulfide shampoo (Selsun) rosacea metro-gel, oral tetracycline or doxycycline treatment of associated meibomitis

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add lid massage and gland expression to lid hygiene regimen associated keratoconjunctivitis artificial tears and lubricants as needed topical antibiotic such as bacitracin, erythromycin or Polytrim as needed STAPHYLOCOCCAL BLEPHARITIS ETIOLOGY: *caused by Staphylococcus aureus and S. epidermidis *cosmetics are subject to microbial contamination and can be a source of bacterial infection DIAGNOSIS: *Symptoms 1) foreign body sensation 2) matting of the lids in the AM 3) itching, tearing, burning *Signs 1) inflammation of lid margin with collarette 2) chronic, papillary conjunctivitis 3) superficial punctate keratitis on inferior third of cornea 4) mucopurulent discharge 5) thickened lid margins (tylosis cillaris) and madarosis in chronic cases 6) occasional marginal corneal infiltration, phlycentenules, and hordeolum MANAGEMENT: *aggressive and consistent management mandatory 1) lid hygiene warm compress and lid scrubs 15 to 20 minutes BID to QID (esp. in the AM) 2) topical antibiotic ointment such as bacitracin or erythromycin or drops such as Polytrim 3) oral antibiotics for severe cases of blepharitis such as doxycycline 4) artificial tears and lubricants 5) culture and sensitivity if suspect resistant organism or considerable failure of therapy FOLLOW-UP: *initial follow-up: 2-3 days *if signs and symptoms improve follow-up: 1 week MIXED SEBORRHEIC-STPHAYLOCOCCAL BLEPHARITIS *combination of staphylococcal and seborrheic involvement

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MANAGEMENT: lid hygiene as described under seborrheic blepharitis QID; especially in the AM topical antibiotic ointment >>>bacitracin or erythromycin only if needed, steroid-antibiotic combination (to reduce inflammation and pain) treat associated dermatological condition indications and dosage as described previously under seborrheic blepharitis if not treated properly; can lead to a) chronic conjunctivitis b) permanent thickening of the lid margin (tylosis) c) madarosis MALIGNANT TUMORS OF THE EYELID SYMPTOMS: *most often asymptomatic *mild irritating eyelid lump SIGNS: *skin ulceration *inflammation of the eyelid *distortion of the normal eyelid structure DIFFERENTIAL DIAGNOSIS 1) seborrheic keratosis affects middle-aged to elderly patients presents as an elevated, brown-black well circumscribed crustlike lesion 2) hordeolum (stye) 3) chalazion 4) keratoacanthoma similar appearance to basal and squamous cell carcinoma elevated, central ulcerative crater grows rapidly to large size ( 1 to 2cm) AND slowly shrinks 5) cysts (epidermal inclusion, sudoriferous, sebaceous) well defined small white or yellow lesions 6) molluscum contagiosum multiple small papules with umbilicated centers viral origin associated chronic follicular conjunctivitis WORK UP: history a) lesion, present for how long? b) rapid or slow growth? c) previous skin malignancy? other malignancy? external examination thorough observation of the skin for additional lesions

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palpate preauricular and submaxillary nodes for possible metastasis slit lamp examination look for: loss of eyelashes destruction of meibomian gland openings telangiectasias over the margins photograph/draw include: size, location, color

BASAL-CELL CARCINOMA (most common malignant eyelid tumor) affects middle-aged to elderly patients does not metastasize, but can be locally invasive usually flesh colored but may be darkly pigmented - nodular firm mass with rolled borders; telangiectasic vessels may cross the borders; ulcerated center may be present MANAGEMENT: Surgically excise lesion due to locally invasive nature SQUAMOUS-CELL CARCINOMA variable presentation Characterized as scaly erythematous (reddish) lesion with a central ulceration, typically elevated More commonly found on upper lid Most commonly found on fair skinned older adults on sun exposed areas Metastasis may occur. Can be fatal. Premalignant lesions called actinic keratosis can turn into squamous cell carcinoma MANAGEMENT: Lesion surgically excised. Systemic oncology evaluation due to possible metastasis. SEBACEOUS-GLAND CARCINOMA presentation a) elevated lesions which resemble chalazion or blepharitis b) arises from the meibomian gland c) multifocal; upper & lower eyelids involved equally d) loss of eyelashes e) destruction of the meibomian gland orifices at the site of the tumor affects middle-aged to elderly patients metastasis or orbital extension can occur must be ruled out in cases of recurrent chalazion or resistant blepharitis - carefully evaluate for loss of lashes and changes to meibomian gland orifices.

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MANAGEMENT: Lesion surgically excised and biopsy. Systemic oncology evaluation due to possible metastasis.

MALIGNANT MELANOMA Presentation Darkly pigmented but color can vary Irregular borders Vary in size but most 6mm or larger Can arise from a dysplastic nevus or lentigo maligna metastasis from primary source (breast or lung) MANAGEMENT: Lesion surgically excised and biopsy. Systemic oncology evaluation due to possible metastasis.

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LACRIMAL DISORDERS CANALICULITIS Canaliculitis can be caused by bacterial, viral or fungal infections. Allergic etiology has also been reported. CLINICAL PICTURE: The patient will often present with a unilateral, red eye, not responsive to antibiotic treatment. Epiphora can also occur. Patients typically complain of tenderness or pain on the nasal portion of the lid and have chronic discharge. Hyperemia and edema of the nasal lid along with an enlarged puncta is typically noted. During probing of the canaliculus, compression or wrinkling of the medial canthal skin occurs in the presence of internal obstruction. MANAGEMENT: Obstructing concretions which can be removed either by expressing or surgically along with material expressed from the puncta should be sent for microbiologic testing. Irrigate the lacrimal system with an antibiotic solution. Treat according to the results of the micobiological testing. Oral antibiotics may be necessary. DACRYOADENITIS Inflammation of the lacrimal gland. Can be due to traumatic insult or a number of disease processes such as: orbital psuedotumor, sarcoidosis, dermoid, lymphoid, syphilis, TB, leukemia, lacrimal gland cyst. May also be a result of a benign epithelial tumor, malignant epithelial tumor or adenoid cystic carcinoma, CLINICAL PICTURE: The temporal one-third of the upper lid is swollen, causing an S-curve to the upper lid, which is diagnostic for dacryoadenitis. Also characterized by unilateral local pain, redness, conjunctival swelling and discharge. Proptosis may be present. Also a preauricular adenopathy can be present if the adenitis is due to a viral infection. MANAGEMENT: Neuroimaging may also need to be performed. Biopsy and histopathologic studies should be done to rule out lacrimal gland carcinoma or pleomorphic adenoma. Usually these tumors will not have signs of inflammation associated with them. Malignant tumors are treated with chemotherapy and radiotherapy. Benign tumors are surgically excised. Acute dacryoadenitis will most times respond rapidly to a dose of systemic corticosteroids. Bacterial dacryoadenitis, which is typically, mucopurlent should be ruled out. Bacterial dacryoadenitis should be treated with systemic antibiotics. Viral adenitis associated with systemic disease should be treated locally with ice and Tylenol.

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Systemic work up may be needed to rule out systemic conditions. DACRYOCYSTITIS (ACQUIRED or CONGENITAL) Infection or inflammation of the lacrimal sac due to an obstruction in the lacrimal system. Congenital form it is a frequent complication of congenital dacryostenosis. CLINICAL PICTURE: Patients present with a distension of the lacrimal sac complaining of pain and epiphora. Diffuse inflammation, chronic conjunctivitis or preseptal cellulitis may also be present. Typically congenital cases present more with a chronic presentation which has less severe signs than the acute form. Masses above this tendon should be considered mucoceles or until proven otherwise. The presence of a pus-filled sac with orbital cellulitis is a life-threatening condition due to potential pathogen spread to the brain tissue. MANAGEMENT: Attempt to determine cause of obstruction. Rule out mucocele (solid tumor), preseptal cellulitis. Culture nonresponsive cases. Acute cases: DO NOT PERFORM D&I!! Hot compresses can be used along with a broad-spectrum antibiotic (such as erythromycin or gentamycin) . Oral antibiotics such as amoxicillin. Especially in children, co-manage. Chronic cases: Massage lid vigorously at least qid and combine this with hot packs. In adult patients, a D&I can be performed. Topical antibiotics can be used if discharge is noted. OBSTRUCTIONS ACQUIRED Not uncommon in adults especially the elderly. Most common cause idiopathic but can be caused by dacryostenosis (narrowing of the canaliculi). Various disease processes of the eyelid margin, topical medication or trauma can cause dacryostenosis or mispositioning of the punctum. A mechanical obstruction such as from a tumor, occult matter or scarring due to trauma can also cause epiphora.

CLINICAL PICTURE/DIAGNOSIS: Patients will present with complaints or signs of epiphora. After ruling out signs of inflammation or infection, a tumor, punctal anomalies and nasal/sinus disease, the Jones number 1 test should be done. If fluorescein is present on the tissue, this is a positive result, indicating patent lacrimal drainage system. If no fluorescein is detected, this

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indicates a blockage in the drainage facility. Next one drop of proparacaine should be instilled. A punctal dilator is then used to widen the punctal opening. The Jones number 1 test is then repeated. If the test is positive, the occlusion has been opened, at least temporarily, and the patient should be re-evaluated in about 1 month. If the test is still negative, the Jones number 2 test should be done. If the patient tastes the saline or performs a gag reflex, the drainage system has opened and the patient should return in one month. If the Jones number 2 test fails to open the system, a dacryocystorhinostomy is required. CONGENITAL Most common cause is due to incomplete opening of Hasners membrane. CLINICAL PICUTURE: An infant will present with epiphora and possibly a dacryocystitis. Prevalence may be up to 6%. Neonatal conjunctivitis must be ruled-out in the presence of mucopurulent discharge. Often, pressure over the lacrimal sac will cause expression of purulent material through the puncta, indicating a dacryocystitis. MANAGEMENT: Conservative treatment is most often used. Topical antibiotics and decongestants are appropriate if the epiphora is associated with inflammatory signs. Also the parents should perform hydrostatic nasolacrimal massage. A finger placed over the common canaliculus and then stroked firmly downward in attempt to rupture Hasners membrane. This is repeated 5 times BID, and should be continued until the epiphora resolves or the child is 3 months old. After the age of 13 months, the lacrimal drainage sac can be probed under general anesthesia, with good surgical results. Failure of this procedure to work usually indicates nasal pathology. DISORDERS OF THE TEAR FILM LIPID DEFICIENCY Usually occurs secondary to inflammatory lid changes due to chronic blepharitis. CLINICAL PICTURE: Patients will usually present with staphylococcal or seborrheic blepharitis. Also a reduced tear break up time (TBUT) is present. MANAGEMENT: The blepharitis needs to be controlled with good lid hygiene and possible antibiotics (if staphylococcal). Tear substitutes and ointment will provide symptom relief while the blepharitis is still present. AQUEOUS DEFICIENCY A reduction of aqueous production occurs as a result of increasing age, certain systemic medications, and Sjogrens Syndrome. Lack of aqueous production at birth is called

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congenital alacrima. Keratoconjunctivitis sicca is a common clinical entity in older patients, and affects women more than men. CLINICAL PICTURE: KCS usually presents as gritty, foreign body sensation, with increasing severity as the day progresses. Examination will reveal tear film debris, reduced tear prism, rose Bengal/lissamine green staining of the conjunctiva and cornea, fluorescein staining to the cornea, and reduced TBUT. MANAGEMENT: Initial therapy usually includes artificial tears QID. Ointment at bedtime for more severe cases. Therapeutic(bandage) soft contact lenses are appropriate, especially if the abnormality of the tear film is causing recurrent corneal erosions. Punctal plugs are also used to help preserve the patients tears. Collagen disposable plugs are used diagnostically to determine if silicon plugs will be beneficial to the patient. Ocular lubricants containing vitamin A are excellent, especially if goblet-cell malfunction or surface abnormalities are present. MUCIN DEFICIENCY Due to goblet-cell dysfunction. Most often due to hypovitaminosis A, conjunctival insult, or disease. Ocular cictricial pemphigoid (OCP) is a chronic inflammation of the conjunctiva, leading to shrinkage and scarring. OCP results in a mucin deficiency. CLINCIAL PICTURE of Ocular Cicitricial Pemphigoid In OCP, normal conjunctiva is replaced with scar tissue. This disease is rare and affects women twice as often as men. It is an autoimmune condition of the elderly. OCP will usually begin with typical dry eye complaints. The conjunctival shrinkage and scarring can involve the lacrimal ducts, leading to aqueous deficiency. Progressive scarring can cause entropion, trichiasis, and lagophthalmos with exposure keratitis. End stage results are keratopathy with eventual keratinization of the cornea. Also corneal neovascularization and ulceration can occur. MANAGEMENT: Heavy lubrication is needed including nightly ointment. Any associated blepharitis needs to be treated. Surgical correction of early entropion and trichiasis is possible. Recent studies show topical vitamin A and immunosuppressive Tx to be helpful in treating OCP.

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CONJUNCTIVAL DISEASE CONJUNCTIVITIS ALLERGIC CONJUNCTIVITIS ALLERGIC CONJUNCTIVITIS Two types: 1. Immediate (type I) eosinophilic cellular response, mediated by serum immunoglobulins, and delayed (type IV) mononuclear cellular response, mediated by lymphocytes sensitized to antigens. CLINICAL PICTURE: Subjective: Itching, onset hours to days, unilateral or bilateral, possible history of allergy, seasonal. Signs: Conjunctival chemosis, possible lid edema, clear mucousy discharge, papillary response (with occasional follicles), and conjunctival injection. MANAGEMENT: 1. Pharmacologic agents, cold compresses, and avoidance of allergens are used to manage allergic conjunctivitis. Typically self limiting. If seasonal allergic reaction consider treatment just before season starts. 2. Therapeutic agents used include ocular decongestants, antihistamines (topical and systemic), topical NSAIDS, mast cell stabilizers, mast cell stabilizersantihistamine combinations and if severe topical corticosteroids. Allergies to cosmetics and topical medications are type IV and therefore do not respond well to antihistamines. These cases need topical steroids. ATOPIC DERMATITIS: Hypersensitivity state caused by hereditary factors. There is no seasonal exacerbation. Eczema may be present on the face, trunk and/or extremities. There is an association with atopy and keratoconus. Clinical picture includes erythema and scaling on the eyelids (secondary blepharitis may occur) papillary reaction on the palpebral conjunctiva, hyperemia and chemosis of the superior bulbar conjunctiva. Ropy discharge is sometimes present. Sterile (shield) corneal ulcer and pannus can occur. Therapy for the skin: corticosteroids Conjunctivitis: Topical corticosteroids and Mast Cell stabilizers are effective in providing symptom relief and may prevent pannus and corneal melting. Topical ocular decongestants, antihistamines and cold compresses should also be used.

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VERNAL CONJUNCTIVITIS ETIOLOGY: Typically occurs in the spring and lasts through summer/early fall. More prevalent in warm climates More prevalent in young males who are age 3 to mid 20s with a history of atopic conditions. Can reoccur for up to 10 years. CLINICAL PICTURE: Symptoms: Intense itching with stringy discharge. Foreign body sensation Signs: Bilateral inflammation of upper palpebral and/or limbal conjunctiva Pseudoptosis Cobblestone papillae Horner Trantas dots Limbal follicles Thick ropy discharge Corneal punctate keratitis Sterile shield ulcer can develop on cornea

MANAGEMENT: Cold compresses Topical antihistamine or antihistamine-vasoconstrictor combinations Mast cell stabilizers or mast cell stabilizer/ antihistamine combinations Topical NSAIDS Topical corticosteroids Oral antihistamine Consider lower maintenance dose once the condition is under control. If shield ulcer is present, treat with aggressive lubrication, patch or use bandage contact lens (reduce mechanical trauma), monitor for infectious keratitis (use prophylactic broad spectrum antibiotic), use topical and systemic agents aggressively to decrease inflammation. Some patients have benefited moving to a cooler climate (severe cases) ANGULAR CONJUNCTIVITIS Caused by Moraxella lacunata or Staphylococcus CLINICAL PICTURE:

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Sectoral inflammation of the nasal or lateral canthus. A papillary or follicular response may be present. MANAGEMENT: Caused by Moraxella: Treat with zinc sulfate erythromycin or tetracycline. Caused by Staphylococcus: Treat with Trimethoprim/ polymyxin B. AXENFELDS CHRONIC CONJUNCTIVITIS Found in children. A chronic follicular conjunctivitis with follicles of upper tarsus and fornix. Patients usually asymptomatic and without corneal involvement. Minimal discharge is usually present. Self limiting. Treatment is not needed. BACTERIAL CONJUNCTIVITIS ACUTE BACTERIAL ETIOLOGY Most common causes: Staphylococcus. Other causes: Streptococcus, Haemophilus, Moraxella, Chylamidia, and Pseudomonas aeruginosa All ages affected Associated with history of blepharitis SIGNS/SYMPTOMS: Initial tearing and foreign body sensation followed by conjunctival injection. Conjunctival injection usually more marked in the cul du sac;limbal area relatively clear Diffuse superficial punctate keratopathy may be present Mild mucopurulent discharge which may cause morning lash matting Unilateral or bilateral Papillae on palpebral conjunctiva No preauricular lymphadenopathy MANAGEMENT: Isolated, bacterial conjunctivitis is self-limiting and will resolve in 7-10 days To relieve patient symptomology Supportive therapy such as warm compresses and lavage. *Broad spectrum topical antibiotic bacterial conjunctivitis such as: gentamicin QID, tobramycin QID, trimethoprim/polymyxin (Polytrim) Q3H. Can supplement ung at bedtime if necessary. Vigamox 1gt TID x 7 days also an option.

*If significant inflammation: consider topical steroid-antibiotic combinations

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*Polysporin, gentamicin, tobramycin or erythromycin ung. May be supplemented QHS or QID for children. Consider more specific treatment, if causative organism is known: Staphylococcus (most common cause, exotoxins cause inferior punctate keratitis), Streptococcus (petechial hemorrhages common), Haemophilus (associated with upper respiratory infections and more common in children), Moraxella (causes angular conjunctivitis, common in alcoholics), Chylamidia (nonresponsive with topical medications, 2 to 4 week history) If unresponsive to treatment, culture to determine causative organism. HYPERACUTE BACTERIAL EPIDEMIOLOGY N. gonorrhoeae and N. meningitidis Found more frequently in children and young adults as well as parents of children CLINICAL PICTURE: Rapidly advancing Marked hyperemia Conjunctival chemosis Severe lid edema Foreign body sensation Copious purulent discharge Prominent preauricular lymphadenopathy. Corneal epithelial staining (possible ulceration) True or pseudomembranes may form MANAGEMENT: Laboratory work up indicated Conjunctival scrapes for Giemsa stain, Gram stain and culturing with blood agar (grows most organisms), Thayer-Martin (grows Neisseria) and chocolate agar (grows Neisseria and Haemophilus). (Rule out/detect syphilis and chlamydia.) Prompt, aggressive treatment is necessary to prevent globe perforation. Topical and systemic medication administered concurrently: Topical therapy: Topical fluoroquinolone Q 2h. Include frequent ocular lavage to reduce the purulent exudates maceration of the corneal epithelium. Oral / intramuscular therapy: Penicillin, Cephalosporin, or erythromycin. If N. gonorrhea or N. meningitides is suspected should receive full dose of systemic therapy. Refer sick children with bilateral hyperacute conjunctivitis due to the possibility of N. meningitides When results of cultures are known modify treatment for causative organism. Follow up

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Reexamine within 24 48 hours due to possible corneal complications. Modify therapy based on progression or resolution of condition. CHLAMYDIAL CONJUNCTIVITIS Caused by an obligate intracellular organism. Two species: Chlamydia trachomatis and Chlamydia psittaci. Trachoma is still a major medical concern in some under-developed nations, and is still occasionally seen in the United States among American Indians. Inclusion conjunctivitis is the most common acute follicular conjunctivitis, and tends to become chronic. CLINICAL PICTURE: Trachoma: Starts as chronic follicular, superior palpebral conjunctivitis. This is followed by papillary hypertrophy and inflammatory infiltration, which masks the follicular component. Photophobia and lacrimation are common. The papillary hypertrophy is followed by conjunctival scarring (Arlts line = white line) which can cause cicatricial entropion or trichiasis. This can lead to vision threatening, corneal ulceration and scarring. Corneal involvement usually includes a superior punctate keratopathy, marginal infiltrates, and pannus. The whitish depressions caused by limbal follicles cicatrization are known as Herberts pits. Inclusion conjunctivitis: Occurs in young adults who usually have been with a new sex partner. It is characterized by acute follicular conjunctivitis with mucopurulent discharge. Usually presents unilaterally with a preauricular lymphadenopathy. During the second week of clinical infection, a keratitis may develop in addition to corneal infiltrates and pannus. Iritis can develop later in the disease. Seems to affect the lower fornix and palpebral conjunctiva. (No Herberts pits; No Arlts line in inclusion conjunctivitis.) With inadequate treatment condition becomes recurrent or chronic. MANAGEMENT: Trachoma will usually respond to oral tetracycline 250 mg tab PO QID for 3 weeks, erythromycin 500 mg tab PO QID for 3 weeks, topical tetracycline-polymixin ung x 6 weeks or oral azithromycin. For inclusion conjunctivitis, conjunctival scraping and culture for diagnosis (inclusion bodies with Giemsa staining), oral doxycycline, tetracycline or erythromycin is taken. Concurrent with systemic treatment: vasoconstrictors, antibiotic steroid combinations. Comanage with physician for systemic condition. CHRONIC / TOXIC CONJUNCTIVITIS Caused by prolonged exposure to substances causing toxic conjunctival reaction.

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ETIOLOGY Secondary to marginal blepharitis or chronic dacryocystitis S. aureus most common bacterial cause Irritating causes such as airborne irritants, allergens, dry conditions Toxic causes such as chemicals, UV radiation, topical drugs (such as echothiophate, phenylephrine, and pilocarpine), systemic medications., or bacterial or viral toxins External factors such as contact lenses, wind Irritating conditions which affect lids such as pediculosis (see lid section) CLINICAL PICTURE: Chronic red eye presentation either unilateral or bilateral Bulbar hyperemia Papillary or follicular conjunctivitis (dependant on cause) If follicular conjunctivitis: preauricular lymphadenopathy typically not found May see phylectenule MANAGEMENT: Rule out other causes of red eye. Establish primary cause. Treat for or eliminate primary cause. Consider: lid hygiene, cold packs, oral antibiotic such as Doxycycline, topical broad spectrum antibiotic, oral antihistamine, lubricants. If unknown etiology stop or change all ophthalmic meds except preservative-free tears. GIANT PAPILLARY CONJUNCTIVITIS ETIOLOGY: Contact lens wear (especially hydrogel extended wear) NOT as common with the introduction of planned replacement lenses and disposables Prosthesis Exposed PKP suture Other mechanical irritants CLINICAL PICTURE: Symptoms: Range: may be asymptomatic or complain of itching, fluctuating vision, stringy discharge, and if a contact lens wearer a loose lens/toric rotation Signs: Papillary response of upper palpebral conjunciva. Size of papillae - large. Mucoid discharge (typically severe) May have punctate keratitis Bilateral

MANAGEMENT: GPC caused by CLs:

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Mild GPC can be resolved with discontinuation (or significant reduction) of CL wear alone. Frequent replacement of lenses or use of disposable lenses is usually effective in reducing chance of developing GPC, or resolving mild GPC. If needed, can prescribe topical agents listed below. GPC from all causes o Topical antihistamine-vasoconstrictor combinations o Mast cell stabilizers Mast cell stabilizers/antihistamine combination o Topical NSAIDS o Topical corticosteroids (site specific or those with mild or moderate strength)

OPHTHALMIA NEONATORUM Neonatal conjunctivitis occurs by 3 routes of inoculation: 1. contact with infection of cervix or vagina due to early membrane rupture 2. passage through infected birth canal 3. contact with infected material soon after birth PROPHYLAXIS AGAINST NEONATAL CONJUNCTIVITIS Diagnose and treat infections in mother during prenatal care Credes prophylaxis: Mechanically clean the neonates eyelids before the eyes open. Instill 1% silver nitrate into the conjunctival sac. Has greatly reduced ophthalmia neonatorium. It is a cause of chemical neonatal conjunctivitis. Alternative 0.5 % Erythromycin or 1% tetracycline ointment. Advantages: as effective as silver nitrate and low conjunctival toxicity. Neither Silver nitrate, tetracycline nor erythromycin provide coverage against chlamydia. BACTERIAL Most common. S. aureus, S. pneumonia, S. pyogenes Less common: Haemophilus, P. aeruginosa, N. gonorrhea N. gonorrhea Clinical signs: hyperacute conjunctivitis, mucopurulent. Onset: 3-5 days Other bacterial Clinical signs: diffuse conjunctival injection, mucopurulent Onset: >5 days MANAGEMENT: Rule out congenital dacryocystitis Culturing is mandatory. Gram stain, Thayer-Martin or chocolate agar Infants with gonococcal conjunctivitis should be hospitalized and treated with intravenous or intramuscular antibiotics (ceftriaxone). Nongonococcal bacterial conjunctivitis can be treated with broad spectrum antibiotics such as: Ointment bacitracin, erythromycin, tetracycline Solution gentamicin, tobramycin, or polytrim.

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CHEMICAL Occurs secondary to topical 1% silver nitrate (Credes prophylaxis). CLINICAL PICTURE: Watery discharge associated with conjunctival hyperemia No treatment needed CHLAMYDIAL Leading cause of neonatal conjunctivitis in the U.S. Usually occurs 5 -10 days after birth. Clinical presentation usually is a mucopurulent conjunctivitis with lid edema, chemosis and conjunctival membrane or pseudo membrane. Conjunctival scrapings for diagnosis (Giemasa stain: basophilic cytoplasmic inclusion bodies). MANAGEMENT: Rule out congenital dacryocystitis Chlamydial conjunctivitis should be treated with erythromycin orally. Parents of the child should be evaluated and treated. VIRAL Viral neonatal conjunctivitis is rare. Causes: herpes simplex and cytomegalovirus. CLINICAL PICTURE: Similar to adults. MANAGEMENT: Rule out congenital dacryocystitis Herpes simplex conjunctivitis is treated with trifluridine. PARINAUDS OCULOGLANDULAR SYNDROME History of exposure to cats. Ocular cat scratch fever Acute, unilateral presentation of large follicles with deep yellow core, conjunctival granulomas and ipsilateral lymphadenopathy. Fever and generalized malaise is often present. MANAGEMENT Self limiting Ocular: broad spectrum antibiotic (Gentamycin, Bacitracin, and Tetracycline). Systemic treatment: antibiotics and NSAIDS.

PHLYCTENULAR CONJUNCTIVITIS

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Hypersensitivity reaction to tuberculin protein and Staphylococcus antigens. Inflammatory conjunctivitis with development of conjunctival or corneal inflammatory nodules. CLINICAL PICTURE: Symptoms: - Foreign body sensation, discomfort , pain or photophobia. - Localized bulbar conjunctival or corneal inflammatory nodules, which are small, raised and pinkish white. If caused by Staphylococcus antigens blepharitis and conjunctivitis may be present. MANAGEMENT: Topical ocular decongestants can be used to treat mild cases. Topical steroids are needed for more advanced cases. Eliminate underlying cause. Treat blepharitis and conjunctivitis as specified in those sections. All patients should be evaluated for tuberculosis (CXR and PPD). SUPERIOR LIMBIC KERATOCONJUNCTIVITIS Chronic and recurrent inflammation of the superior conjunctiva. CLINICAL PICTURE: Symptoms: Foreign body sensation, photophobia, and lacrimation Signs: Bilateral superior conjunctival and limbal hyperemia. Papillary reaction on the superior palpebral or tarsal conjunctiva. Punctate kerititis (rose bengal staining) of the superior cornea is present. Filamentary keratitis of superior cornea is common although etiology is unknown. Some patients may have thyroid dysfunction. MANAGEMENT: In mild cases, lubricants (artificial tears or bland ointment) or soft contact lens can relieve symptoms. In more advanced cases, topical corticosteroids or mast cell stabilizers are used. If filaments present, consider 10% acetylcystine QID. In severe cases, .5% silver nitrate is used followed by irrigation. If still no relief, consider mechanical scraping, cautery, cryotherapy or surgical resection. Perform testing to rule out thyroid dysfunction. VIRAL CONJUNCTIVITIS Follicular ACUTE HEMORRHAGIC CONJUNCTIVITIS Causes: enterovirus 70. CLINICAL PICTURE:

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Fast incubation period. Bilateral, follicular conjunctivitis, prominent subconjunctival hemorrhages, duration up to 5 days, and teary discharge. Systemic and corneal involvement rare. Highly contagious. MANAGEMENT: Resolves without treatment within one week. Lubricants, ocular decongestants, and cold compresses used. Topical antibiosis if secondary bacterial infection occurs. EPIDEMIC KERATOCONJUNCTIVITIS Associated with Adenovirus 8, 19, 21, 37. Any age affected. CLINICAL PICTURE: Symptoms: Patient usually complains of foreign body sensation and tearing. Patient may report expose to another case. Signs: - Follicular reaction on the palpebral conjunctiva - Diffuse pinkish-purplish bulbar hyperemia - Conjunctival petechial hemorrhages - Chemosis - Lid edema - Preauricular lymphadenopathy. - Pseudomembrane formation is common in severe cases. RULE OF 8s First 8 days conjunctivitis; Eighth day superficial punctate keratitis develops.: By the end of the second week (approx. day 16), subepithelial infiltrates form which cause a reduction of vision if over the visual axis. The keratopathy resolves in 1 month and the infiltrates resolve usually within 4 months, but may take years to resolve. MANAGEMENT: Normally self-limiting. Lubricants, cold compresses, and ocular decongestants are used as supportive therapy. Prophylactic antibiotics can be used to prevent secondary bacterial infection. Corticosteroids are controversial since herpes simplex can mimic EKC. Corticosteroids are usually used to increase patient comfort, if the infiltrates reduce vision, or to reduce pseudomembrane formations. Need a long, slow taper to prevent the infiltrates from returning. Antivirals are not indicated, due to their toxicity and the self-limited course of EKC. Sanitary conditions are a must to help prevent the transmission of EKC. HERPES SIMPLEX 90% of herpes simplex virus infections remain asymptomatic and do not cause ophthalmic involvement. HSV 1 is most commonly associated with ocular manifestations, but HSV 2 is possible in newborns and adults. CLINICAL PICTURE:

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Clinical presentation is same for HSV 1 and 2. Follicular conjunctivitis with prior or simultaneous vesicular lesion of the eyelids. Conjunctivitis can last for 3 to 4 weeks. Herpetic punctate keratitis with dendritic corneal ulceration is common. (Carefully evaluate cornea). Tends to be unilateral with watery discharge. Recurrence is common with 50% of patients having another episode within 2 years. NOTE: SEE CORNEAL SECTION FOR DETAIL ON CORNEAL LESIONS. MANAGEMENT: Trifluridine 1%: First drug of choice. Vidarabine 3% ointment Used in individuals who are not responding to trifluridine or are sensitive to idoxuridine. NOT COMMERCIALLY AVAILABLE. Idoxuridine 0.1%:. IDU is usually tolerated well, but some moderate allergic reactions have been reported. Corticosteroids are contraindicated in herpes simplex keratitis with epithelial defects. HERPES ZOSTER SEE CORNEAL SECTION Follicular conjunctivitis treated with cool compresses, erythromycin ung Oral antiviral PHARYNGOCONJUNCTIVAL FEVER Most frequently caused by adenovirus 3, but sometimes 4, 7 or 14. Mostly affects children and teens that have a recent history of sore throat and fever. Known as swimming pool conjunctivitis due to possible mechanism in which virus is spread. CLINICAL PICTURE: Symptoms: Watery eyes, irritation, photophobia and foreign body sensation. Signs: Triad of follicular conjunctivitis with history of pharyngitis and fever. Lower palpebral conjunctival fornices usually affected. Preauricular node enlargement. Usually self-limiting in 1 to 3 weeks. MANAGEMENT: No curative therapy; only supportive. Ocular decongestants, lubricants, cold compresses and Tylenol. Topical antibiotics only needed with secondary bacterial infection. VIRAL CONJUNCTIVITIS SECONDARY TO SYSTEMIC DISEASE.

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LYME DISEASE Follicular conjunctivitis is one of the most common ocular manifestations of Lyme disease. MEASLES Upper respiratory tract is the primary entry site. Conjunctiva is a secondary entry area. No racial or sex predilection. Mild catarrhal conjunctivitis with occasional chemosis. MUMPS Caused by a RNA virus. Mumps is a childhood disease. Characterized by mild conjunctivitis without discharge. Rarely, interstitial keratitis, dacryoadenitis, and optic neuritis have been reported. NEWCASTLE DISEASE A disease of poultry. Characterized by mild follicular conjunctivitis, preauricular lymph adenopathy, and mild upper respiratory infection symptoms.

MANAGEMENT: 1. All of these may have a secondary bacterial infection causing mucopurulent discharge. The bacterial component should be treated with a broad spectrum topical antibiotic (gentamicin) four times daily. 2. Treatment of these viral conjunctivides is supportive (ocular decongestants, cold compresses, and lubricants). 3. The topical antibiotics should be used prophylactically. OTHER CONJUNCTIVAL DISEASE CONJUNCTIVAL LACERATION AND ABRASIONS CLINICAL PICTURE: Conjunctival hyperemia, chemosis, and conjunctival hemorrhage Moderate pain and foreign body sensation is common MANAGEMENT: Must rule out ocular penetration before pressure is applied to the globe. Any globe perforations should be immediately referred after a Fox shield has been applied. Lavage is needed to remove retained foreign bodies. Cycloplege the eye, apply erythromycin or Gentamicin ointment, then pressure patch if needed. Usually only large lacerations need surgical repair. SEE CORNEAL LACERATION AND ABRASIONS FOR MORE INFORMATION. MUCOUS MEMBRANES DISORDER OCULAR CICATRICIAL PEMPHIGOID (OCP):

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Bullous condition of skin and mucous membranes affecting patients 55 and older. An immunocomplex disease. Can occur secondary to Stevens-Johnson syndrome. CLINICAL PICTURE: Symptoms: Dry eye, tearing and photophobia Signs: Typically bilateral chronic, progressive shrinking of inferior conjunctiva which can cause entropion and trichiasis. Symblepharon formation may occur inferiorly. The symblepharon formation can reduce ocular motility. Chronic, severe dry eye can lead to keratinization of the cornea as well as ulcers and pannus. Bullous vesicles can form on the nose, mouth , pharynx, etc. MANAGEMENT: Intensive lubricants are needed. Artificial tears Q1H with lubricant ointment QHS is recommended. Consider using punctal plugs and bandage contact lenses. Bacitracin Polymyxin B is used to treat the concurrent blepharitis. Treat complications such as entropian and ulcers as stated in their sections. Oral corticosteroids and immunosuppressive agents have been used to treat these cases. STEVENS-JOHNSON SYNDROME Bullous disease of skin and mucous membranes usually affecting young men (lips appear black). Mild form involving skin only: erythema multiforme. Precipitating factors are sometimes associated with development of the disease: Toxic or allergic reactions to drugs (including topical sulfonamides), radiation exposure, neoplasm, and herpes simplex infection. CLINICAL PICTURE: Dry eye, keratitis sicca, corneal ulceration, entropia, destruction of puncta. Conjunctival scarring and symblepharon formation. Macules, papules, vesicles and bullae on extremities. MANAGEMENT: Intensive lubricants are needed. Artificial tears Q1H with lubricant ointment QHS is recommended. Consider using punctal plugs and bandage contact lenses. Antibacterial ointment to prevent secondary infection. Treat complications such as entropion and ulcers as stated in their sections. Oral corticosteroids have been used to treat these cases. OCULODERMATOLOGIC DISORDERS PSORIASIS

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Chronic papulosquamous disorder with sharply demarcated erythematous patches overly gray scales. If try to remove these scales, get pinpoint bleeding (Auspitz sign). Tends to affect the scalp, knees, and elbows. The clinical picture is yellowish red plaques on the conjunctival form secondary to psoriatic blepharitis. Topical and oral steroids are used to treat this condition. Phototherapy (PUVA) is used to treat severe psoriasis. (A side effect of PUVA is cataracts.) ROSACEA Papular-pustular eruptions with cutaneous erythema affecting women ages 30-50 (about 60%). Systemic signs are chronic congestion of the capillaries, telangiectasias, and sebaceous gland hypertrophy of the face and rhinophyma of the nose. Patient complains of foreign body sensation and recurrent chalazia. CLINICAL PICTURE Lids may be thickened and hyperemic with telangiectasic vessels on the lid margins. Conjunctival hyperemia and chronic blepharitis are most common. Corneal superficial punctate keratitis especially on the lower third of the cornea may be present. Corneal neovascularization, corneal infiltrates and corneal ulcers are less common. MANAGEMENT: Oral doxycycline 100mg BID x 2 wks, then taper or oral tetracycline 250 mg tab PO QID tapered over several weeks. Oral erythromycin may be used if tetracycline is not tolerated. May consider a chronic treatment with a low dose of oral medication. Topical bacitracin-polymyxin B lid scrubs qhs is recommended to eliminate the common staphylococcal blepharitis. Topical antibiotic-steroid combination or steroid may be used to treat blepharoconjunctivitis or corneal infiltrates. Treat chalazion and SPK as mentioned in those sections. PTERYGIUM Triangular hyperplasia of conjunctival tissue which extends onto the cornea. Associated with environmental exposure and UV radiation. It is also common in individuals who work outdoors and welders. As the pterygium approaches the cornea, it first appears as a subepithelial halo called a cap. This grayish-white avascular zone is considered the leading edge of the head of the pterygium. Stockers line (iron line) may be seen on the cornea preceding growth. Corneal dellen formation may occur adjacent to the pterygium. Artificial tears may prevent symptomology related to a mild pterygium. Ocular lubricants should be used if dellen is present to prevent corneal punctate keratitis. Surgical removal may result in a recurrent pterygia, which is more aggressive and harder to treat SYMPTOMS: Redness, photophobia, tearing, foreign body sensation. SIGNS: Triangular, fleshy lesion found in the interpalpebral zone. May be red or inflamed. An iron line may be seen in static pterygia more central to the cap. This is a Stockers line. Induction of corneal astigmatism is a possibility.

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MANAGEMENT: Small pterygium with mild symptoms: a) Sunglasses with UV-A & UV-B protection b) Topical lubricants PRN c) Avoid smoke and dust Larger pterygium: possible visual axis involvement Surgical excision Mitomycin C during or after the procedure to decrease reoccurrence rate SUBCONJUNCTIVAL HEMORRHAGE Usually due to valsalva maneuver (heavy lifting), chronic cough, constipation, bleeding disorders, trauma, surgery, blood-borne diseases. Recurrent subconjunctival hemorrhages without cause should be investigated to rule out anemia, diabetes, hypertension, or other microvascular abnormalities. Treatment is to reassure the patient. Cold compresses the first 24 hrs; warm compresses thereafter.

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SCLERAL AND EPISCLERAL DISEASES EPISCLERITIS 1. -Simple 2. -Nodular EPIDEMIOLOGY: benign typically unilateral inflammation occurs in young adults; females > males peak incidence between second and fourth decade relatively common/can occur spontaneously ETIOLOGY: majority of cases unknown etiology *associated with herpes zoster, gout, syphilis, rheumatoid arthritis, Crohns disease, hepatitis B, systemic lupus erythematous, and Lyme disease SIMPLE EPISCLERITIS SYMPTOMS: mild discomfort photophobia pain-located to eye; may radiate to head tearing SIGNS: Sectoral injection and episcleral congestion; may involve entire globe brick red appearance no bluish tinge episcleral tissues may be infiltrated with deposits which turn yellow under red free light MANAGEMENT: * depends on severity * without treatment improve 50% within 1st week - can be completely resolved within 32 weeks * Mild episodes - cold compresses - vasoconstrictors * Moderate or Severe episodes -mild topical steroids -oral NSAIDS * if recur, refer out to internist to R/O systemic disease

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NODULAR EPISCLERITIS *symptoms similar to simple episcleritis localized cellular infiltrate forming a nodule with surrounding congestion nodule is movable nodule may be single or multiple increased edema and infiltration throughout sector MANAGEMENT: * resolves more slowly than simple episcleritis * MILD to MODERATE episodes -topical corticosteroids every 2-4 hours * SEVERE episodes or nonresponsive cases Oral steroids * referral to internist to R/O systemic disease SCLERITIS EPIDEMIOLOGY/ETIOLOGY: relatively rare females>males fourth sixth decade in life associated systemic conditions: Connective tissue disease Rheumatic disease, Lupus, ankylosing spondylitis Herpes zoster, TB, gout, syphilis, Ocular surgery SYMPTOMS: * HALLMARK -severe pain; can radiate to jaw, sinus, temple pain * tender to the touch * pain may be greater than ocular findings * inflammation is a prominent feature * tearing SIGNS: * usually a purplish or bluish color (more easily seen in natural light) compared to the bright red of episcleritis * superficial and deep layered vessels become engorged and tortuous, lose their normal radial pattern and become beaded in appearance (do not bleach with phenylephrine) * inflammation can involve small segment or whole anterior segment (brawny/red meat appearance when superficial tissues are severely inflamed). * sclera edematous and thinned * corneal: peripheral thinning, stromal and endothelial disruption * Anterior uveitis

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MANAGEMENT: * treat underlying cause *treat with systemic medications : steroids, NSAIDS, or immunosuppressive therapy (methotrexate, cyclosporin) * referral for complete physical examination * hematology studies -CBC -WBC with differential -ESR, ANA, FTA-ABS, RPR -uric acid level * x-ray tests -chest, hands and feet, lumbosacral spine COMPLICATIONS OF SCLERITIS: * occur late in disease or during very acute inflammation * occur most frequently with posterior scleritis and necrotizing scleritis with inflammation -decrease VA -uveitis -glaucoma -cataracts -RD -Optic nerve swelling ANTERIOR SCLERITIS DIFFUSE ANTERIOR SCLERITIS most common form NODULAR ANTERIOR SCLERITIS *scleral tissue is immobile and edematous *edematous episclera adherent to nodule and tender to the touch *sclera may become transparent beneath nodule *sclera does not become necrotic and inflamed NECROTIZING ANTERIOR SCLERITIS (WITH INFLAMMATION/WITHOUT SCLEROMALACIA PERFORANS) * most serious form of scleritis extreme pain * highest complication rate *> 60% develop complications other than scleral thinning 40% loss of VAs * begins in localized area with acute congestion of vessels * vessels become distorted or occluded * gradual onset of painful, injected eye * underlying sclera becomes transparent

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* uveal layer may be observed when viewed through daylight (choroidal pigment seen) * High association with systemic inflammatory disease MANAGEMENT: * if advanced stage, immediate referral NSAIDS and H2 blocker, systemic steroids, and immunosuppressive Tx NECROTIZING (WITHOUT INFLAMMATION/WITH SCLEROMALACIA PERFORANS) * characterized by complete lack of symptoms * occurs predominantly in females, between 50-75 years who suffer from longstanding polyarticular rheumatism POSTERIOR SCLERITIS * can be an extremely serious/destructive disease * can lead to loss of vision -severe pain -perforation of globe -loss of involved eye * difficult to diagnose because unable to view posterior sclera * Dx may be made by ultrasound * suspicion for disease high if pain or discomfort outweighs signs * can be an extension of anterior scleritis * very few signs unless anterior sclera involved * May also find: proptosis, choroidal folds, may simulate an amelanotic choroidal mass, RD, disc edema, macular edema, retinal hemorrhages or angle closure glaucoma PIGMENTATION ABNORMALITIES Blue Sclera robins egg-blue -hallmark of osteogenesis imperfecta and other collagen disorders -defect in the development stage of sclera -thinning of sclera with uvea showing through (blue) -white ring Saturns ring can present around limbus due to direct contrast to adjacent thinning sclera -choroidal tumors can cause localized black area THINNING (ECTASIA) * softening and bulging of sclera Staphylomas a) posterior staphyloma -most common -usually due to posterior uveitis -high myopia b) anterior staphyloma -due to anterior inflammation

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c) d)

i.e. pars planitis -trauma intercalating staphyloma -due to inflammation around limbal area total staphyloma -total bulging of eyes (Buphthalmos) -commonly seen in congenital glaucoma

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CORNEAL DISEASE CONTACT LENS RELATED CORNEAL PROBLEMS: 3-9 OCLOCK STAINING Desiccation of the peripheral cornea, which is the result of poor tear film-lens, edge interaction. Lens movement, position, and excessive peripheral lens edge lift may be responsible. Patients are usually asymptomatic. Modifications of the peripheral curve or cutting down edge thickness may alleviate the problem. Lenses may also need to be refit. CORNEAL STRIAE Vertically, centrally located, grey or black lines in the deep cornea. Indicates corneal hypoxia and stromal edema greater than 6%. Patient may be mildly symptomatic. Treatment is to switch to a thinner lens or higher water content lens. DIMPLE VEILING (STIPPLING) Small indentations into the epithelium secondary to bubbles or debris trapped under a lens. May be seen more easily with fluorescein staining. Patient is asymptomatic. Treatment is to flatten or loosen the fit of the lens. Discontinue lens wear, if it is persistent. EPITHELIAL MICROCYSTS Pockets of cellular debris and fluid. Edema within the cornea results in fluid accumulation in the intraepithelial cellular spaces. Microcyst formation is a direct result of corneal hypoxia. TX: Refit into a different material, higher DK, decrease lens wear, or fit flatter. INFILTRATES Can be due to a number of causes such as overwear. Often infiltrates may be seen as sterile inflammation secondary to trapped debris under a tight fighting CW lens. Patients may report redness, discharge, photophobia, and lens intolerance. One must always rule out an infectious etiology! Deprivation of lenses until the infiltrates clear is the treatment. Should clear within 1-2 weeks. May initiate topical steroid if apperars noninfectious. Refit the lens and recommend daily wear. NEOVASCULARIZSATION Perilimbal vascular loops, twigs or branches which extend more than 1.5 mm into the corneal tissue. Stromal swelling allows blood vessel advance to provide nutrients and oxygen to hypoxic areas. Also neovascular growth may be a response to chemotaxic factors released during epithelial injury. Patients are asymptomatic. Rule out inflammatory disease and mechanical aggravation. Treatment is to fit a looser lens with higher Dk, discontinue continuous wear, and measure the neovascular growth. POLYMEGATHISM Characterized by change in the size of endothelial cells. Normal age-related changes occur to the endothelial cells, however, soft hydrogel lens wear can accelerate these changes. Patients are usually asymptomatic but may experience decreased lens tolerance

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and increased rate of corneal de-swelling. Patients should be discontinued from continuous wear lenses or be worn with extreme caution. Also, increase the permeability of oxygen to the cornea. PSEUDODENDRITES Branching, infiltrative, epithelial surface lesions which is usually secondary to solution toxicity. Mostly reactions to chlorhexidine and thimerosal. Resemble slightly raised lesions. Must rule out Herpes Simplex and Herpes Zoster. Discontinue lens wear temporarily and chemical disinfection. Return in 1 week. SUPERFICIAL PUNCTATE KERATITIS SEE IN CORNEAL SECTION CORNEAL DEGENERATIONS ARCUS SENILIS SYMPTOMS: Asymptomatic. SIGNS: Depending on appearance. Usually bilateral, light or dense white annular ring around the peripheral cornea with a clear zone between the white ring and the limbus. MANAGEMENT: 1) Patient under 40. Baseline serum cholesterol Medical work-up to rule out cardiovascular disease. 2) Older than 40 Consider work up for hypercholesteremia based on number of risk factors for cardiovascular disease (hypertension, obesity, stress, smoking, diet, etc.) 3) Internist to manage serum cholesterol, if elevated, to reduce risk of cardiovascular disease. BAND KERATOPHATHY SIGNS: Calcium plaque formation in the interpalpebral zone of the cornea which is typically bilateral. It can move across the cornea. Whitish-yellowish haze in epithelium and Bowmans layer. Holes within the plaque give the appearance of swiss cheese. CAUSES: Chronic iritis (Juvenile rheumatoid arthritis (JRA)), interstitial keratitis, long standing hypercalcemia (hyperparathyroidism, vitamin D intoxication, etc.), Exposure to irritants and gout. SYMPTOMS: Asymptomatic or decreased vision.

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TREATMENT: 1) Attempt to determine underlying cause. a) Younger patients: Work-up for Stills triad: JRA b) Workup for hyperparathyroidism. 2) Mild Cases lubricant therapy 3) Chelation of the band keratopathy more severe cases. a) EDTA ethylenediamine tetraacetic acid Debride cornea with 5% cocaine solution. EDTA is placed on a swab and wiped on band keratopathy. 4) PTK (Phototherapeutic keratectomy) in severe cases. BULLOUS KERATOPATHY Due to advanced, prolonged corneal edema interfering with the ability of the endothelial cells to pump out water from the cornea. PREDISPOSING FACTORS: Post-Intraocular surgery aphakia, pseudophakia Intraocular inflammation Vitreous, IOL touching endothelium Fuchs endothelial dystrophy Chronic elevated intraocular pressure Progressive loss of endothelial cells SYMPTOMS: Pain, photophobia, decreased vision, tearing. Eye may be red. SIGNS: Corneal edema with irregular corneal surface, bullae. scarring, and guttata in Fuchs disease. May see neovascularization,

MANAGEMENT: Medical management based on symptoms present. Treat aggressively prior to formation of bullae. Hypertonic salt solution 5% QID to Q 1-2h Hypertonic salt ointment 5% UNG HS Hair dryer in the A.M. back in forth at arms length for 5-10 minutes. Increases evaporation. High water soft contact lens To treat bullae. If bullae produce significant epithelial defects, this may require pressure patching or therapeutic contact lenses. May require surgery, if related to IOL. Penetrating Keratoplasty Improve acuity in centrally involved cases. Poor prognosis when neovascularization is present. DELLEN

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Focal peripheral depressions or thinning usually associated with an adjacent elevated mass such as a pingueculum. It is stromal thinning associated with disiccation and is reversible. SYMPTOMS: Asymptomatic SIGNS: Intact epithelium, occurs usually between 3 and 9 oclock. May have the appearance of a small hole. ASSOCIATED LESIONS: Raised lesions such as pinguecula, large sub conjunctival hemorrhage, filtering bleb, chalazia, thick edge of a contact lens, etc. MANAGEMENT: Attempt to reduce the mass, cause Topical lubricants Q 2-3H QID, UNG HS MARGINAL FURROW DEGENERATION Can be idiopathic in nature or have an underlying systemic disease entity. In the elderly, peripheral thinning may be seen adjacent to areas of arcus senilis. This is benign and does not lead to perforation. However, furrowing changes as well as ulcers can occur with systemic disease entities. Each disease may present differently but can cause peripheral corneal changes and ultimately, in some cases, perforation. SYMPTOMS: Asymptomatic or can present with pain and photophobia. SIGNS: Peripheral corneal thinning. possible. Peripheral infiltration, ulceration, and perforation are

TREATMENT: 1) Differential diagnosis: - infectious vs. sterile ulcer. - idiopathic vs. systemic cause - peripheral thinning disorders. 2 ) May require systemic work up to rule out underlying disease: (ANA, CBC, Rheumatoid factor, ESR) 3) Look for other signs such as hypopyon, iritis, dendrites, scarring as signs of other processes. Consider scraping or culturing ulcers to rule out infectious etiology. 4) Treat by severity and signs: Idiopathic: no treatment. This usually occurs.

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Ocular lubricants Q1h and UNG HS PTERYGIUM SEE CONJUNCTIVA SECTION SALZMANNS NODULAR DEGENERATION SYMPTOMS: Asymptomatic but may be symptomatic with epithelial breakdown. Blurred vision SIGNS: Bilateral non-inflammatory formations of bluish-white nodules in the superficial cornea. Found around the pupillary area and may be central. TREATMENT: Asymptomatic: no treatment Bandage contact lens if epithelium chronically breaks down Penetrating keratoplasty or PTK if desired. CORNEAL DYSTROPHIES Inherited, bilateral, non-inflammatory lesions found in the cornea. The presentation may be asymmetric between the two eyes. There is no history of any systemic or inflammatory disease and typically onsets early in life. Usually one layer of the cornea is involved and there is slow progression throughout life. In general, most require monitoring on a normal routine basis. Photodocumentation and diagnostic testing will demonstrate change over time. In some instances, the patient may present with corneal erosions and that must be properly addressed. If vision begins to decrease, the patient may require more frequent appointments. Surgical management is available for poor acuity. Examination of family members should be attempted to determine if there is a dominant (50%) or recessive mode of inheritance. A genogram can be made and genetic counseling can be offered. ANTERIOR CORNEA EPITHELIAL BASEMENT MEMBRANE DYSTROPHY (EBMD) (COGANS MICROCYSTIC, MAP-DOT-FINGERPRINT) Most common corneal dystrophy. SYMPTOMS: Usually asymptomatic, but a common complaint is a mild foreign body sensation more noticeable in wind, dry air or air conditioning. If pain, usually because of recurrent corneal erosion. SIGNS:

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Map, Dots, Fingerprint Recurrent corneal abrasions TREATMENT: 5% NaCl ointment HS and lubricants. If needed, add 5% NaCl drops in the day. If moderate hair dryer at arms length to increase evaporation and soft contact lens. If severe, debridement. HEREDITARY EPITHELIAL DYSTROPHY (MEESMANS) Rare, autosomal dominant, bilateral, symmetrical dystrophy usually appears in the first year of life. SYMPTOMS: Usually asymptomatic until cysts aggregate or surface. Can cause blurred vision or irritation SIGNS: Tiny, epithelial vacuoles appearing as gray blebs or bubbles within palpebral tissue of cornea Can develop recurrent corneal erosion. TREATMENT: Most individuals remain minimally symptomatic and require no treatment. Treat recurrent corneal erosion Lamellar or Penetrating keratoplasties if significant scarring (often develop cystic formation because host epithelium is still present). REIS-BUCKLERS DYSTROPHY Bilateral, symmetrical dystrophy which is autosomal dominant. First appears in the first few years of life as superficial corneal opacification with unilateral recurrent erosions. During the second and third decades, fishnet patterns of opaque rings appear in the central cornea and migrate outward. Superficial stromal haze accompanies that change. By age 30, the erosions are less frequent, but denser opacification and irregular corneal surfacing may affect visual acuity. SYMPTOMS: Pain, photophobia, redness during recurrent erosion phase. Blurred or decreased vision. SIGNS: Gray-white opaque rings in the central cornea forming a fishnet pattern at the level of Bowmans layer. Recurrent corneal erosions. TREATMENT: 1) Treat the recurrent erosions

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2) Lamellar or penetrating keratoplasty to improve acuity ( dystrophy can occur in graft.) STROMAL DYSTROPHIES GRANULAR (GROENOUW TYPES I) Discrete, crystal-like deposits within clear areas of stroma. Autosomal dominant. Most patients asymptomatic or report mild photophobia .

MACULAR DYSTROPHY (GROENOUW II) Gray-white opaities with poorly defined edges within the cloudy cornea. Autosomal recessive. Decreased VA at young age. Patients report photophobia. LATTICE DYSTROPHY (BIBER-HAAB-DIMMER) Lattice work of lines and dots (glassy appearance) which scar the central corneal stroma. Recurrent erosions are common. Decreased vision from progressive stromal clouding and scarring from recurrent erosions. Autosomal dominant. TREATMENT: Bandage contact lens (for treatment of recurrent corneal erosion) , penetrating keratoplasty, or excimer laser (if vision severely affected or patient significant symptomology) (condition can reoccur in the graft). ENDOTHELIAL DYSTROPHIES CONGENITAL HEREDITARY Two forms of the dystrophy exist. An autosomal recessive form, which presents at birth accompanied with nystagmus. This is stationary. A second autosomal dominant variety exists which presents in the first to second year of life. Nystagmus is absent but is accompanied by photophobia and discomfort. Slow progression occurs over 510 years. SYMPTOMS: Photophobia and discomfort in the dominant form. SIGNS: Cornea has a bluish-gray, ground glass appearance with focal gray spots. Epithelial and stromal edema may accompany the findings. Secondary changes such as band keratopathy can cause a marked decrease in vision. Density of the stromal opacification may change form one area to another but not anterior to posterior. Small macula opacities may be present. Normal corneal diameter. No guttata. Normal IOP. MANAGEMENT: Rule out congenital glaucoma. Treat corneal edema.

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Hypertonic saline drops QID-Q 1-2h Hypertonic saline ointment HS Hair Dryer 5-10 minutes at arms reach in the A.M. High water Soft Contact Lens Penetrating keratoplasty indicated to prevent deep amblyopia, nystagmus, or esotropia in a young child. Opacification occurs in a large number of cases due to poor endothelial function. Genetic Counseling. CORNEAL GUTTATA New collagenous formations produced by the endothelial cells which lie posterior to Descemets. If seen in the peripheral cornea called Hassall-Henle bodies. Can be seen in cases of inflammation, degenerative corneal disease, and trauma. Sometimes, severe inflammation can produce guttata, which subside when the condition clears. More common as age of patient increases. Seen in middle age to elderly patients. Unclear inheritance pattern. Watch progression to Fuchs dystrophy. FUCHS ENDOTHELIAL DYSTROPHY Occurs primarily in older women, is bilateral, asymmetric, and may follow an autosomal dominant hereditary pattern. First, excrescences formed by hyperproduction of Descemets are seen in the posterior cornea (guttata). Endothelial cells over these areas prematurely die. As the guttata become more numerous, overall endothelial function is compromised resulting in stromal edema followed by epithelial edema and bullous keratopathy. This condition is termed Fuchs dystrophy. Chronic edema can lead to a host of problems, which includes corneal scarring. Epithelial adhesion to the basement membrane is compromised and breaks in Bowmans may occur. Pannus and cellular debris may invade into the compromised zones. Can be accelerated after intraocular surgery. High intraocular pressure works against the pumping mechanism. SYMPTOMS: Pain, foreign body sensation, blurred vision, glare. Can be produced from recurrent corneal erosions and bullous eruptions. SIGNS: Corneal guttata, corneal edema stromal, Beaten-metal appearance of the posterior cornea. Edema initially is axial and may spread peripherally. Epithelial microcystic edema may be seen as well as areas of bedewing (ground-glass). Patches of epithelial edema form over the stroma edema and results in bullous keratopathy. When the bullae erupt onto the surface, the patient is symptomatic. Long-term effects can produce connective tissue scarring in the sub-epithelial zone. DIFFERENTIAL DIAGNOSIS:

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Aphakic or Pseudophakic Bullous Keratopathy Corneal Edema Congenital Hereditary Endothelial Dystrophy Posterior Polymorphous Dystrophy Endothelial edema secondary to anterior segment inflammation High intraocular pressure

MANAGEMENT: Medical management based on symptoms present. a) Hypertonic salt solution 5% QID to Q 1-2H b) Hypertonic salt ointment 5% UNG HS c) Hair dryer in the A.M. back in forth at arms length for 5-10 minutes. Increases evaporation d) High water soft contact lens relieve symptoms of bullous keratopathy e) Myopic correction may be helpful in AM myopic shifts f) Penetrating Keratoplasty Improve acuity in centrally involved cases g) Follow patient every 3-6 months. POSTERIOR POLYMORPHOUS DYSTROPHY (PPD) Bilateral, asymmetric, endothelial dystrophy which is inherited either autosomal dominantly or recessively. Congenital or begins early in life. SYMPTOMS: Usually asymptomatic. Blurred vision from edema or opacities. SIGNS: Irregular-shaped opacities (vesicles) with central pigmentation can be seen in the endothelium and Descemets. Vesicles can be grouped together, in a peripheral ring, or in a Swiss cheese pattern. Descemets membrane may be thickened either diffusely or localized. Very slow changes occur. Iridocorneal adhesions may occur (rule out ICE syndromes). TREATMENT: 1) Monitor 2) Periodic checks of intraocular pressure. 3) If Visual acuity is reduced, consider a penetrating keratoplasty ECTATIC DISORDERS KERATOCONUS A non-inflammatory, axial ectasia of the cornea. Incidence in the population is estimated at 0.15%-0.6%. Initial presentation first occurs in the mid-teens. There does not seem to be a sexual predilection. It also occurs bilaterally in the majority of cases (90%). Frequent history of asthma, allergies, atopic disease, and eye rubbing, have been associated with the condition leading many to assume environmental influence. Hereditary influences are also possible. Keratoconus has also been linked with systemic

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connective tissue disorders and dysplastic conditions. The literature also offers reports of PMMA lens wear as a triggering factor. Anterior keratoconus can progress slowly or quickly. Refractive error changes can take place while the best-corrected visual acuity may drop. SYMPTOMS: Patients may report glare, discomfort, blurred vision, diplopia or polyopia, photophobia. SIGNS: 1) Scissors motion on retinoscopy 2) Keratometry values begin to steepen and mires distort. Autokeratometers can obtain peripheral cornea information. 3) Keratoscope or Placidos Disc may show areas of irregular mire patterns. 4) Computerized corneal topography may indicate areas of steepening. 5) Decreased VA, may improve with pinhole or rigid contact lens. 6) Distance ophthalmoscopy yields image of the cone in the red reflex. 7) Munsons sign Lower lid indentation secondary to protrusion of the cone in downgaze. 8) Minimal corneal thickness is decreased with pachymetry. 9) Biomicroscopy: Early stages Increased concavity or dewdrop: of the posterior cornea, thinning of the stroma adjacent to the cone, striae, dulling of the anterior apical reflex. Intermediate stages Fleischers ring (broken ring of hemosiderin deposition at the base of the cone), Irregular, superficial scars at the apex, posterior cornea shows vertically oriented stress lines (Vogt), increased visibility of corneal nerves. Advanced stages Marked thinning, pronounced cone, hydrops (stromal edema secondary to aqueous flowing through breaks in Descemets), dense scarring. 10) Types of cones: Nipple Oval Globus MANAGEMENT: Rule out pathological disorders or thinning disorders Spectacle correction for early cases Rigid gas permeable lenses to provide best acuity Variety of fitting philosophies: CLEK Study Surgical Management Penetrating Keratoplasty Thermokeratoplasty Lamellar Keratoplasty Epikeratoplasty Manage hydrops with hypertonic solutions and hypertonic ointments. Tell patients not to rub their eyes.

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KERATOGLOBUS SYMPTOMS: Blurred vision, pain if acute hydrops is present. SIGNS: Overall thinning of the cornea with thinnest points in the midperiphery. Munsons sign present on downgaze. May see folds and areas of thickness in Descemets membrane. Fleischers rings are not usually present. There may be associated ruptures in descemets and acute hydrops, but not as common a finding as in keratoconus. Perforation is more common than seen in keratoconic patients and often do not report history of trauma. Slow progression or non-progression is usually the rule. TREATMENT: Spectacle correction Rigid Gas Permeable Lens Penetrating Keratoplasty requires large grafts, higher failure rate. Ocular protection prophylaxis for perforation Treat hydrops with hypertonics, bandage lens, etc. Tell patients not to rub their eyes POSTERIOR KERATOCONUS Congenital, and non-progressive. Involves alterations in the curvature of the central or paracentral posterior cornea. It is non-inflammatory in nature. Anterior curvature changes can be manifested but not to the degree of anterior keratoconus. There is no association to anterior keratoconus. Rarely does it have an effect on the vision due to the fact that the posterior surface doesnt contribute significantly to the physiological light refraction in the eye. There may be stromal scarring. SYMPTOMS: Thinning of the central or paracentral posterior cornea. Stromal scarring may be evident. TREATMENT: 1) Spectacle lenses or contact lenses. 2) Penetrating keratoplasty, if vision is decreased INFLAMMATORY CONDITION FILAMENTARY KERATITIS Is a chronic disease process caused by a number of specific problems. Characteristically, filaments are found on the anterior surface of the cornea. They are usually produced by aberrant epithelial healing. Often mucous strands are attached at the free ends. CONDITIONS ASSOCIATED: 1) Dry Eye Syndrome

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2) 3) 4) 5) 6) 7) 8) 9) 10) 11)

Superior limbic keratoconjunctivitis Atopic keratoconjunctivitis Toxic keratitis Prolonged patching following surgery Herpes infections simplex, zoster Thygesons SPK Vernal Keratoconjunctivitis Systemic problems: diabetes, psoriasis, ectodermal dysplasias Recurrent corneal erosions Chronic Bullous Keratopathy

SYMPTOMS: Moderate to severe discomfort, photosensitivity, hyperemia, sandy, itchy feeling. SIGNS: Conjunctival hyperemia, punctate keratitis, mucous strands adherent to the cornea. Stains with fluorescein. MANAGEMENT: Consider debridement: If not too many filaments o Drop of anesthetic o Mechanical removal at the base with forceps or cotton tipped applicator Topical lubricant therapy Artificial tears Q 1-2H Bland ophthalmic ointment QHS OR Sodium Chloride 5% drops QID, ointment HS OR Acetylcysteine (Mucomyst) QID x 24 hrs Consider bandage soft contact lens, if severe Consider topical steroids, if severe

HERPES ZOSTER OPHTHALMICUS PRIMARY VARICELLA ZOSTER INFECTION (CHICKEN POX) CORNEAL SIGNS: Punctate or dendritic (see specifics under recurrent HZ) kerititis. HERPES ZOSTER (REACTIVATION) CORNEAL SIGNS: Acute: a) Punctate epithelial keratitis: Edematous, raised, epithelial cells in the periphery. May be adjacent subepithelial infiltrate. b) Pseudodendritic keratitis: Coalescence of corneal vesicles, forming pseudodendrites in the periphery. Blunt-ended and more superficial than

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dendrites in HSV, pseudodendrites are slightly raised, thick and have no terminal end bulbs. They stain with rose bengal and poorly with sodium fluorescein. c) Infiltrates in the anterior stroma. * Punctate keratitis and pseudodendrites indicate active viral replication is currently present in the epithelium. Delayed: a) Mucous plaque keratitis: Chronic keratitis which can develop weeks to many months after the skin eruption. They are raised, grayish-white lesions on the surface of the cornea. Stains well with Rose Bengal b) Disciform keratitis Round, defined area of diffuse stromal edema. c) Serpiginous ulceration large geographical ulcer that can potentially lead to corneal melt. Chronic: a) Neurotrophic keratitis indolent ulcer with round margins and rolled edges, requires re-epithelializing b) Interstitial Keratitis DIFFERENTIAL DIAGNOSIS 1) Herpes Simplex 2) Other causes of follicular conjunctivitis 3) Temporal arteritis neuralgia 4) Infectious Keratitis - infiltrative MANAGEMENT: Keratitis a) Punctate Epithelial Keratitis i. artificial tears and bland ung ii. Use topical antivirals if herpes simplex cannot be ruled out. iii. Prophylactic topical antibiotics BID Pseudodendrite i. artificial tears and bland ung Topical steroid (i.e. prednisolone acetate 1%) Use topical antivirals if herpes simplex cannot be ruled out. Disciform keratitis i. Steroids topical 1% prednisolone; Taper over several weeks ii. Topical broad-spectrum antibiotics for prophylaxis QID dosage (especially if epithelial defect present) d) Indolent ulcers/Neurotrophic keratitis i. Bland ointments ii. Bandage contact lens or pressure patching iii. Prophylactic antibiotics iv. NO Steroids v. Non-responsive cases may require tarsorrhaphy, conjunctival flap, or transplant vi. Watch for perforation

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INFECTIOUS KERATITIS (CORNEAL ULCERS) SYMPTOMS: *red eyes *mild to severe pain *photophobia *decreased vision *discharge *tearing *foreign body sensation ETIOLOGY: *bacterial (most common) *fungal (more common in Southern United States; after organic, traumatic corneal injury) Recent rise in cases secondary to contact lens solution/care in US. MPS pulled from the market. *acanthamoeba (extremely painful stromal infiltrate; seen with contact lens wearer who practices poor hygiene or uses tap water/non-preserved saline) *Herpes simplex virus (may see accompanying eyelid vesicles or corneal epithelial dendrites) ACANTHAMOEBA KERATITIS Acanthamoeba is a protozoan which is found in many sources of water, soil and the oral cavity of humans. SYMPTOMS: Early stages: Lid swelling, *Pain which exceeds physical appearance of existing inflammation, *Edematous, necrotic cornea with overlying punctate staining and /or epithelial defects, dendritiform-like epithelial lesion, patchy paracentral infiltrates, nummular infiltrates (coin sized and coin shaped), dense white central infiltrates which associated thinning, midstromal radial infiltrates, associated hypesthesia, and recurrent erosions. Uveitis and scleritis may be present. Associated findings include: conjunctival chemosis, pseudomembranes, and preauricular lymphadenopathy. Late stages: RING infiltrates (hall mark sign of acanthamoeba keratitis) formed by the confluence of stromal infiltrates over a 2-3 month period Further progression can lead to vascularization, scleral melt, descemetocele, perforation, and loss of the eye. Inflammation is varied from cell and flare, keratic precipitates, and hypopyon. Transient hyphema has been reported. Symptoms may wax and wane. RISK FACTORS: Contact lens wear has been reported with all types of lenses including disposable. Primarily soft lenses.

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2) Homemade saline solution 3) Exposure to contaminated water hot tubs, swimming pools, etc.. 4) Penetrating trauma MANAGEMENT: 1) Rule out differential diagnoses: Herpes simplex keratitis - Bacterial Keratitis/Ulcer - Fungal Ulcer - Corneal abrasion - Foreign body abrasion - Contact lens overwear - Epidemic Keratoconjunctivitis - Toxic Keratoconjunctivitis - Allergic Keratoconjunctivitis - Sterile Infiltrates 2) Corneal smears with calcofluor white. Deep corneal involvement, consider a biopsy. 3) Most therapy is controversial and ineffective: (antifungal/antibacterial agents) Kertoconazole (Nizoral) Miconazole (Monistat) Neomycin Propamidine isethionate 0.1% (Brolene) Adjuncts: Topical steroids, bandage lenses, pain relievers. 4) Discontinue Lens Wear. 5) Penetrating keratoplasty as surgical means of treatment - excision of the infected cornea BACTERIAL CORNEAL ULCERS SIGNS: *focal white opacity in the corneal stroma along with an overlying epithelial defect *significant conjunctival injection, chemosis *epithelial defect typically >3 mm from limbus *significant corneal edema and inflammation surrounding the infiltrate *folds in Descemets membrane *AC reaction *can have hypopyon *mucopurulent discharge *upper eyelid edema may be present *pupil may be miotic *IOP can be raised *May see associated neovascularization PREDISPOSING FACTORS:

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1) Contact lens wear a) Soft lens wear incidence of 2-3/10,000 person-years b) Continuous wear incidence of 22-32/10,000 c) Dirty lenses d) Old lenses >6 months e) Poor compliance f) Poor hygiene and/or handling techniques g) Poor fit h) Ineffective disinfection or contaminated solutions 2) Chronic keratitis ( due to Ectropion, entropion, lacrimal obstruction, trichiasis) 3) trauma surgical abrasions or non-surgical DIFFERENTIAL DIAGNOSIS: *corneal infiltrates from an immune reaction to contact lenses/solution -diagnosis of exclusion -typically <2mm from limbus -minimal: lid edema, to no AC reaction, corneal edema (limited to around lesion) -minimal to moderate conjunctival injection -Visual acuity not significantly affected *residual corneal foreign body or rust ring *sterile ulcer (non-infectious) -dry eye syndrome -rheumatoid arthritis (collagen vascular diseases) -vernal keratoconjunctivitis (shield ulcer) -neurotrophic keratopathy -vitamin A deficiency *staphylococcal hypersensitivity (peripheral corneal infiltrates(s)) signs/symptoms of staph -multiple -bilateral -minimal to no AC reaction *subepithelial infiltrates (viral) -i.e. epidemic keratoconjunctivitis signs/symptoms -bilateral -history of acute, red eye with discharge -history of cold/flu-like symptoms PROGRESSION: 1) Staphylococci days 2) Streptococci 1-2 days 3) Gram negatives within 24 hours 4) Neisseria 12-24 hours 5) Psuedomonas within hours

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MANAGEMENT: *Controversial: Current standards are to culture every suspected ulcer however some feel this is unnecessary in some cases. *swab the palpebral conjunctival if discharge present and send specimen for cultures *obtain corneal scrapings for smears and cultures *Slide preparations are stained with Gram and Giemsa stains *Medium: i. Blood agar bacteria ii. Sabourands without cycloheximide Fungi iii. Thioglycolate broth Aerobic & anaerobic bacteria iv. Chocolate agar Hemophilus, Neisseria v. Thayer-Martin Neisseria vi. Non-nutrient with E.coli overlay - Acanthamoeba *do not delay commencement of therapy -initial antibiotic therapy should be broad and intensive -as final culture and sensitivity reports become available, treatment may require adjustment. *topical antibiotics fluoroquinalones Typically initially taken around the clock. See pharmacology section for dosage. *cycloplegic (type dependant on severity of ulcer/AC reaction) *analgesics >>>acetaminophen (po) PRN *no contact lens wear *admission to hospital necessary if: -sight-threatening infection exists -patient unable to self medicate -patient likely to be non-complaint -patient may be lost to follow-up Follow-up: *monitor daily until epithelial defect is resolved -evaluate -degree of eye pain -size of epithelial defect over the infiltrate -size and depth of the infiltrate -AC reaction -IOP *Alter dose schedule dependant on improvement in signs/symptoms (healing rate, size of epithelial defect, AC reaction, etc.) - May consider adding tobramycin ung before bed if it is acceptable for patient to take fluoroquinolone during waking hours only - gradually taper antibiotic regimen - if necessary, adjust antibiotic regimen according to culture and sensitivity results

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*If lesion not healing as expected -consider referral -consider alternating tobramycin with gentamycin or tobramycin -Consider subconjunctival antibiotics -Venereal disease organisms should be treated with oral cephalosporins, penicillins, tetracyclines, erythromicin. -Appropriate agents to be used for acanthamoeba, fungi. *If epithelial defect is healed but significant AC response still present consider adding topical steroid with concurrent antibiotic therapy. *If original cultures are negative and still not responding -REFER to corneal specialist -reculture the ulcer -corneal biopsy may be necessary *If originally not cultured and now worsening -consider referral -perform cultures and stain -alter treatment as needed *Corneal transplant or patch graft may be necessary for dense scarring or if cornea perforates FUNGAL KERATITIS OR ULCER Fungal keratitis, rarer than viral and bacterial keratitis, is an entity, which is most commonly found in the southern and southwestern United States. Of over 35 genera of fungi that have been known to cause infection of the human cornea, 4 are the most commonly found. These include Fusarium, Cephalosporium, Aspergillus, and Candida. Filamentous fungi such as Fusarium and Cephalosporium usually initiate infection following abrasive trauma with vegetable material. Both can be found in soil, air and organic waste material. Candida, a yeast fungi, is more common in the Northern and coastal regions of the United States. It is an opportunistic fungi and is the most common ocular fungal pathogen found in the lids, conjunctiva, and retina. It is most associated with an altered host defense. PREDISPOSING FACTOR: 1) History of trauma, organic/vegetative material (i.e. tree branch, airborne debris, etc.) 2) Candida commonly occurs in eyes with compromised host defense: a) Exposure keratitis b) Keratitis sicca c) Post-operative keratoplasty

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d) e) f) g) h)

Chronic use of corticosteroids (topical or ocular) Herpes Simplex keratitis AIDS patients Anticancer therapy Post-lasik

SIGNS: Gray or dirty white corneal stromal infiltrate, serpiginous (creeping) ulcerative process. Surface and marginal epithelium is elevated over the infiltrate. Ulcerated area has feathery borders with feathery extensions (hyphae), and rough textured surface. There may be satellite lesions (focalized areas of infiltration) separate from the central lesion. An immune ring or partial/complete ring abscess may surround the primary lesion. Associated findings: Conjunctival hyperemia, mucopurulent discharge, anterior chamber reaction, hypopyon, and posterior corneal infiltration. Descemets membrane is a barrier for most bacteria but is penetrable by fungi. Therefore, must watch for anterior chamber involvement. MANAGEMENT: 1) Rule out other forms of infectious keratitis. a) LABORATORY DIAGNOSIS! i. Prepare cultures and slide material ii. Gram, Giemsa, PAS, Gomori methenamine-silver stains iii. Sabourands or blood agar at room temperature b) Fluorescein-Rose Bengal staining i. Will have fluorescein staining of the leading edge (live-vital cells) and rose bengal staining of the rear (necrotic-devitalized) edge. 2) Initiate broad-spectrum anti-fungal therapy: a) Natamycin (Natacyn, Pimaricin) drug of choice i. Only antifungal under FDA approval for topical ophthalmic use. Available as a 5% suspension in the U.S. and 1% ointment outside the U.S.. ii. Initial dosage is Natacyn 5% gtts Q1H during waking hours and Q2H during waking hours and Q2H during sleep. iii. Viscous suspension with prolonged contact time but poor penetrating ability. Not as effective for deep infections. iv Warrants daily follow up. Consider Corneal specialist with expertise in treatment or hospitalization to insure instillation of medication. v. Can usually reduce to Q2-3 hrs after 3-4 days. Continue for 14-21 days or until resolution of active keratitis occurs. vi. NO STEROIDS in active phases. 3) Treat underlying iritis. Use cycloplegics atropine 1%, scopolamine 0.25%, 5% homatropine in high doses: Q2H QID b) No steroids.

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Check IOP to rule out decreased outflow and increased pressure. Treat with Timoptic .5% BID. 4) Monitor cornea for thinning or perforation. 5) If no response: a) Consider referral to corneal specialist b) Check laboratory diagnosis. Filamentous vs. Yeast c) Change to anti-fungal agent most specific for pathogen. May require one or more agents. i. Topical Amphotericin B 0.15% solution Q1h (made from parental solution by pharmacist or doctor) 6) Surgical intervention may be required in progressive cases. a) Penetrating Keratoplasty HERPES SIMPLEX KERATITIS PRIMARY HERPES SIMPLEX EPITHELIAL KERATITIS SIGNS: Cornea Epithelial involvement SPK, dendrites (stain well with Rose Bengal and fluorescein), infiltrates depending on severity. Keratitis is epithelial, not stromal. MANAGEMENT: Keratitis Superficial epithelial keratopathy Topical Trifluridine (Viroptic) Q2H up to 9x daily Topical lubricants Cool compresses NO STEROIDS! - not in the presence of replicating virus Monitor closely for dendritic formation 3 day intervals Dendritiform keratitis See treatment recommendations under RECURRENT HERPES SIMPLEX KERATITIS RECURRENT HERPES SIMPLEX KERATITIS Deep stromal involvement can be seen in recurrent disease. Patient reports history of HSV infection and factors associated with reactivation. SYMPTOMS: Similar to Primary Herpes infection. Depends on the severity of the inflammatory response. SIGNS: Unilateral red eye, follicular conjunctivitis, preauricular adenopathy, keratitis, and various levels of corneal involvement. Cornea: 1) Dendritic lesions: Fine, lacy linear defect; branching patterns with terminal end buds. Surrounded by edema and SPK; Stains with Rose-Bengal. Trough-like so that fluorescein pools in the base of the lesion. Can be central or peripheral.

c)

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2) Trophic Ulcer (metaherpetic or indolent ulcers): Can progress from dendrite. Round margins with rolled edges. May cause scarring as healing begins. Stains with Rose Bengal. - Is sterile, caused by viral damage upon the basement membrane retarding proper mechanical repair and epithelial overgrowth. Clue is persistent ulcer in patient under adequate treatment with antivirals. 3) Stromal Keratitis: Interstitial keratitis, Wessely immune ring. Type III hypersensitivity reaction. Irregular stromal infiltration occurs which may be partial to full thickness. Neovascularization into the deep stroma. Vessels can be full or ghost vessels. Epithelial and stromal edema. Infiltrative ring formations in the anterior cornea. KPs on the posterior cornea along with secondary guttata. Can develops scarring and lipid deposition. Most interstitial keratitis in nonsyphiltic patients is assumed to be HSV. Rule out corneal dystrophy. 4) Stromal Disciform Lesion: Type IV hypersensitivity reaction. Epithelium is usually intact with a dense solid disk of stromal edema surrounded by fine edema. Rarely, a Wessely immune ring may be seen. Uveitis is present. KPs may be viewed as well as folds in Descemets. MANAGEMENT: Oral Acyclovir may help to reduce reoccurrence rates as evidenced by the Herpetic Eye Disease Study Group. DENDRITE KERATITIS a) Rule out other forms of dendritiform keratitis b) Topical Antiviral therapy i. Topical Trifluridine (Viroptic 1%) drops - Q2H up to max. dose of 9 drops/day - drug of choice, least toxic - Continue until the cornea begins to respond. Cut dosage in half Q4H ii. Topical Virabidine (Vira-A) ointment: Not commercially available - use at night in conjunction with Viroptic drops c) Alternate therapy in order of preference i. Vira-A ointment 5x/day and HS ii. Idoxuridine .5% ointment (Stoxil) - if allergies to other antivirals iii. Idoxuridine (Herplex, Stoxil) .1% drops d) Consider corneal debridement i. Can be done initially or later if no response to antivirals after several days. Frequent pressure patching and virabidine ointment is used 45 times a day following debridement. e) NO STEROIDS! f) Cycloplegic agents if anterior chamber reaction is present g) Adjunct therapy

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i. Topical broad spectrum antibiotic drops as prophylaxis ii. Oral acyclovir 400 mg PO 5x/day (not FDA approved) iii. Topical lubricants iv. Warm compresses h) Monitor for complications, toxic side effects i. Follow-up every 24-48 hours ii. Monitor for Trophic ulcer formation If epithelial defects are not resolving after several weeks, consider toxicity, non-herpetic mechanism. - Switch to antibiotic ointment or lubricant and monitor. Can be fooled by toxic staining which can occur as fast as 10 days into therapy. May think that the condition is not improving or worsening. i) Some recommend 200mg to 400mg of acyclovir (Zovirax) PO 5xday for 14-21 days. TROPHIC ULCER Continue with treatment as specified for dendrite keratitis Debridement - repeat as needed Bandage soft contact lens-high water Prophylactic broad spectrum antibiotic drops BID Topical lubricants QID Monitor for corneal melt or thinning may require cyanoacrylate to avoid perforation Cycloplegics, only if needed Most heal with little scarring. However, repeated attacks can produce scarring. STROMAL (INTERSTITIAL) KERATITIS Consider referral to corneal specialist Topical Steroids: must be attentive to epithelial condition Prophylactic antiviral drops Viroptic 5x/day if steroids used more than BID Prophylactic broad-spectrum antibiotic at least BID if steroid use exceeds BID Cycloplegics Penetrating keratoplasty in severely scarred eyes Oral acyclovir for recurrent casese Since syphilis can cause a similar presentation: - consider FTA-ABS for inactive cases, add VDRL for active cases to rule out syphilitic cause. STROMAL DISCIFORM KERATITIS Consider referral to corneal specialist Topical steroids Prophylactic antiviral drops Viroptic 5x/day up to 9x/day Cycloplegic

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Penetrating keratoplasty if indicated

INTERSTITIAL KERATITIS SYMPTOMS: Red eye, pain, photophobia SIGNS: Corneal epithelial and stromal edema, vascular growth in the stroma, diffuse or secotral infiltrates in the stroma. Symptomatic cases may present with cell and flare, KPs and corneal edema. Ghost vessels may be seen in old cases of inflammation along with stromal thinning. ETIOLOGY: Usually a systemic etiology. Congrenital syphilis Herpes Zoster Acquired syphilis Trachoma Tuberculosis Acanthamoeba Lyme disease Herpes Simplex Mumps Be most aware of Syphilis (90%) and Tuberculosis Congenital syphilis: Presents bilaterally in most cases as an immunologic reaction to the treponeme. Ages in which the patient is usually symptomatic is 5 to late teens. Clinical appearance is indistinct infiltration, endothelial edema, KPs, and small stromal opacities. As it progresses, the areas of vascularization begin to proliferate. Is not seen as much now clinically. Look for other signs such as Hutchinsons teeth, saddle nose, deafness, salt and pepper fundus, loss of temporal eyebrow. Old cases show scarring, ghost vessels, opacification, residual vasculature. SEE SYSTMETIC DISEASE SECTION for more information. 2) Acquired syphilis; Usually a unilateral presentation. Vascular growth is usually limited to a sector of the cornea. SEE SYSTMETIC DISEASE SECTION for more information 3) Tuberculosis: Unilateral, and affects the cornea sectorally and peripherally. Dense infiltration is usually present. Systemically tuberculosis presents with history of productive cough, weight loss and low grade fever. 4) Herpes simplex: SEE CORNEAL SECTION. 5) Herpes Zoster: SEE CORNEAL SECTION. 6) Lyme Disease: SEE SYSTEMIC DISEASE SECTION MANAGEMENT: Determine systemic cause with appropriate testing. Treat systemic cause if appropriate. Ocular treatment typically: a. Topical cycloplegics b. Topical steroids q1h-qid depending on severity

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c. Penetrating keratoplasty for cases of dense scarring MOORENS ULCER An entity of progressive, inflammatory thinning and ectasia of the cornea. Two clinical forms have been recognized: Type I: Older patients, benign, unilateral, less severe inflammation, with good response to treatment. Type II: Younger patients, bilateral and severe. Aggressive and poor response to treatment. SYMPTOMS: Red eye, pain, tearing, photophobia. SIGNS: It may appear as a painful ulceration of the peripheral cornea. (Serpiginous or creeping ulcer.) Initially, it begins as grayish infiltration in the nasal and temporal perilimbal region. Overlying epithelium is broken down and involvement extends into the anterior stroma within weeks. Coalesces of these areas result in larger lesions which form a circumferential pattern. Stromal infiltration advances are followed by breakdown of the overlying epithelium. This results in the characteristic overhanging central edge of the ulcer. Progression can lead to stromal thinning, neovascularization, and ultimately, perforation. Watch for central involvement. MANAGEMENT: Rule out Noninflammatory thinning disorders Terriens, Pellucid, Marginal furrow Staphylococcal keratitis Infectious ulcers Fungal keratitis Medical Treatment may not respond well. a) Topical broad spectrum antibiotic - prophylaxis b) Cycloplegics c) Eye Protection. Consider adding: Topical prednisolone 1%q1h (Pred-Forte) Systemic steroids 60-100 mg QD PO +H2 antihistamine PO Immunosuppressive agents (Cyclophosphamide or methotrexate). Collagenase inhibitor Surgical procedures: a) Conjunctival excision or flap b) Cryotherapy c) Lamellar keratoplasty d) Cyanoacrylate adhesion to prevent perforation.

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Penetrating keratoplasty after inflammation has decreased PELLUCID MARGINAL DEGENERATION Bilateral, inferior, clear, peripheral thinning disorder. The area most often involved is between 4-8 oclock. This can result in astigmatism. SYMPTOMS: Asymptomatic SIGNS: Inferior zone of thinning of the inferior cornea. Normal corneal sensation is present. Scarring can occur but perforation or significant scarring does not occur. Found with keratoconus occasionally. MANAGEMENT: Rule out peripheral corneal degenerations Terriens, Moorens. Routine Follow up; Monitor for signs of keratoconus. PHARYNGOCONJUNCTIVAL FEVER SEE VIRAL CONJUNCTIVITIS PHLYCTENULAR KERATOCONJUNCTIVITIS SYMPTOMS: Tearing, foreign body irritation, pain, photophobia SIGNS: Conjunctival involvement appears as a localized hyperemic area at or near the limbus. A small elevated nodule may be centered in the hyperemic area. Most commonly found at 4 and 8 oclock. It may extend onto the peripheral cornea with surrounding or overlaying neovascular growth. May go 1-3 mm into the cornea. Corneal involvement can lead to scarring, ulceration, and perforation. Usually unilateral. If due to Staphylococcal exotoxins, blepharitis and conjunctivis may be present. ETIOLOGY: May be caused from local Staphylococcal exotoxins. However, it is also associated with tuberculosis. MANAGEMENT: Eliminate causative agent: Treat blepharitis and conjunctivis if present SEE APPROPRIATE SECTIONS Test (PPD) / Refer to rule out Tuberculosis Mild symptoms topical vasoconstrictors Topical steroids such as Pred Forte 1% or Rimexolone. *If needed can prescribe antibiotic/steroid combination.

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ROSACEA KERATITIS SIGNS: Corneal involvement presents as peripheral opacities and subepithelial infiltrates and superficial punctate keratopathy. Vascular proliferation may follow and subsequent ulceration and scarring can result. Basement membrane changes may also present in the peripheral cornea. Progressive epithelial erosion also progress to ulcerative and thinning changes. The inferior cornea is the most common site. Secondary bacterial infections may result. Other associated findings: Blepharoconjunctivitis, blepharitis, recurrent lid disease, iritis, meibomitis, frothy tear film, dermatological changes such as telangiectasia, erythematous skin changes, papules, pustules, sebaceous gland hypertrophy, and rhinophyma . MANAGEMENT: SEE OCULODERMATOLOGIC DISORDERS SCLEROKERATITIS SYMPTOMS: Pain photophobia, and irritation SIGNS: Cornea adjacent to area of scleritis can develop edema, stromal infiltration and peripheral thinning. Areas of opacification may extend centrally but often spare the cornea. Total opacification of the cornea may occur. Vascularization of the cornea may be seen at any depth. In addition, corneal opacities may become crystalline, or develop surrounding rings or lipid deposition. Anterior chamber reaction is usually present. MANAGEMENT: Medical treatment: Treat underlying scleritis and systemic disease Cycloplegics Topical steroids based on amount of anterior segment inflammation Follow-up based on amount of inflammation present SUPERFICIAL KERATITIS Defects in the cornea caused by a variety of processes. Superficial punctate epithelial erosions: Focal defects in the corneal epithelium. Best visualized with staining. Often associated with desiccation. Superficial punctate keratitis: Focal areas of inflammation within the epithelium. Can be seen in viral and bacterial cases as well as rosacea. Pattern distribution may aid in the diagnosis: Diffuse or non-specific: Staphylococcal (bacterial), viral, medicamentosa, solution, edema, mechanical, toxic Inferior distribution: Staph blepharokeratoconjunctivitis, lagophthalmos, trichiasis, medicamentosa

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Interpalpebral: Keratitis sicca, exposure keratitis, UV exposure, incomplete blinking, dry eye Superior: Superior limbic keratoconjunctivitis, trachoma, vernal conjunctivitis, atopic keratoconjunctivitis, inclusion keratoconjunctivitis. Linear: Trichiasis, Mechanical - FB

Chronic toxic exposure or hypersensitivity may result in neovascular changes. SYMPTOMS: Patients may report gritty sandy feeling or foreign body sensation. MANAGEMENT: External examination - Lymph node palpation - associated lid and conjunctival signs Differential diagnosis: - Infectious vs. sterile - What type of infectious etiology Medical management: a) Topical broad spectrum antibiotic qid b) Topical steroids * Indicated in non-infectious keratitis only * Indicated when there is tissue damage, infiltration, inflammation c) Cycloplegics * Indicated in moderate to severe presentations * Decreases risk of secondary inflammatory response d) Lubricants e) Other * Heat lid hygiene and comfort * Hypertonics to reduce edema SUBEPITHELIAL INFILTRATES (MARGINAL) Focal opaque spots of localized inflammation of the superficial stroma. DIFFERENTIAL DIAGNOSIS: Sterile infiltrates seen in herpes, adenoviral, chlamydial, and staphylococcal (in response to infections exotoxins released by local bacteria). Infectious corneal infiltrates bacterial, herpetic, fungal, acanthamoeba. Herpes simplex is extremely important. No steroids in an eye with active virus ( unless treating stromal HSV keratitis in combination with topical antiviral) Sterile Ulcers Dry eye, collagen vascular disease, thinning disorders, neurotrophic ulcer, vernal keratoconjunctivitis. Subepithelial infiltrates EKC Corneal infiltrates secondary to contact lenses/solutions Scar or residual rust ring/inflammation

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Corneal ulcer (anterior stromal involvement, anterior chamber reaction, significant pain and redness, epithelial defect is depressed). SYMPTOMS: Acute red eye can experience photophobia and pain. Foreign body sensation. SIGNS: Raised grayish lesions in the peripheral cornea with well-defined edges. Stains superficially with fluorescein. May have slight edema around it and is separated from the limbus by clear cornea. Can have sectoral injection of the conjunctiva. Lesions are usually where the lower lid hits the limbus. Involvement can be 360 degrees. Usually no anterior chamber reaction is present. MANAGEMENT: Determine cause (infectious, inflammatory, etc) and treat accordingly (as specified under that section).When in doubt, treat as a bacterial corneal ulcer until proven otherwise. SPK OF THYGESON SYMPTOMS: May be asymptomatic. May experience FB sensation, tearing, or photophia. SIGNS: Bilateral and asymmetric presentation of superficial punctate keratitis and infiltrates. Keratitis is usually greater centrally. Keratitis may be fine to coarse and if dense, may decrease acuity. Infiltrates are epithelial in depth and may be elevated. They can produce areas of negative staining. No associated signs of inflammation or infection. ETIOLOGY: Unknown etiology but hypothesized as a viral entity or immunotheory. Most often found in young females ages 15-40 years old. MANAGEMENT: Rule out other forms or keratitis infectious, sterile, CL wear. Medical management: a) Mild Presentations: * Topical Lubricants b) Moderate to severe presentations - symptomatic Mild steroids Prednisolone .125% or FML Q2-4 hrs x 3-4 days then taper. May use 1 drop per week afterwards * Soft bandage contact lens may be used to reduce symptoms. Monitor patients wearing contacts closely. SUPERIOR LIMBIC KERATOCONJUNCTIVITIS SYMPTOMS: Photophobia, tearing, foreign body sensation, redness.

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SIGNS: Bilateral but asymmetric presentation. Thickening and inflammation of the superior limbal area. Positive staining with fluorescein of the superior bulbar conjunctiva and superior cornea. Conjunctival injection may be seen from the superior rectus muscle to the limbus. May see papillary changes on the superior tarsus. Subepithelial infiltrates may be present in severe presentations. Pseudodendrites may also be seen. Keratinization of these region may be seen. Additional findings include filaments and micropannus. ETIOLOGY: Unknown etiology but has been linked to contact lens wear, thimerosal, and hyperthyroidism. Two entities have been classified. SLK of Theodore occurs in middle age women with hyperthyroidism (50%) and presents with dense papillae. Contact lens related SLK presents at any age without systemic disease. Patients may notice lens irritation and show fine papillae, unless GPC is present. The disease runs a course of 110 years with remissions. MANAGEMENT: 1) Case History Ocular and systemic history a) Symptoms of hyperthyroidism b) Contact lens history thimerosal products? 2) External examination proptosis, motility restrictions - cornea, conjunctiva 3) Medical treatment: a) Mild cases may be treated by lubricants. b) STANDARD TREATMENT for moderate to severe cases: * .5%-1% Silver Nitrate solution placed on the superior bulbar and superior tarsal conjunctiva after topical anesthetic. Irrigate the eye. Antibiotic ointment coverage for 1 week. * Do not place on the cornea. * May provide temporary relief. c) Topical steroids QID for 2-3 weeks may be helpful. d) Mast cell stabilizer QID e) Mucomyst (Acetylcysteine) TID for filamentary keratitis f) Bandage contact lens in severe cases or pressure patch g) Cryotherapy, scraping, resection in severe cases. 4) Contact lens-related SLK a) Discontinue any thimerosal containing products b) Discontinue lens wear c) Lubricants 5) 6) Thyroid function tests Follow-up weekly

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TERRIENS MARGINAL DEGENERATION Unilateral (sometimes bilateral) thinning of the superior cornea. Occurs in 20-50 year old patients (Usually males). Condition usually stabilizes after initial attack. SYMPTOMS: Typically a painless presentation. decreased acuity. Astigmatism can be induced which can cause

SIGNS: First signs may be marginal corneal opacification, similar to corneal arcus. As the disease progresses, superior nasal thinning occurs with opacification parallel to the limbus and separated from the limbus by a clear zone. Thinning can cause corneal distortion and astigmatism. Corneal inflammation and neovascularization can accompany acute presentations. MANAGEMENT: Rule out other marginal degenerations and inflammatory causes such as Moorens ulcer and fungal ulcer. o Corrective lenses spectacles or gas permeable contact lenses o Primary surgical REPAIR of ectatic area, sclero-corneal autotransplantation, corneo- scleral lamellar keratoplasty, and annular keratoplasty. VERNAL CONJUNCTIVITIS - CORNEAL SEE ALLERGIC CONJUNCTIVITIS SECTION SIGNS: Cobblestone PAPILLAE. Limbal papillae form and move onto the adjacent cornea. Semi-opaque masses called Horner-Trantas dots. Thick ropy white discharge is noted. Superior distribution of SPK may be seen. Filamentary keratitis may be present in some situations. Limbal infiltrates and Horner-Trantas dots in more severe cases. SHIELD ULCER 1. Topical cycloplegia 2. Topical antibiotic 3. Bandage contact lens to reduce lid-corneal mechanical irriatation 4. Upon re-epithelialization: add topical steroid 5. Mast cell stabilizers IRRITATIONS AND INJURIES CORNEAL ABRASION CORNEAL FOREIGN BODY CORNEAL LACERATION

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PERFORATING INJURY SEE OCULAR EMERGENCIES SECTION DRY EYE SYMPTOMS: Foreign body sensations, excessive tearing, burning, frequent blinking, itching, photosensitivity, pain, AM irritation, discharge, and blurred vision. SIGNS: Decreased tear film, poor tear quality, decreased tear breakup time, papillae, hyperemia, superficial punctate staining, discharge, staining of cornea and conjunctiva, dellens, and filaments. * Some pathological and tear film problems will be discussed below. CONTRIBUTING FACTORS TO DRY EYE: By understanding the underlying mechanism, one can use specific agents to reduce signs and symptoms. AGE CONTACT LENS WEAR ENVIRONMENTAL/OCCUPATIONAL Controlled environments: air conditioning, low humidity, heat, wind drafts Irritants: Smoke, smog, dust, fumes; Computer screens; Studying/paperwork MEDICATIONS: Causes or worsens dry eye Anticholinergics Tri-cyclic antidepressants Antihistamines Diuretics Beta-blockers MAO Inhibitors Phenothiazines Narcotics Antianxiety medications Oral contraceptives? Retinoids - acne SYSTEMIC DISEASE: Mostly autoimmune 1) Rheumatoid arthritis 2) Systemic Lupus Erythematous 3) Polyarteritis Nodosa 4) Scleroderma 5) Polymyositis 6) Sarcoidosis 7) Lymphoma 8) Sjogrens syndrome TEAR FILM ABNORMALITIES: 1) Aqueous deficiency reduced tear prism a) Commonly referred to as keratoconjunctivitis sicca (KCS) b) Sjogrens syndrome middle aged women c) Others: CN VII palsy, lacrimal gland neoplasm 2) Mucin Deficiency Rapid Tear Breakup Time (TBUT)

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3) 4)

5)

6)

Chemical burns, conjunctival destruction secondary to injury Chronic conjunctival inflammatory disease -Pemphigoid, Stevens-Johnson Hypervitaminosis A Trachoma Lipid Abnormality rapid TBUT a) Meibomian gland disease b) Blepharitis, Rosacea, atopic keratoconjunctivitis Lid Position Abnormality poor lid-tear film interaction a) Ectropion, entropion, b) Neurologic palsy, paralysis c) Poor blinking, closure d) Proptosis e) Disease or Trauma Epithelia disruption, surface disease dry areas a) Pterygia b) Corneal trauma scarring, etc.. c) Medicamentosa d) Disease EBMD, etc LID ABNORMALITIES a)Blink rate infrequent? b)Incomplete blinking, Bells reflex, Blepharoplasty, Nocturnal lagophthalmos c)Lid disease. d)Puncta stenosed, plugged, overgrowth, poor apposition

MANAGEMENT: Testing Vital Dyes Sodium Fluorescein accumulates in depressions; stains interpalpebral portion of cornea may extend superiorly or inferiorly in severe cases; stains desquamated cells Rose Bengal (Lissamine green) stains dead (devitalized) cells Tear Breakup Time Fluorescein (invasive; indicates tear volume), keratometer (noninvasive indicates tear stability) Tear Secretion Tests Schirmer #1 without anesthetic (reflex tear volume); <5 mm in 5 mins is abnormal Schirmer #1 with anesthetic (basal tear) Schirmer #2 response to nasal stimulation; secretion reflex Phenol red thread test Lysozyme Agar Diffusion test Lysozyme levels a) Impression cytology goblet cell density b) Tear Osmolarity TREATMENT:

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Determination of underlying cause dictates treatment. Modify the environment/occupational conditions to best of ones abilities. Abnormal blinking educate patient about more forceful blinking. Aqueous deficiencies artificial tear supplements, punctal occlusion e) Mucin deficiencies artificial tears with mucomimetics (i.e.Theratears) f) Lipid abnormalities i) Treat the active lid disease! May require topical or oral antibiotics, hygiene; po doxycycline/omega 3 fatty acids ii) Artificial tears with lipid component g) Lid abnormalities i) Mild cases may require topical lubricants and/or bland ophthalmic ointment at night ii) Lid taping or bandage lenses may be required for exposure type problems in addition to lubricants. iii) Surgical means may be necessary to fully correct the problem. h) Corneal/Surface disease i) Medicamentosa discontinue toxic agent General treatment *Treatment based on severity and response to therapy. a) Low viscosity artificial tear substitutes i) Preserved vs. Unpreserved ii) Medicamentosa warrants a change. Moderate viscosity artificial tear substitutes High viscosity artificial tear substitutes Gel formulations blurs vision only for a very short time e) Bland ophth ointments -usually HS schedule , can be used during the day but blurs the vision f) Punctal occlusion i) Collagen plugs lasts for approximately 1 week ii) Silicone plugs more permanent Punctal cautery Surgical g) Preservation of Moisture i) Taping lids at night ii) Goggles or plastic wrap shields at night iii) High water bandage contact lens h) Tarsorrhaphy i) Consider broad spectrum antibiotic if there is risk for infection. j) Consider a pulse dose of topical steroids for moderate to severe cases k) Cyclosporin A: Restasis: 1gt BID; may take from 3-6 months for full therapeutic effect; expensive MEDICAMENTOSA SYMPTOMS:

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Red eye, irritation, itching SIGNS: Injection of the conjunctiva, papillae and follicles, may have lid swelling. Chronic toxicity can lead to corneal SPK (typically diffuse) which prominently stains with Rose Bengal. Corneal edema can appear in severe cases. ETIOLOGY: Reaction to topical agents or preservatives after use > 2 weeks. These agents/ preservatives include antibiotics, anti-glaucoma, anti-virals medications, contact lens solutions and BAK preserved solutions. It can develop minutes to hours after exposure. MANAGEMENT: Discontinue causative agent and switch to another agent. Cold compresses and topical unpreserved lubricants. RECURRENT CORNEAL EROSION Recurrent corneal erosions are a problem, which can be pinpointed to an abnormal basement membrane. Although many disease processes can lead to erosions, treatment is invariably the same. For proper epithelial adhesion, an intact membrane is required. Trauma, EBMD, corneal dystrophies, dry eye are a few of the many types of entities, which can alter the basement membrane, thus affecting epithelial adhesion. Typically, patients report symptoms upon awakening. Epithelial edema may form during sleep, which may loosen adhesions. As the lid is opened, the loosened epithelium is abraded off. The patient is then symptomatic. A full 6-8 weeks may be required to have a new basement membrane regenerated. PREDISPOSING FACTORS: Trauma fingernail, paper cut, twig Corneal dystrophies EBMD Chemical-thermal injuries Diabetes Dry eye Bullous keratopathy Band keratopathy Salzmanns nodular degeneration Infectious & non-infectious keratitis Post-operative RK or cataract surgery SYMPTOMS: Pain, photophobia, foreign body sensation, sandy feeling upon awakening SIGNS: Mild presentation: SPK and staining Moderate to severe: Edema, abraded area with flap edges, hyperemia.

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MANAGEMENT: Treat acute presentations of recurrent erosion as an abrasion Cycloplegic Broad spectrum antibiotic ointment Gentamicin, Tobrex in the cul-de-sac Topical antibiotic 1gt QID Pressure patch or bandage contact lens Follow-up, no epithelial defect present. a) Warm compresses QID b) Hypertonic saline drops 5% Q4H x 4-6 weeks (Muro 128 or adsorbonac) c) Hypertonic saline ointment 5% QHS x 6-12 weeks (Muro 128ung) d) Monthly for 4-6 months Larger or more severe erosions may require additional management: a) If loose epithelial edges or flaps exist, they must be debrided. Bandage soft contact lens a) Extended wear, medium to high water content lens b) Worn 8-12 weeks c) Use hypertonic saline drops qid Alternative Surgical management Anterior stromal puncture Epithelial debridement Phototherapeutic keratectomy PUPILLARY ANOMALIES Various conditions result in anisocoria. The premise for anisocoria is that one pupil is abnormal. Evaluation of pupil size in bright and dim illumination is necessary to determine if the sympathetic or parasympathetic system is involved. Flash photography allows the most accurate measurement of pupil size, but black semicircles are usually adequate. Diagnosis of pupillary abnormality can usually be made by observation, review of old photographs, and biomicroscopy, but occasionally pharmacologic testing is needed. ADIES TONIC PUPIL Idiopathic; Usually affects females 20-40 years old. Relative mydriasis in bright illumination, which decreases over years as the pupil becomes more miotic. Unilateral accommodative paresis (>0.50 D initially, decreasing difference over months). Miosis after sustained near effort. Iris sector paralysis, stromal streaming, and stromal spread. (vermiform movements) Impaired deep tendon reflexes (greater in ankles and triceps). Pharmacologic testing: Pilocarpine:

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0.125% pilocarpine will cause the pupil in the eye with Adies Tonic Pupil to constrict more than the unaffected eye. MANAGEMENT: Reassure patients Unequal reading additions can be prescribed to reduce asthenopia. ARGYLL-ROBERTSON PUPIL Pupils irregular and react poorly to light. Pupils constrict normally to near stimulus. (Light near dissociation) MANAGEMENT: Test to rule out syphilis (RPR or FTA-ABS, VDRL) Refer to internist to manage syphilis HORNERS (OCULOSYMPATHETIC PARESIS) SYNDROME Either congenital or acquired SIGNS: Unilateral partial ptosis of upper and lower eyelid. Ipsilateral miosis. Anisocoria which is greater in dim illumination. Dilation lag of ipsilateral pupil. When changing from bright to dim illumination, the anisocoria will be greater after 5 seconds than it is after 15 seconds. Possible facial or body anhydrosis Hypopigmented ipsilateral iris if the lesion is congenital or onset before age two Ipsilateral conjunctival hyperemia due to sympathetic paresis It is important for prognosis to determine where the lesion is located. First order neurons leave the hypothalamus, run through the brainstem and cervical cord, and terminate at C8-T2 in the ciliospinal center of Budge. Second order neurons leave C8-T2, run through the stellate ganglion at the pulmonary apex and synapse at the superior cervical ganglion near the bifurcation of the internal and external carotid arteries. The postganglionic (third order) neurons leave the superior cervical ganglion at the level of the jaw and travel through the internal carotid plexus and penetrate the base of the skull along with the internal carotid artery. Fibers for sweat and piloerection of the face most often follow the external carotid artery. Sometimes fibers to the forehead above the eye will follow the ICA allowing a postganglionic lesion to cause anhydrosis in this area. The oculosympathetics course through the cavernous sinus, then accompany the long ciliary nerves to the iris dilator. These fibers also innervate the Muellers muscle in the upper and lower eyelid. Pharmacologic testing: Not needed if unequivocally no ptosis or dilation lag (physiologic anisocoria).

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Cocaine: As a rule, instillation of topical cocaine will result in reduced or absent pupillary dilation of a Horners pupil. Perform test by instilling 2 drops of 4% or 10% cocaine separated by 5 minutes into each eye. Hydroxyamphetamine: Hydroxyamphetamine is an indirect acting sympathomimetic which causes pupil dilation only in the presence of endogenous norephine. Postganglionic lesions result in a reduced supply of norepinephrine in the presynaptic nerve terminals, resulting in lack of or reduced mydriasis. Preganglionic lesions do not affect the concentration of norephinephrine available for pupil mydriasis, therefore hydroxyamphetamine will cause dilation in preganglionic Horners. Pupils which fail to dilate with paredrine have a better prognosis (postganglionic) than those which dilate (preganglionic lesions). Various studies have found hydroxyamphetamines predictive (mnemonic = Fail Safe) accuracy to approach 100%. Currently there are no pharmacologic tests to determine a central from a preganglionic lesion. MANAGEMENT: In the presence of congenital Horners (heterochromia irides and photodocumentation of long-standing ptosis and miosis), no further diagnostic testing is required. If an acquired Horners is suspected, one needs to determine preganglionic (First or second order neuron) vs. postganglionic etiology with pharmaceuticals. Unless one can rule out trauma or neck surgery, a complete neurologic workup is needed. Preganglionic lesions: refer to internist for radiologic testing and to rule out systemic conditions such as Pancoasts tumor of the lung apex. Postganglionic lesions usually carry a better prognosis. IRIS SPHINCTER DAMAGE Causes include high IOP, trauma, and synechia. Diagnosis can usually be made with the biomicroscope. Depending on iris damage, puillary constriction with miotics will be variable. MANAGEMENT: Reassure the patient and manage any causative problem. If due to trauma rule out retinal detachment and angle recession. PHARMACOLOGIC MYDRIASIS Most often a history of contact with mydriatics can be elicited, including scopolamine for motion sickness, accidental instillation of mydriatic/cycloplegic, and handling plants such as belladonna plant. Ask about other side effects of anticholinergic drugs. Since muscarinic sites on the iris sphincter have been affected, the pupil will not constrict to 0.5% PILOCARPINE. MANAGEMENT: Reassure the patient.

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 PHYSIOLOGIC (ESSENTIAL) ANISOCORIA Normal near and light pupillary reflex. No dilation lag (discussed under Horners syndrome). Equal anisocoria in dim and bright illumination No associated neurologic signs (i.e. ptosis, EOM weakness). Both pupils will dilate with cocaine No Predilection for sex iris color, or time of day THIRD NERVE PALSY SEE OCULOMOTOR SECTION Sudden Ptosis, diplopia and possibly pain. MANAGEMENT: All patients with third nerve palsies should receive prompt neurological evaluations.

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UVEAL TRACT UVEITIS SYMPTOMS: Pain, photophobia, blurred vision and lacrimation SIGNS: Cells and flare in anterior chamber (can be Grade 1+ to 4+), perilimbal injection, keratic precipitates (vary in size and quality), iris nodules, sluggish miotic pupil, IOP typically lowered but can be elevated due to synechia or inflammatory debris, anterior or posterior synechiae and vitreal cells. CLASSIFICATION Type of inflammation: Non-granulomatous: acute onset, prominent redness and pain, small to medium KPs, no iris nodules, typically doesnt affect vitreous, choroid or retina. Granulomatous (need to perform diagnostic to determine cause): often chronic, minimal symptomolgy / redness, large (mutton fat) keratic precipitates and iris nodules, may see vitreal exudates or choriod/ retina involvement. Location of Inflammation Anterior uveitis Intermediate uveitis (pars planitis) Posterior uveitis Panuveitis Non specific treatment of anterior uveitis Treatment of anterior uveitis is aimed toward preventing synechial formation, reducing severity of the uveitis, preventing iris blood vessel damage, relieve discomfort, and preventing secondary cataracts. The possibility of synechia formation is minimized by keeping the pupil immobile. This is accomplished by the use of cycloplegics. 5% homatropine is used about 90% of the time in treating active uveitis. The dosage can very from TID to Q2H depending on the severity of the inflammatory response. Scopolamine and atropine should be reserved for very severe inflammation. Corticosteroids, when indicated, are used to eliminate the inflammation as quickly and completely as possible. For this reason a strong steroid, which penetrates the cornea well, is indicated. 1% prednisoone acetate is typically used. The minimum starting dose should be Q2H, with Q1H preferable. In severe cases periocular or oral steroids are used. When the uveitis subsides, a gradual taper (about 2 weeks) is needed to prevent inflammatory exacerbation. CAUSES OF ENDOGENOUS UVEITIS

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CYTOMEGALOVIRUS Congenital infection usually causing bilateral chorioretinitis (posterior uveitis) and multiple cerebral defects. Acquired infection usually occurs when the patient is immunocompromised e.g. AIDS. Uveitis may not be pronounced in immunocompromised patients. TREATMENT: SEE RETINAL SECTION HERPES SIMPLEX Usually accompanies herpes simplex with epithelial or stromal disease. Granulomatous iritis with keratic precipitates and occasional Koeppe nodules. May occur due to direct viral involvement, hypersensitivity reaction or as result of adjacent inflammation. May see increase in IOP. SEE SYSTEMIC DISEASE SECTION TREATMENT: Cycloplegia, antiviral for treatment of herpes simplex (see cornea) and antiglaucoma medications (if needed). HERPES ZOSTER Onset may be delayed. Uveitis may be caused by ischemic necrosis of the iris. SEE SYSTEMIC DISEASE SECTION TREATMENT: Uveitis is treated with cycloplegia and topical cortiocosteroids. With IOP increase use site specific steroids and topical antiglaucoma medications (beta-blockers or topical CAI) if needed. LYME DISEASE SEE SYSTMEIC DISEASE SECTION - get iritis, choroiditis, vitritis DIAGNOSTIC PROCEDURES: ELISA, IFA (can get false positives or negatives) Clinical picture TREATMENT: Treat systemic condition Treat uveitis with nonspecific measures.

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ONCHOCERCIASIS = RIVER BLINDNESS Onchocerca volvulus is a leading cause of blindness in underdeveloped countries. The parasite is passed through the bite of a blackfly. The worm develops over the next 1-2 years. The classic lesion is a subcutaneous nodule of the skin. The nodules are formed by several adult worms and typically occur near joints. Microfilariae can be seen in the anterior chamber. Iridocyclitis (granulomatous and nongranulomatous) with posterior synechiae, sclerosing keratitis, secondary glaucoma, optic atrophy, and chorioretinal lesions (which spare the fovea and perifoveal retina until late in the course of the disease) can all be seen. DIAGNOSTIC PROCEDURES: Examination of skin samples reveals the microfilaria. Microfilaria can be seen in the aqueous humor during active iritis and corneal disease. TREATMENT: Ivermectin 150 micrograms/kg once a year or twice a year. Prevention of infection. RUBELLA CHORIORETINOPATHY Characterized by scattered retinal hyperpigmentation, microphthalmus, congenital cataracts, deafness, heart disease, uveitis, and posterior synechiae. DIAGNOSTIC PROCEDURES: RIA for rubella IgM, hemagglutination inhibition (HAI), EIA, CF, and positive hemagglutination (HA). TREATMENT: No treatment for rubella. Topical cycloplegia and corticosteroids for intraocular inflammation. Posterior uveitis may need systemic corticosteroids. SYPHILIS Presents as anterior or posterior uveitis either granulomatous or nongranulomatous. Iridocyclitis is usually typical of secondary syphilis and may be seen with cutaneous eruptions of the palms and soles of the feet. Iridocyclitis tends to be recurrent chronic, and relatively resistant to topical corticosteroid therapy in isolation. SEE SYSTEMIC DISEASE SECTION DIAGNOSTIC PROCEDURES: RPR (rapid plasma reagin) is a screening test for primary or secondary syphilis. FTA-ABS or MHA-PT (A positive RPR is confirmed with FTA-ABS or MHA-TP) FTA-ABS is specific for syphilis but will remain reactive forever in a patient with a history of treponemal infection, even after adequate therapeutic therapy. VDRL if positive provides evidence of active syphilitic infection, but beware of false positives. TREATMENT: Treat systemic condition.

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Treat uveitis with nonspecific measures (often recalcitrant to treatment) TOXOCARIASIS See RETINAL SECTION TOXOPLASMOSIS Exudative, necrotizing, chorioretinitis, with possible disorganization of vitreous with subsequent retinal tears. Occasionally disc edema will occur. Anterior uveitis can be severe and accompanied by synechiae and increased IOP. DIAGNOSTIC PROCEDURES: Toxoplasma dye test (sabin-Feldman methylene blue dye test (SFDT), Indirect fluorescent antibody test (IFA) most common, enzyme immunoassay, and enzyme-linked immunosorbent assay for toxoplasmosis (ELISA). TREATMENT: Retinochoroiditis usually only treated if visual function is threatened. Pyrimethamine 75 mg loading dose then 25 mg daily for weeks, folic acids, triple sulfonamides 2 g loading dose than 1 g QID for weeks, clindamycin 300 mg QID for 3 weeks, and prednisone 80 mg every other day tapered over 4 weeks. Colitis is a side effect of clindamycin which is treated with vancomycin 500 mg QID for 10 days. No steroids! TUBERCULOSIS Ocular findings include: granulomatous iritis, necrotizing retinochoroiditis, and subacute endophthalmitis. Also found are iris nodules, posterior synechiae, and cataracts. DIAGNOSTIC PROCEDURES: PPD, Chest x-ray, ACE (elevated), and biopsy of peripheral retinal granulomatous lesions. TREATMENT: Anti-tuberculin therapy, nonspecific measures for uveitis. NONINFECTIOUS CAUSES OF UVEITIS IDIOPATHIC ANTERIOR UVEITIS Nongranulomatous Bilateral but one eye at a time. DIAGNOSTIC PROCEDURES: HLA-B27 ( positive result shows predisposition for condition not diagnostic) CBC and ESR (non-specific; overall health) TREATMENT: Nonspecific

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ANKYLOSING SPONDYLITIS Usually affects young men aged 15 to 40 years. Acute, recurrent, symptomatic nongranulomatous iridocyclitis with possible posterior synechiae and fine keratic precipitates. Vitreal exudation can occur from cyclitis, causing obscuration of fundus details. Most often affects one eye at a time. Many patients will admit to lower back pain or stiffness, but some will be asymptomatic. Cataract, posterior synechiae, secondary glaucoma, and macular edema can occur from the iridocyclitis, causing a reduction in visual acuity. Attacks subside in 4-6 weeks with treatment, but patients can expect several attacks before disease burns out later in life. DIAGNOSTIC PROCEDURES: HLA-B27 ( positive result shows predisposition for condition not diagnostic) Sacroiliac joint X-rays (Patients with uveitis who are HLA-B27 positive, but radiologically negative for AS, should be re-evaluated for evidence of sacroiliac involvement.) ESR TREATMENT: Uveitis responds well to topical corticosteroids and cycloplegics. Oral anti-inflammatory agent (Indomethacin) and oral analgesics can also be used. BEHCETS Recurrent ulcers of mouth, tongue, genitalia, and nonulcerative skin eruptions. Characterized by bilateral, nongranulomatous uveitis with hypopyon (cold hypopyon). Other finding include: Arthritic changes, gastrointestinal lesions, central nervous system disorders, and cardiovascular changes. Rare in caucasians, but occurs in those with Mediterranean and Far East decent. DIAGNOSTIC PROCEDURES: HLA-B5, skin puncture test TREATMENT: Topical cycloplegia and topical corticosteroids. Periocular and systemic steroids and immunosuppressives may be needed. FUCHS HETEROCHROMIC IRIDOCYCLITIS Hypochromia of the iris (lighter eye affected) Small, diffuse Keratic precipitates diffusely distributed over the corneal endothelium Neovascularization of the iris and anterior chamber angle. Cataracts and secondary glaucoma. Mild anterior uveitis Affects individuals in their 30s and 40s. DIAGNOSTIC PROCEDURES: Diagnosis is usually made on the basis of clinical signs, and no medical work-up is needed. TREATMENT:

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Periodic gonioscopic evaluations to rule out neovascularization. Uveitis is chronic. Cycloplegia is needed only if concern over synechia development (rare). Topical corticosteroids are only needed if the patient becomes symptomatic (rare ). Treat secondary glaucoma. INFLAMMATORY BOWEL DISEASE Including Crohns disease, colitis, Whipples disease. Unilateral or bilateral acute iridocyclitis that is synchronized with attacks of inflammatory bowel disease. Uveitis may be presenting sign of disease. DIAGNOSTIC PROCEDURES: HLA-B27 ( positive result shows predisposition for condition not diagnostic) Upper and lower GI series. Based on clinical signs of stool changes and findings consistent with bowel disease. TREATMENT: Treat uveitis nonspecifically Treat systemic disease. INTERMEDIATE UVEITIS (PARS PLANITIS) Snow bank exudation seen overlying the pars planitis especially inferior. Peripheral vasculitis and vitreal cell and debris are usually present. Typically affects young adults. Macular edema and subcapsular cataracts are possible sequelae. There is some evidence suggesting that pars planitis may be a sequela of many disease processes. Differential diagnosis includes: Sarcoidosis (If iris nodules or keratic precipitates are seen, a sarcoidosis work-up is indicated.), Multiple Sclerosis, Lyme disease, Birdshot choroidopathy, Idiopathic vitritis, and syphilis. TREATMENT: Treatment is indicated in the cases where cystoid macular edema is reducing visual acuity to 20/30 or worse. Treatment includes sub-Tenons injections of corticosteroids. If the CME does not respond to this treatment, 100 mg/day prednisone for 1 week is used. Cryotherapy has been used successfully to treat the pars plana exudation. JUVENILE RHEUMATOID ARTHRITIS Two patterns of uveitis in JRA: 1. Chronic iridocyclitis, usually in young girls, associated with pauciarticular (less than 5 joints) arthritis and 2. Acute, recurrent iridocyclitis, usually in young boys, associated with polyarticular (more than 5 joints ) joint arthritis and predisposing to ankylosing spondylitis. If systemic or polyarticular JRA low uveitis risk. If pauciarticular JRA high uveitis risk. The chronic uveitis is usually insidious in onset, without pain, photophobia, or conjunctival injection. The chronic uveitis is very devastating, causing posterior synechiae, cataracts, and secondary glaucoma. Band keratopathy is also often seen. The pathophysiology of JRA is not well understood, although a particulate foreign antigen may be involved. JRA is a seronegative form of rheumatoid arthritis, but 20% of these children will

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have rheumatoid factor. Eighty-eight percent of children with JRA have antinuclear antibodies (ANA). DIAGNOSTIC PROCEDURES: The diagnosis is usually suspected in any child with arthritis of the knee or finger. X-ray examination reveals joint fluid or swelling ESR, ANA, Rheumatoid factor (Rh factor) TREATMENT: Treat with topical cycloplegic and steroid. Flare and cell may never go away. May need long term cycloplegic therapy. Treat secondary glaucoma if present. Comanage with pediatrician or rheumatologist. POSNER SCHLOSSMAN SYNDROME (GLAUCOMATOCYCLITIC CRISIS) young to middle age patient with unilateral attacks of high IOP (40-60 mm ltg) which usually last several days Patient may report blurred vision and halos and mild ocular discomfort Patient should be considered glaucoma suspect after attack SIGNS: Mild inflammation, open angles, no synechiae, mild anterior chamber rxn (some cells and flare). TREATMENT: Beta blocker, CAI (topical or oral) and topical steroid/ cycloplegic if patient has discomfort. REITERS SYNDROME Triad of findings: 1. Nongonococcal urethritis, 2. Polyarthritis and 3. Mucopurulent conjunctivitis. Other features: Acute iridocyclitis similar to ankylosing spondylitis and with hypopyon sometimes. HLA-B27 is positive in up to 85% of the cases. Males more often affected than females. It is postulated that certain infections act as a trigger to initiate uveal, urethral, and joint inflammation (occurs post venereal or dysenteric). DIAGNOSTIC PROCEDURES: HLA-B27 ( positive result shows predisposition for condition not diagnostic) ESR Chlamydia complement-fixation test. X-ray of hands, feet, sacroiliac joint. TREATMENT: Cycloplegics and topical corticosteroids are used to treat the iridocyclitis. Treatment systemically for chlamydia if a possibility. RHEUMATOID ARTHRITIS The iritis can often be insidious and produce no symptoms. Often the iritis is diagnosed after the presence of cataract and band keratopathy. Scleral inflammation followed by necrosis can occur due to occlusive vasculitis.

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DIAGNOTIS PROCEDURES: Rheumatoid factor HLA-DW4 is present in 70% of patients with rheumatoid arthritis. TREATMENT: Topical cycloplegics and topical or systemic corticosteroids. Also NSAIDs are often used. Many times immunosuppressive drugs are needed to control inflammation. SARCOIDOSIS Anterior or posterior, granulomatous or nongranulomatous bilateral uveitis is associated with this condition. Often there will be sudden onset of pain, photophobia, and lacrimation. Examination will often reveal mutton fat KPs, and conjunctival or skin nodules. Granulomatous iridocyclitis with posterior synechiae, corneal edema, and secondary glaucoma is possible. In the later stages of the disease, retinal vasculitis and focal retinitis may be seen. Optic nerve inflammation is possible with sarcoidosis of the central nervous system. Pathophysiology of sarcoidosis is still unknown. Histopathologically, there are nodular, non-caseating granulomas containing giant cells, lymphocytes, and epithelioid cells. SEE RETINAL SECTION DIAGNOSTIC PROCEDURES: Chest X-ray (hilar adenopathy) ACE (increased) Serum lysozyme Gallium scan of eyes, etc. Kveim test Biopsy of suspected granulomas ANA TREATMENT: Treat anterior uveitis with nonspecific measures Treat posterior uveitis with systemic or periocular steroids. Refer to internist. SYMPATHETIC OPHTHALMIA A rare, bilateral choroiditis and iridocyclitis that follows penetrating ocular injury or surgery. The inflammation occurs at least 2 weeks following the ocular insult. The initiating eye is chronically red, with anterior chamber cells and flare and vitreal cells. The uveiits is typically granulomatous (large keratic precipitates). Yellow-white, deep retinal exudates can be found scattered throughout the fundus. There is diffuse multifocal choroiditis. The etiology is most likely an autoimmune reaction to the damaged pigment epithelium or uveal body. DIAGNOSTIC PROCEDURES: Diagnosed on clinical signs and patient history. No medical work-up is needed for diagnosis.

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TREATMENT: Treat aggressively. Topical corticosteroids are used Q1H then tapered gradually. Systemic and periocular steroids. Immunosuppressive Tx (methotrexate, cyclophosphamide, cyclosporine, etc.) should be considered. VOGT-KOYANAGI-HARADA SYNDROME Chronic, progressive panuveitis of unknown origin. The iridocyclitis may be granulomatous or nongranulomatous and usually has acute onset several days after generalized malaise and tinnitus. Later in the disease, posterior synechiae, vitreous infiltration, choroiditis, peripapillary edema, and serous retinal detachments can occur. VKH is a diffuce granulomatous process. The ocular inflammation may have a genetic predisposition. VKH patients may show cerebrospinal fluid pleocytosis and elevated protein levels in the first two weeks of the disease. Audiograms will often show loss of sensitivity for high-pitched sounds. Vitiligo, poliosis and alopecia commonly occur. TREATMENT: High dose oral corticosteroids (prednisone 100mg/day) and frequent topical corticosteroids are needed if an anterior uveitis is present. MANAGEMENT FOR ANTERIOR UVEITIS Always dilate patients with uveitis to evaluate the posterior segment health and for inflammation. These findings may help identify the causative factor for the uveitis. Medical management should be accompanied by appropriate laboratory testing and subspecialty care. TREATMENT Steroids: Pulse dose Start with a stronger medication used frequently for a short amount of time instead of a weaker agent used less frequently for a longer period. Mild Presentations Mild Signs/Symptoms Nongranulomatous uveitis Treatment: *Mild cycloplegic: Tropicamide 1%, Homatropine 2% on a BID to TID schedule *Topical steroid: - Prednisolone acetate (Pred-Forte) 1% QID - Vexol (Rimexolone) or Lotemax (loteprednol etabonate) use for patients at risk for steroid response Follow-up in 48 hrs to 3-5 days or sooner, if condition worsens 2. a. Moderate Presentations Moderate Signs

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Nongranulomatous or Granulomatous uveitis b. Treatment: * Moderate cycloplegic: Homatropine 5% QID *Topical steroid: Prednisolone acetate (Pred-Forte) 1% Q2h Vexol (Rimexolone) or Lotemax (loteprednol etabonate) use for patients at risk for steroid response * Supportive therapy: - Ibuprofen (up to 1600 mg) - Sunglasses - Correction for near * Follow-up in 48 hours or patient to RTC sooner, if condition worsens. * If stabilizing, RTC in 3-5 days *RTC in 3-5 days following discontinuation of medications. 3. Severe Presentations a. Severe Signs: Nongranulomatous or Granulomatous uveitis l) Treatment: * Strong cycloplegia: - Homatropine 5% Q2H or Scopolamine /25% BID QID or Atropine 1% BID * Topical: - Prednisolone acetate 1% or rimexolone or loteprednol etabonate Q2H or Q1H depending on severity - Consider steroid ointment for use when sleeping to decrease taking drops in during the night. - Supportive Therapy: - Oral Ibuprofen (up t o 2000 mg) - Sunglasses - Correction for near * May need periocular or oral steroids. * Follow-up in 24 hrs. * If stabilizing, follow in 48 hrs. Follow-up appointments: a. Monitor symptoms b. Monitor for changes in the anterior chamber reaction c. Check the dilation: * Check for reactivity of the pupil * If the pupil is reactive, consider poor compliance or patient requires a stronger cycloplegic. d. Check the IOP: * May see an increase in IOP with steroid responders after a short period of therapeutic management. * Moderate cases: Maintain steroid and treat glaucoma with a topical beta blocker. Can also modify or discontinue steroid.

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Continuation of medications: * If the condition is stable: - Steroids use at least 7-10 days. - Cycloplegics use at least 5-7 days. - Follow-up in 3-4 days. * Tapering of steroids: - Required to avoid a rebound phenomenon. - May require a longer taper, depending on the circumstances * Tapering of cycloplegics is optional. Patients may experience eye pain as the cycloplegia wears off due to long term inactivity of the involved muscles. * Breaking posterior synechiae: - May require multiple dilating agents in high frequency or strong dilating agents.

LABORATORY EVALUATION Indicated for: Bilateral cases Granulomatous disease Recurrent disease Initial Laboratory work-up should include: ESR CBC CXR-Chest XRAY PPD with anergy panel HLA-B27 Specific testing for systemic disease: See previous section ENDOPHTHALMITIS POST-SURGICAL See CATARACT SURGERY SECTION BACTERIAL SIMILAR MANAGEMENT TO POST-SURGICAL See CATARACT SURGERY SECTION TRAUMATIC SIMILAR MANAGEMENT TO POST-SURGICAL See CATARACT SURGERY SECTION In addition CT scan

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GLAUCOMA PRIMARY OPEN ANGLE GLAUCOMA SYMPTOMS: Asymptomatic; may notice side vision problems or decreased vision in advanced cases. Silent blinding eye disease SIGNS: Elevated intraoculare pressure o may have large diurnal fluctuation o may have normal pressure with NTG Open anatomical angle without pathology Optic nerve changes asymmetry between eyes (> 0.2) (without significant anisometropia) progressive enlargement of C/D localized focal enlargement of C/D Neuroretinal rim thinning vertical elongtion Regional pallor of nerve Drance (splinter) hemorrhages adjacent to optic nerve Bayoneting of blood vessels Obliteration of rim Normal optic nerve rim should follow the ISNT rule. Inferior is the widest, then the superior rim, Nasal, with the Temporal the narrowest. Peripapillary atrophy 2 types Alpha Mottled appearance, seen in normals Beta white areas of atrophy, choriocapillaris changes Borders the disc and may indicate pathology Baring of Lamina Dots Nerve fiber layer defects Nerve fiber layer loss as evidenced by new technology: OCT/GDX HRT for optic nerve topography Pre-perimetric nerve fiber loss APD in advanced cases Visual field defects : o Nasal step o Arcuate Scotoma o Altitudinal defect o End stage defects islands Correspond optic nerve defects to visual field defects.

RICK FACTORS: 1) Positive Family History 2) Age

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- Increased prevalence with increase in age 3) Race - Blacks at higher risk 4) Systemic disease a. Cardiovascular disease b. Carotid stenosis c. Diabetes d. Thyroid disease 5) Ocular a. Increased Intraocular Pressure b. Glaucoma in the contralateral eye c. Steroid response (increased IOP) PATHOPHYSIOLOGY: Mechanical (direct compression) Vascular (ischemic) GLAUCOMA EVALUATION: Complete history reviewing risks, systemic health, medications, allergy etc Tonometry - Serial tonometry or have patient return at various appointment times to catch spike in IOP ( map out diurnal curve) Evaluation of the optic nerve Stereo disc photography Threshold Visual Fields Patient fatigue, arthritis, old age, poor mobility can affect patient performance. Patients subject to learning curve. Early detection may include using new technology such as Humphrey Matrix ( frequency doubling technology) or SITA-SWAP (updated blue-yellow technology) Pachymetry: Measuring central corneal thickness Nerve Fiber Analysis Confocal scanning laser ophthalmoscopy GDX-VCC HRT OCT MANAGEMENT: Biggest dilemma is when to initiate treatment. The goal of therapy is to prevent further loss of vision by decreasing intraocular pressure. Initiate treatment when the following signs are present or the patient is at high risk to develop them: Optic nerve cupping, glaucomatous visual field defects corresponding to the optic nerve damage, and elevated IOPs. Risk factors to develop glaucoma

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The greater the existing damage i.e. cupping, field loss, the lower the target pressure should be. The ultimate goal is to use the minimal number of agents with the minimal number of side effects to provide adequate IOP control. Typically treatment initiated if IOP>30 mmHg even without characteristic optic nerve or visual field findings (increased prevalence of glaucoma). Consider central corneal thickness; review OHTS study. Treatment may be initiated earlier and be more aggressive in monocular patients or younger patients. Consider initiating treatment in: a. Fellow eye of patient with angle recession with elevated IOP; high correlation to development of primary open angle glaucoma. b. Fellow eye of patient with diagnosed glaucoma in one eye. INITIATING MEDICAL MANAGEMENT * Look under each specific medication for contraindications. 1. Assess patients systemic/ ocular health and current medications. a. Determine contraindications to all potential medications. b. Assess potential problems with compliance: *Financial, frequency of dosage, dexterity Initiate treatment with Beta-blocker, Prostaglandin analog or alpha agonist. Non-selective beta-blockers not used with history of asthma or chronic obstructive pulmonary disease. Use another agent outlined in next sectionor selective B-blocker (Betoptic 0.25%). Before initiating therapy, take a blood pressure and a pulse! Contact physician if unsure of patients medical status. Determine target IOP based on glaucomatous damage. Educate patient on drop instillation and punctal occlusion. May utilize a uniocular trial to see how much IOP is lowered.

2.

FOLLOW UP VISITS: Reevaluate patient 2-6 weeks after starting therapy. Prostaglandin analogs may take up to 6 weeks to lower IOP fully. Ask about specific systemic and ocular side effects (symptoms) of each drug.. Blood pressure and Pulse (for those taking Beta blocker) If IOP not lowered, consider compliance as an issue. Reeducate patient on drop instillation and punctal occlusion. Long term drift. May not be as effective over time especially with Beta-blockers. Frequency of follow-up is dictated by the severity of the disease and control of IOP. If the patient is stable, see at 3-6 month intervals. If more severe disease, may elect to see every 6-8 wks. If medication added reevaluate IOP in 2-6 weeks. Fundus examination at least yearly. More frequently in patients with retinal disease or pathology and on miotics. Visual field at least once per year more frequent if progression of glaucoma is suspected.

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3. If inadequate IOP control is not obtained (or agent is ineffective, discontinued or not appropriate for the patient) or glaucomatous progression occurs add /start treatment with another agent. Review side effects /mechanism of action to determine which would be best suited for the patient (SEE PHARMACOLOGY SECTION) Consider feasibility of dosing schedule. NOTE: It can be beneficial to use agents which lower IOP by different mechanisms. Prostaglandin analog (Lumigan/Travatan/Xalatan) Topical CAI (Trusopt/Azopt) Brimonidine (Alphagan/Alphagan P) Dipevefrin (propine). 1% BID dosage i. Used as an adjunct drug for a small drop in IOP. ii. Be concerned about side effects: CME, etc Rarely prescribed in practice Pilocarpine QID dosage i. Unless contraindicated younger patients, lens changes, etc. ii. Start with 1% in white patients, 2% in black patients. iii. Monitor for inflammation, lens changes, RD iv. If drops not efficacious, increase concentration. v. Poor compliance switch to Pilogel 4% ointment HS Less miosis in the day time. Consider for a younger patient. vi. Compliance can be assessed by checking pupils. 4. If additional medication is needed to further lower IOP add additional medication as outlined in the previous section. Consider contraindications, side effects, mechanism of action and feasibility of dosing of schedule. Add additional medications as needed until target IOP reached.

After maximal medical therapy is reached using the previously mentioned agents consider: a. Argon Laser Trabeculoplasty (ALT) Or Selective Laser Trabeculoplasty ( SLT) i. May be considered if patient is non-compliant, does not tolerate the medications or for whom medications are contraindicated. ii. ALT may be repeated twice. Usually 180 degrees. SLT can be repeated; selective treatment. iii. Long term drift. Patient may need additional surgical treatment. b. Carbachol 3% i. Stronger miotic used on a TID schedule ii. Replaces pilocarpine 4% iii. Important to remember contraindications and side effects. c. Echothiophate (Phospholine Iodide) 0.5% BID i. Switch the miotic drug to a longer acting miotic. ii. More side effects with the drug

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Oral carbonic anhydrase inhibitors: Neptazane or Acetazolamide (Diamox) i. May be an option if the patient refuses an ALT or used prior to an ALT. ii. Be aware of systemic side effects. iii. Discontinue use of topical CAI.

Other options if glaucoma is not controlled using maximum medical therapy: a. Argon Laser Trabeculoplasty (if not performed) i. Repeat, if necessary. b. Trabeculectomy (Filtering surgery) i. Partial thickness ii. Full thickness iii. Anti-metabolites Mitomycin or 5-FU iv. Setons c. Cyclodestructive procedures i. Intent is to decrease aqueous production by destroying portions of the ciliary body. ii. Underproduction can lead to extreme hypotony. iii. Done by freezing, lasers, heat, and ultrasound. NOTE: Typically in practice in the year 2006, first line therapy is a prostaglandin analog. For select patients or those with contraindications to PGs, alternatives include once a dosing with a topical beta-blocker, alpha-agonists (brimonidine) or a topical CAI . Second line therapy (following a PG) may include either a beta-blocker, brimonidine, CAI, or the fixed combination Cosopt. There is no clear "best" choice among these and each has pros and cons (side effects, dosing frequency, efficacy, etc.), evaluate every patient individually. Third line treatment usually includes referral for laser trabeculoplasty (SLT or ALT). The TMOD may cover older/traditional therapy ( ie Pilocarpine/ Propine/ Phospholine Iodide, so therefore the traditional therapies need to be reviewed and covered) but current use of these agents for POAG is extremely limited. Glaucoma therapy and treatment philosophy may differ from one practitioner to the next. One must take into account current health status, age, cost of medications, insurance formulary drugs etc,. however there is one common treatment goal: prevent further visual loss while maintaining quality of life. GLAUCOMA LASER PROCEDURES 1) ARGON LASER TRABECULOPLASTY (ALT) a. Requires visible trabecular meshwork by gonioscopy. b. May elect to treat 180 or 360 degrees. i. 180 is favored so that a retreatment can be done, if needed c. Indications: i. When medical treatment is not satisfactory or is contraindicated in reducing the IOP. ii. Progressive damage to the nerve and/or visual field with maximum medical therapy. * IOP must be in the high teens for it to have an appreciable effect.

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e.

f. h.

i.

iii. Attempted prior to filtration surgery. iv. Patient has extremely poor compliance. Efficacy: * Effective with: POAG, Pseudoexfoliation, Pigmentary. * Works poor in :angle recession, Congenital glaucoma, Uveitic glaucoma, and ICE syndrome. Contraindications: * No view of the TM, significant PAS, hyphema, ICE, uveitic glaucoma, young patients, steroid induced, and mesodermal dysgenesis, hazy media, uncooperative patient. Complications: * Corneal abrasion, peripheral anterior synechiae formation, acute rise in IOP, corneal burns, iritis, corneal edema, and hemorrhaging. Other * Tends to work better in older patients * Worse results in African American patients * Tends to work better if performed prior to cataract surgery. * Better results in pigmented trabecular meshwork. * ALTs become less efficacious as time goes on. After 5 years, 25% of ALTs have lost effect. At 10 years, about 50% Pre-op and Post-op i. Patient given 1 drop apraclonidine 1% prior to procedure. ii. Topical anesthetic, goniosol, lens application. iii. Procedure is completed. iv. After irrigation, 1 drop of apraclonidine 1% is placed in the eye to reduce spiking v. May use prophylactic anti-glaucoma medications. vi. Pressure is measured at 1 hour, 1 day and 1 week post-op. vii. Topical steroid is administered qid for 4-5 days and then tapered.

2) SELECTIVE LASER TRABECULOPLASTY Selective laser trabeculoplasty works by Using a specific wavelength to irradiate and target only the melanin containing cells of the trabecular meshwork No damage to the non-pigmented cells Produces a biologic response which leads to the release of cytokines that trigger macrophage recruitment leading to IOP reduction Unlike ALT, SLT can be repeated several times with overall good intraocular pressure reduction. 2) LASER PERIPHERAL IRIDOTOMY a. Function is to create a communication between the anterior and posterior chamber. Reduces pressure build-up behind the iris. b. May be performed with a Nd: YAG and/or Argon Laser. c. INDICATIONS:

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Primary angle closure glaucoma acute, sub-acute, or chronic. Secondary angle closure with pupil block Eyes with high risk for angle closure (prophylaxis) * Fellow eye of one with angle closure * POAG with coincidentally narrow angles * Plateau iris * Narrow angles by gonioscopy with a shallow anterior chamber * Fellow eye of one with malignant glaucoma iv. * Prior to ALT, if not all the TM is visible. v. Non-patent surgical iridectomy d. Procedure: i. Drop of pilocarpine 2% or 4% 30-60 minutes prior. ii. Drop of apraclonidine 1% iii. Anesthetic, Abraham lens iv. Laser Nd: YAG for light iris, combination of Nd: YAG and Argon or Argon alone for darker irises. v. Argon may be used first and then finished with the Nd: YAG vi. Focus on a superior located iris crypt. vii. After completed procedure, 1 drop apraclonidine 1% viii. Prophylactic anti-glaucoma meds may be given ix. Topical steroids Q1H for 2 days, QID for 5 hrs. x. IOP check at 1 hr, 1-2 days, 1 week xi Follow-up for closure over six week period. e. If procedure is ineffective: * Iridotomy is not patent * Plateau iris syndrome * Peripheral anterior synechiae * TM damage (outflow problem) * Malignant glaucoma f. Complications: * Iritis, PAS, corectopia, Retinal burns, Lenticular opacity, Corneal burn, Closure of the iridotomy, Hyphema, elevated IOP. *Pigment migration into the iridotomy occurs during the first couple of weeks post-op. Complete closure may occur by 6 weeks. LASER IRIDOPLASTY (GONIOPLASTY) Used to alter the contour of the iris by contraction of iris tissue. This is done in cases where there is a prominent last iris roll which may occlude the TM or potentially cause angle closure such as with plateau iris. Laser energy is applied to the peripheral iris in hopes of contracting the iris out of the angle GONIOPHOTOCOAGULATION Used when active or progressive growth of vessels continues after PRP. Laser energy is directly focused upon vessels in the angle. Attempts to eradicate neovascular vessels. Does not treat the cause of the neovascular vessels. Can cause hyphema, iritis, and increased IOP. CYCLOPHOTOCOAGULATION

i. ii. iii.

4.

5.

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Indicated in failed filters or refractory glaucomas. Vision is poor but IOP may be causing discomfort. Goal is destruction of the ciliary body to result in decreased aqueous production. Complications: PAIN, INFLAMMATION, hypotony, flat chamber, choroidal detachment, transient increase in IOP phthisis.

GLAUCOMA SURGERY 1. FILTRATION SURGERY a. Surgically created fistula from the anterior chamber to the subconjunctival space. Aqueous is kept in a conjunctival reservoir called a bleb. Fluid is absorbed by the conjunctival veins or into the tears. b. Indicated when medical or laser treatment cannot control IOP at such a level to prevent damage from occurring. c. Poor results with neovascular glaucoma, uveitic glaucoma, and congenital glaucoma. d. Risk factors for poor surgical results: * Previously failed glaucoma surgery * Conjunctival scarring * Aphakia * Younger patients * Intraocular inflammation * African American patients may have less success than Caucasians e. Procedures i. Full-thickness ii. Partial thickness (Trabeculectomy) iii. Setons - Aqueous drainage implants, which are usually reserved for repeated failed filters. - Purpose is to shunt and maintain aqueous flow from the anterior chamber through a tube into the subconjunctival space. * Molteno Implant iv. Antimetabolites used to reduce scarring of the fistula and in patients with high risk of failure or history of previous failed filter * 5-Fluorouracil Serial subconjunctival injections placed during the postoperative period. * Mitomycin C Potent antimetabolite which is used during the course of surgery. v. Releasable sutures may be used to control outflow f. Surgical procedure entails formation of a full or partial thickness filtration site. Usually surgical iridectomy is performed (removal of piece of the peripheral iris). g. Post-operative care: i. Topical steroids, cycloplegics, antibiotics. ii. Follow-up 1-2 days, 1 week, 2 weeks, 1 month, 2 months. iii. Evaluate the bleb. Avascularity is a good sign. - Look for encapsulation, leaking, fistular obstruction. - May require surgical or laser treatment to reopen the fistula. iv. Anterior chamber: hypopyon, flat chamber, hyphema, cell/flare v. Cornea: edema, toxicity or erosion.

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vi. Tonometry: hypotony vs. elevated IOP vii. Choroidal detachment from hypotony (b-scan) viii. Posterior segment/nerve. ix. Digital massage can lower IOP 180 degrees away from the filter Complications * Expulsive hemorrhage, hemorrhage, choroidal effusion, lens injury, vitreous loss during surgery. *Hypotony, flat Ant. Chamber, high IOP, Uveitis, Endophthalmitis, CME, loss of vision, cystic bleb, encapsulated bleb, leaking bleb, cataract.

NORMAL-TENSION GLAUCOMA (NTG) Characterized by an open angle, visual field loss, optic nerve changes, and an IOP of less than 21 mm Hg. A controversial topic which has lead to a number of theories about its mechanism of action. These include vascular insufficiency and progressive hereditary optic atrophy. Associated findings are a family history of glaucoma, myopia, disc hemorrhages, low blood pressure, and migraines. It is typically found in the elderly, and more so in women. Higher diurnal fluctuation is seen as well as aberrant aqueous dynamics. May also be asymmetric. SYMPTOMS: Asymptomatic. Family history of NTG. SIGNS: More common in NTG compared to POAG: - Focal visual field defects typically closer to fixation - Larger than average optic disc - Higher incidence of optic pit - Rim tissue lost more commonly inferior and temporal - Higher incidence of Drance hemorrhages (disc hemes) - Localized defect in NFL. DIFFERENTIAL DIAGNOSIS: 1) High diurnal variation in IOP. 2) Burned-out glaucoma damage from past history of high IOP or old pigmentary glaucoma 3) Intermittent angle closure 4) Posner-Schlossman 5) Congenital nerve defects: coloboma, pit, physiologically large c/d, tilted disc, drusen etc.. 6) Ischemic optic neuropathy 7) Compressive lesions to the chiasm and optic nerve. 8) Cardiovascular disease. Previous hemodynamic crisis or hypoperfusion. MANAGEMENT: 1) Consider neuro evaluation, neuro-imaging, blood studies, ESR when there is an atypical presentation or progression. 2) Medical management is similar to POAG. Use of nonselective beta blockers or epinephrine controversial due to possible vascular (vasoconstriction) response. * If progression is seen add additional medication or consider surgical procedure

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Surgical intervention may be needed: * ALT (results mixed, questionable benefit) * Filtering surgery 4) If due to preceding hemodynamic crisis or sudden vision loss No TX necessary. Note: Important to review the Normal Tension Glaucoma Study ( NTG Study). This is a landmark study that concludes lowering IOP by approximately 30% in patients with NTG may reduce the progression of VF loss. The science of this study does reveal that even in NTG, intraocular pressure plays a role in glaucomatous disease. Two-thirds of the untreated patients showed no progression for first 3 years of follow-up, while approximately 1/6 of the treated group still showed progression despite lowered intraocular pressure. This is why many practitioners will refer to NTG as progressive or non-progressive. Further research will shed more light on who to treat vs observe. CONGENITAL GLAUCOMA SIGNS: Epiphora, large cornea diameter greater than 12 mm, corneal edema (haze), breaks in Descemets membrane, Haabs striae, elevated IOP, buphthalmos, corneal asymmetry greater than 1.5 mm, enlarged c/d, is bilateral. MECHANISM: Many different mechanism Primary infantile or developmental abnormalities such as aniridia, Axenfelds, phakomatoses, and metabolic disorders. Primary infantile glaucoma is due to the trabeculodysgenesis which either a flat or concave iris insertion. Pressure can be devastating to ocular structures and may lead to nerve damage and amblyopia. DIFFERENTIAL DIAGNOSIS: Megalocornea, birth trauma, high myopia, inflammatory corneal disease, corneal malformations, metabolic disorders, secondary infantile glaucoma. TREATMENT: 1) Early surgery is the desired treatment. Often infants and juveniles do not respond well to therapy. 2) Medical therapy may be effective in some cases, such as Axenfelds. If not, surgical means is necessary. * Medical treatment is same for POAG. 3) Goniotomy is the preferred treatment, especially in infantile glaucoma. This involves cutting into the trabecular meshwork. There is good success in infants under age 2. 4) Trabeculotomy is a similar type of surgical procedure in which the trabecular meshwork is removed to aid aqueous outflow. Is used in children older than 3 years old or juvenile cases. 5) May require trabeculectomy. Follow patients once the IOP is stabilized once a year. Increase in IOP may be seen at a later point in time. PIGMENTARY GLAUCOMA SYMPTOMS: Asymptomatic or may complain of blurred vision or halos after dilation or exercise.

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SIGNS: NOTE: Patients can have PIGMENT DISPERSION SYNDROME without having glaucoma! This means all the classic signs are present but no apparent ocular damage. 1) Heavily pigmented Trabecular Meshwork 2) Krukenberg spindle Fine, dust like pigment on the corneal endothelium in a linear pattern. 3) Mid-peripheral iris transillumination defects 4) Pigment anterior to Schwalbes line (Sampaolesis line) 5) Pigment on the anterior and posterior lens as well as equatorially and peripherally on the lens and on the zonules (Zentmayers ring or Scheie line) 6) Periodic free pigment in the anterior chamber 7) Peripheral iris has a concave configuration. 8) Glaucomatous optic nerve cupping, Visual field defects, and /or Increased intraocular pressure EPIDEMIOLOGY: Pigment dispersion syndrome is found equally among young myopic males and females. However, Pigmentary glaucoma is typically seen more in males. It is a bilateral condition. Young, myopic males (35-45) are the most characteristic of those with the disease. Women developing pigmentary glaucoma are slightly older (40-50). It primarily afflicts the Caucasian population but African Americans and Asians can also be affected. MECHANISM: Liberation of pigment from the pigmented epithelium may arise from pupillary mydriasis or be spontaneous. Deposition of the pigment occurs in various structures of the anterior segment. Concavity of the peripheral iris results in rubbing of the pigmented epithelium of the iris on the adjacent lens zonules. Deep anterior chamber depth of myopes increases likelihood of this condition. Most authorities directly attribute the subsequent accumulation of pigment in the trabecular meshwork as the primary cause of increased IOP. Deposition of the pigment occurs to all structures in the anterior segment. For example, convection currents in the aqueous are responsible for linear deposition of pigment onto the corneal endothelium Krukenberg spindle. Patients may experience a sharp rise in IOP following exercise and dilation. DIFFERENTIAL DIAGNOSIS: 1) Any condition producing pigmentation on the TM. 2) Psuedoexfoliation glaucoma (cental iris atrophy) 3) Inflammatory/Uveitic KPs on the cornea, cells in the anterior chamber 4)Iris melanoma MANAGEMENT: Based on findings, treat appropriately like POAG. i) Medical therapy Miotics may not be well tolerated in young patients although they decrease iridozonular touch and pull open the trabecular meshwork. ii) Argon Laser Trabeculoplasty works well in patients with pigmentary glaucoma. However, long term follow up indicates decreased success. Consider surgical intervention trabeculotomy (filtering procedure) for advanced cases.

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Experimental trials have been performed with laser peripheral iridotomies in hopes of equalizing pressure between anterior and posterior chambers.

PSEUDOEXFOLIATION GLAUCOMA SYMPTOMS: Usually asymptomatic. SIGNS: NOTE: Patients can have PSEUDOEXFOLIATION (EXFOLIATION) SYNDROME without having glaucoma! This means all the classic signs are present but no apparent ocular damage. 1) Open Angle 2) White dandruff material on the cornea, lens, iris, angle, zonules. 3) Anterior lens capsule: Center translucent disc surrounded by clear zone which is surrounded by gray-white ring with scalloped borders (peeling appearance) This is best seen postdilation. 4) Pigment in the trabecular meshwork especially inferior 5) Pigment anterior to Schwalbes line Sampaolesis line 6) Pupillary ruff loss or moth-eaten appearance 7) Peripupillary iris transillumination defects 8) Fine pigment dotting on the iris stroma 9) Glaucomatous cuping, file defects, increased IOP EPIDEMIOLOGY: High prevalence among the Scandinavian and Spanish American populations although it is scattered throughout the world. Usually appears within the fifth and seventh decades. If often bilateral but asymmetrical. DIFFERENTIAL DIAGNOSIS: Pigmentary glaucoma Primary amyloidosis amyloid deposition on the ant. Lens True exfoliation Glass blower Exposure to heat, trauma, uveitis can cause peeling of the lens. MANAGEMENT: Treat appropriately based on findings A. Psuedoexfoliation syndrome i. Monitor every 6 month B. Psuedoexfoliation Glaucoma i. Medical Management similar to POAG (Less favorable response to medical treatment than POAG) ii. Argon Laser Trabeculoplasty works well iii. Surgical intervention if medical treatment fails

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ANGLE RECESSION GLAUCOMA SYMPTOMS: asymptomatic SIGNS: Torn iris processes, Deeper areas in the Ciliary body band seen with gonioscopy. Sectoral widening may be seen. Trabecular damage may be evident early or late. May see peripheral synechiae. IOP is unequal between the two eyes, higher in the eye with a history of trauma. Patient may have history of hyphema, iridocyclitis, iridodialysis, retinal detachment, vitreal hemorrhage or choroidal rupture secondary to trauma. Glaucoma can develop years to decades after trauma. MECHANISM: Angle recession results from blunt injury to the eye. Concussive forces are believed to force aqueous at high speed into the angle and causes visible damage to the ciliary body and trabecular meshwork. Splitting of the circular and longitudinal muscles is seen as a widened ciliary body band with gonioscopy. Development of glaucoma is higher among patients with a hyphema at the time of injury and large areas of angle recession. Many of these patients go on to develop POAG in the fellow eye, especially those with a family history of glaucoma. Glaucoma may onset within a month, to years or decades after the initial injury. Initial trabecular damage and trauma to the ciliary body result in decreased outflow and decreased production. Ciliary body function can return to normal and IOP goes up. Over months time, decreased outflow can present as increased IOP. Over the course of many years, 10 or more, changes occur to the trabecular meshwork, which impedes its normal outflow. TREATMENT: Medical management: Follow the same guidelines as POAG. - Miotics such as Pilocarpine may not be as effective as in POAG. ALT is of limited benefit. STEROID INDUCED GLAUCOMA SYMPTOMS: Asymptomatic SIGNS: Elevated IOP following use of corticosteroids. MECHAMISM: Efficiency of the aqueous outflow through the TM is compromised. Can be seen following systemic and ocular steroid usage in about 2-6 weeks time. Is reversible, if discontinued. Will return to baseline in 1-3 weeks. Genetically determined factor. MANAGEMENT:

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Treat like POAG. Remember that effects are reversible upon cessation of the medication. Patients that must remain on the steroids should be treated with anti-glaucoma agents. Discontinuation of the drop will often solve the problem. Remember that steroids should be tapered. Ocular steroids may be switched to medrysone, fluoromethalone, rimexolone or loteprednol which have less hypertensive effects; or switch to NSAID. Differential diagnosis is uveitic glaucoma, which may be difficult to distinguish from a steroid response. Patients may develop POAG in the future. POSNER-SCHLOSSMAN (GLAUCOMACYCLITIC CRISIS) SEE UVEITIS SECTION FOR MORE DETAIL - unilateral, high IOP, mild, cyclitis - recurrent, younger patients OPEN ANGLE MANAGEMENT -Rule out open angle inflammatory glaucoma, Fuchs heterochromia, pigmentary, angle closure Medical therapy NO MIOTICS! - Topical beta-blocker BID - CAI topical or oral - May consider topical steroid to control the inflammation, but be aware of steroid induced IOP increases - Follow every few days then on a weekly basis until attack is over. - Once the attack is over, discontinue meds. Medications do not prevent recurrences. UVEITIC OR INFLAMMATORY GLAUCOMA SYMPTOMS: Pain, photophobia, blurred vision. SIGNS: May present similar to acute angle closure. Elevated IOP, red eye, corneal edema, and a miotic pupil. However, significant cell and flare, KPs synechiae, and an open angle are the signs, which may be used, in the differential diagnosis. MECHANISM: Inflammation in the eye can lead to a tremendous increase in IOP. Trabeculitis, the inflammation of the trabecular meshwork, may reduce pore size. Inflammatory debris and increased viscosity of the aqueous contributes to decreased outflow. May be caused by a host of ocular inflammatory conditions. Chronic uveitis can cause a host of problems such as cataracts, peripheral synechiae, posterior synechiae, and nodule formation. MANAGEMENT:

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Gonioscopy Rule out angle closure, Posner Schlossman, neovascular, etc -any type of acute glaucoma. Rule out synechial angle closure. Medical therapy: * Prednisolone acetate (Pred-forte) 1% Q 1-2H * Cycloplegics to prevent or break posterior synechiae * Topical beta-blocker (Timoptic .5% BID), Brimonidine, Dorzolamide. * MIOTICS! CONTRAINDICATED IN INFLAMMATION. * Prostaglandin analogs also contraindicated. * If IOP stays elevated, consider steroid response and switch to a medication less likely to create steroid response (Vexol or Lotemax). * Ineffective topical treatment with steroids may need subconjunctival and/or oral steroids. * Uveitic glaucoma does not do well with ALT or filtration surgery. May require systemic work-up to find out etiology of uveitis. MISCELLANEOUS GLAUCOMA PHACOMORPHIC GLAUCOMA Shallowing of the anterior chamber secondary to a swelling lens. May induce pupillary block or non-pupillary block angle closure. MANAGEMENT: Tonometry/Gonioscopy determine if there is a pupillary block component. Laser Iridectomy is the treatment of choice. See if the angle deepens after the procedure. If ineffective and the angle is narrowing, lens removal may be the only option. PHACOLYTIC GLAUCOMA Occurs when protein material leaks from a mature cataract and obstructs outflow of aqueous through the trabecular meshwork. SYMPTOMS: Patient may complain of unilateral pain, photophobia, and redness. SIGNS: May cause an increase in IOP. A mature cataract should be present. Angles open, significant cell and flare, corneal edema, ciliary injection. MANAGEMENT: Immediate control of IOP is required. Use medications similar to angle closure glaucoma. NO MIOTIC. Topical cycloplegics and steroids a (Pred forte 1% Q 1H). Lens removal when the IOP is controlled and inflammation is reduced. Usually in 2-3 days. * If it occurs follow cataract surgery:

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Medical management should include cycloplegic, topical or oral medications, and topical steroids. If medical management is not effective, surgical management may be needed to remove the obstructing materials. Consider phacoanaphylactic endophthalmitis.

GLAUCOMA ASSOCIATED WITH HYPHEMA SEE OCULAR EMERGENCIES SECTION SYMPTOMS/SIGNS: Pain, blurred vision, history of trauma. Blood in the anterior chamber to various degrees. Outflow of aqueous may be compromised by the presence of blood in the anterior chamber. Often rebleeds can occur and do so in the first 5 days. Rebleeds increase the likelihood that a rise in IOP may occur. Those filling at least 50% of the anterior chamber are more apt to cause a rise in pressure, MANAGEMENT: 1) For treatment of Hyphema (see ocular emergencies) 2) Treatment of associated glaucoma: Topical beta blockers may be used. Neptazane, not Diamox, should be used if an oral CAI is needed; otherwise, Trusopt. NO MIOTICS! Surgical intervention may be required after 1 week. ANGLE CLOSURE GLAUCOMAS PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK Symptoms: Pain, nausea, vomiting, blurred vision, colored haloes around lights, headache SIGNS: Mid-dilated pupil, corneal edema, conjunctival hyperemia, shallow anterior chamber, no visible structures with gonioscopy, markedly elevated intraocular pressure (40-50 or more), anterior chamber inflammation. EPIDEMIOLOGY 1) Race (indecending order) - Eskimos: 40 times incidence of ACG than whites - Caucasians - 0.1% - Asians - African American 2) Sex - Women 3-4 times incidence of men 3) Age - Peaks at 50s-60s

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Refractive Error - associated with hyperopia

MECHANISM: Posterior iris-lens contact at the pupillary margin impedes normal aqueous flow. Aqueous accumulation in the posterior chamber displaces the peripheral iris into appositional closure of the drainage angle. This mechanism can also occur with an intumesecent lens and posterior synechia. Can occur spontaneously or be triggered by pupillary dilation. Adrenergic agonist agents, such as phenylephrine, pull the relaxed peripheral iris posteriorly towards the lens. Pupillary block occurs when the pupil is in the mid-dilated state; as the pupil constricts several hours post dilation. * Loss of vision can occur after several hours and blindness after 2-3 days. OCULAR EMERGENCY. MANAGEMENT: COMPLETE HISTORY a. Time of onset b. History of previous attacks in affected or fellow eye c. Current Medications including over-the counter - Many sympathomimetic and parasympatholytic drugs can precipitate an angle closure attack. External Ocular Examination: - Signs of previous angle closure attacks glaucomflecken, iris atrophy, irregular pupil GONIOSCOPY * Check the status of the angle. - Angle is open or closed - Check for signs of previous angle closure - synechiae - Use 4 mirror lens to perform indentation gonioscopy. - Determine if the angle is closed by a pupillary block or non-pupillary block mechanism to the best of your ability. * Check fellow eye for narrow angle or signs of intermittent angle closure. MEDICAL MANAGEMENT: If the pressure is greater than 40 mm Hg. Medications Topical Beta-blocker (verify not contraindicated) 1 drop q 30 minutes Adrenergic Agonists 2 drops q 30 minutes i. apraclonidine 0.5% or 1% (Iopidine) ii. brimonidine 0.2% (Alphagan) Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) NO Sequels for acute situation ii. Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients.

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1 or 2% Pilocarpine 1 drop q 15-30 minutes *Note: not effective if IOP >40mmHg. Pilocarpine contraindicated in aphakes and pseudophakes. Make sure you have ruled out types of glaucoma for which pilocarpine is contraindicated. . Topical steroid such as 1% prednisolone acetate to decrease anterior chamber reaction 1 drop 4-8 times /day Recheck IOP every 30 minutes. If IOP not lowered readminister medications as specified. If IOP not lowered in 60 minutes or if truly emergent situation include: Oral Hyperosmotic Agents i. Oral Isosorbide (1.5 ml/lb. Of body weight) Best for diabetics ii. Oral Glycerin (1.5 g/kg of body weight or 2-3 ml/kg) (roughly 4-6 ozs. Per individual) OR iii. Intravenous Hyperosmotic Agent * IV Mannitol (1-2 g/kg in 45 minutes) Verify attack has been broken: IOP back to normal, angle open by gonioscopy, pupil is miotic. If attack broken, continue topical Beta-blocker, pilocarpine until patient has a peripheral laser iridotomy. Continue topical steroid until the eye quiets. Attack not broken if 4-6 hrs., patient should be sent for a LPI or surgical iridectomy. Consider another mechanism of angle closure. Prophalactic LPI is typically performed in the fellow eye. Long-term: evaluate for open angle glaucoma component as mixed mechanism glaucoma may be present. CHRONIC ANGLE CLOSURE GLAUCOMA Chronic areas of appositional closure which over time develop peripheral anterior synechial formation. As more of the angle is closed, the intraocular pressure rises and glaucomatous damage occurs. First starts in the superior angle. SYMPTOMS: Usually asymptomatic. Can manifest angle closure symptoms with pupil block. SIGNS: Narrow peripheral anterior chamber with possible areas of peripheral anterior synechiae. May see glaucomatous damage. MANAGEMENT: Gonioscopic evaluation:

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-Indentation gonioscopy to determine areas of synechial closure. Compression will not allow deepening of the peripheral angle if synechiae are present. Laser peripheral iridotomy indicated as early as possible. If too many areas of synechial closure, medical management is indicated. If pressure remains elevated, consider rabeculectomy (filtering procedure). NARROW ANGLES Determine if patient reports symptoms of previous angle closure attack SEE ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK section or has a family history of angle closure attacks. Look for evidence of previous angle closure attacks: segmental iris atrophy, glaucomflecken (focal infarcts of the lens epithelium), peripheral anterior synechiae, irregular pupil, glaucomatous changes. If dilation is performed follow procedure for provocative mydriatic test Procative testing can be performed Dark room lying prone for 1 hr. recheck IOP immediately; IOP elevation of 8 mmHg or more is a positive test Mydriatic- consider performing on one eye; dilate with 0.5% Tropicamide only; Symphathomimetics such as phenylephrine not used since may make pupillary block more difficult to break; Mid-dilated pupil is desired for this test since pupillary block more likely to occur with mid-dilated pupil; Check IOP after 3060 minutes if elevated by 5 mmHg or gonioscopy shows narrower angle test is positive. Reverse with 1-2% pilocarpine or follow management under ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK Consider LPI : Positive provacative test History or evidence of previous angle closure attack Full trabecular meshwork not visible in at least one quadrant No access immediate medical attention should an angle attack occur SUBACUTE ANGLE CLOSURE GLAUCOMA Intermittent periods of angle closure which spontaneously break after minutes to hours. May be broken by changing environments. SYMPTOMS: Intermittent episodes of blurring of vision halos around lights, headache, pain. These may precipitate at night, in dimly-lit environment, times of stress, fatigue, hunger, anger, reading and taking certain medications. SIGNS:

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Shallow peripheral anterior chamber, segmental iris atrophy, glaucomflecken (focal infarcts of the lens epithelium), peripheral anterior synechiae, irregular pupil, cataract, glaucomatous changes.

MANAGEMENT: LPI is the treatment of choice to prevent intermittent episodes of pupil block. PRIMARY ANGLE CLOSURE GLAUCOMA NON-PUPILLARY BLOCK PLATEAU IRIS TYPES: PLATEAU IRIS CONFIGURATION: Normal central anterior chamber depth with a flat iris plane from the pupillary margin to the midperiphery at the midperiphery the iris turns sharply posteriorly before insertion into the ciliary body. Gonioscopic evaluation may reveal a last large iris roll. When the pupil dilates, may develop acute angle closure due to the iris bunching up and blocking the trabecular mechwork. PLATEAU IRIS SYNDROME Similar to appearance to Plateau iris configuration. Angle closure is not prevented by LPI. SYMPTOMS: Asymptomatic unless angle closure attack presents. SIGNS: Anatomical variation diagnosed by gonioscopic evaluation. Signs indicative of angle closure SEE ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK SECTION. MANAGEMENT: If angle closure is precipitated, treat as specified in ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK section. Laser peripheral iridectomy is the long-term treatment of choice for PLATEAU IRIS CONFIGURATION as dilation could precipitate an angle closure attack. To differentiate between PLATEAU IRIS CONFIGURATION and PLATEAU IRIS SYNDROME: One week after LPI, dilate with 0.5% Tropicamide. If IOP increases, plateau iris syndrome may be diagnosed. Treat acutely as specified in ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK section.

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Long-term treatment includes either weak topical miotic such as 1% Pilocarpine QID or argon laser gonioplasty. If this is ineffective consider trabeculectomy. SECONDARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK PHAKOMORPHIC GLAUCOMA Manage acutely like ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK primary acute angle closure After attack is broken remove cataract PSEUDOPHAKIC AND APHAKIC PUPILLARY BLOCK Management: acutely like ACUTE PRIMARY ANGLE CLOSURE GLAUCOMA PUPILLARY BLOCK in addition consider tropicamide and phenylephrine UVEITIS WITH 360 POSTERIOR SYNECHIA Topical Beta-blocker Adrenergic Agonists i. apraclonidine 0.5% or 1% (Iopidine) ii. brimonidine 0.2% (Alphagan) Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients. Uveitis is treated as specified in the UVEITIS SECTION (prednisolone acetate 1% and homatropine 5%); LPI; trabeculectomy SECONDARY ANGLE CLOSURE GLAUCOMA NON-PUPILLARY BLOCK CILIOCHOROIDAL EFFUSION Topical Beta-blocker Adrenergic Agonists Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients. prednisolone acetate 1% and homatropine 5% oral steroids CYST OF CILIARY BODY OR IRIS Treat cysts with laser INTRAOCULAR TUMOR

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Topical Beta-blocker Adrenergic Agonists Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients. INFLAMMATION/UVEITIS Same as :UVEITIS WITH 360 POSTERIOR SYNECHIA see preceding section ICE IRIDOCORNEAL ENDOTHELIAL SYNDROME Young to middle age adults; more common in women; not inherited; uniocular SYMPTOMS: Abnormalities in their iris appearance, blurred vision and pain. SIGNS: All ICE syndromes have uniocular progressive iris atrophy, PAS and iris holes. PAS can cause angle closure. PROGRESSIVE IRIS ATROPHY Iris atrophy, PAS with iris hole adjacent to it, abnormal corneal endothelium

CHANDLERS SYNDROME Corneal edema, corneal endothelium dysfunction (hammered silver appearance), iris atrophy, iris holes, displaced pupil and PAS. COGAN-REESE SYNDROME Iris nevi (iris nodules), corneal edema, corneal endothelium dysfunction (hammered silver appearance), iris atrophy, iris holes, displaced pupil and PAS.. TREATMENT: If glaucoma is present, Topical Beta-blocker Adrenergic Agonists Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients. Filtering surgery. Hypertonic saline solutions to decrease edema. Keratoplasty, in advanced edematous conditions. MALIGNANT GLAUCOMA (CILIARY BLOCK GLAUCOMA) aqueous misdirection into the vitreous cavity; differentiate from pupillary block glaucoma Management: Topical Beta-blocker Adrenergic Agonists

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Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients. prednisolone acetate 1% and homatropine 5% Laser treatment to ciliary processes NEOVASCULAR GLAUCOMA An angiogenic factor is released in situations where intraocular ischemia is present. As result, new iris vessels or rubeosis irides, begin to grow. Initiating at the pupillary margin, the vessels grow toward the angle. Vessels move over the trabecular meshwork along a gonioscopically-invisible fibrous support membrane and impede outflow (open angle). However, contraction of the fibrovascular tissue causes zippering of the angle. Tent-like PAS may be seen initially, then broad-based synechiae as the angle zippers up 360 degrees. This is the non-pupillary block angle closure mechanism which is symptomatic. Complete closure can occur in days to a couple of weeks. CAUSES: MOST COMMON CRVO, Diabetes Mellitus, Carotid occlusive disease, Ocular ischemic syndrome, CRAO. Extraocular disease, intraocular vascular occlusion/disease neoplasms, retinal detachment, and uveitis may be responsible for neovascular glaucoma. SYMPTOMS: Similar to acute angle closure glaucoma. Red, painful, eye. SIGNS: Corneal edema, elevated IOP, iris neovascularization, injection, synechial angle closure, mild cell/flare. MANAGEMENT: Prophylactic: PANRETINAL PHOTOCOAGULATION (PRP), Retinal cryoablation PRP may also prevent rubosis irides from progressing to neovascular glaucoma. Acute episode of neovascular glaucoma Topical Beta-blocker Adrenergic Agonists Oral Acetazolamide i. 500 mg PO (2 x 250 mg tabs) Substitute dorzolamide or brinzolamide or IV Acetazolamide 500 mg for nauseated patients. prednisolone acetate 1% and atropine 1% * * PANRETINAL PHOTOCOAGULATION (PRP) is indicated immediately to decrease hypoxic demand, if retinal disease is the underlying cause. May involute the iris neovascularization over several weeks. PANRETINAL CRYOTHERAPY is used to treat hypoxic retina if there is poor

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visibility and PRP cannot be performed. In severe cases, TRABECULECTOMY / ANTIMETABOLITES Seton Implants are often used.

Angle is closed and there is no useful vision: * Topical steroids and cycloplegics (atropine) to control inflammation * If the eye becomes painful, consider ciliary body ablation or retrobulbar alcohol injection. CORNEODYSGENESIS POSTERIOR EMBRYOTOXON Bilateral prominently anterior displaced Schwalbes line. Low risk for glaucoma. AXENFELDS ANOMALY Posterior embryotoxon plus prominent iris processes which insert into the prominent Schwalbes line. 50% of the patients may develop glaucoma (Axenfelds syndrome). Bilateral RIEGERS ANOMALY Posterior embryotoxon, iris strand attachments to Schwalbes line, and changes to the iris. Midperipheral iris adhesions to the cornea, areas of iris stroma hypoplasia and iris holes can displace the pupil (corectopia). Dental and skeleto-facial anomalies also are present (Riegers syndrome). High association with glaucoma. Bilateral. TREATMENT: No treatment unless glaucoma is present. If glaucoma is present in infants, goniotomy or trabeculotomy is performed. In older children, medical management as in POAG is attempted prior to surgical procedures. PETERS ANOMALY (Anterior Chamber Cleavage Syndrome) Congenital iris hypoplasia with iris attachments to the posterior cornea. Central corneal clouding can occur. Other findings include: myopia, cataracts, aniridia and microphthalmos. Risk for glaucoma (syndrome) is 50%. Bilateral. TREATMENT: No treatment unless glaucoma is present. If glaucoma is present in infants, goniotomy or trabeculotomy is performed. In older children, medical management as in POAG is attempted prior to surgical procedures. May require keratoplasty if the scar decreases vision.

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 ANIRIDIA Inherited condition in which there is bilateral absence or almost absence of the iris at birth. May be associated with childhood onset glaucoma, amblyopia, nystagmus, foveal hypoplasia, cataract, and corneal opacification. In sporadic cases, WILMS tumor (kidney) may be present. TREATMENT: No treatment unless glaucoma is present. If glaucoma is present in infants, goniotomy or trabeculotomy is performed. In older children, medical management as in POAG is attempted prior to surgical procedures. -Caution with a beta-blocker. Monitor cataract and cornea. May require surgical intervention when vision is decreased. Correct the amblyopia. Systemic work-up by pediatrician to rule out disease. Glaucoma Overview: This study guide by far only provides just a summary of glaucoma differentials and therapy. Glaucoma therapy is evolving and consistently changing as new research is completed. As a practicing optometrist, is is imperative to review major clinical trials and the literature for updates in therapy in technology. Consider review of the following: 1. NTG Study 2. AGIS Study ( Advanced Glaucoma Intervention Study) 3. OHTS ( Ocular Hypertension Treatment Study) 4. CIGTS ( Collaborative Initial Glaucomat Treatment Study) 5. OCT 6. HRT 7. GDX-VCC 8. Pachymetry 9. SITA-SWAP technology 10. Humphrey Matrix ( Frequency Doubling Technolgy) 11. Nocturnal IOP 12. Structure versus Function

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PRE-OPERATIVE AND POST-OPERATIVE CATARACT MANAGEMENT AGE-RELATED CATARACTS Is the major cause of PREVENTABLE blindness throughout the world. Often seen more in warmer climates with greater sun exposure, in females, patients with diabetes mellitus, and exposure to radiation. 1. NUCLEAR SCLEROSIS Thought to be alteration in lens metabolism which increases the concentration of insoluble proteins. Nucleus becomes a dense, solid mass. Index changes in the nucleus can cause myopic shifts in refractive error. Tends to be slow progression and may not affect acuity. Distance vision may be affected more than near. Patient may experience glare of diplopia. CORTICAL (CUNEIFORM) CATARACTS Due to osmotic imbalance and alteration of the distribution of electrolytes. Accumulation of extracellular water occurs resulting in disruption of the lens fibers Can cause a hyperopic shift. As the lens changes progress, hydration increases and the lens swells. Axial length of the lens may increase to a point where secondary pupillary block can occur. Called an intumescent cataract. Morgagnian cataract complete liquefaction of the cortex and the lens nucleus may sink to the bottom or float - May experience blurred vision (if visual axis involved), diplopia or glare. Obstructs peripheral fundus views. POSTERIOR SUBCAPSULAR CATARACT Visually disabling, if on the nodal point of the eye. Typically affects near vision more than distance. May cause more glare than NS or CS Vision may be better in dim illumination when the pupil is larger. Acuity may drop with pinhole. May be caused by medications such as steroids, uveitis, RP, trauma, neoplasms, and radiation

2.

3.

CAUSES OTHER THAN AGE CONGENITAL ANOMALIES Intrauterine infection: Syphilis, rubella, measles etc. Genetic Gestational disturbances drugs, malnutrition or irradiation. METABOLIC CATARACTS: a. Galactosemia develop in the first few months of life. - Oil drop in the nucleus

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b. c. d. e. f. TRAUMA

- May note diplopia Galactokinase deficiency Hypoglycemia increased risk in premature infants. Diabetes mellitus Hypocalcemia Hypoparathyroidism

a. Rosette cataract disruption of lens fibers b. Penetrating injury c. High-voltage shock anterior capsule changes RADIATION a. Ultraviolet b. infrared glass-blower or exfoliation c. X-rays PSC TOXIC a. b. c. d. Pilocarpine Anterior subcapsular Corticosteroids Posterior subcapsular Cholinesterase inhibitors Ant. subcapsular Thorazine Pigment deposition on the cortex and capsule

SYSTEMIC a. Diabetes snow-flake cataracts, can affect refractive error, and accelerate aging changes of lens. b. Wilsons Hepatolenticular degeneration - Copper deposition in ant. Lens capsule: Sunflower c. Myotonic dystrophy: - Iridescent subcapsular cataract Christmas-tree d. Atopic dermatitis - PSC; anterior polar cataract e. Hypercalcemia small, white, ant. And post. Cortical cataracts INTRAOCULAR DISEASE Uveitis, RP, RD, Tumors, Anterior Segment Ischemia CONGENITAL OPACITIES 1. Capsular small flakes, unilateral or bilateral 2. Polar found at subcapsular cortex - can be anterior or posterior - can affect vision if on the central posterior pole - Anterior form can extend into anterior chamber 3. Anterior axial embryonic small white dots in anterior lens - no effect on vision 4. Sutural anterior and posterior stellate

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5. 6. 7.

- In the Y sutures, Bilateral, Bluish or white - no effect on vision Pulverulent Bilateral, autosomal dominant - White dots on the fetal nucleus - no effect on vision Cerulean cataract Small opaque blue dots in the lens - common, May not affect vision Rubella unilateral, dense, white nucleus - Maternal transmission of the virus in the first trimester

INDICATIONS FOR CATARACT SURGERY 1. Dislocated or subluxed lens 2. Reduced vision and reduced activities of daily living 3. Phacomorphic glaucoma 4. Phacoanaphylactic uveitis 5. Phacolytic glaucoma 6. Poor view of the fundus with active retinal disease. 7. In the case of a child, if significant vision loss is present, early surgery may prevent deep amblyopia and allow for better results with vision training. Always rule out other systemic problems. CATARACT EVALUATION * Determination of the time for cataract extraction is usually based upon the patients functional ability. Visual needs vary as well as tolerance to the symptoms on an individual basis. SUBJECTIVE TESTING SYMPTOMS: Decreased visual acuity Glare Decreased contrast Aniseikonia Asthenopia Unequal acuity: near vs far Monocular diplopia, polyopia Binocular diplopia Ghost images Decreased color sense VISUAL FUNCTION Potential visual function i. Interferometry Laser or white light ii. Guyton-Minkowski Potential Acuity Meter (PAM) iii. McIntyre Super Pinhole Glare testing i. Contralight test acuity chart blacklit by sun exposure

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ii. Miller-Nadler iii. Mentor Brightness Acuity Tester (BAT) Contrast sensitivity i. Vis-tech OBJECTIVE TESTING 1. 2. 3. 4. KERATOMETRY PUPIL TESTING a. Cataracts do not cause afferent pupillary defects SPECULAR MICROSCOPY Endothelial cell count ULTRASONOGRPHY a. A-scan: i. Axial length measurements of IOL calculations. b. B-scan: i. Indicated in cases where there is no view of the posterior pole, assessment of retinal detachment, neoplasms, disc drusen, etc

PREOPERATIVE MEDICATIONS Topical antibiotics Topical antihypertensives (1% Iopidine) Pupillary dilation Topical NSAID SURGICAL PROCEDURES 1. ICCE INTRACAPSULAR CATARACT EXTRATION a. Entire lens is extracted along with the capsule. b. Not done as frequently as in the past. c. Performed in third world countries d. Used in cases such as dislocated lenses. e. Requires zonulytic agent (alpha-chymotrypsin) to be placed into the eye prior to surgery. f. Cryoprobe is placed on the capsule g. Lens is removed through a large wound near the limbus. h. More prone to complications: vitreal prolapse, CME, increased risk of retinal tears, etc i. Not indicated in younger patients due to strong hyaloid-lens adhesions. 2. ECCE EXTRACAPSULAR CATARACT EXTRACTION Surgery in which the posterior capsule is left intact. Allows placement of posterior chamber intraocular lens implant (PC IOL). Relative contraindication in patients with chronic uveitis. Usually, a limbal incision is made and a viscoelastic substance such as Healon is placed in the anterior chamber to protect the endothelium. The anterior capsule is then cut by a cytostome and removed The nucleus is physically express out of the capsular bag.

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The remaining cortex and capsular debris are cleared. PC IOL is implanted into the posterior chamber, ideally supported by the posterior capsule (in the bag). Miotic is placed in the anterior chamber. Evacuation of the viscoelatic substance. Wound is closed. Reduced risk for CME, RD, vitreous loss or vitreous-induced pupillary block. 3. ECCE - PHACOEMULSIFICATION Similar opening surgical technique as planned ECCE. Involves ultrasound instrument which vibrates at high frequency and fragments the lens material. c. Can use a smaller incision since whole nucleus is not being expressed. d. New techniques have allowed the procedure to have 1 stitch or no-stitch closure. i. Foldable IOLs ii. Capsulorhexis 3-5 mm circle is cut from the central anterior capsule. e. After Healon is placed in the ant. chamber, capsulorhexis is performed. g. Phacoemulsification probe is placed in through the hole in the capsule and emulsification of the nucleus begins The cortex and capsule are aspirated as in planned ECCE. Healon is removed and the wound is closed. May have higher risk for endothelial damage, zonular damage. May not be able to phacoemulsify dense nuclear sclerotic cataracts with most phacoemulsification equipment. Not recommended in dislocated lenses, shallow anterior chamber, miotic pupils, compromised endothelium, non-transparent corneas. Quicker recovery, less astigmatic error induced, less discomfort. a. b.

INTRAOCULAR LENS IMPLANTS 1. IRIS CLIP LENSES or IRIS FIXATED LENS a. Loop haptics which clip onto the iris or sutured into place b. Characterized by square pupil. c. Instability seen with iris movement: shaking d. Was popular in the 70s as a solution to lens dislocation and IOL-corneal touch. Higher change of endothelial damage and CME *f. DO NOT DILATE PATIENT! Unsutured or IOLs without synechiae formation may dislocate with dilation 2. a. b. c. d. e. f. ANTERIOR CHAMBER LENSES Placed in the anterior chamber with the haptics in the angle. Easier to insert and remove. Can be used as a secondary implant. Can dilate the patient Caused UGH syndrome uveitis, glaucoma, and hyphema Caused corneal problems, endothelial damage

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g. 3. a. b. c. d.

CME is more common in secondary implants POSTERIOR CHAMBER LENSES Lens of choice by most surgeons. Placed in the capsular bang in the posterior chamber. May come in rigid and foldable forms. Major drawback is difficulty with removal.

COMPLICATIONS DURING SURGERY Descemets membrane torn or detached Capsular bag tear May lead to vitreous loss Breakage of lens zonules Endothelial damage Trauma to the iris Expulsive choroidal effusion Retrobulbar hemorrhage secondary to injection Choroidal hemorrhage DETERMINATION OF POST-OPERATIVE CYLINDER Very little induced cylinder with clear cornea technique. Varies with - Amount of pre-operative corneal toricity - Location of the incision to the cornea. Closer greater. - Size of the incision. Larger greater amount. - Sutures: Tightness, Material - Method of suturing POST-OPERATIVE MANAGEMENT 1. IMMEDIATELY FOLLOWING SURGERY Topical antibiotic Topical steroid Antihypertensive if needed (1 drop 1% Iopidine) Metal Eye Shield for at night Acetaminophen or Ibuprofen PRN Avoid activities, which may require a valsalva maneuver or heavy lifting for 24 hours. RTC next day DAY ONE Post-operative a. Examination i. History/chief complaint ii. Visual acuity with pinhole iii. Pupils iv. External examination

2.

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* * * * * *

* Tonometry b. Management i. Treat complications appropriately i.e. abrasions, elevated IOP, hyphema * See section on early complications. ii. Topical antibiotic/steroid drops QID Most surgeons prescribe topical fluoroquinolone and prednisolone acetate iii. Topical glaucoma medications, if IOP is elevated. iv. Eye shield at night for 2-4 more days. v. Acetaminophen or ibuprofen PRN vi. RTC in 1 week post-op vii. Frequency of follow-up visits are dictated by complications. Patients to return if there s any unexpected changes -lid edema, increased pain or decreased vision. 3. STATUS POST-OP 1 WEEK Case history and elicit symptoms Visual acuity with pinhole Pupils Keratometry Consider refraction f. External examination g. Tonometry h. Treat any complications i. Maintain medications j. Return in 1- 3 weeks, unless problems arise a. b. c. d. STATUS POST-OP 2 - 4 WEEKS Case history Visual acuity with pinhole Keratometry Attempt Retinoscopy/Refraction *Remember that corneal toricity should equal refractive cylinder

Lids - equal fissure size? Conjunctiva May see chemosis, hemorrhages, injection - Seidal Test for wound leakage Cornea -May see edema, striae Anterior Chamber - Check depth, may see cell and flare, few RBCs, and debris Iris, Pupil - intact, intact pupil IOL - Centration Post. Chamber

4.

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e. f. g. h. i. j. k. 5.

in a psuedophakes or aphakes in most cases. External Examination/pupils Tonometry Consider dilation, if acuity is worse than 20/50 Treat any complications Begin taper of medications (topical), if cell and flare are diminishing. Eye protection, cautious with physical activity, hygiene Return in 3-4 weeks, unless problems arise.

STATUS POST-OP 5 8 WEEKS a. Case history b. Visual acuity with pinhole c. Keratometry d. Retinoscopy/Refraction for spectacle e. External Examination/pupils f. Cut sutures if high astigmatism is present g. Tonometry h. Fundus Evaluation/Capsule evaluation i. Manage any complications j. Continue taper or Discontinue medications k. Return in 4-6 weeks, unless problems arise a. b. c. d. e. f. g. h. STATUS POST-OP 3 MONTHS History Visual acuity with pinhole Pupils External examination Posterior capsule evaluation ;Consider posterior capsulotomy, if dense membranes has formed. Tonometry Fundus evaluation, if acuity is decreased Follow-up six months to annual.

6.

EARLY POST-OPERATIVE COMPLICATIONS ENDOPHTHALMITIS A devastating clinical entity which rarely occurs. Very poor prognosis. Acute post-operative endophthalmitis *Incidence of .05-.17% Post-operative Endophthalmitis Infectious - fungal or bacterial *Wound leak, contamination, airborne, local flora b. Sterile *Reaction to IOL *Chemical insult *Foreign material - powder from gloves, sponge a.

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c.

*Manipulation of the vitreous Long-term post-operative - (months - years later) *Access through inadvertent filtering blebs *Activation of liable bacteria *wound dehiscence

Most common bacteria is Staphylococcus Epidermidis in acute endophthalmitis. Also other Staph, Strep., and gram negs such as Psuedomonas. Chronic post-operative: Propionibacterium acnes Other forms: From penetrating trauma From endogenous sources - drug use, abdominal surgery, etc SYMPTOMS: Patients complain of pain, redness, discharge, reduced vision. SIGNS: Increasing inflammation, possible Hypopyon, chemosis, lid swelling, conjunctival, scleral and episcleral injection, corneal edema, infiltrates, vitreous cell, retinal phlebitis and hemes, reduced red reflex, decreased vision, possible APD, elevated IOP. *NOTE: A hypopyon is not expected in the routine post-operative period. Always consider endophthalmitis as a possibility and refer out! DIFFERENTIAL DIAGNOSIS: Phacoanaphylactic uveitis Post-operative uveitis Forms of posterior uveitis Sterile inflammatory reaction TREATMENT: HOSPITALIZATION Immediate referral for cultures for bacteria, fungus i. Anterior chamber paracentesis vitreous aspiration Slides with Giemsa and gram staining Consider vitrectomy Modify treatment/ Treat based on culture results Treat with intravitreal and topical (fortified) antibiotics Topical, subconjunctival, and intravitreal steroids after fungal cause ruled out Cycloplegics Determined to be sterile after multiple cultures: *Increase steroid dosages, will respond.

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Patient is followed closely every 4-8 hours. PTOSIS May be mechanical or secondary to retrobulbar or peribulbar anesthetic or steroids. Follow. Most early postoperative ptosis will resolve, but some may not requiring oculoplastics POST-OPERATIVE HYPHEMA a. b. c. d. e. f. Source of bleeding usually from incision site however can occur from erosion into the iris root by the ACIOL or erosion into the ciliary body from the ciliary body. Most often seen with: incision posterior to the limbus, iris neovascularization, Fuchs heterchromic iridocyclitis, manipulation of the iris, history of anticoagulant therapy and intrinsic clotting defects. May cause an increase in intraocular pressure. TREATMENT: continue with antibiotic/steroid drops, consider cycloplegia, avoid anticoagulants (unless already prescribed), monitor IOP (treat IOP if elevated) and limit activity. Persistent or extensive cases: bedrest, evacuation of the anterior chamber. Rare complications include: Corneal blood staining, endocapsular hematoma, vitreous hemorrhage, and hemolytic glaucoma.

FLAT ANTERIOR CHAMBER a. Determine clinical entities which may be responsible. Pupillary block glaucoma *Elevated IOP with synechia, iris ballooning, lens dislocation and vitreous prolapse. *May require surgical management by glaucoma specialist - Wound leak *Positive Seidel sign, Low IOP *May indicate poor suturing, wound healing, or trauma to the area *Consult with surgeon may require surgical management *Minor leak can be treated with pressure patch and antibiotic - Choroidal detachment *Negative Seidel sign, Low IOP *Consult with surgeon regarding surgical management IRIS PROLAPSE May be seen as a peaked pupil or visible iris through open wound. Eye may be inflamed Vitreous prolapse may be present. May have hypotony, risk of endophthalmitis with open wound. Requires surgical management

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CORNEAL EDEMA a. Commonly seen response secondary to lens surgery. b. Endothelial damage secondary to surgery may result in chronic Edema, bullous keratopathy. c. Treatment with Hyperosmotic solution or ointment, NSAIDS or topical steroids. OCULAR HYPERTENSION WITH OPEN ANGLE(EARLY, CHRONIC OR LATE) Possibly due to: residual materials, inflammation, hyphema, intraocular lens haptics, neovascular changes Consider Treatment if IOP over 25 or if concern of optic nerve damage In EARLY cases: Do not treat with epinephrine compounds (link to CME) Avoid miotics TRANSIENT ANTERIOR IRITIS - Expect mild/moderate response - Continue treatment with antibiotic/steroid compound - Monitor for keratitic precipitiates and hypopyon OCULAR HYPERTENSION DUE TO NARROW OR CLOSED ANGLE a. Appositional pupil block *Elevated IOP with synechia, iris ballooning, lens dislocation and vitreous prolapse. More common with iris fixated and anterior chamber IOL Can occur with posterior IOLs if inserted in the sulcus rather than the bag or if vitreous prolapses into the anterior chamber. b. Malignant glaucoma * Uniformly narrow anterior chamber * High IOP with corneal edema *Aqueous misdirected behind vitreous causing lens dislocation, vitreous prolapse c. Medical management until surgical intervention is indicated. Laser peripheral iridectomy is typically performed in these cases. EXPOSED SUTURES a. May cause irritation (foreign body sensation) to the patient. Also discharge and tearing. b. Suture removal is indicated. c. Treat with topical antibiotics and lubricants. LATE COMPLICATIONS CYSTOID MACULAR EDEMA = IRVIN-GASS SYNDROME.

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One of the most common causes of vision loss following cataract surgery. Possibly due to intraoperative trauma or postoperative inflammatory response. Signs Appearance of macular thickening, cystic honeycomb retinal thickening, small parafoveal hemeorrhages, and yellowing of the macular area. Fluorescein angiography reveals characteristic "flower-petal" pattern of hyperfluorescence OCT shows increased foveal thickening Positive amsler grid /visual field findings. Symptoms Patients report blurred vision. CME occurs more frequently after cataract surgery with complications and in those with epiretinal membranes, diabetic retinopathy or those aphakes and pseudophakes taking topical epinephrine or propine. Prophylaxis for CME includes postoperative topical steroids and NSAIDS and periocular steroid injections. Visually significant CME occurs most commonly 4 weeks to 6 months following cataract surgery. Note: Most patients will demonstrate angiographic CME post-operatively with no affect on acuity. Differential Diagnosis Capsule Opacification, vitreous clouding, uveitis, corneal edema, retinal pathology. TREATMENT: Combination therapy with topical steroid and topical NSAID New therapy: topical Nevanac ( ALCON NSAID: TID DOSING) Topical/Oral steroids Discontinue epinephrine or propine. Surgical management *No real efficacy proven with any regimen. POSTERIOR CAPSULAR OPACIFICATION Occurs by two mechanisms: i. Epithelial cells from the lens periphery migrate to the posterior capsule ii. Metaplasia of epithelial cells into myofibroblasts. This results in fibrosis and contraction of the posterior capsule. Is a frequent complication of ECCE surgery, especially in young patients. Epithelial cells and cortical material left in the capsular bag during surgery. TREATMENT: Nd: YAG Laser Capsulotomy

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FOLLOW UP: recheck IOP due to possibility of spikes (within first week) perform DFE one month following procedure; most common complication retinal detachment. IOL DISPLACEMENT a. May result in glare, halos, reduced vision, diplopia. b. May be result of wrong size, wrong positioning, dilation of an Iris-supported lens. c. Pharmacological management may include miotics. d. May treat with contact lens e. May ultimately require some form of surgical correction. RETINAL DETACHMENT More prevalent with ICCE than ECCE. Risk factors: posterior capsule rupturing during ECCE surgery and vitreous loss during surgery. Risk factors: pre-existing vitreo-retinal disease, axial myopia, young myopic males, and previous detachment. Symptoms: flashes, floaters, vision loss. Refer out to retinal surgeon BULLOUS KERATOPATHY/ PERSISTENT CORNEAL EDEMA a. Loss of endothelial cells secondary to surgery may result in chronic edema of the cornea. b. Bullae formation. c. Patients complain of decreased vision and foreign body sensations. d. Treat with hypertonic solution/ ointments, topical steroids or NSAIDS, bandage contact lens (for pain), lower IOP if elevated with appropriate agent, consider surgical repositioning or exchange of IOL, and in severe cases a PKP may be needed. CHRONIC UVEITIS a. May be a response to physical contact with IOL b. Treat with low-dose steroids. Monitor carefully to avoid side effects of chronic steroid use. SECONDARY GLAUCOMAS Can be due to a number of mechanisms: *epithelial downgrowth, steroid response, peripheral anterior synechiae, uveitis, hyphema, pigmentary dispersion, trauma to the trabecular meshwork or pseudophakic angle closure. Monitor closely for IOP changes. Routine fields, gonioscopy, fundus and nerve evaluations Treat accordingly.

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SPECTACLE CORRECTION CONSIDERATIONS FOR APHAKES: 1. 2. Younger patients usually do not have implantation due to incomplete Eye growth. Spectacles for aphakes can cause lots of problems: a. Magnification - 30% or so. b. "Jack-in-the-box" phenomenon. -ring scotoma. c. Visual field limitation to about 30 degrees. d. Difficulties with fusion due to prismatic effects e. Anisophoria in down gaze f. Peripheral aberrations g. Image distortion h. Poor cosmesis i. Weight of the spectacles Conditions are worse for unilateral aphake. a. Consider size lenses. Consideration of contact lenses. a. Reduction of distortion, induced prism. b. Increased peripheral vision. c. Magnification is 5-9%. d. Must take into consideration the patient's age and anterior segment. i. Elderly patients have more problems. ii. Lids - blepharitis, decreased tension, ectropion, entropion, lagophthalmos, etc. iii. Tear film - decreased, epiphora, poor wetting action iv. Cornea - endothelial disease, EBMD, erosions v. Dexterity vi. Systemic disease - diabetes, rosacea, depressed immune response. vii. Young adults usually acquire cataracts through trauma. One may have to deal with a scarred or deformed anterior segment. Lens fit may commence 6-12 weeks post-operatively, if the anterior segment has healed. Lens choices: a. Rigid gas permeable b. Hydrogel lenses ii. May be worn continuous wear or daily wear. Contact lens complications seen are similar to other contact lens wearers. Prescribe ultraviolet protection over the contact lenses.

3. 4.

5. 6.

7. 8.

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NOTES:

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DISORDERS OF THE VITREOUS AND THE RETINA RETINAL VASCULAR DISORDERS CAROTID OCCLUSIVE DISEASE Based on ocular symptoms, the patient may be required to undergo a battery of tests necessary to quantify potential areas of occlusion. AMAUROSIS FUGAX May be an isolated symptom or in company with other neurological symptoms. Patients may report transient visual loss, which may last minutes to an hour. Vision returns to normal after the attack. Most often it is the result of emboli of cardiac or carotid origin. Cholesterol (Hollenhorst) plaques may be visible at the bifurcations of the retinal arteries. There are a host of emboli other than cholesterol plaques that can cause this. There is a strong association with systemic diseases such as carotid stenosis and cardiovascular disease. Risk factor for development of stroke. MANAGEMENT: *Blood pressure on both arms *Carotid auscultation for carotid bruit *Medical examination *Carotid Duplex scan (Doppler and ultrasound) *Blood studies including fasting blood sugar *Echocardiography *Cardiology consult * Angiography ARTERIAL OCCLUSION SEE RETINAL ARTERY OCCLUSION SECTION. OCULAR ISCHEMIC SYNDROME (OIS) Ocular ischemic syndrome (OIS) is typically found in patients around the age of 65 with severe carotid obstruction. Males seem to be afflicted twice that of their female counterparts.

CLINICAL PICTURE Typically unilateral Dull ache, which starts over the eye and migrates to the temple. Slow recovery time to bright light. Unilateral red eye. Corneal edema with Descemets folds Rubeosis irides or neovascularization glaucoma. Mild cell and flare response. Rapidly developing lens opacities. History of unilateral monocular blindness.

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Unilateral arterial narrowing, venous dilation Equatorial hemorrhages. Fluorescein angiography may reveal prolonged circulation/transit time, vascular staining, and non-perfusion. Hypertension is present in two-thirds and diabetes one-half of the patients. MANAGEMENT: *Blood pressure on both arms *Carotid auscultation for carotid bruit *Fluorescein Angiography *Medical examination *Carotid Duplex scan (Doppler and ultrasound) *Blood studies including fasting blood sugar *Westergren sedimentation rate, if giant cell is suspected *Cardiology consult *Ocular Treat the neovascular glaucoma. *See Neovascular glaucoma. PRP or gonioplasty. Monitor or treat retinal findings.

DIABETIC RETINOPATHY Occurs with increasing frequency as duration of disease increases. Strict glucose control seems to favorably affect the course of retinopathy. Duration of disease is the greatest risk for development of retinopathy. Microvascular compromise is first initiated as loss of pericytes from the walls of the capillaries. In combination with increased vascular resistance, the loss of pericytes results in ballooning of the weakened vessel wall. This is called a microaneurysm. Microaneurysms may leak fluid which present as intra-retinal edema. Weakened areas of the vessels may release blood resulting in dot-blot hemes. Hard exudate formation or lipid accumulation indicates long-standing edema. As the disease progresses, capillary occlusion can produce localized signs such as cotton-wool infarcts, venous beading, and flame hemes. Capillary closure can create more areas of retinal non-perfusion which respond with intraretinal microvascular abnormalities (IRMA) and neovascularization. Interaction of the neovascularization and its fibrotic scar tissue with the vitreous-retina interface can result in tractional retinal detachments, vitreous hemorrhages, and subsequent loss of vision. In addition, ischemic retina can produce neovascularization of the iris which can lead to subsequent forms of glaucoma. CLINICAL PICTURE: Early Treatment Diabetic Retinopathy Study Severity Scale/Classification MILD NPDR MODERATE NPDR

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 SEVERE NPDR VERY SEVERE NPDR PDR MILD PDR MODERATE PDR HIGH-RISK PDR ADVANCED PDR

Nonproliferative diabetic retinopathy (NPDR) Mild, Moderate Blot hemorrhages, flame hemorrhages, cotton wool spots , hard exudates, IRMAS, venous beading Severe nonproliferative diabetic retinopathy *Venous beading, IRMA present in at least two quadrants OR *Moderate IRMA present in at least one quadrants OR *Moderate hemorrhages and microaneurysms present in all quadrants 4-2-1 Rule Very Severe diabetic retinopathy nonproliferative 2 or more of the above criteria Mild/Moderate proliferative diabetic retinopathy *New vessel growth on the disc or elsewhere and *Definition for High-risk proliferative diabetic retinopathy or worse not met. High-risk proliferative diabetic retinopathy *New vessels on or within 1 DD of the optic disk (NVD) which is greater or equal to 1/3 1/2 disk area, with or without vitreous hemorrhage, or *Vitreous or preretinal hemorrhage and NVD less than 1/3 1/2 disk area, or *Vitreous or pre-retinal hemorrhage and neovascularization elsewhere (NVE) greater than 1/2 the disk area. Diabetic Macular Edema Macular edema is the major cause of vision loss. It can occur with NPDR or PDR Clinically Significant Macular Edema (CSME) defined as one of the following: (1) retinal thickening within 500 microns (1/3 of disc diameter) of the center of the macula (2) hard exudates within 500 microns (1/3 disc diameter) of fovea, if associated with thickening of surrounding retina. (3) Retinal thickening 1 disc diameter or greater and within 1 disc diameter of fovea.

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MANAGEMENT: Educate patient to strive for controlled blood sugar level. Focal, grid laser photocoagulation remains the treatment of choice for clinically significant macular edema. Newer treatments include intravitreal steroid injections and vitreal implants. This new research is showing promise for alternatives to the laser destructive procedures. OCT imaging is becoming a more widely used way evaluating diabetic macular edema. Panretinal photocoagulation laser treatment is considered for severe NPDR and PDR. Mild non-proliferative DR: no macular edema present. *Low percentage progress to PDR in 1 year *Follow up in at least one year Clinically significant macular edema: *Fluorescin angiography to identify areas of leakage *Treatment of choice for focal edema is traditionally Focal laser photocoagulation Moderate non-proliferative DR: *12-27% risk of progression to PDR in one year *Follow up every 3 months *Identify risk factors Severe non-proliferative DR: *52% risk of progression to PDR in 1 year *Retinal consultation fluorescein angiography to identify areas of non-perfusion *Consider panretinal photocoagulation Early Proliferative DR: *Retinal consultation fluorescein angiography to identify areas of non-perfusion *Consider panretinal photocoagulation High-risk Proliferative DR: *Retinal consultation fluorescein angiography to identify areas of non-perfusion *Consider panretinal photocoagulation. If there is a poor view of the retina, peripheral retinal cryotherapy is performed *Vitrectomy is indicted for: -Long-standing non-clearing vitreous hemorrhage -Tractional retinal detachment involving the macula -Displacement of the macula -Unresponsive neovascularization and fibrous proliferation after attempted laser procedures -Hidden retinal detachments under fibrosis/Traction RD -Recurrent vitreous hemorrhage

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EALE'S DISEASE (RETINAL PERIPHLEBITIS) A disease of young men, age 20 - 40, most often bilateral. Characterized by peripheral retinal venule inflammation. It may be the result of tubercular hypersensitivity. CLINICAL PICTURE: Inflammatory perivascular sheathing and vitritis. Neovascularization can lead to vitreous hemorrhage. Macular edema can be due to perimacular telangiectasia. MANGEMENT: Must rule out tuberculosis with a PPD and chest x-ray. Refer for treatment of Tuberculosis if indicated. Laser photocoagulation is used to treat neovascularization/nonperfusion and edema. HYPERTENSIVE/ARTERISCLEROTIC RETINOPATHY Prolonged or chronic hypertension can lead to the breakdown of the blood-retina barrier. Most often, chronic hypertension, in conjunction with arteriosclerosis, results in vessel changes. This may be appreciated by observing color, contour, reflex, and with changes of the retinal vasculature. Retinal hemorrhaging, infarction, and vascular closure may be seen in severe disease. CLINCIAL PICTURE -Asymptomatic and bilateral -Retinal arteriole constriction, minimal tortuosity -A-V crossing changes -Artery sheath changes -Flame shaped (typically) Hemorrhages -Exudates. -Focal arteriolar constriction -Cotton-wool spots. -Disc edema -Retina edema -Macular star -Choroidal infarction. Four phases can be identified in hypertensive disease. The first, the vasoconstrictive phase, shows hypertension stimulating constriction of the vasculature by a myogenic autoregulatory mechanism. The sclerotic phase of hypertension produces changes to the walls of the affected vessels. During the exudative phase, damage to vascular smooth

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muscle and endothelium results in breakdown of the blood-retina barrier. Flamed-shaped hemes, dot-blot hemes, hard exudates, and nerve fiber layer infarctions (cotton-wool spots) are present. Lastly, complications from sclerosis produce venous occlusion, artery occlusion, ischemic syndrome, and epiretinal membranes. Ischemic syndrome may produce neovascular glaucoma and vitreous hemorrhage. Optic nerve head changes such as disc edema may be noted in malignant hypertension. Arteriosclerotic changes: Broadened light reflex with little or no A-V compression Salus sign bending of veins at A-V crossing Copper-wire arteriole appearance Gunns sign-venous compression which appears as tapering or deflections Bonnets sign A-V crossing with dilation (distal to the crossing) and banking of the vein Silver-wire changes to the arterioles and severe A-V crossing changes. Sclerosis of the vessels result in progressive changes to the appearance of the light reflex and thickening of the wall which reduces the size of the lumen. In addition, normal acute angle crossing patterns become more obtuse or even 90 degrees. Choroidal involvement may also be seen in hypertensive disease. Patients who develop accelerated hypertension, pre-eclamptic and eclampti changes in pregnancy, renal disease, and connective tissue disease may develop hypertensive choroidopathy. Fibroid necrosis of choroidal vasculature can cause focal non-perfusion. This may best be demonstrated with fluorescein angiography. Overlying RPE above the choroidal infarcts appear yellow and leak fluorescein. These are called Elschnig spots. Healing will result with hyperpigmentation of these areas. Linear areas of hyperpigmentation called Siegrists streaks may be seen overlying choroidal arteries. Localized exudative bullous detachments can also occur. TREATMENT: Control the hypertension. Follow the patient every 1-3 months based on findings. Consider retinal consult if macular edema, disc edema or other hypoxic complications are present. RETINAL ARTERY OCCLUSION CENTRAL RETINAL ARTERY OCCLUSION Occlusion of the central retinal artery after it branches from the ophthalmic artery. Occurs more commonly in men vs. women of about 65 yrs. of age Causes:

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Carotid artery occlusive disease, cardiovascular disease, collagen vascular disease, coagulopathies, GCA. Systemic hypertension and diabetes are often found in those who have had a CRVO. CLINICAL PICTURE: Symptoms: Unilateral, sudden, painless loss of vision, may have history of amarosis fugax Signs: *Vision of hand motion to counting fingers or light perception *milky, white edematous appearance of the retina in the posterior pole *Cherry red spot in center of macula *marked APD *Narrowed retinal arterioles *boxcarring/segmentation of the blood column of the arterioles *occasionally, cilioretinal artery sparing of the foveola Giant cell arteritis should be ruled out in those with CRAO who are older than 60 yrs. old. ETIOLOGY: *embolus (carotid or cardiac) *thrombosis *Giant Cell Arteritis (GCA) *collagen vascular disease -Systemic Lupus Erythematous -Polyarteritis Nodosa *hypercoagulation disorders -oral contraceptives -polycythemia *rare causes -migraine -Bechets disease -syphilis -sickle-cell disease *trauma TREATMENT/MANAGEMENT (IMMEDIATE, IF CRAO < 24 HRS. OLD): Massage of the globe, inhalation of a combination of carbon dioxide and oxygen, paracentesis of the anterior chamber and IV or oral acetazolamide. All management methods have minimal effects. Treatment should begin as soon as possible following CRAO. The upper time limit for treatment has yet to be determined.

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A general systemic exam needs to be done, especially with reference to cardiac, aortic, and carotid disease *stat ESR and C reactive protein to R/O GCA *check BP *order blood tests -fasting blood sugar or 3 hour glucose tolerance test -CBC with differential and platelets -lipid profile -PT/PTT -ANA -RF -FTA-Abs -serum protein electrophoresis and hemoglobin electrophoresis *carotid duplex -doppler -ultrasound *cardiac studies -echocardiogram *FA and/or electroretinogram Follow-up: *check for neovascularization in 3-6 weeks at first then every 3 months BRACH CENTRAL RETINAL ARTERY OCCLUSION Similar to CRAO but only affects a sector of the retina. CLINICAL PICTURE: Similar to CRAO but less severe. MANAGEMENT: No immediate therapy. Systemic work up similar to CRAO. RETINAL VENOUS OCCLUSION BRANCH RETINAL VEIN OCCLUSION (BRVO) Most common vascular occlusion. A blockage of a sectoral retinal vein. The following are considered as risk factors for vein occlusions: systemic hypertension, cardiovascular disease, male sex, increased body mass index at 20 yrs of age, higher serum levels of alpha-2globulin and history of primary open angle glaucoma.

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Most commonly occurs in the seventh decade (60s) of life. SIGNS: Sector of retina with dilated and tortuous venules with associated superficial and deep retinal hemorrhages, retinal edema (usually macular), cotton wool spots, and lipid exudates along a single retinal arcade. Sectoral disc swelling may be present. Iris neovascularization rarely occurs. The area of capillary nonperfusion which, is determined by a fluorescein angiogram determines whether BRVO is perfused (nonischemic) vs. nonperfused (ischemic). Preretinal and optic disc neovascularization along with vitreous hemorrhage are sequelae, which are more common in nonperfused BRVOs. Collateral vessels must be differentiated from neovascular vessels. Macular edema more common in the patient who develops a BRVO. MANAGEMENT: Controversial since the natural course of the disease is usually promising. Anticoagulants, fibrinolytic agents, and antithrombotic agents have been used without proven efficacy. Laser photocoagulation remains the mainstay for treatable retinopathy (neovascularization or macular edema with visual acuity of 20/40 or worse). CENTRAL RETINAL VEIN OCCLUSION (CRVO) Blockage of the central retinal vein. Peak prevalence in people older than 50. Also occurs in younger individuals. Causes are similar to those of BRVO and include: ocular hypertension or glaucoma, arteriosclerosis, systemic hypertension, optic disc edema, optic disc drusen, hypercoagulation diseases such as leukemia, vasculitis such as from sarcoid, drugs, abnormal platelet function, retrobulbar external compression and migraine. CLINICAL PICTURE: Usually, a unilateral painless loss of vision. Superficial and deep retinal hemorrhages from optic nerve to peripheral retina which are present in all four quadrants,

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dilated/tortuous venules, macular edema, optic nerve head swelling, lipid exudation and cotton wool spots. Blood and thunder. The area of capillary nonperfusion which, is determined by a fluorescein angiogram determines whether the CRVO is perfused (nonischemic) vs. nonperfused (ischemic). Typically nonischemic CRVOs have visual acuity better than 20/200 with no APD while those with ischemic CRVOS have visual acuity worse than 20/200 with an APD. A common sequela is rubeosis irides that can occur along with neovascular glaucoma. The risk for development is based on the amount of capillary nonperfusion. Neovascularization can develop on the disc or elsewhere on the retina which must be differentiated from collateral vessels. MANAGEMENT: Nonischemic CRVO should be followed every month for 6 months to detect the progression into ischemic CRVO. Ischemic CRVO should be followed every 2-3 weeks for the development of neovascularization. Gonioscopy at each visit. Panretinal photocoagulation remains the treatment of choice for neovascularization of the iris or anterior chamber angle and severe ischemic retina. Use of anticoagulants like coumarin, heparin, and warfarin are controversial. Consider treatment of IOP in fellow eye if elevated. Studies to review for diabetic retinopathy and venous occlusive disease: (a) ETDRS Early Treatment Diabetic Retinopathy Study (b) CRVO Study Group (c) BRVO Study Group RETINOPATHY OF PREMATURITY (ROP) RISK FACTORS: * * * * prematurity (<30 weeks gestation) low birth weight (<1250 to 1750 grams) supplemental oxygen other possible factors

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-vitamin E deficiency, cyanosis, apnea, mechanical ventilation, intraventricular hemorrhages, seizures, exchange transfusions, septicemia, in utero hypoxia, and anemia CLINICAL PICTURE LOCATION OF THE INVOLVED RETINA ZONE I: imaginary circle which is centered around the disc and whose radius is twice the distance from the disc to the center of the macula ZONE: II: from ZONE I to the nasal ora serrata and the temporal equator ZONE III: the remaining temporal periphery EXTENT OF ROP Specified by the number of clock hours involved STAGES OF RETINAL CHANGES STAGE 1: demarcation line -separates the anterior, avascular retina from the posterior, vascularized retina more prominent in temporal retina STAGE 2: demarcation line develops into ridge. Neovascular tufts may be seen posterior to it. STAGE 3: ridge with extraretinal fibrovascular proliferation; Ridge turns pink; Retinal vessels may be dilated and tortuous; retinal and vitreal hemorrhage may occur. STAGE 4a: retinal detachment, extrafoveal STAGE 4b: subtotal RD involving macula STAGE 5: total RD Plus disease: dilation of veins and tortuosity of the arterioles; a plus sign is added to the stage number. May also find: * bilaterally, decreased visual acuity * dragged disc * leukocoria * nystagmus DIFFERENTIAL DIAGNOSIS: * familial exudative vitreoretinopathy * retinoblastoma * persistent hyperplastic vitreous

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* *

congenital cataracts X-linked retinoschisis

WORK-UP: * protocol for examination -infants < or = 1500 grams at the age of 6 weeks -dilated retinal examination with scleral depression (taking care to recognize stimulation of oculocardiac reflex). -dilate with cyclopentolate with or without phenylephrine.

TREATMENT: *STAGE 1 and 2: STAGE 2+ or 3: STAGE 4 and 5: no treatment required consider cyrotherapy surgical repair of retinal detachment with buckle, vitrectomy or both

FOLLOW-UP: * reexamine every 2 wks until age 14 wks, then every 1-2 months initially and later every 6-12 months * monitor for development of glaucoma, cataracts, and amblyopia MACULAR DISORDERS AGE-RELATED MACULAR DEGENERATION (AMD) The leading cause of blindness in patients 65 years old and over. There are 2 types of AMD: nonexudative (dry form) and exudative (wet form). Affects mainly patients >50 years old. RISK FACTORS Dietary deficiencies Exposure to UV light Family history of ARMD Increasing age Light colored irides Smoking Hypertension Caucasian Increased body mass index NONEXUDATIVE (DRY FORM) SYMPTOMS:

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visual symptomology ranges from being completely asymptomatic to having a reduction in visual acuity and a loss of central vision.

SIGNS: * macular drusen (bilateral) * pigment clumping * focal areas of RPE atrophy * absence of a foveal reflex * Amsler grid defect Atrophy of RPE can progress to atrophy in the outer retinal layers and choriocapillaris leading to Geographic Atrophy. This form of dry AMD can cause significant vision loss. DIFFERENTIAL DIAGNOSIS: autosomal dominant drusen Basal laminar drusen Pattern dystrophy Idiopathic central serous chorioretinopathy TREATMENT: * no proven treatment modality known to date * prophylactic antioxidants and zinc vitamin supplementation possibly prevents further vision deterioration especially with intermediate or advanced AMD * include green leafy vegetables in diet * low vision aids and devices FOLLOW-UP: * patient to perform monocular home Amsler grid daily * reexamine every 6 months, if not sooner EXUDATIVE (WET FORM) SYMPTOMS: * rapid, dense vision loss * distortion/metamorphopsia * central or paracentral scotoma SIGNS: * choroidal (subretinal) neovascular membrane (CNVM) * hemorrhagic sensory or RPE detachment * subretinal hemorrhages, exudates, pigment ring * exudative retinal detachment * disciform scar * vitreous hemorrhage

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DIFFERENTIAL DIAGNOSIS: * ocular histoplasmosis syndrome * angioid streaks * traumatic choroidal rupture * drusen of the optic nerve * myopic degeneration MANAGEMENT: * Home amsler grid; Monitor dependant on prognosis * Perform a fluorescein angiogram or ICG angiography if suspect choroidal (subretinal) neovascular membrane and treatment possible TREATMENT: MPS ( Macular Photocoagulation Study) showed that argon laser photocoagulation applied to classic well defined extrafoveal (>200 microns from the center of the foveal avascular zone (FAZ) ) CNVM was more effective than no treatment in preventing vision loss. MPS showed that argon laser photocoagulation applied to classic well defined juxtafoveal (1 to 200 microns from the center of the FAZ) CNVM was somewhat more effective than no treatment in preventing vision loss. High rate of reoccurrence of CNVM after treatment Patients with CNVM < 2DD have a better prognosis than > 2DD Patients with CNVM <2 DD and VA less than 20/160 may have a greater initial drop in VA than if no treatment however may have better VA over long term than if no treatment Poor candidate for laser treatment is CNVM > 2 DD and VA 20/160 or better. CNVM in 1 eye chance of occurrence if fellow eye 6% / year. Photodynamic therapy (PDT) with verteporin can reduce chance of vision loss ( versus no treatment) and cessation of fluorescein leakage when applied to subfoveal CNVM which are >50% classic. Macular translocation ? studies in progress. FOLLOW-UP: If treatable lesion: * monocular home Amsler grid daily * reexamine in 2 weeks, 6 weeks, 3 months, and then every 6 months post-treatment * repeat fluorescein angiography as needed * consider further treatment if necessary

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New Therapeutic Therapy for Wet AMD (1) Phototherapeutic Therapy (PDT) (2) Vascular Endothelial Growth Factor Inhibitors ( VEGF) (a) Macugen ( Pegaptanib sodium): FDA approved; approved for treatment of all forms and sizes of wet AMD; typically dosed via intravitreal injection every six weeks up to a year; clinical studies showed preservation of vision in most patients relative to sham therapy, with results varying considerably. (b) Lucentis ( ranibizumab) anti-VEGF intravitreal injection dosed every 4 weeks; pending FDA approval (3) Retaane ( anecortave acetate) corticosteroid that blocks the angiogenic pathway. Administered as a juxtascleral depot injection every six months; pending FDA approval AREDS: Age Related Eye Disease Study Designed to evaluate the natural history and risk factors of AMD and cataract Evaluate the effect of high doses of antioxidants and zinc on the progression of AMD and cataract Results from the AREDS showed that high levels of antioxidants and zinc significantly reduce the risk of advanced age-related macular degeneration (AMD) and its associated vision loss. These same nutrients had no significant effect on the development or progression of cataract. Note: Many different OTC formulas available. Important to recommend lutein formulation for patients who smoke due to increased chance for lung CA with betacarotene formulation. PreserVision-AREDS formula; beta-carotene: non-smokers PreserVision-Lutein formula: substitute for smokers ICAPS-AREDS formula Ocuvite

CYSTOID MACULAR EDEMA Occurs secondary to surgical procedures such as cataract extraction (Irvine-Gass Syndrome), retinal vascular disease such as BRVO and diabetic retinopathy, retinal degeneration such as retinitis pigmentosa, intraocular inflammation such as iridocyclitis and retinitis or choroiditis, drug incuded such as from the use of epinephrine, propine, betololol, or Tamoxifen and other causes such as ocular tumors and epiretinal membranes.

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CLINICAL PICTURE: The appearance of cystic spaces in the foveal area, retinal thickening and loss of foveal reflex. Metamorphopsia and decreased vision may be present. Fluorescein angiography can aid in the detection of CME which is sometimes difficult to detect ophthalmoscopically. Most recently, OCT has been employed in practice as a great adjunctive tool in evaluation CME as well as diabetic macular edema. MANAGEMENT: Topical anti-inflammatory agents such as steroids and NSAIDS. Systemic carbonic anhydrase inhibitors Steroidal injections Laser photocoagulation Vitrectomy EPIRETINAL MEMBRANE Many names have been used to describe this including surface wrinking retinopathy, cellophane retinopathy, and macular pucker. The diagnosis of epiretinal membrane proliferation usually occurs in patients over the age of 50 and presents bilaterally in 10-20% of cases. Etiology: idiopathic, PVD, following retinal or intraocular surgery, trauma, uveitis, and retinal vascula disease. SYMPTOMS: Patients be asymptomatic or complain of blurred vision and metamorphopsia. SIGNS: Initially, the membrane is thin and transparent and does not cause distortion. It can be seen by detection of an abnormal reflex or glistening sheen. The membrane may stop growth or continue to contract and cause wrinkling of the inner retinal layers, which appear as radial folds. Thick membranes may be gray or white and can have associated pigmentation. Also look for "pseudo-macular hole", straightening of vessels, edema, and detachments in the macular area. After contracting, most membranes remain stable. Some may spontaneously separate from the retina. Fluorescein angiography or OCT can be used as an adjunct to help with the diagnosis.

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MANAGEMENT: Mild Cases: Monitor and home amsler grid. Moderate to Severe Cases: 20/60 or worse or metamorphopsia. *Vitreous surgery in which the epiretinal membrane is dissected from the retina. Usually performed <1 year from first symptoms (easier removal). *Acuity improvement seen in most cases. Treat underlying retinal vascular disorder, if indicated. Treat CME ( SEE CME Section) IDIOPATHIC CENTRAL SEROUS CHOROIDOPATHY (ICSC) Characterized by the development of a serous, sensory retinal detachment. Occurs due to focal leakage from a defect in the retinal pigment epithelium. Most common in males, age 30 -40, with stressful living conditions. CLINICAL PICTURE: Blurry vision, micropsia, metamorphopsia, and color distortions. May have delayed photostress recovery time. Smooth dome shaped elevation in the macular area. Evaluate the nerve head for possibility of optic pit. Fluorescein angiography will reveal characteristic smokestack or ink blot appearance. Young patients rarely will develop subretinal neovascularization with ICSC. MANAGEMENT: Monitor; Spontaneous resolution occurs after a few months. Nonresolving cases can be treated with laser photocoagulation. INFLAMMATORY MACULAR DISORDERS OCULAR TOXOCARA CANIS Toxocara is a parasite usually transferred to humans through canine feces. Children and young adults are most often infected. Occurs in visceral, ocular, and oculovisceral forms. ELISA is used to diagnose the condition and either blood or aqueous can be used. CLINICAL PICTURE: Typically unilateral Chronic endophthalmitis Leukocoria, unilateral vision loss

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Posterior pole granuloma Peripheral granuloma Retinal lesions (granulomas) are round, elevated, whitish lesions about 1.5 mm in size. Fibrotic tissue (bands) may migrate from the lesion to the disc. Anterior uveitis and vitiritis are usually not present. MANAGEMENT: Periocular and systemic steroids are used to treat the ocular condition. Oral antihelminthic, thiabendazole and prednisone are also used to treat inflammation. Victrectomy can be done to release traction bands. OCULAR TOXOPLASMOSIS Toxoplasma gondii is an obligate intracellular protozoan, which is one of the leading causes of posterior uveitis. Usually congenital with prevalence of 0.01% of live births. Reoccurs most frequently between age 10 and 35. The indirect fluorescent antibody and enzyme immunoassay test are used to confirm the diagnosis. Acquired toxo is on the upswing, most noticeably in AIDs patients. CLINICAL PICTURE: Active form: Yellow-white patches of retinochoroiditis with associated vitritis (headlights in a fog), anterior uveitis from hypersensitivity, and occasional papillitis. Sometimes subretinal neovascularization can occur. Reassurances present as sattelite lesions adjacent to areas of previous scarring. Inactive form: Chorioretinal scars which may be bilateral. May have fibrosis in the vitreous as a result of the previous vitritis. MANAGEMENT: Active form: The chorioretinitis is usually treated if the lesion involves the macula or papillomacular nerve bundles due to potential acuity loss, near or on the optic nerve because of potential vision or field loss, or if the inflammation can cause significant vitreoretinal traction with possible sequelae including retinal detachment. Immunocompromised patients are typically treated irrespective of location of lesion or severity of the condition. Treatment may include: Pyrimethamine Sulfonamides Clindamycin

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Systemic steroids Clindamycin Co-trimoxazole Azithromycin Atovaquone The common anterior chamber reaction needs to be controlled with topical corticosteroids and cycloplegics. ONCHOCERCIASIS = RIVER BLINDNESS SEE UVEITIS / CAUSES OF ENDOGENOUS UVEITIS

PRESUMED OCULAR HISTOPLASMOSIS SYNDROME Thought to be caused by prior infection of the fungus, Histoplasma capsulatum. The disease is typically found among Caucasians who have resided along the Ohio/Mississippi River Valley. Chest radiography and the histoplasmin skin test can be used to diagnose the primary disease of histoplasmosis. CLINICAL PICTURE: Typically the patient is asymptomatic. Classic triad: peripapillary atrophy, maculopathy and atrophic peripheral chorioretinal scars. Maculopathy can progress to subretinal neovascularization (CNVM) which can lead to disciform macular lesions. MANAGEMENT: Monitor for development of a CNVM. Fluorescein angiography if CNVM identified or suspected. If a well defined CNVM in the macula is present, laser photocoagulation or surgical removal can be performed. Patients with peripapillary and extrafoveal (nasal to the fovea) CNVM can benefit from laser treatment. Macular translocation for submacular CNVM MACULAR HOLE ETIOLOGY: * * * tangential traction on the macula trauma cystoid macular edema

SYMPTOMS: * decreased vision (20/200) for full thickness hole * distortion/metamorphopsia * predilection for middle-aged to elderly women

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SIGNS: Stage 1A: yellow deposit, loss of foveal reflex and foveolar depression. Stage 1B: yellow ring, prefoveal vitreous still attached Stage 2: Separation of the pseudo operculum from the hole with associated separation of the vitreous. Stage 3: Full thickness retinal hole; + Watzkes sign, yellow-white opacities in the hole. Stage 4: Full thickness retinal hole with PVD. DIFFERENTIAL DIAGNOSIS: Drusen, macular pucker, solar retinopathy, ICSC, CME, pseudohole, geographic RPE atrophy. TREATMENT: *Vitrectomy (TPPV) with internal limiting membrane peel and gas tamponade with face down positioning. MANAGEMENT: * daily Amsler grid * examination every 6 months * warn patients of risk of RD in high myopes and traumatic macular holes. * small risk in the development of a macular hole in the fellow eye CYTOMEGALOVIRUS RETINITIS (CMVR) CMV is an opportunist organism that typically infects immunocompromised hosts such as AIDS or organ transplant recipients. Occurs usually after AIDs Dx and when CD4 count < 50. CLINICAL PICTURE: Symptoms vary dependant on the part of the retina affected. Ophthalmoscopically white granular patches with irregular, feathered borders and retinal hemorrhages. Begins as a focal area of necrosis(similar to cotton wool spots) and the progresses. Other signs include: vitreous opacities, perivascular sheathing affecting veins and arteries, hemorrhage and ischemic areas of retina. Can lead to retinal detachment. TREATMENT: (slows it, does not kill it.) 1) Ganciclovir IV, oral and intraocular implant device.

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2) Foscarnet 3) Cidofovir 4) Fomiversen Repair of retinal detachment. INHERITED RETINAL-CHOROIDAL DYSTRPOHIES CONE DYSTROPHIES Cone dystrophies encompass a number of cone dysfunction syndromes which are inherited primarily in an autosomal dominant pattern but autosomal recessive and X-linked forms exist. Patients with progressive cone dystrophy have acuity loss in childhood to early adulthood. SYMPTOMS: Photophobia, bilateral slowly progressing decreased vision (to the level of 20/200), color vision, difficulty, patients report best vision at dusk. Vision is worse in the day than at night (opposite RP). SIGNS: 1. Four types of macular lesions seen: a. Pigment stipling and diffuse pigment clumping b. progressing to Bull's eye appearance (most common) c. Atrophy of the choroidal vessels and the choriocapillaris. d. Stargardt's-like lesions 2. Optic atrophy - first seen as temporal pallor but can acquire a waxy appearance. Central scotoma on visual field testing 4. Electrodiagnostics ERG - Single flash response is decreased or extinguished. Flicker response is also depressed b. Dark adaption - abnormalities in the cone segment of the curve. c. EOG - Affected in severe cases. *Early on in the disease, the fundus may have a normal appearance but the acuity is decreased. TREATMENT: Genetic counseling/Pedigree analysis Low Vision rehabilitation RETINITIS PIGMENTOSA (RP) Retinitis Pigmentosa refers to a collection of disorders characterized by progressive retinal pigment epithelium and photoreceptor dysfunction. RP is hereditary in nature and

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can be transmitted autosomal recessive, autosomal dominant, and X-linked modes. However, often there is no family history of RP present. Mean age of onset is the 9-19 years. SIGNS & SYMPTOMS 1. 2. Nyctalopia or night blindness Visual Field Loss: Progressive field loss begins as relative scotomas in the midperiphery which increase in size and coalesce to form ring-shaped scotomas. Field loss expands into the periphery, sparing islands of depressed retinal sensitivity. Vision loss is usually symmetrical. Central Vision Loss: May depend on associated pathology such as cystoid macular edema. Sector RP usually retains good acuity, whereas X-linked patients may have acuity, whereas X-linked patients may have acuity of 20/200 or worse by the third or fourth decade. Autosomal dominant and recessive forms may drop to 20/200. Color Vision Loss: Remains good until central acuity falls below 20/40. Retina: Early signs of RP are vessel attenuation and granular mottling of the midperipheral and peripheral RPE. Pigment from the degenerating RPE migrates into the retina and coalesces around the vessels in "bone-spicule" formation. In severe forms, the vessels may appear as threads. Classically the optic nerve will obtain a waxy, pallor appearance. Cystoid macular edema may occur in 10% of RP patients. Vitreous: Fine pigment dusting, PVD, cotton-like opacities, and asteroid hyalosis Photopic ERG extinguished

3.

4. 5.

6. 7.

Differential Diagnosis: Pseudo RP 1. Syphilis - salt and pepper fundus, asymmetric fields. 2. Congenital rubella - Normal ERG, microphthalmos 3. Phenothiazine toxicity 4. Pigmented paravenous retinochoroidal atrophy 5. RPE changes following exudative detachment or trauma. 6. Congenital stationary night blindness - normal fields, non-progressive. 7. CMV, toxoplasmosis, herpes infections: May produce pigment mottling in some cases. 8. Cancer-associated retinopathy MANAGEMENT: Low Vision rehabilitation: field expanders, tinted lenses, mobility training. Genetic counseling, examination of family members. Cystoid macular edema may be treated with macular grid laser photocoagulation. MALIGNANCIES CHOROIDAL MELANOMA

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Choroidal melanomas are rare but are the most common intraocular tumor in adults. Uveal melanoma tends to be diagnosed in the fifties and sixties and is predominantly found in Caucasians. Some of the literature demonstrates hereditary risk factors in the development of uveal melanoma. Other risk factors, which have been noted, are lighter irides, lighter hair color, sunlight exposure, and occupational and chemical exposure. SIGNS: Lesions appear as a well pigmented and defined nodular mass with overlying serous fluid or yellow pigment. They may appear grey-green or brown, and yellowish if amelanotic. Also noted may be orange lipofuscin pigment granules overlying the tumor. Choroidal melanoma can appear as a mushroom-shaped tumor with an associated exudative detachment. Other associated signs include a large amount of subretinal fluid, elevated greater than 2 mm, rubeosis irides, vitreous pigment or hemorrhage, lens subluxation, or choroidal neovascular membrane. Most tumors are greater than 10 mm in size. DIAGNOSTIC TESTING: 1. Fluorescein angiography - intrinsic circulation, leakage, and multiple subretinal leaks which enlarge in the late staining phase. A & B Scan Ultrasonography - medium low reflectivity with A scan and orbital Shadowing, choroidal excavation, and acoustically empty space within the tumor With B-scan. 3. CT or MRI - may help differentiate detachments from solid tumors or orbital extension. 4. Radioactive phosphorus uptake test (P-32) - 99% accurate with large tumors but difficulty with small melanomas and other ocular tumors. Test is diagnostic when the decay count over the tumor is twice that of uninvolved choroid. 5. Fine needle aspiration biopsy - May be performed transvitreal or transscleral. MANAGEMENT: Management: Depends on the size, secondary metastases, etc a. Enucleation - time honored treatment b. Irradiation therapy - plaques or external beam are methods of delivery. c. Chemotherapy d. Photocoagulation - may be useful for small tumors. e. Cryotherapy Prognosis: a. Peak incidence of metastases occurs 1 year post-enucleation. b. The liver is usually the first site - survival time is several months. Other sites include the lungs and brain. c. Half of those who develop metastases do so in the first 3 years following enucleation: d. Other factors which affect metastases following enucleation: *Cell type, extrascleral extension, tumor volume, and location. Full medical work-up by an internist. a. Blood work, carcinoembryogenic antigen

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b. Liver function tests Chest X-ray Brain scan RETINOBLASTOMA Retinoblastoma is a malignant tumor, which is almost exclusively diagnosed in young children. It is the most common intraocular malignancy in children. The tumor can occur sporadically or be inherited through an autosomal dominant pattern. Inherited tumors are typically bilateral. SIGNS: The presence of a white pupil or leukocoria is the sign often associated with retinoblastoma. Other presenting manifestations include strabismus and a red painful eye. Other findings may include poor vision, glaucoma, pseudo-orbital cellulitis, hypopyon, iris lesions, indolent endophthalmitis, granulomatous uveitis, iris neovascularization, and retinal detachment. Early on in the course of the tumor, small yellow-white, discrete, smooth shaped nodules are seen in the retina. It appears as a dull, elevated, chalky lesion as it grows larger. Calcium content in the tumor makes it highly reflective with ultrasonography. Retinal feeder vessels may be apparent on larger tumors but may require fluorescein angiography to discern vascularization in smaller tumors. Factors which may determine prognosis are size, location, number, retinal detachment, and vitreous seeding. Lesions located behind the equator, less than 10 DD in size, without retinal detachment, and lack vitreous seeding have better prognosis. In addition, early treatment is essential in reducing risk for metastatic disease. MANAGEMENT: Diagnostic work-up a. Diagnostic imaging *CT scan, ultrasonography, MRI is least helpful b. Fluorescein angiography c. Determination of possible metastasis *Bone marrow aspiration *Lumbar tap and cerebrospinal fluid analysis *Fine needle biopsy *Bone scans Management a. Enucleation - when vision is not salvageable and there is no view of the optic nerve head. Must remove a large portion of the optic nerve to prevent metastasis. b. Radiotherapy - most common form of therapy is cases where attempts to salvage the eye are made. c. Radioactive plaques - used if vitreous seeding is present. d. Cryotherapy - small tumors anterior to the equator confined to the sensory retina.

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e. f. Follow-up: a. b.

Photocoagulation - small posterior retinoblastomas confined to the sensory retina. Chemotherapy Most metastatic disease arises in the first 18 months following treatment. Work-up for secondary tumors should take place at least every 6 months with a yearly bone scan. Prognosis: *Metastatic tumors - Poor prognosis. Life expectancy is 6-12 months following survival of 5.8 months. *Overall survival rates are reported to be quite high following treatment 86-93% but probably skewed by specialty oncology centers.

VITREAL AND PERIPHERAL RETINAL FINDINGS Those requiring more than routine monitoring: ACQUIRED RETINOSCHISIS A bilateral condition (70% of the cases) of the retina in which there is a split in the sensory retina at the level of the outer plexiform layer and the inner nuclear layer. Its prevalence in the population is 4% and is 7% in those over 40 years old. There is no sex predilection. Retinoschisis is most often seen in the inferior temporal quadrant and can extend past the equator. Field testing demonstrates absolute defects if the lesion extends posterior to the equator. The lesions are asymptomatic and may progress to retinal detachment. SIGNS: Acquired retinoschisis is separated into two subgroups: Flat or typical retinoschisis is believed to be a form of cystoid degeneration. It is typically located anterior to the equator and does not possess retinal holes. Its inner layer is thin and smooth resembling beaten metal. Bullous retinoschisis is characterized by a thin, transparent elevation of the retina. The tissue is taut and does not quiver like a detachment. In 70% of cases white flakes are deposited on the surface. Cystoid degeneration is observed from the edge of the lesion out to the ora serrata. Choroidal detail under the lesions are not as clear as under the surrounding retina. The lesion has a honeycomb appearance to its surface. Inner and outer layer breaks may be present. Inner layer breaks appear as oval and clear spots where as outer layer breaks are viewed as small pink holes with rolled edges. One quarter of patients with schisis will have breaks in one layer and 40% in both the inner and outer layers. Sixty percent have liquified vitreous associated with the lesion. Most cases do not progress, however, outer layer breaks may lead to rhegmetogenous retinal detachment. MANAGEMENT: Retinoschisis without breaks or with inner layer break anterior to the equator: *Document size and location. Educate patient on symptoms of retinal

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detachment. Return every 6 12 months for dilated fundus examinations. Monitor for progression. Retinoschisis which has outer layer breaks: *Document size and location. Educate patient on symptoms of retinal detachment. Return every 6 months for dilated fundus examinations. Monitor for progression. Retinoschisis which has inner and outer layer breaks: *Refer to a retinal specialist for consultation. *Risk for rhegmetogenous detachment is variable. Progressing retinoschisis threatening the vision.. *Refer to a retinal specialist for consultation. *Little risk for vision loss but prophylactic photocoagulation may flatten the schisis. Retinoschisis with progressive retinal detachment: *Immediate referral to retinal specialist. *May require scleral buckle, cryopexy, or photocoagulation. ATROPHIC RETINAL HOLES Atrophic holes are round full-thickness breaks in the peripheral retina which may be observed in a variety of conditions such as snail-track, cystoid, and lattice degeneration. It is the most common form of retinal break (70%). SIGNS: As its name implies, atrophic holes are resultant of progressive retinal thinning. These lesions may range in size from pinpoint to 2 DD and occur in 2-3% of the population. These may appear red in color due to the RPE and choroid becoming more apparent secondary to the absence of overlying retina. Cuffs of subretinal fluid may surround the lesion and put the patient at risk for a sub-sensory retinal detachment (between the sensory retina and RPE). Pigment may be present in lesions present for over one month. Vitreoretinal traction may also be observed. Approximately 7% develop progressive retinal detachment. MANAGEMENT: Isolated asymptomatic atrophic holes: *Document location and size, Scleral depression. Educate patient on symptoms of retinal detachment and to immediately return if these should happen. Return for annual dilated fundus examination. b. Isolated asymptomatic atrophic holes with a cuff of sub-retinal fluid or edema less than 1 dd in size: *Document location and size. Scleral depression. Educate patient on symptoms of retinal detachment and to immediately return if these should happen. Return at 6 month intervals for dilated fundus examination.

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c. d. e.

Isolated asymptomatic atrophic holes with a cuff of sub-retinal fluid or edema greater than 1 dd in size: *Retinal consult Symptomatic atrophic holes: *Retinal consult Isolated atrophic holes with strong vitreoretinal adhesions or flap tears: *Retinal consult

CHORIODAL PIGMENTATION BENIGN CHOROIDAL NEVUS SIGNS: Generally appear as slate grey lesions which may be flat or slightly elevated with defined margins (not sharply demarcated). They range in size from 0.3 DD to 7 DD, are usually ovoid, and are less than 2 mm in thickness. Pigmentation may vary from amelanotic types to heavily pigmented. The overlying tissue may undergo changes thus altering the appearance. These include drusen, coalesced drusen, and lipofuscin pigment. Most nevi are smaller than two DD. Because of there location deep in the retina, nevi disappear when viewed with a red free filter. Choroidal nevus have the potential to turn into a melanoma. Differential Diagnosis: 1. Choroidal freckles - areas of increased choroidal pigmentation with irregular borders. Not composed of atypical cells. 2. Subretinal hemorrhages 3. RPE hypertrophy 4. RPE hyperplasia 5. Small choroidal melanoma Highly suspicious lesions: Thickness greater than 2mm Size greater than 5 mm in diameter. Orange pigment (lipofuscin) overlying the lesion. Documented growth of a nevus. Fluorescein angiography demonstrates multiple pinpoint sub-retinal leaks which enlarge in the late staining phase. MANAGEMENT: Ancillary testing (See choroidal melanoma) *A & B-scan Ultrasonography *Photography *Fluorescein angiography Management:

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a. b. c.

0.5-2 DD in size: Benign Photograph and follow in at least one year. 2-5 DD in size: Suspicious - May be slightly elevated, have overlying drusen, subretinal fluid. Consider ancillary testing. Photograph and return in 6 months 5 DD or larger. Work up as malignant melanoma.

LATTICE DEGENERATION Lattice degeneration is the thinning of the retina due to loss of the inner layers down to the outer nuclear layer. Most common of all hereditary vitreoretinal degenerations and appears in 6-8% of the general population. It presents bilaterally in 50% of cases. There is no racial or sexual predilection. It is more common among eyes with long axial length and first appears in young patients. SIGNS: The lesions may take the form of varied shapes and colors. Pigmentation (RPE hyperplasia) is observed in 82% of lattice lesions. Also noted in high frequency are whitish-yellow flecks and white lines. Classic white lines or fishboning within the lesions represent altered or sclerosed blood vessels. Lesions are usually found between 11 and 1 o'clock and 5 and 7 o'clock. Variation in the size of the lesions is great. Reports have noted lesions from one-fifth to twelve DD in size. Usually they are 1-4 DD in size and 0.5 -2 dd in width. Lattice is usually situated around pars plana and is circumferentially oriented. Sometimes the lesions may be seen radially oriented adjacent to retinal vessels. The borders of the lesions have exaggerated vitreoretinal adhesions and overlying the lesions are pockets of liquified vitreous. White without pressure may also occur along the borders. The border is an area of interest since it represents an area which is a potential retinal traction tear. Atrophic holes are signs of progressive retinal thinning. Approximately, 18-30% of lattice lesions have atrophic holes and 25% develop retinal breaks in or around the lesions. Look for signs of progression such as enlargement, presence of atrophic holes, vitreal traction, and vitreous degeneration. A small percentage of eyes go on to develop retinal detachments (0.3-0.5%). Those lesions with atrophic holes detach with a frequency of 3-14%. Approximately 20-30% of eyes with retinal detachment have lattice degeneration. MANAGEMENT: Lattice degeneration without holes or symptoms *Document size, appearance, and position of the lesion. Educate the Lattice degeneration with symptoms. patient on the signs and symptoms of retinal detachment and to immediately return if these should occur. Follow-up with annual dilated examinations. Scleral indentation when indicated.

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*Document size appearance, and position of the lesion. Scleral depression looking for holes and breaks. Educate the patient on the signs and symptoms of retinal detachment and to immediately return office if these should soccur. Dilated fundus examinations every 6 months. Lattice degeneration with asymptomatic atrophic holes. *In the absence of any risk factors: Document size, appearance, and position of the lesion. Scleral depression looking for holes and breaks. Educate the patient on the signs and symptoms of retinal detachment and immediately return if these should occur. Dilated fundus examinations every 6 12 months. Lattice degeneration with associated risk factors: *Retinology consult for prophylactic treatment Lattice degeneration with retinal breaks at the edges. *Retinology consult for prophylactic treatment Prophylactic treatment: *Performed on patients with high risk for RD *Retinal cryopexy *Laser photocoagulation OPERCULATED RETINAL HOLES SIGNS: As opposed to atrophic retinal holes, operculated holes result from anomalous vitreoretinal adhesion. As the vitreous pulls away from the retina, the anomalous point of adhesion tugs away removing a plug of retinal tissue. The free-floating piece of retina remains in the vitreous and atrophies. This piece is referred to as the operculum and usually appears smaller than the hole. Hemorrhaging may accompany this event. Drusen may be seen in the bottom of the hole. The holes are typically found in the peripheral retina from the equator to the ora serrata. The main concern is the seepage of liquified vitreous under the sensory retina causing a detachment. MANAGEMENT: a. Asymptomatic operculated hole: *Document size and location. Scleral indentation. Educate patient on the symptoms of RD and to return immediately if they should occur. Patient to return for annual dilated fundus examinations. b. Multiple asymptomatic operculated holes: *Document size and location. Scleral indentation. Educate patient on the symptoms of RD and to immediately if they should occur. Patient to return for dilated fundus examinations every 6 12 months. c. Retinal consultation needed when: *Surrounding edema cuff greater than 2 DD *Patient is symptomatic *High risk for RD: traction, tear, aphakia, history of detachment in the fellow eye.

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 POSTERIOR VITREOUS DETACHMENT (PVD) SYMPTOMS: * floaters (described as "cobwebs," "flies," or "spiders") * flashes of light SIGNS: * vitreous opacity -usually in the shape of a ring -suspended over the optic disc -moves with eye movements * vitreous hemorrhage * peripheral retinal and disc margin hemorrhage * pigmented cells in the anterior vitreous * retinal break or detachment MANAGEMENT: * no treatment necessary for PVD alone * if retinal break present or cryotherapy indicated to prevent development of retinal detachment FOLLOW-UP: -repeat dilated retinal examinations with scleral depression in 1 month, and 3 months, following initial development of symptoms if no breaks or hemorrhage present. -repeat dilated retinal examinations with scleral depression in 2-3 weeks, 2-3 months, then 6 months following initial development of symptoms no breaks, but mild vitreous and retinal hemorrhage present. -repeat dilated retinal examinations with scleral depression in 1-2 weeks, 4 weeks, 3 months, and 6 months after initial symptoms marked vitreous hemorrhage which obscures view of the retina -if no retinal view, perform B-scan (rule-out retinal detachment or tumor) RETINAL TEARS GIANT RETINAL TEARS Term reserved for retinal dialysis involving greater than 90 degrees. It is seen four times more frequently in males and is associated with high myopia (>8D). Causes: Idiopathic - 70%, Trauma - 20%, and chorioretinal degeneration - 10%. There is also associated considerable vitreous liquefaction. Traction may cause the retina to double

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back upon itself to form a "taco". Prognosis is poor with progression toward retinal detachment highly likely due to presence of vitreoretinal traction. MANAGEMENT: a. Immediate retinal consultation b. Retinologist may perform cryopexy or scleral buckle. *Must watch the fellow eye - higher risk for RD *Poor prognosis after surgery due to proliferative vitreo-retinopathy. HORSESHOE TEARS Similar to the mechanism of an operculated hole, strong vitreo-retinal adhesions are usually responsible for the formation of retinal tears. Areas of atrophied retinal tissue are more predisposed to tearing than healthy retinal tissue. The apex of the tear usually points toward the posterior pole and is attached to the vitreous. Hemorrhaging may be present if retinal vasculature is damaged in the process. Thirty percent chance of retinal detachment. Risk Factors: Myopia, persons over 40, patients with lattice, aphakia, traums. SYMPTOMS: Flashes, floaters MANAGEMENT: a. Immediate retinal consultation b. Symptomatic retinal tears and/or fresh full thickness tears should be prophylactically treated with photocoagulation or cryopexy. Breaks requiring prophylactic treatment: a. Symptomatic breaks b. Fresh horseshoe tears with vitreous traction c. High myopia d. Strong family history of retinal detachment or breaks in the follow eye. e. Patient on miotics f. Large or multiple breaks g. Aphakia h. Trauma induced i. Traction tears associated with lattice j. Subclinical detachment around the break k. Vit. Heme, uveitis, cataract RETINAL DIALYSIS Retinal dialysis is tearing of the peripheral retina near the border of the ora serrata. Most commonly it is associated with trauma. Bilaterial spontaneous cases have been reported in young patients and are found in the inferior temporal quadrants. The involved retinal tissue appears gray due to edema or atrophy. Vitreous hemorrhage

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may be present. Noted slow progression toward retinal detachment. May have multiple demarcation lines. This is reserved for cases in which less than 90 degrees are involved. Is usually asymptomatic. TREATMENT: Complete eye examination with indirect ophthalmoscopy. a. Scleral indentation - a must in cases following trauma. Management: a. Retinal consultation b. Retinologist may perform cryopexy or scleral buckle. VITREOUS HEMORRHAGE SYMPTOMS: * sudden, painless loss of vision * sudden appearance of a "shower" of black dots with flashing lights SIGNS: * red blood cells in the vitreous * red fundus reflex may be absent * poor to no fundus view * mild afferent pupillary defect ETIOLOGY * diabetic retinopathy * retinal break * retinal detachment * retinal vein occlusion * posterior vitreous detachment * age-related macular degeneration (ARMD) * sickle-cell disease * trauma * intraocular tumor * subarachnoid or subdural hemorrhage (Terson's syndrome) * Eale's disease * Coat's disease * retinopathy of prematurity * von Hippel-Lindau retinal angiomatosis * hypertension * radiation retinopathy DIFFERENTIAL DIAGNOSIS: * vitritis * retinal detachment MANAGEMENT:

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* *

B-scan ultrasonography (rule-out retinal detachment or intraocular tumor) bed rest with head elevated for 2-3 days -reduces rebleeds and hastens the blood to settle inferiorly * aspirin, nonsteroidal anti-inflammatory drugs, and other anti-coagulant agents are strictly contraindicated * cryotherapy or laser photocoagulation for retinal breaks * scleral buckling, cryotherapy, or pneumatic retinopexy for retinal detachments * laser photocoagulation for proliferative retinal vascular complications * vitrectomy indicated -in chronic cases -when vitreous hemorrhage is associated with a retinal detachment -when neovascularization of the iris is present -in hemolytic glaucoma FOLLOW-UP: * reexamine in 1-3 days * perform B-scan ultrasonography every 1-3 weeks to rule-out subsequent development of a retinal detachment.

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OPTIC NERVE DISEASE OPTIC NERVE EDEMA CONGENTAL OPTIC NERVE ANOMALIES LEBER'S OPTIC NEUROPATHY ETIOLOGY: *a visually debilitating optic atrophy that occurs as a sequel to acute or subacute optic disc edema *strong hereditary component -transmission by mitochondrial DNA -affects males.>>females (6.7:1) *age of onset -males 15 to 30 years of age -women 20 - 40 years of age SIGNS AND SYMPTOMS: * bilateral acute or subacute vision loss (20/200 to counting fingers) *mild disc edema with early circumpapillary telangiectatic microangiopathy *permanent optic atrophy usually affecting the temporal sector *central scotoma that can extend out to the periphery *cecocentral visual field defect *associated with headaches, vertigo and nervousness MANAGEMENT: *poor prognosis *no effective treatment *genetic counseling PSEUDOPAPILLEDEMA Disc Drusen Myelinated Retinal Nerve Fibers Tilted Disc Syndrome INTRAOCULAR INFLAMMATION/HYPOTANY Uveitis Blunt Trauma ISCHEMIC OPTIC NEURITIS ARTERITIC ISCHEMIC OPTIC NEUROPATHY (Giant Cell Arteritis) SYMPTOMS: *sudden, painless, nonprogressive visual loss *unilateral or bilateral *age > 50 years *associated headaches *jaw claudication (pain upon chewing) *scalp tenderness (pain upon hair combing)

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*muscle and joint aches *weight loss *fever SIGNS: *decreased VAs-counting fingers or worse *APD *disc pallor, disc edema, hemorrhage *altitudinal visual field defect, typically inferior *palpable and tender temporal artery *elevated ESR (men > .5 x age, women > .5 x (age + 10) *elevated C reactive protein *optic atrophy as edema resolves *temporal artery biopsy indicative of giant cell arteritis. *CRAO-may occur *cranial nerve palsy especially sixth nerve palsy) may occur DIFFERENTIAL DIAGNOSIS: *nonarteritic ischemic optic neuropathy -younger age group (40-60 yrs. old) -less severe visual loss but sudden onset -normal ESRs, C reactive protein -negative temporal artery biopsy -usual no symptoms of GCA Disc edema (sectoral or full) -positive APD -Altitudinal visual field defect. *inflammatory optic neuritis (papillitis) -younger age group -less severe and sudden onset of visual loss -pain with eye movement -optic disc swelling; more hemorrhage -no symptoms of GCA *compressive optic nerve tumor -slowly progressive visual loss -few to no symptoms of GCA *central Retinal Vein Occlusion -severe visual loss -APD usually present -disc swelling -retinal hemorrhages spreads out to periphery *Central Retinal Artery Occlusion -sudden, painless, severe visual loss -APD present -disc not swollen -retinal edema

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-cherry red spot -Buergers disease MANAGEMENT: *order STAT ESR (Westergren) -guideline for top normal ESRs -male=age/2 female=(age+10)/2 *Order C reactive protein *temporal artery biopsy (to rule in/out GCA) *STAT neurology or neuroophthalmology consult to initiate treatment with IV than oral steroids. *with or without steroids fellow eye may become involved within 24 hours OPTIC NEURITIS (PAPILLITIS) (RETROBULBAR OPTIC NEURITIS) SYMPTOMS: *acute vision loss, deteriorating over hours to days (usually over a 2-3 day span) *vision stabilizes and improves about one week after onset *usually unilateral in adults and bilateral in children *typical age 18-45 years *pain upon eye movement *acquired color vision defect *reduced brightness sense *Uhthoffs and L'Hermitte's symptoms Uhtoffs symptoms-decrease in vision with an increase in body temperature L'Hermitte's symptoms-sensation of electric-like shocks/tingling when head is Flexed forwards with chin touching chest *neurologic symptoms or an antecedent viral syndrome (i.e. URI, GI) COMMON SIGNS: *relative afferent pupillary defect (except in symmetrical, bilateral cases) *reduced color vision *central, cecocentral, or arcuate visual field defects SIGNS OF PAPILLITIS: *swollen hyperemic disc with or without peripapillary flame hemorrhages *posterior vitreous cells (white cells) *SVP may be absent *optic nervehead cupping filled in the early stage *hard retinal exudates SIGNS OF RETROBULBAR OPTIC NEURITIS: (more common in adults) *normal optic nervehead appearance - no edema ETIOLOGY:

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*idiopathic Noninfectious *demyelinating disease (i.e. multiple sclerosis) *collagen vascular disease *syphilis *sarcoidosis *intraocular inflammations (i.e. uveitis) Infectious *viral infections -mononucleosis -herpes zoster -encephalitis -measles, mumps chicken pox *granulomatous inflammations -tuberculosis Neuroretinits *contiguous inflammation of the menenges, orbit or sinuses MANAGEMENT: *order blood tests -CBC -RPR -FTA-ABS -ESR *order chest x-ray *CT scan and/or MRI Treatment: *management of optic neuritis is controversial Possibly quicker visual recovery and delay onset of MS but no long term benefits proven. *pulse steroids -methylprednisolone IV then oral for 14 days. Note: Oral steroids should not be used alone as a Tx. PAPILLEDEMA *increased intracranial pressure causing optic disc swelling SYMPTOMS: *bilateral, transient loss of vision (visual obscuration lasting only seconds) *headache *double vision *nausea *vomiting SIGNS: *optic disc characteristics:

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-bilaterally swollen -hyperemic -blurry disc margin -papillary or peripapillary retinal hemorrhages -loss of pre-existing spontaneous venous pulse -dilated tortuous retinal veins -cotton woll spots -enlarged blind spot -unilateral or bilateral sixth cranial nerve palsy -macular edema -chorioretinal folds *may have abnormal head CT scan and MRI **elevated cerebral spinal fluid pressure EITOLOGY: *primary and metastatic intracranial tumors *subdural and epidural hematomas *arteriovenous malformation *brain abscess *meningitis *encephalitis MANAGEMENT: -blood pressure measurement -visual fields Refer to neurologist or neourophthalmologist *STAT CT scan and/or MRI *lumbar puncture may be indicated Treatment: *treat underlying cause of the elevated intracranial pressure PSEUDOTUMOR CEREBRI SYMPTOMS: *headaches *visual obscurations (transient vision loss lasting only for seconds) *diplopia *tinnitus *dizziness *nausea/ vomiting *back/neck ache *acute onset *paresthesias

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SIGNS: Same signs and symptoms as papilledema but normal head CT scan and MRI Elevated intracranial pressure as measured by lumbur puncture normal cerebral spinal fluid compostition ETIOLOGY: *idiopathic *occurs predominantly in females who are around 40 years old and who are >20% over their ideal body weight. *pregnancy *specific medications -oral contraceptives -tetracyclines -nalidixic acid -vitamin A -systemic steroid withdrawal MANAGEMENT: *visual field testing refer to neurologist or neuro ophthalmologist *STAT CT scan (axial and coronal views) or MRI or orbit and brain Lumbar puncture Treatment: 1. programmed weight loss, if >20% over their ideal body weight. 2. diuretics (acetazolamide) 3. discontinue any causative medication 4. systemic steroids-short term therapy 5. optic nerve decompression surgery 6. lumbo-peritoneal shunt Follow-up: *assess -visual acuities -visual field -color vision -weight status -optic nerve examination SECONDARY TO OCULAR DISEASE Central retinal vein occlusion Diabetic papillitis Leukemia Malignant hypertensive retinopathy Sarcoid

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Trauma TOXIC (DEFICIENCY) /METABOLIC OPTIC NEUROPATHY SYMPTOMS: *painless, progressive, bilateral loss of vision SIGNS: *visual acuities range from 20/50 to 20/200 *color vision desaturation *possible disc edema followed by optic atrophy *temporal disc pallor *bilateral cecocentral or central visual field defect *alcoholism *poor nutrition ETIOLOGY: *tobacco/alcohol abuse *thiamine (vitamin B-1) deficiency from severe malnutrition *pernicious anemia (defect in vitamin B-12 absorption) *toxic substances -chloramphenicol -chloroquine -digitalis -disulfiram (Antaabuse) -ethambutol -ethchlorvynol (Placidyl) -isoniazid -lead -streptomycin MANAGEMENT: *visual field testing *CBC *serum vitamin B12 level and zinc level *heavy-metal screen (i.e. lead, thallium ) Treatment: Eliminate toxic agent Nutritional supplementation Thiamine, folate, fortified multivitamin Counseling for substance abuse R/O neoplasm TUMOR Infiltrative optic neuropathy

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Compressive optic neuropathy

VISUAL PATHWAYS Visual field analysis allows the clinician to identify where along the visual pathway a lesion may lie. In addition, other signs may help isolate the lesion, especially when the lobes of the brain are involved. Neuroimaging, such as Magnetic Resonance Imaging (MRI) and Computed Tomography (CT scan) is required for diagnosis. 1. Visual field testing a. Amsler grid b. Confrontation fields c. Red cap or Subjective brightness comparison testing d. Tangent Screen Automated / Goldmann perimetry Other tests: Optokinetic nystagmus, neutral density filters, Pulfrich stereo phenomenon, photostress

VISUAL FIELD PATHWAYS: 1. PRECHIASMAL Unilateral field loss Generalized defect Cataract, generalized retinal disease Central defect ARMD or other macular lesion, optic neuritis Nasal step, arcuate defect, Glaucoma Enlarged blindspot Glaucoma, papilledema, disc drusen Altitudinal defect Advanced Glaucoma, Ischemic optic neuropathy, hemiretinal artery/vein occlusion Cecocentral. Toxic optic neuropathy, Lebers optic neuropathy CHIASMAL DISEASE a. Bitemporal Hemianopsia *Classic field defect resulting from involvement of the decussating nasal optic nerve fibers. Respects the vertical midline. *Binasal field defects are possible but are extremely rare. Junctional Scotoma *Ipsilateral central scotoma and contralateral superior temporal field defect. *Lesion is positioned at the Anterior Knee of von Wilbrand (where the optic nerve meets the chiasm).

2.

b.

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c. d. e.

Etiology : *Pituitary adenoma, Meningioms, craniopharyngioma, aneurysms. Other signs: temporal pallor of disc, reduced stereopsis decreased visual acuity Mimickers of bitemporal hemianopsia: Tilted disc, optic disc drusen, Sector RP, bilateral RD, drugs, eyelids, Fuchs coloboma, papilledema with large blind spots, centrocecal scotomas.

3.

OPTIC TRACT a. b. Very incongruous partial homonymous hemianopsia. *Contralateral to the lesion. *In early lesions, defect may first appear to be unilateral. Isolated tract lesions are uncommon due to the close proximity to surrounding structures. *Can have a posterior junctional syndrome which affects the anterior tract at the posterior chiasm: Ipsilateral central scotoma and contralateral homonymous hemianopsia. Causes: Craniopharyngioma, pituitary aenoma, internal carotid aneurysms, gliomas. Other signs: *Contralateral Afferent pupillary defect due to the greater number of contralateral nasal hemiretina fibers in the tract. *Temporal pallor of the ipsilateral disk, Bowtie pallor (nasal and temporal) of the contralateral optic nerve. *Wernickes pupil: Rarely seen.

d. e.

4.

LATERAL GENICULATE NUCLEUS (LGN) a. b. c. The termination point of ganglion cell axons. Rare to isolate the LGN due to its particular location. Type of field defects seen: *Incongruous homonymous hemianopsia *Wedge-shaped homonymous sectoranopia *Relatively congruous *Situated around the horizontal midline *Incongruous Homonymous Hemianopsia with sector sparing around the horizontal midline. Causes: Tumors, ischemic-vascular, A/V malformations, and trauma. Other signs: *Pupils are not disturbed since the fibers exit prior to entering the LGN. *Retrograde optic atrophy *May see contralateral hemiparesis, if adjacent internal capsule is involved. *May see hemisensory loss, if adjacent thalamus is involved.

d. e.

5.

TEMPORAL LOBE

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a. b. c.

d. e. f.

Characterized by Superior Homonymous Quadranopsia or Pie-in-the-sky. VF loss is contralateral to the lesion. Mnemonic PITS Parietal lobe Inferior, Temporal lobe Superior. May present as congruous or incongruous superior quadranopsia or hemianopsia more dense superiorly. *If incongruous, the ipsilateral field loss is more dense, and involves more central, inferior, and peripheral areas than the contralateral eye. Area involved is geniculocalcarine radiations or Meyers loop Causes: Tumor (slow) vs vascular, demyelinating (quick onset) *Tumors, primarily gliomas, are more common than vascular dz. Other signs: *May have contralateral hemiplegia. *Aphasia (speech impairment), poor comprehension, Hemianesthesia *Normal stereoacuity, Optokinetic nystagmus (OKN) *No pupil involvement and no optic atrophy

6.

PARIETAL LOBE a. b. c. d. e. Characterized by Inferior Homonymous Quadranopsia or *Cake-on-the-floor. VF loss is contralateral to the lesion. *Visual field loss is denser inferiorly. More often, they present as complete homonymous hemianopsia. Superior retina fibers course through the parietal lobe. Cause: *Tumors (gliomas, meningioma, metastases) vs Ischemic-vascular (middle cerebral artery) is about 1:1. Other signs: *Abnormal OKN *Decreased stereoacuity *Spasticity of conjugate gaze *Astereoagnosia: Inability to recognize by touch *Language and visual recognition disturbance *Neglect of contralateral space *Spatial disorientation *If left angular gyrus is involved, it can cause impaired reading, arithmetic, left-right orientation, and finger counting. *Right-sided lesions typically produce inability to copy simple figures, drawing clocks and flowers, and construction apraxia. *No pupil involvement and no optic atrophy. *May have contralateral hemiparesis.

7.

OCCIPITAL LOBE a. Characteristic field loss is congruous homonymous hemianopsia with macular sparing. Contralateral to the lesion.

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b.

*Hemianopsia is very congruous. Vascular disease is the most common cause of occipital lobe involvement. Metastatic tumors and trauma are other causes. c Other signs: *Normal OKN & Stereoacuity *No pupil involvement and no optic atrophy *Loss of comprehension *May be associated with vertebro-basilar disease: *Transient hemianopia, diplopia, oscillopsia, gray-outs, ocular motility disturbances such as gaze palsies, nystagmus, Drop attacks, headache, hemiparesis. CORTICAL BLINDNESS a. b. c. d. e. f. Refers to subnormal visual acuity secondary to bilateral retrogeniculate lesions. Binocular and symmetric visual acuity and field impairment May be the consequence of consecutive events, first affecting one hemisphere and then subsequently the other. Patients have normal fundus appearance and normal pupillary activity. Causes: Ethanol toxicity, trauma, stroke, multiple sclerosis, meningitis, tumors, A/V malformations, heavy-metal toxicity, encephalitis, electric shock, etc. Other signs: *Antons syndrome: Denial of blindness. *Failure to recognize familiar faces or objects. *Memory loss and dementia One fourth of patients with occipital strokes may progress to bilateral cortical blindness within four years. VEP is helpful in making the diagnosis.

8.

g. h.

GENERAL TREATMENT: 1. 2. Diagnostic Imaging: CT or MRI. Contrast enhancement with such agents as Gadolinium helps increase sensitivity. Determine the underlying cause and treat: a. Tumors may require medical and/or surgical treatment or irradiation. b. Vascular disease requires further investigation of embolic material, carotid occlusion, and blood panel. Angiography or arteriograms may be needed to locate aneurysms or malformations. Should be medically treated.

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OCULOMOTOR DISORDERS This section tries to cover some of the more common oculomotor disorders. It does not include saccadic or nystagmoid syndromes. THIRD (III) NERVE PALSY SYMPTOMS: *binocular double vision *ptosis droopy eyelids *pain (present or absent) SIGNS: *complete palsy -restricted/limited ocular movement in all fields of gaze except temporally (out) -ptosis -pupil (involved or spared) *superior division palsy -restricted superiorly (up gaze) -ptosis *inferior division palsy -restricted nasally or inferiorly (in or down gaze) *aberrant regeneration -elevation of upper eyelid with gaze inferiorly or nasally -segmental pupil constriction with gaze superiorly, inferiorly, or nasally -exotropia/ hypotropia *aberrant regeneration of the primary type without preceding third-nerve palsy, usually caused by a cavernous sinus tumor or aneurysm ETIOLOGY: *pupil involvement 1. aneurysm (at the site of the posterior communicating artery) 2. microvascular disease (diabetes, hypertension, tumor, trauma, congenital) 3. uncal herniation, cavernous sinus mass lesion, orbital disease, herpes zoster, leukemia *pupil sparing 1. microvascular disease, especially diabetes 2. cavernous sinus syndrome (no excyclorotation with cover test) DIFFERENTIAL DIAGNOSIS: *myasthenia gravis -diurnal variation of the signs and symptoms -pupil spared -increased lid droop after fatigue (e.g. Sustained up gaze) -weak orbicularis oculi muscle -positive edrophonium chloride test (Tensilon)

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*thyroid eye disease -lid lag -stare -injection over rectus muscle -proptosis -positive forced duction testing -abnormal CT scan of orbits -no ptosis *Chronic Progressive External Ophthalmoplegia -bilateral -slowly progressive ptosis -restriction of ocular motility -pupil spared -no double vision *orbital inflammatory pseudotumor -pain -proptosis *mid-brain lesion -restricted up and down gazes -pupils react sluggishly to light and briskly to convergence -no ptosis -variable lid retraction -variable retraction nystagmus -bilateral *Blow out fracture SEE UNDER ORBIT SECTION MANAGEMENT: *immediate hospitalization and CT scan and/or MRI of the brain indicated for the following: -pupil involved third-nerve palsies -pupil spared third-nerve palsies in patients: With incomplete third nerve paresis <50 years old (unless history of established diabetes or hypertension) with additional cranial nerve or neurologic abnormality patients who develop aberrant regeneration except those following traumatic thirdnerve palsies whose third nerve palsy is >2-3 months old with no signs of improvement. * Complete third-nerve palsies with pupil spared not meeting above requirements should have a work-up including blood pressure testing, CBC with differential, gluconse tolerance test, sedimentation rate, VDRL, FTB-ABS and ANA. Management: *treat underlying vasculopathic risk factors (e.g. Diabetes or hypertension) or abnormality *patch involved eye if double vision presents from third-nerve palsy *re-check every 6 weeks; should regain function within 3 months *no improvement or if additional neurologic abnormality develops after 3 months,

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obtain MRI *cerebal angiography may be indicated for all patients -> 20 years old -who meet the criteria for CT scan and/or MRI and whose CT scan and/or MRI is unremarkable. FOURTH (IV) NERVE PALSY The IV cranial nerve innervates the SO muscle on the contralateral side of its exit point. The fourth nerve has the longest intracranial course and is the slenderest of all the cranial nerves. Isolated IVth nerve palsy 1. Congenital: Large vertical fusional amplitude, Head tilt *May see in young children or in adults when fusion decompensates, may have amblyopia. *Unilateral 2. Acquired: Acute onset *Head tilt to opposite shoulder *Chin down, face turned opposite *Causes: Adults: Children: 40% Trauma 60% Congenital 30% Unknown 35% Trauma 20% Ischemic 5% Inflammation 10% Neoplasm or aneurysm SYMPTOMS: Patients may report binocular vertical diplopia which may cause difficulty reading or objects to appear tilted. SIGNS: The involved eye should appear higher and will be unable to look inferionasally. Look at the fellow eye, it may be depressed depending on the fixating eye. Head tilt to the opposite side. *DIFFERENTIAL DIAGNOSIS: Myasthenia Gravis, Thyroid eye disease, Third nerve paresis, Orbital tumor, Inflammation, blow-out fracture, skew deviation (3-step test can not isolate one muscle), Browns vertical hypertropia. MANAGEMENT: Patient is under 20 no work up required if: *Long standing head tilt or hyper by old pictures. *Increased vertical fusional vergences. *No other neurological signs present. Neuroimaging CT *History of trauma *Other neurological signs or symptomatic

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Neuroimaging CT or MRI Vascular disease work-up in patients over 40. Rule out diabetes and hypertension Sedimentation rate in patients over 65. Rule out Giant Cell Arteritis May be able to use prisms of VT. Surgery in extreme cases. SIXTH (VI) NERVE PALSY The sixth cranial nerve lies in the mid-portion of the pons below the floor of the fourth ventricle, in close proximity to the faciculus of the seventh nerve. So a nuclear lesion cannot present as an isolated sixth nerve palsy. Emerging fibers pass ventrally and leave the brain stem at the pontomedullary junction. It then enters the prepontine basilar cistern, passes the pons, is crossed by the anterior inferior cerebellar artery, pierces the dura, and makes an angle bend over the tip of the petrous bone. It then goes through the inferior petrosal sinus and Dorrelos canal into the cavernous sinus. It innervates the lateral rectus muscle. Location of the Lesion: 1. Brainstem: Usually will involve other structures such as Medical longitudinal fasciculus - INO PPRF Ipsilateral conjugate gaze palsy Pyramidal tract contralateral hemiparesis Oculosympathetic central neuron ipsil. Horners

2.

Sub-arachnoid space: Elevated intracranial pressure induces downward displacement of the Brainstem, stretching the VI Hemorrhage, inflammation, meningeal infection, infiltration. Petrous apex: Cerebello-pontine angle tumor Nasopharyngeal carcinoma Petrous bone fracture due to trauma Otitis media (Gradenigos)

3.

4. 5.

Cavernous sinus:

presents with other involved CNs (III, IV and V1)

Orbit: - May see proptosis as an early sign. Tumor Trauma Inflammation Cellulitis

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6.

Isolated sixth nerve palsies: Younger patients: Post viral, or neoplasm *50% chance of neoplasm (Brainstem glioma) in non-traumatic sixth nerve palsy in a child. Older patients: *Acute Ischemic vascular Chronic: Compressive mass

Differential diagnosis: 1. Thyroid eye disease injection and lid signs 2. Myasthenia Gravis is variable 3. Duanes retraction syndrome Type I fissure narrowing 4. Medial wall blow out fracture 5. Intermittent esotropia 6. Spasm of near reflex pupils constrict SYMPTOMS: Horizontal binocular diplopia. Worse at distance. SIGNS: Affected eye is turned inward. Eye does not turn out with versions. MANAGEMENT: Tests: *Optokinetic nystagmus myopathic vs neuropathic *Forced duction testing *Cranial nerve testing rule out other involvement Laboratory studies: Age <14: Consider MRI Age 15-40:neurologic evaluation with CBC, glucose testing Age .40:most likely vascular if over 55 obtain Westergren sed rate and possibly temporal artery biopsy. *Consider MRI and Lumbar puncture if other neurologic signs present. Treatment: 1. Treat the underlying disease. 2. Prisms 3. Surgery, 1 year after onset at least INTERNUCLEAR OPHTHALMOPLEGIA (INO) INO results from a lesion(s) of the medial longitudinal fasciculus, which interrupts internuclear messages between the abducens necleus and the oculomotor nucleus. However, in the case of INO, a lesion in the MLF disrupts the message to the medial rectus nucleus. There is no action or lag of the medial rectus (lag or no adduction). This will be more obvious with repeated saccadic eye movements. *THE LESION IS ALWAYS ON THE SIDE WITH THE ADDUCTION DEFICIT!

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*May also see jerky pseudo-nystagmus is seen in the abducting eye! (Not needed for diagnosis of INO) Associated signs and symptoms: Transient blurred vision and diplopia. Unilateral internuclear ophthalmoplegia. *Vascular disease *MS *Brain stem infarction May also present bilaterally known as BINO. *Associated with demyelinating disease and vertibrobasilar disease. MANAGEMENT: Neuroimaging. INO indicates lesion of the MLF. *Determine etiology ischemic-vascular vs. tumor *Diagnosis of multiple sclerosis Full neurological and systemic evaluation Treatment: Treat underlying cause. ASSOCIATED SYNDROMES: WEBINO Wall-Eyed Binocular Internuclear Ophthalmoplegia Caused by a midline lesion which affects both medial rectus subnuclei and both MLFs. Patient is exotropic and has no convergence abilities and abducting nystagmus. One and Half syndrome: A lesion which encompasses the MLF and PPRF (or VI nerve nucleus) on the same side. As a result, horizontal gaze is paralyzed on the ipsilateral side and an INO is present on the opposite side (Adduction deficit). Treatment is similar to INO. Pathological etiologies are pontine hemorrhage, brainstem infarct, multiple sclerosis, and pontine glioma. DORSAL MIDBRAIN SYNDROME or PARINAUDS SIGNS: 1. Loss of saccadic upgaze 2. Light-near dissociation pupils, mid-dilated pupils 3. Convergence retraction nystagmus 4. Papilledema 5. Lid retraction Colliers sign

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6.

Skew deviation

May see accompanied: IVth nerve palsy, downbeat nystagmus, loss of upgaze pusuits, loss of downgaze saccades, spontaneous convergence-retraction movements. CAUSES: Pinealoma, aqueductal stenosis, tumors of the aqueduct and superior colliculus, multiple sclerosis, syphilis, trauma, stroke.

MANAGEMENT Neuroimaging - MRI Consider treponemal studies, especially if neuroimaging is clean Neurological examination DUANES RETRACTION SYNDROME THREE TYPES: I -Palsy of abduction and widening of palpebral aperture; with retraction on -adduction. II. -Palsy of adduction with retraction, and intact abduction. III. -Palsy of adduction and abduction and retraction with attempted adduction. Vertical upshooting and downshooting may occur in the adducting eye. Patients may have good binocular function in positions of gaze are aligned, but suppress in the diplopic fields. Microtropia and poor convergence may also be present. Patients may report sudden onset of diplopia. Patients show a compensatory head turn toward the involved eye. MANAGEMENT: Congenital anomalies Must rule out other forms of muscle restriction such as orbital tumor, medial wall fractures, Graves dz, and orbital metastasis. Duction testing may help. Surgery is only indicated for cosmetically unacceptable strabismus and disturbing head positions. BROWNS SYNDROME Limitation of the eye to move up when in the adducted position. Due to restriction of the superior oblique tendon through the trochlea. The affected eye is usually hypotropic. May be due to congenital problem of a short and tethered tendon or can be acquired through trauma or inflammation. Forced ductions can be used in the diagnosis. Many congenital cases spontaneously improve by age 12 yrs. Rule out inflammatory or IV nerve palsy. MYASTHENIA GRAVIS (MG)

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Major ophthalmologic symptoms are: Alternating or transient ptosis, diplopia which may also be transient or worse at the end of the day. SIGNS: Ocular motilities: *Patient may have phorias and tropias which vary throughout the exam or on a day to day basis. Lid Position *Push down on frontalis muscle and remeasure to see amount of actual ptosis. *Have patient go into sustained upgaze will fatigue. *Have patient squeeze lids tight will open up. *Place an icepack on the lid lid will retract. *Cogans lid twitch overshoot of the lid in upgaze in some pts. *Test orbicularis function: have patient try to shut lids while the doctor attempt to pull them apart. *May have secondary kerititis MANAGEMENT: Ocular Treatments: *Patching *Prisms *Ptosis crutch CAVERNOUS SINUS SYNDROME Patients present with peri-orbital or hemi-cranial pain, ipsilateral oculomotor palsies (CNs III, IV, V1, VI), oculosympathetic paralysis, loss of sensation along the ophthalmic branch of CNV. Orbital apex syndrome (on the other side of the superior orbital fissure) involves the optic nerve in addition to oculomotor palsies. Horners (ptosis and miosis), or dilated pupil if III CN is involved. May have proptosis or an orbital bruit. CAUSES: 1. Vascular aneurysm, fistulas, thrombosis 2. Neoplasm meningiomas, metastasis, pituitary adenoma, Craniopharyngioma, lymphoma, nasopharyngeal tumor, etc 3. Inflammation Bacterial, fungal, syphilis, viral, sarcoid, Tolosa-Hunt Look for: Intracavernous aneurysm, nasopharyngeal tumor, orbital cellulitis, Carotid-cavernous fistula, cavernous sinus thrombosis, giant cell arteritis, pituitary apoplexy, Tolosa-Hunt, Herpes Zoster, and meningiomas. Differential Diagnosis: 1. Myasthenia 2. Dysthyroid disease 3. Orbital disease 4. Diabetic ophthalmoplegia

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5.

Giant cell arteritis

MANAGEMENT: NEUROIMAGING. Systemic disease work-up. (See Tolosa-Hunt). Treat the underlying disease process. May require biopsy, steroids, surgery, radiation, etc. TOLOSA-HUNT SYNDROME Granulomatous inflammation of the cavernous sinus. It presents as a painful, reoccurring entity which may last days to weeks. Spontaneous remissions may occur with months to years in between. The syndrome may affect CNs, II, III, IV, V, VI, VII, and oculosympathetic pathway. Pain may precede, coincide or follow the onset of the ophthalmoplegia. Pain may be described as severe periorbital or hemi-cranial pain. Accompanying signs may be proptosis, optic nerve involvement, conjunctival injection, peri-orbital edema, and chemosis. Typically, a cavernous sinus picture can appear. However, the optic nerve, the maxillary and mandibular branch of the trigeminal nerve and the facial nerve may be involved. Neurological deficits such as hemiparesis and psychosis have been noted. Tolosa-Hunt is a diagnosis of exclusion. Diagnostic tests must be done to rule out other cavernous sinus disease entities. MANAGEMENT: Refer to neurologist. Hematologic tests: CBC, ESR, ANA, FTA-ABS, Serum protein electrophoresis, serum chemistry, lupus erythematosus cell prep. Cerebrospinal fluid: Pressure, serology, cytology, protein, glucose, cell count with differential, culture for fungi, bacteria Neuro-imaging and angiography of the cranium Nasopharyngeal examination. Treatment plan is similar to cavernous sinus syndrome. Systemic steroids (Prednisone) have been shown to vastly improve the condition in a couple of days.

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URGENT AND EMERGENT EYE CARE AMAUROSIS FUGAX Amaurosis Fugax may be in isolated symptom or in company with other neurological symptoms. Patients may report transient visual loss which may last minutes to an hour. Vision returns to normal after the attack. Most often it is the result of emboli of cardiac or carotid origin. Cholesterol (Hollenhorst) plaques may be visible at the bifurcations of the retinal arteries. There are a host of emboli other than cholesterol plaques that can cause this. MANAGEMENT: SEE RETINAL SECTION ACUTE ANGLE-CLOSURE GLAUCOMA SEE ACUTE ANGLE-CLOSURE GLAUCOMA IN GLAUCOMA SECTION SYMPTOMS: *pain *blurred vision *colored halos around lights *frontal headaches *nausea / vomiting SIGNS: *closed angles *elevated IOP *corneal edema *conjunctival injection *fixed mid-dilated pupil *shallow AC ETIOLOGY: *pupillary block *neovascular or inflammatory membrane *lens-induced/Phacomorphic *choroidal detachment *choroidal effusion syndrome *posterior segment tumor *malignant glaucoma *plateau iris syndrome DIFFERENTIAL DIAGNOSIS: *Glaucomatocyclitic crisis/Posner-Schlossman Syndrome *inflammatory POAG

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*retrobulbar hemorrhage or inflammation *traumatic glaucoma *phacolytic glaucoma -look for -KPs -posterior synechiae -iris neo -swollen lens -AC cells and flare MANAGEMENT: 1. carbonic anhydrase inhibitor acetazolamide 250-500mg 2. an oral osmotic agent Glycerin (1.5g/kg) or Isosorbide (1-2 g/kg) in diabetics 3. topical B-blocker 4. apraclonidine 0.5 1% 5. recline px in supine position to allow lens to fall backward with vitreous dehydration 6. recheck IOP and ocular findings after one hour 7. consider adding pilocarpine if IOP under 40 8. recheck IOP and ocular findings after 30 minutes 9. a) if IOP drops and the angle is open: treat patient with pilocarpine .5%-2% QID B-blockers BID steroids-prednisolone 1% QID acetazolamide 500mg (po) BID when and if the eye quiets, perform a laser iridotomy b) if IOP unchanged or elevated and the angle remains closed: -suspect lens-related angle closure -perform argon laser iridoplasty to break attack BURNS CHEMICAL BURN (STAT) TRUE OCULAR EMERGENCY! Types of chemical burns: Aromatic compounds Acids Alkali Continue to penetrate through ocular structures after initial reaction. INITIAL TREATMENT: *initiate treatment IMMEDIATELY -irrigate eyes copiously with Saline Solution or Ringers lactated solution (if not available, nonsterile water should be used) for at least 20 - 30 minutes *irrigate thoroughly -pull down lower lid and, singly or doubly, evert upper lid

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*determine substance causing injury and determine any specific treatment needed *test pH for neutrality MILD TO MODERATE BURNS SIGNS: *corneal epithelial defect -ranging from SPK to small focal epithelial loss to denuded epithelium *small area(s) perilimbal or random ischemia *conjunctival chemosis *hyperemia *hemorrhages may be present *mild eyelid edema *possible first and second degree burns of the periocular skin TREATMENT AFTER IRRIGATION: *remove any residual caustic particles and necrotic conjunctiva by further irrigation or use of a cotton-tipped applicator 1. 2. 3. cycloplegic >>>homatropine topical antibiotic ointment >>>erythromycin or tobramycin topical steroid >>>prednisolone acetate 1% OR >>>dexamethasone 0.1% analgesic >>>acetaminophen with/without codeine as needed

4.

FOLLOW-UP: *recheck every 24 hours until corneal defect is healed *repatch with cycloplegic drop and antibiotic ointment until corneal defect is healed MODERATE TO SEVERE BURNS SIGNS *severe chemosis *perilimbal blanching *corneal edema *corneal opacification (with no or very limited views of AC, iris, lens) *moderate to severe AC reaction * IOP may be significantly increased *second and third degree burns of the periocular skin *local necrotic retinopathy

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(from direct penetration of alkali through sclera) TREATMENT AFTER IRRIGATION: hospitalization may be necessary *same as for mild to moderate burns in addition: *monitor IOP and corneal healing *debride necrotic tissue *remove sequestered foreign matters *bandage contact lens *antiglaucoma medications if IOP is elevated >>>acetazolamide (Diamox) or methazolamide (Neptazane) ADD topical beta-blocker *lysis of conjunctival adhesions to prevent symblepharon *Collagenase inhibitors (such as acetlycysteine) FOLLOW UP: *monitor closely until cornea healed *taper topical steroids after seven days to prevent corneal melting *Consider artificial tear substitute and/or lubricating ointment *if severe dry eye, consider tarsorrhaphy, conjunctival flap or mucous membrane graft CENTRAL RETINAL ARTERY OCCLUSION (CRAO) SEE UNDER RETINAL VASCULAR CONDITIONS CORNEAL ABRASION SYMPTOMS: *pain *photophobia *foreign body sensation *tearing *history of scratching/abrading the eye SIGNS: *epithelial staining defect with sodium (NaF1) fluorescein *conjunctival injection *swollen eye lid *mild anterior chamber reaction NO INFILTRATE

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MANAGEMENT: - Broad spectrum antibiotic such as: gentamycin, trimethoprim sulfate-polymyxin B sulfate or tobramycin - Dependent on severity of epithelial defect, AC reaction and patient symptoms consider: cycloplegic and/or pressure patch for 24 hours only. - For small abrasions in non-CL patient BCL works great with concomitant antibiotic therapy - Discontinue contact lens wear in CL patients Follow-up: -RTC 24 hours -Evaluation of epithelial defect a. if abrasion resolved or only SPK remains, continue topical antibiotics for 4 more days b. if abrasion healing and remaining abrasion is small and non-central, treat as above, topical antibiotics for 4 more days c. if abrasion remains large or central, continue antibiotic, repeat cycloplegic and pressure patch if needed as above RTC 24 hours Resume contact lens wear after through contact lens evaluation and deemed safe If repeat abrasion, consider bland ointment before bedtime for at least 3 weeks after reepithelialization. CORNEAL FOREIGN BODY SYMPTOMS: *foreign body sensation *tearing *photophobia *pain *history of foreign body to the eye *visual acuity typically not significantly affected SIGNS: *eyelid edema *imbedded material on palpebral conjunctival *conjunctival injection *corneal foreign body *rust ring *SPK *mild anterior chamber reaction * perforating injury: entry site in cornea, conjunctiva, or iris (positive Seidel sign); decreased VA, marked conjunctival hemorrhage or chemosis, hypotany, flattening of the anterior chamber, vitreous hemorrhage, cataract formation.

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MANAGEMENT: Determine cause of foreign body especially if object propelled at high velocity (lathes, firearms, hammers, drills, or grinder). * Determine if perforating injury, look for penetrating wound in conjunctiva, cornea and iris. If a perforating injury do not remove foreign body, follow guidelines in PERFORATING OCULAR INJURY. Immediate referral! Remove foreign body DFE must be performed if corneal foreign body is a by high velocity particle. If DFE unremarkable for intraocular foreign body consider X-ray, ultrasound or CT scan NOT MRI if foreign body is suspected to be of metallic nature. *Foreign body removal 1. anesthetize cornea with 2 drops of proparacaine 2. Technique/ Instrument -irrigate -remove with Foreign Body Spud (Golf Club Spud), hypodermic needle, Jewelers forceps, or Nylon loop 3. if rust ring develops, remove -anesthetize cornea with 2 drops of proparacaine -remove with alger brush or hypodermic needle -Rust ring does not need to be removed at one time. Can be removed at a later time when cells have migrated more superficially. *After removal - Broad spectrum antibiotic such as: gentamycin, trimethoprim sulfate-polymyxin B sulfate or tobramycin - Dependent on severity of epithelial defect, AC reaction and patient symptoms consider: cycloplegic and/or pressure patch for 24 hours only. Follow-up: FOLLOW GUIDELINES UNDER CORNEAL ABRASIONS Remove rust ring as needed. CORNEAL LACERATION *partial-thickness laceration -anterior chamber and globe intact, anterior chamber is not entered and globe not penetrated R/O PENETRATING INJURY: *History: Injury caused by object propelled at high velocity (lathes, firearms, hammers, drills, or grinder. *Look for penetrating wound in conjunctiva, cornea and iris *R/O uveitis

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*anterior chamber depth -R/O hyphema *perform the Seidel test *measure IOP by applanation tonometry (if sure no penetrating injury has occurred) MANAGEMENT: Remove foreign body (if present) DFE must be performed if corneal laceration caused by high velocity particle. If DFE unremarkable for intraocular foreign body consider X-ray, ultrasound or CT scan NOT MRI if foreign body is suspected to be of metallic nature. 1. cycloplege scopolamine, homatropine, or cyclogel 2. antibiotic >>>a)gentamicin ointment or erythromycin ointment OR b)gentamicin IF NEEDED: 3. pressure patch OR therapeutic SCL collagen shield or bandage SCL Re-evaluate every 24 hours until cornea epithelializes PERFORATING OCULAR INJURY (FULL THICKNESS LACERATION) SYMPTOMS: *pain *decreased vision *history of trauma, especially high velocity particle SIGNS: *full thickness scleral or corneal laceration (if penetrating injury) *subconjunctival edema and hemorrhage *flat/shallow AC *hypotany *hyphema *prolapse of intraocular contents *irregular pupil *iridodialysis *periorbital ecchymosis *cataract *subluxed lens *commotio retinae *choroidal rupture *retinal breaks/detachment

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*traumatic optic neuropathy MANAGEMENT: *avoid applying any pressure (no tonometry or gonioscopy) *protect the eye with a fox shield *refer immediately for surgical repair CORNEAL ULCER ETIOLOGY: *bacterial (most common) *fungal (more common in Southern United States; after organic, traumatic corneal injury) *acanthamoeba (extremely painful stromal infiltrate; seen with contact lens wearer who practices poor hygiene or uses tap water/non-preserved saline) *Herpes simplex virus (may see accompanying eyelid vesicles or corneal epithelial dendrites) SEE CORNEAL SECTION FOR DETAILS HYPHEMA SYMPTOMS: *pain, blurred vision *history of trauma (to the iris or ciliary body) SIGNS: *blood in the anterior chamber from microhyphema to eight ball hemorrhage *Elevated IOP *Uveitis MANAGEMENT: -R/O ruptured globe -do not perform gonioscopy or scleral depression -R/O orbital step-down/blow out fracture *order sickle-cell prep and hemoglobin electrophoresis in African American patients *perform B-scan ultrasound to R/O RD if poor view of fundus *CT scan of the orbits and brain, when indicated *hospitalization (confinement) for children with extensive hyphema -bedrest with head elevated 30 degrees -limited activity *shield (not patch) involved eye *no aspirin or blood thinners *Consider treatment with atropine and topical steroid *-if IOP is elevated - topical or oral antiglaucoma medications

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Follow-up: Monitor for: new bleeds, increase in IOPs, corneal blood staining *Surgical removal of the hyphema and/or clot may be indicated Long term Follow-up -Monitor for angle recession glaucoma ORBITAL CELLULITIS SEE UNDER ORBIT SECTION PRESEPTAL CELLULITIS SEE UNDER ORBIT SECTION RETINAL DETACHMENT EXUDATIVE RETINAL DETACHMENT SYMPTOMS: *minimum to severe visual loss *VF defect SIGNS: *serous elevation with shifting SRF *smooth surface of RD *APD (may be present) ETIOLOGY: Result of accumulation of fluid, hemorrhage, serous fluid or neoplasm under the sensory retina. *neoplasm -choroidal malignant melanoma, metastasis, choroidal hemangloma *inflammatory disease -VKH syndrome, posterior scleritis, other chronic inflammatory processes *congenital abnormalities -optic pit, morning glory syndrome, choroidal coloboma, Coats disease *nanophthalmus *Uveal Effusion Syndrome MANAGEMENT:TREATMENT: Traction al RD not involving or threatening the macula are semi-urgent -should be repaired within 1-2 days *chronic RD are repaired within one week RHEGMATOGENOUS RETINAL DETACHMENT ETIOLOGY: Break or thinning of peripheral retina

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*horseshoe tears *operculated tears *retinal holes *PVD *lattice degeneration *chronic rhegmatogenous RD -pigmented demarcation line -intraretinal cysts -folds SYMPTOMS: * increase or sudden onset of flashes of light *increase or sudden onset of floaters *curtain/shadow vision loss SIGNS: *elevation of retina with flap tear or break with underlying choroidal detail obscured *serous elevation with non-shifting SRF *corrugated appearance of detached retina *retina is mobile *absence of RPE hyperplasia around tear *vitreous hemorrhage *anterior uveitis *tobacco dust/Shaffers sign in anterior vitreous from blood cells and retinal pigment *PVD *APD (may be present) MANAGEMENT: Fresh retinal detachment should be referred immediately to a retinal surgeon. Long standing retinal detachments should be referred to a retinal surgeon within 1 to 2 days TRACTIONAL RETINAL DETACHMENT ETIOLOGY: Occurs secondary to other retinal conditions such as proliferative diabetic retinopathy, posterior uveitis, sickle cell or retinopathy of prematurity. *contraction of the fibrous bands with subsequent pulling of the retina off the RPE *perforating injuries SYMPTOMS: *peripheral and/or central visual loss *VF defect *With or without symptomology listed under RHEGMATOGENOUS RETINAL DETACHMENT SIGNS:

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*vitreous membranes/fibrous bands pulling on retina *smooth, concave surface of the detached retina *retina is immobile *APD (may be present) *usually in posterior pole MANAGEMENT: Retinal detachment should be referred immediately to a retinal surgeon especially if it involves the macula. Long standing retinal detachments should be referred to a retinal surgeon within 1 to 2 days THIRD NERVE PALSY SEE UNDER OCULOMOTOR DISORDERS

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SYSTEMIC DISEASE PRESENTATION IN THE EYE CARDIOVASCULAR DISORDERS CAROTID OCCLUSIVE DISEASE The three most common ocular findings associated with carotid artery disease are: amaurosis fugax, arterial occlusion, and ocular ischemic syndrome. Based on ocular symptoms, the patient may be required to undergo a battery of tests necessary to quantify potential areas of occlusion. AMAUROSIS FUGAX See retinal section ARTERIAL OCCLUSION Arterial occlusion is a process with grave consequences. Embolic obstruction of the retinal vasculature can produce ischemia, in turn, leading to vision loss. Most commonly, the ophthalmic or the central retinal artery is involved. Smaller branch occlusions may also manifest themselves. Sudden and painless, occlusion of the central retinal artery can decrease vision to less than 20/400. See retinal vascular conditions. OCULAR ISCHEMIC SYNDROME See retinal vascular section. MANAGEMENT: 1. 2. Blood pressure on both arms Other testing: *Carotid bruit turbulent blood flow as a result of plaque build-up. Heard if there is 50-90% occlusion. Medical examination *Including echocardiogram *Blood studies including 2 hr post-prandial sugar *Westergren sedimentation rate, if giant cell is suspected *Periorbital Doppler *Carotid Duplex scan (Doppler and ultrasound) *Intraarterial or Intravenous Digital Subtraction Angiography Carotid vascular work-up by neurologist. Consultation for carotid endaterectomy. Fluorescein Angiography may be helpful in retinal disease.

3.

4. 5.

HYPERTENSION

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Most often, chronic hypertension, in conjunction with arteriosclerosis, results in vessel changes. This may be appreciated by observing color, contour, reflex, and with changes of the retinal vasculature. Ocular side effects of HYPERTENSION/ARTERIOSCLEROSIS See retinal vascular section MANAGEMENT: Blood pressure: classified based on two or more readings JNC Report: New Classification Scheme (a) Pre Hypertension: SP=120-139 DP=80-89 (b) Stage 1: SP=140-159 DP=90-99 (c) Stage 2: SP= >160 DP=>100 Old Classification Scheme: Category Optimal Normal High-Normal Hypertension Stage 1 Stage 2 Stage 3 Referral criteria Systolic BP <130 130-139 140-159 160-179 >180 Diastolic BP <85 85-89 90-99 100-109 >110 Referral criteria Recheck within 2 yrs Recheck within 1 yr. Confirm within 2 months Refer within 1 month Immediate referral Systolic BP <120 <130 130-139 140-159 160-179 > 180 Diastolic BP <80 <85 85-89 90-99 100-109 >.110

ENDOCRINE DISORDERS DIABETES MELLITUS OCULAR COMPLICATIONS OF DIABETES 1. Refractive changes: Typically myopic shifts are seen with elevated blood glucose.

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2. 3. 4. 5. 6. 7. 8. A. B. C. D. E.

However, vision may fluctuate and undergo a hyperopic shift as the sugar level is placed under medical control. Early onset of presbyopia and decreased accommodation have been noted. Visual performance: Reduced acuity, blue-yellow color defects, abnormal contrast sensitivity, and field loss. Nerve Palsies: May effect the oculomotor nerves. The third nerve is the most common nerve affected. It is characterized by sparing of the pupillary fibers. Cornea: The basement membrane is altered in diabetics and the cornea tends to heal more slowly while sustaining injury much easier. There is also decreased sensitivity. Cataracts: Age-related cataractogenesis begins at an earlier age and progresses faster. Christmas-tree cataracts, resultant from chronic states of hyperglycemia are rare. Rubeosis irides: Neovascular response to ocular ischemia secondary to diabetes. can be responsible for open and closed angle glaucoma. (See neovascular glaucoma). Optic nerve disease: Non-arteritic ischemic optic neuropathy. Diabetic retinopathy: See retinal section. MACULAR EDEMA CLINICALLY SIGNIFICANT MACULAR EDEMA MILD NONPROFLFERATIVE DIABETIC RETINOPATHY MODERATE NONPROLIFERATIVE DIABETIC RETINOPATHY SEVERE NONPROLIFERATIVE DIABETIC RETINOPATHY *Soft exudates, venous beading, IRMA all definitely present in at least two of fields 4-7, or *Two of the three lesions above present in at least two of fields 4-7 and hemorrhages and microaneurysms present in each of fields 4-7, equaling or exceeding standard photograph 2A in one of them, or *IRMA present in each of fields 4-7 and equaling or exceeding standard photograph 8A in at least 2 of them, and *Definition for Early Proliferative DR or worse is not met. EARLY PROLIFERATIVE DIABETIC RETINOPATHY *New vessel growth and *Definition for high-risk proliferative diabetic retinopathy or worse not met. HIGH-RISK PROLIFERATIVE DIABETIC RETINOPATHY *New vessels on or within 1 DD of the optic disk (NVD) which is greater or equal to standard photograph 10A (1/4 1/3 disk area of NVD), with or without vitreous hemorrhage, or *Vitreous or preretinal hemorrhage and NVD less than standard photo 10A, or *Vitreous or pre-retinal hemorrhage and neovascularization elsewhere (NVE) greater than the disk area.

F.

G.

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TREATMENT: 1. Case History: a. Ocular decreased vision, fluctuation of vision, pain, etc. b. Personal Hx Polyphagia, polydipsia, frequent urination Last blood sugar, home monitoring, diet vs medical control Associated vascular disease or pregnant. Duration of diabetes. Juvenile vs. Adult onset c. Family history of diabetes, blindness d. Medications 2. Complete eye examination: a. Refraction may require several attempts to get stable Rx. b. Pupils, Amsler Grid Motilities c. Iris check for rubeosis, may also require gonioscopy d. Check cornea, lens e. Dilated fundus examination with stereoscopic viewing f. Blood pressure g. Fundus photography Management: OCULAR: a. Mild non-proliferative DR: Microaneurysms, dot-blot hemes, hard exudates and, if there is no macular edema present. *Low percentage progress to PDR in 1 year *Follow up in at least one year *Associated hypertension, kidney disease or pregnancy requires special medical attention i.e. closer follow-up and medical management. This applies to any stage. b. Clinically significant macular edema: *Fluorescin angiography to identify areas of leakage *Treatment of choice is Focal laser photocoagulation c. Moderate non-proliferative DR: *12-27% risk of progression to PDR in one year *Follow up every 3 months *Identify risk factors d. Severe non-proliferative DR: *52% risk of progression to PDR in 1 year *Follow up every 2-3 months *Retinal consultation fluorescein angiography to identify areas of non-perfusion e. Early Proliferative DR: *Refer to retinal specialist *Follow up closely 1-2 months f. High-risk Proliferative DR: *Retinal consultation *Flurescein angiography *Scatter Pan-retinal photocoagulation is the treatment of choice for

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neovascular proliferation of the disk, retina, and iris. If there is a poor view of the retina, peripheral retinal cryotherapy is performed. *Vitrectomy is indicated for: -Long-standing vitreous hemorrhage -Tractional retinal detachment involving the macula -Displacement of the macula -Unresponsive neovascularization and fibrous proliferation after attempted laser procedures -Hidden retinal detachments under fibrosis/Traction RD -Recurrent vitreous hemorrhage *If macular edema is also present, then perform focal/grid laser prior to scatter photocoagulation *Have better visual outcome with early vitrectomy *Requires close monitoring THYROID DISORDERS Ocular Signs: a. Upper lid retraction, lid lag on downgaze (von Graefes), lid edema, vessel engorgement over the insertion of the EOMs, inflammation and hypertrophy of the EOMs, prolapse of orbital fat, chemosis, palpable lacrimal gland, infiltrative changes in the retrobulbar tissue. Proptosis 3mm or more in excess of upper limit, with and without symptoms (a difference of 2 mm or more between the two eyes with exophthalmometry warrants thyroid testing.) Infiltration of the muscles results in enlargement, loss of elasticity and fibrosis of the muscles. Muscles become injected and full. Resistance on forced duction testing present. Ultrasonography and neuroimaging may be used. Chronic exposure keratitis severe corneal disease such as ulceration, necrosis. Perforation may occur. e. Optic nerves may show papilledema, papillitis, and retrobulbar neuritis. pre-tibial mixedema, skin and hair texture, tremor, etc. Symptoms: a. Patients may note lid and conjunctival appearance as well as proptosis. b. Sandy, gritty feeling, tearing, photophobia. c. May begin to experience diplopia Typically, Graves disease initiates within 18 months of the diagnosis of hyperthyroidism. The clinical course may be quite unpredictable. MANAGEMENT: Standard testing such as color, visual fields, and pupils should be performed. CT scan, MRI, or ultrasound. Thyroid testing Serum TSH, Free T4, anti-thyroid antibodies, FTI TREATMENT: Lid edema: Elevating head during sleep, cold compresses; surgical procedures for severe

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cases. Lid retraction: adrenergic blocking agents have been used or surgery for severe cases. Corneal changes: Ocular lubrication, broad-spectrum antibiotic ointment for severe cases. EOM involvement: Fresnel prisms, exercises, patching, Botulinum Toxin injected into the affected muscles, systemic prednisone, and muscle surgery, in stable and unresponsive cases. Orbital decompression. HEMATOLOGIC DISORDERS SICKLE CELL RETINOPATHY TYPES: SS sickle cell anemia (4% of sickle-cell syndromes) *severe systemic complications *mild ocular manifestations SC sickle-cell C disease (1-2% of sickle-cell syndromes) Sthal sickle cell thalassemia (10% of sickle-cell syndromes) *milder anemia *less severe systemic complications *most severe ocular presentations SA sickle-cell trait (80% of sickle-cell syndromes) *do not express significant systemic disease * ocular complications uncommon OCULAR FEATURES: -Non-proliferative retinopathy *characterized by

-venous tortuosity -black sunbursts (chorioretinal atrophy) -salmon patch (pink superficial hemorrhages) -refractile spots (hemosiderin deposits) -silver-wiring of peripheral arterioles -retinal breaks -angioid streaks -dark without pressure *symptomatic complications -CRAO -macular arteriole occlusion -retinal vein occlusion -choroidal vascular occlusions

-Proliferative sickle retinopathy *most severe forms of retinopathy associated with SC and Sthal disease *Proliferative stages

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-stage 1: peripheral arteriolar occlusion -stage 2: peripheral arteriovenous anastomoses -stage 3 sea-fan neovascularization -flat, new vessels taking on a fan shaped configuration -fed by a single arteriole and drained by a single vein -sea-fan tufts lift off the retinal surface due to traction and become adherent to the vitreous gel and eventually get pulled into the vitreous cavity -stage 4: vitreous hemorrhage -stage 5: vitreous hemorrhage with retinal detachment TREATMENT: 1. scatter panretinal photocoagulation -indicated in the presence of neovascularization -applied to sector of ischemic retina peripheral (anterior) to neovascularization 2. vitrectomy surgery with or without scleral buckle -recommended for chronic vitreous hemorrhage associated with traction/ rhegmatogenous RD -Non-retinal manifestations *conjunctiva-comma or corkscrew shaped, dark red vascular segments a reliable indicator for sickle cell disease *uvea -iris atrophy at the pupillary margin -rubeosis INFECTIOUS DISORDERS HERPES SIMPLEX SEE CONJUNCTIVA (Follicular conjunctivitis) CORNEA, UVEAL and LID (Inflammatory conditions) SECTIONS FOR MORE INFORMATION PRIMARY HERPES SIMPLEX KERATITIS Lid margin signs and conjunctivitis is more common as an initial presentation in Primary Herpes while dendrites are more common in recurrent infections. Primary herpes usually is limited to the anterior cornea. On rare occasions, a dramatic ocular presentation of a primary herpes simplex infection can take place. It is a self-limiting process. SYMPTOMS: Unilateral red eye, mild foreign body sensation, photophobia, burning. Mild to moderate fever. Seen primarily in children under the age of 15. SIGNS: Perioral skin vesicular eruptions around the mouth, lips Andenopathy preauricular, face and neck

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Lid margins, periocular area clear vesicular eruptions on an erythematous base, which may be single or clustered. Can develop scaling. Conjunctiva follicular conjunctivitis, severe cases hemes, pseudomembranes Cornea Epithelial involvement SPK, dendrites (stain well with Rose Bengal), infiltrates depending on severity. Keratitis is epithelial not stromal. DIFFERENTIAL DIAGNOSIS: Bacterial infections (follicles not papillae) Chlamydial infections (not mucopurulent) Viral or follicular conjunctivitis (EKC, PCF)(corneal involvement too severe, no rule of 8s) Herpes Zoster (Pain not severe. Midline not respected.) MANAGEMENT Skin and lid eruptions: Topical acyclovir ointment q4h x 21 days NO STEROIDS! Conjunctivitis No corneal involvement Prophylactic broad-spectrum antibiotic QID Gentamicin, Polytrim, etc Topical lubricants as needed Alternate warm and cold compresses Topical vasoconstrictors NO STEROIDS! Check every 5 days or so to watch the cornea. Keratitis Superficial epithelial keratopathy Topical Trifluridine (Viroptic) 4-5x/day Topical lubricants, vasoconstrictors Cool compresses NO STEROIDS! - not in the presence of replicating virus Monitor closely for dendritic formation 3 day intervals Dendritiform keratitis See treatment recommendations under RECURRENT HERPES SIMPLEX KERATITIS Monitor for complications, toxic side effects If epithelial defect do not resolve after several weeks, consider toxicity, nonherpetic mechanism, neurotrophic ulcers Switch to antibiotic ointment or lubricant and monitor closely RECURRENT HERPES SIMPLEX KERATITIS TRIGGERING FACTORS FOR REOCCURENCE: Direct mechanical or pharmacological insult to the neuron/tissue Trauma, Surgery, Epilation Systemic changes in immune modulators or neurotransmitters a) Infectious diseases, fever

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b) Steroids Extreme heat or cold SYMPTOMS: Similar to Primary Herpes infection. Depends on the severity of the inflammatory response. SIGNS: Unilateral red eye, follicular conjunctivitis, preauricular adenopathy, uveitis, keratitis, and various levels of corneal involvement. Cornea: 1) Dendritic lesions: Fine, lacy linear defect; branching patterns with terminal end buds. Surrounded by edema and SPK; Stains with Rose-Bengal. Trough-like so that fluorescein pools in the base of the lesion. Can be central or peripheral. Trophic Ulcer (metaherpetic or indolent ulcers): Can progress from dendrite. Round margins with rolled edges. May cause scarring as healing begins. Stains with Rose Bengal. - Is sterile, caused by viral damage upon the basement membrane retarding proper mechanical repair and epithelial overgrowth. Clue is persistent ulcer in patient under adequate treatment with antivirals. 3) Stromal Keratitis: Interstitial keratitis, Wessely immune ring. Type III hypersensitivity reaction. Irregular stromal infiltration occurs which may be partial to full thickness. Neovascularization into the deep stroma. Vessels can be full or ghost vessels. Epithelial and stromal edema. Infiltrative ring formations in the anterior cornea. KPs on the posterior cornea along with secondary guttata. Can develops scarring and lipid deposition. Most interstitial keratitis in nonsyphiltic patients is assumed to be HSV. Rule out corneal dystrophy. 4) Stromal Disciform Lesion: Type IV hypersensitivity reaction. Epithelium is usually intact with a dense solid disk of stromal edema surrounded by fine edema. Rarely, a Wessely immune ring may be seen. Uveitis is present. KPs may be viewed as well as folds in Descemets. MANAGEMENT: Oral Acyclovir may help to reduce reoccurrence rates. HERPES ZOSTER PRIMARY VARICELLA ZOSTER INFECTION (CHICKEN POX) SIGNS: Systemic Rash consists of macules progressing to papules and then to vesicles and eventually crusts. Lesions form initially of the trunk and then spread to the extremities. Scars form if bacterial superinfection occurs. Ocular

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Vesicle can occur on the lids, conjunctiva or cornea. Conjunctiva: Nonspecific follicular conjunctivitis. Cornea: Punctate or dendritic (see specifics under recurrent HZ) kerititis. SYMPTOMS: Fever, malaise MANAGEMENT: Supportive Oral Acyclovir Treat ocular side effects as listed under Herpes Zoster. HERPES ZOSTER (REACTIVATION) PREDISPOSING FACTORS FOR HERPES ZOSTER REACTIVATION: - Childhood varicella - Increasing age - Stress - Immunocompromised patients AIDS - Systemic steroids - Radiation Treatment - Chemotherapy - Trauma - Surgery, especially the trigeminal nerve - Heavy-metal poisoning - Leukemia - Lymphoma - Neoplastic disease - Tuberculosis - Syphilis SYMPTOMS: Prodrome: fever, malaise, pain and burning in the affected dermatome. Skin rash, facial and scalp tenderness or itchiness, red eye, headache, eye, photophobia, malaise, blurred vision, chills. Initially, a patient may experience chills, fever. In 2-3 days time, edema, erythema, and vesicular eruption occurs along the first division of the trigeminal nerve. SIGNS: Skin Acute vesicular or maculopapular rash and edema, which occur at the affected dermatome. Respect the midline. Cutaneous necrosis and crusting. Classic Hutchinsons Sign vesicle on end of nose which indicates nasociliary nerve involvement (higher risk of ocular involvement). Eyelid Acute vesicular or maculopapular rash and edema which occur at the affected dermatome. May see entropion, ectropion, madarosis, and trichiasis.

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Scarring can occur. Conjunctiva Nonspecific follicular conjunctivitis, hyperemia, possible pseudomembranes and symblepharon formation. Sclera Can develop scleritis diffuse or nodular, episcleritis, can develop posterior scleritis. Scleral thinning. May develop anterior uveitis, iris atrophy. Can develop secondary glaucoma. CORNEA: Acute: Punctate epithelial keratitis: Edematous, raised, epithelial cells in the periphery. May be adjacent subepithelial infiltrate. Pseudodendritic keratitis: Coalescence of corneal vesicles, forming pseudodendrites in the periphery. Blunt-ended and more superficial than dendrites in HSV, pseudodendrites are slightly raised, thick and have no terminal end bulbs. They stain with rose bengal and poorly with sodium fluorescein. Infiltrates in the anterior stroma. * Punctate keratitis and pseudodendrites indicate active viral replication is currently present in the epithelium. Delayed: Mucous plaque keratitis: Chronic keratitis which can develop weeks to many months after the skin eruption. They are raised, grayish-white lesions on the surface of the cornea. Stains well with Rose Bengal Disciform keratitis Round, defined area of diffuse stromal edema. Cerpiginous ulceration large geographical ulcer that can potentially lead to corneal melt. Chronic: Neurotrophic keratitis indolent ulcer with round margins and rolled edges, requires re-epithelializing Interstitial Keratitis 7) Other: Iritis (SEE UVEAL SECTION) Optic neuritis, optic atrophy, secondary glaucoma, acute retinal necrosis, progressive outer retinal necrosis, IIIrd, IVth, and VIth nerve palsy, sympathetic ophthalmia, neuroretinitis and vitritis. DIFFERENTIAL DIAGNOSIS Herpes Simplex Other causes of follicular conjunctivitis Temporal arteritis neuralgia Infectious Keratitis - infiltrative MANAGEMENT: Skin Lesions: a) Oral Acyclovir (Zovirax) 800 mg 5x/day, Famciclovir 500 mg tid, or Valacyclovir 1,000 mg tid x 7 10 days - Initiate as early as possible. For best results, within 72 hrs. of vesicle eruptions Caution in those with renal disease. b) Warm saline soaks with Burows solution until lesions start to

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crust over. c) Topical antibiotic ointments as prophylaxis once lesions crust over i. Broad spectrum polysporin, erythromycin BID Conjunctivitis: a) Prophylactic broad spectrum antibiotic (erythromycin ung) b) Cool compresses c) Lubricants Keratitis a) Punctate Epithelial Keratitis i. Artificial tears and bland ung ii. Use topical antivirals if herpes simplex cannot be ruled out. iii. Prophylactic topical antibiotics BID Pseudodendrite i. Artificial tears and bland ung Topical steroid (i.e. prednisolone acetate 1%) Use topical antivirals if herpes simplex cannot be ruled out. Disciform keratitis i. Steroids topical 1% prednisolone; Taper over several weeks v. Topical broad-spectrum antibiotics for prophylaxis QID dosage (especially if epithelial defect present) d) Indolent ulcers/Neurotrophic keratitis ii. Bland ointments iii. Bandage contact lens or pressure patching vi. Prophylactic antibiotics vii. No Steroids v. Non-responsive cases may require tarsorrhaphy, conjunctival flap, or transplant vii. Watch for perforation *SEE EACH SECTION FOR MORE INFORMATION Uveitis Cycloplegics Topical corticosteroids (monitor carefully with epithelial defect.) Episcleritis/ Scleritis Topical or systemic NSAIDS or topical or systemic corticosteroids. Elevated IOP If taking topical steroids consider changing to an agent which is less likely to effect IOP Treat with beta-blocker or topical CAI (decrease

HUMAN IMMUNODIFICIENCY VIRUS (HIV) AND ACQUIRED IMMUNE DIFFICIENCY SYNDROME (AIDS)

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SYSTEMIC HIV RELATED DISEASES AND OPPORTUNISTIC INFECTIONS Candidiasis Cryptococcus Cytomegalovirus Histoplasmosis Hodgkins disease HIV Encephalopathy Karposi Sarcoma Pneumocystis carinii pneumonia Syphilis Toxoplamosis (Meningitis) Tuberculosis OCULAR HIV RELATED DISEASES AND OPPORTUNISTIC INFECTIONS Anterior Segment Molluscam contagiosum Herpes zoster ophthalmicus (See Lid, Corneal sections) Karposi Sarcoma Keratocinjunctivitis Sicca (poor tear film production) Gonococcal conjunctivitis (Gonorrhea also sexually transmitted) Inclusion conjunctivis (Chlamydia also sexually transmitted Ocular manifestations of Syphilis Ocular manifestations of Tuberculosis Posterior Segment Nonspecific HIV Microvasculopathy Intraretinal hemorrhages and cotton wool spots CMV (SEE RETINAL SECTION) Ischemic macular edema Acute retinal necrosis syndrome (ARNS) Progressive outer retinal necrosis (PORN) Toxoplasmosis Retinopathy Papilledema Chorioditis Ocular manifestations of Syphilis Ocular manifestations of Tuberculosis LYME DISEASE OCULAR SIGNS: Stage one: conjunctivitis, periorbital edema Stage two: Cranial nerve palsies III, IV,V,VI,VII *Optic Nerve: AION, Disc edema, Optic neuritis, Optic atrophy, Big blindspot, syndrome.

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*Retina: Vasculitis, hemes, exudative detachments, retinitis, tractional tears, macular edema, artery occlusion. *Uvea: Choroiditis, iridocyclitis, pars planitis *Pupils: Argyll-Robertson, Bilateral tonic pupils, Horners *Cornea: Exposure keratitis, Cogans *Other: Vitritis, Panophthalmitis, Blepharospasm Stage three: Interstitial keratitis, orbital pseudotumor, cortical blindness, episcleritis, Ophthalmoplegia. SYSTEMIC SIGNS: Stage one (acute localized infection): Headaches, erythema migraines, fever, adenopathy, malaise, fatigue, lethargy, arthralgia, myalgia, etc. Stage two (acute localized infection): Facial palsy (unilateral or bilateral), rash, migratory pain in the joints and muscles, arthritic attacks, osteomyelitis, myositis, aseptic meningitis, cranial and peripheral neuropathy, encephalitis, athrioventricular block, bradycardia, tachycardia cardiomegaly, meningopolyneuritis. Stage three (late disseminated disease): Progressive encephalomyelitis (spinal and cerebral), peripheral polyneuritis, chronic fatigue, memory lapse, pyopathy, chronic arthritis with possible joint erosion, skin atrophy. MANAGEMENT: Ocular treatment: *Treat uveitis with appropriate steroids and cycloplegics depending on severity of presentation. *Corneal presentations may require lubrication or prophylactic antibiotics. *Retinal problems are managed in their regular fashion. SYPHILIS ACQUIRED SYPHILIS Ocular Signs: Secondary syphilis: Iridocyclitis, retinal vasculitis, optic neuritis, chorioretinitis, optic atrophy, ptosis, conjunctivitis, dacryoadenitis, dacryocystitis, interstitial keratitis, nodules on the conjunctiva, lens dislocation, papillitis, and fleshy, pink nodules near the iris sphincter. Tertiary Syphilis: Neurosyphilis can present in a variety of fashions. It can involve the cerebral cortex and cause papilledema, confusion, hemiparesis, epilepsy, dizziness, EOM paresis, and tremors may manifest. Argyll-Robertson pupils accompany this disorder. Other ocular signs: optic atrophy, chronic iritis. MANAGEMENT: Serological Testing:

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*RPR or VDRL - screening *FTA-ABS or MHA-TP or TPI temporal tests *HIV testing, if immunosuppression suspected. Ocular external and internal examination -Rule out Interstitial keratitis, Chorioretinitis, Nerve involvement. Active inflammation *Cycloplegics *Topical steroids Pred Forte QID or as needed Early Latent or Tertiary syphilis: *Benzathine Penicillin G IM *Tetracycline or Erythromycin Neurosyphilis: *Penicillin G IV followed by Benzathine Penicillin G. IM . OR Aqueous Procaine Penicillin G IM daily plus Probenicid followed by Benzanthine Penicillin G IM or Benzanthine Pen G IM or Doxycycline PO BID CONGENITAL SYPHILIS Some infants may develop meningitis, hydrocephalus, choroiditis, retardation, and convulsions. Most Characteristic Signs: HUTCHINSONS TRIAD Acute, bilateral interstitial keratitis, eighth nerve deafness, Hutchinsons teeth. OCULAR SIGNS: 1. Interstitial keratitis acute and bilateral in nature. Causes stromal edema and vascular infiltration. (See Interstitial keratitis under Cornea). 2. Chorioretinitis: Presents as salt and pepper fundus. Due to hyperpigmentation adjacent to atrophic areas. May be seen in eyes with bout of interstitial keratitis or no history of inflammation. May be segmental or generalized. Vitritis may be present. 3. Optic Atrophy may lead to blindness. 4. Anterior uveitis may be present from birth. MANAGEMENT: Serological testing: *RPR or VDRL as screening tests. *FTA-ABS or MHA-TP for treponemal testing. *May have false positives which may convert back over time. *May require lumbar puncture. Scrapings from skin and/or mucosal lesions can aid in diagnosis Treat Interstitial keratitis and inflammation, if present. *See Interstitial keratitis under Cornea.

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INFLAMMATORY DISORDERS SARCOID OPHTHALMOPATHY In the presence of a granulomatous uveitis tuberculosis and syphilis should be ruled out. In children, juvenile rheumatoid arthritis should be ruled out (serum ANA titers). CLINICAL PICTURE: Anterior segment: Anterior uveitis, mutton fat keratic precipitates, iris nodules and posterior synechiae. Cataracts and glaucoma can occur secondary to the condition. Posterior segment: Posterior uveitis, vitreal inflammation is characterized by white fluffy, vitreal opacities. Periphlebitis is the most common fundus finding. Periphlebitis appears as a creamy-white exudation around vessels, most commonly in the equatorial region. Multifocal choroiditis can occur. Disc swelling can occur. Neovascularization and subretinal neovascularization also occurs. MANAGEMENT: The uveitis is treated with cycloplegics and topical steroids. Periocular steroid injections can also be used in severe cases. Active fundus lesions can usually be controlled with 60 mg of prednisone daily followed by appropriate taper. Laser photocoagulation can be performed in cases of neovascularization. Systemic condition can be treated with oral steroids. Sarcoid is characterized by exacerbations and remissions. MUSCULAR DISORDERS MYASTHENIA GRAVIS (MG) Major ophthalmologic symptoms are: Alternating or transient ptosis, diplopia which may also be transient or worse at the end of the day. SEE OCULOMOTOR DISORDERS SECTION NEUORLOGICAL DISORDERS MULTIPLE SCLEROSIS Ocular Findings:

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Optic Neuritis (SEE OPTIC NEURITIS SECTION) Oculomotor (INO, BINO, impaired pursuits/saccades, nystagmus) Uveitis Uthoffs sign PHAKOMATOSES STURGE-WEBER SYNDROME OCULAR FEATURES: 1) Diffuse choroidal hemangioma catsup fundus 2) Glaucoma (facial hemangioma of the superior lid increases risk of glaucoma) 3) Buphthalmos (if glaucoma present at less than 3 yrs old) 4) Iris heterochromia 5) Retinal detachment 6) Retinal pigment epithelial atrophy 7) Port wine stain nevus flammeus on the eyelid 8) Choroidal coloboma 9) Retinoblastoma 10) Anomalous vessels in the conjunctiva and sclera WORK-UP: Complete general and ophthalmic examinations CT scan or MRI of the brain TREATMENT: If present, treat glaucoma in the same manner as Primary Open Angle Glaucoma Treat sight threatening serous retinal detachments SKELETAL CONNECTIVE TISSUE DISORDERS MARFANS SYNDROME (dystrophia mesodermalis congenita-typus Marfanus, arachnodactyly) GENERAL AND SYSTEMIC FEATURES: *Typical presentation: tall person with long, thin extremities, long face and hypermobile joints. *Classic triad includes subluxated lenses, skeletal anomalies, and cardiovascular disease OCULAR ABNORMALITIES: 1. Ptosis 2. Pupil miosis secondary to hypoplasia of the dilator muscle of the iris 3. Heterochromia iridis 3. Megalocornea 5. Keartoconus 6. Blue scleras 7. Colobomas of the iris and choroid 8. Strabismus 9. Ectopia lentis

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-found in 80% of the patients -bilateral -dislocation is usually superiorly and temporally -microspherophakia and cataract formation may occur 10. Retinal detachment and peripheral pigmentary retinopathy can develop. Peripheral degeneration such as lattice and white without pressure. 11. Glaucoma (10% of the patients) TREATMENT: 1. Supportive, orthopedic procedures and devices 2 Cataract extraction, if indicated 3. Retinal detachment surgery, if indicated PSEUDOXANTHOMA ELASTICUM Cutaneous findings: peau dorange (yellow coalesed papules) OCULAR INVOLVEMENT: *Angioid streaks (preceded by fundus peau dorange and salmon spots) (Groenblad-Strandberg syndrome) *Also see drusen of the fundus, optic nerve drusen, peripheral punched-out lesions *Ocular complications of angioid streaks -Disciform macular degeneration due to choroidal neovascularization -Subretinal hemorrhages -Retinal pigment epithelial atrophy -Loss of central vision *differential diagnosis of angioid streaks -Pseudoxanthoma elasticum (Groenblad-Strandberg syndrome) -Pagets disease -Sickle-cell disease -Ehlers-Danlos syndrome -Acromegaly -Lead poisoning -Pituitary tumor -Senile (actinic) elastosis -Hypercalcemia OCULAR TREATMENT: -Focal laser photocoagulation if choroidal neovascularization develops outside of the foveal avascular zone

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MYDRIATICS AND CYCLOPLEGICS

SYMPATHOMIMETICS Alpha-agonists COCAINE See topical anesthetics. HYDROXYAMPHETAMINE MECHANISM OF ACTION: Releases norepinephrine from adrenergic nerve terminals. Inhibits the action of MAO inhibitors and reduces re-uptake of norepinephrine into adrenergic nerve terminals. Has a significant effect on accommodative status. Maximal mydriasis occurs in 60 minutes with duration of 6 hours. CLINICAL USES: Dilated fundus examinations, fundus photography, differentiation of preganglionic postganglionic lesions in patient with Horner's syndrome, from dilation of patients with narrow anterior chambers angles. TOXICITY: Ocular - Ocular irritation rare. Systemic - No reported cases of systemic involvement. DISPENSING INFORMATION: 1.0% solution. CONTRAINDICATIONS: Same as phenylephrine, but due to its ineffectiveness in postganglionic denervation and its tachyphlaxis properties, it is safer in high risk patients. PHENYLEPHRINE (0.12%, 2.5 % and 10% solution) MECHANISM OF ACTION: Acts directly on alpha receptors and has a negligible effect on beta receptors. Causes vasoconstriction in blood vessels and acts on the radial muscle of the iris (2.5 and 10%). 0.12%: Causes vasoconstriction with little or no mydriasis. CLINICAL USES: 2.5 and 10%: decongestant, vasoconstriction, and mydriasis (prior to surgery, for ophthalmoscopic examination, to break posterior synechia, ocular decongestion). TOXICITY: Ocular - Keratitis, transient discomfort, allergic conjunctivitis, blurring of vision, rebound hyperemia after chronic use, pigment dispersion with intraocular pressure spikes.

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Systemic Palpitations, tachycardia, arrhythmia, hypertension, occipital headache, rupturing of aneurysms. 10%: Major concern is rise in systemic blood pressure. Significant elevation in blood pressure is rare but may occur. Caution in elderly and small children. Carefully monitor blood pressure in these patients. Three elderly patients died following instillation of 10% solution were positive for cardiovascular disease (no reported deaths with 2.5%). CONTRAINDICATIONS: 10% solution - use with caution in patients with cardiovascular disease, aneurysms, and insulin-dependent diabetes mellitus. Caution in patients taking MAO inhibitors, (tricyclic antidepressants, guanethidine, methyldopa, and reserpine) beta blockers and anesthetics. Use with caution in patients predisposed to angle closure glaucoma, low birth weight infants and elderly patients with atherosclerotic disease or cerebrovascular disease. PARASYMPATHOLYTICS Block effects of acetylcholine at muscarinic receptor sites in iris sphincter and ciliary body producing mydriasis and cycloplegia. Effects potentiated with use of antihistamines, phenothiazines, and tricyclic antidepressants. ATROPINE (0.5 2.0% solution and 1% ointment) Most potent and longest duration mydriatic and cycloplegic agent available. Depending on dosage: mydriasis for 7-10 days and cycloplegia for 7-12 days. Heavily pigmented eyes: slower onset, prolonged duration of cycloplegia and less mydriasis. CLINICAL USES: Treatment of acute inflammation of iris/uveal tract, cycloplegic refractions in children, amblyopia therapy. TOXICITY: Ocular - Stinging and burning on instillation, allergic contact dermatitis, elevation of intraocular pressure, allergic conjunctivitis, blurred vision and keratitis. Systemic - Diffuse cutaneous flush, dry mouth/skin, hallucination, photophobia, urinary retention, tachycardia, and in severe circumstances respiratory depression followed by death. CONTRAINDICATIONS: Care must be taken with narrow anterior chamber angles, in children and patients with Down's syndrome, brain damage or spastic paralysis. Treatment of overdose is physostigmine. CYCLOPENTOLATE (0.5%, 1.0% and 2.0% solution) Depending on dosage: mydriasis for 1 day and cycloplegia for 0.25 days. Less mydriasis

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and cycloplegia in dark colored irides. CLINICAL USES: Cycloplegic refraction in all age groups, dilated fundus examinations, treatment of uveitis. TOXICITY: Ocular - Keratitis, conjunctival hyperemia, elevation of intraocular pressure in glaucoma patients, lacrimation, photophobia. Systemic - Dose related. More central nervous effects than atropine. Hallucinations, drowsiness, ataxia, dysphagia, psychotic reactions/behavioral disorders, dryness of mucous membranes, grand mal seizures, tachycardia. CONTRAINDICATIONS: Use with caution in children, debilitated patients, and patients with narrow anterior chamber angles. HOMATROPINE (2.0% and 5.0% solution) Depending on dosage: mydriasis and cycloplegia for 1-3 days. CLINICAL USE: Treatment of uveitis. TOXICITY: Ocular and systemic effects same as atropine. No death reported. CONTRAINDICATIONS: Same as atropine. SCOPOLAMINE (0.25% solution) Mydriasis for 3-7 days and cycloplegia for 5-7 days. CLINICAL USES: Treatment of uveitis, cycloplegic refractions in children, pre and postoperatively in the treatment of iridocyclitis. TOXICITY: Ocular and systemic effects same as atropine. CONTRAINDICATIONS: Same as atropine. TROPICAMIDE (0.5% and 1.0% solution) Depending on dosage: mydriasis and cycloplegia for 6 hours, with recovery of accommodation first. CLINICAL USES:

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Drug of choice for ophthalmoscopy and fundus photography. TOXICITY: Ocular Transient stinging, elevation of intraocular pressure in glaucoma patients. Systemic - Extremely rare. CONTRAINDICATION: Narrow anterior chambers angles. ALPHA-ADRENERGIC ANTAGONISTS DAPIPRAZOLE (REV-EYES) Dapiprazole is a alpha-adrenergic antagonist which produces miosis and reduces intraocular pressure. (Blocks alpha receptors in iris dilator muscle.) DOSAGE: Concentration: 0.5% solution when reconstituted. Typical dosage: 2 drops followed 5 minutes later by 2 more drops. Packaged as a lyophilized powder which is reconstituted by the practitioner. The solution should be used within 3 weeks. INDICATIONS: 1) Reversal of diagnostic pupillary dilation CONTRAINDICATIONS: When pupillary constriction is undesirable, i.e. acute anterior uveitis. SIDE EFFECTS: 1) Conjunctival hyperemia 2) Burning, irritation 3) Mild ptosis TOPICAL ANESTHETICS Increase the effects of succinylcholine and sympathomimetics. Decrease the effects of adrenergic blockers and sulfonamides. MECHANISM OF ACTION: Act on cell membrane of nerve tissue, where they alter the cell membrane permeability to ions. Combination of two or more anesthetics does not produce an additive effect, but increases the chance of toxic reaction. Patients should never by permitted to self-medicate with any anesthetic due to corneal disruption. NOTE: Topical anesthetics (except Cocaine) are used prior to instillation of mydriatic and

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cycloplegic agents for various reasons: Reduce stinging of cycloplegics. Anesthetic reduce tear flow allowing prolonged cycloplegic contact time. Reduce blink rate allowing prolonged cycloplegic contact time. Anesthetic produces a transient keratopathy which increases the adsorption of the cycloplegic. Topical anesthetics contain preservatives, which have anti-bacterial and anti-fungal properties. Single dose containers without preservative should be used prior to culturing of ocular structures to obtain accurate results. BENOXINATE (0.4% concentration in combination with 0.25% sodium fluorescein (Fluress)) CORNEAL ANESTHESIA Onset: 10 seconds Duration: 10 minutes CLINICAL USES: Applanation tonometry; Sodium fluorescein fluoresces more with benoxinate than any other topical anesthetic. TOXICITY: Ocular - Same as tetracaine Systemic - One case of grand mal seizure possibly associated with benoxinate use. CONTRAINDICATIONS: Known allergy to any component COCAINE (Cocaine HCl and Cocaine Viscous 4 and 10%, Cocaine HCl powder) Clinically, not used often due to ocular/systemic toxicities. MECHANISM OF ACTION: Blocks initiation and conduction of nerve impulses and causes vasoconstriction. Causes sloughing of the corneal epithelium which can results in clouding,pitting and ulceration of the cornea. Peak effect: 1-5 minutes. Duration 30-60 minutes INDICATIONS: Used for corneal epithelial debridement, forced duction testing, and diagnosis of Horner's syndrome of oculosympathetic paresis. SIDE EFFECTS: Ocular - Significant corneal epithelial defects, corneal clouding and ulceration, dilation of pupil. Systemic - Rapid, irregular pulse, decrease heart rate, nausea, vomiting, convulsions, headache, restlessness all with dose of 20mg.

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Death secondary to respiratory arrest with 1.2g DISPENSING INFORMATION: Administration limited to in office use. Solutions can be prepared by diluting the powder from the cocaine (usual 1-4%). Concentrations >4% not advisable due to systemic complications. CONTRAINDICATIONS: 1) Systemic hypertension 2) Patients taking: adrenergic agonists guanethidine, reserpine, tricyclic antidepressants, methyldopa, or monoamine oxidase inhibitors. 3) The concomitant use of epinephrine or phenylephrine is contraindicated. 4) Patients predisposed to angle-closure glaucoma. PROPARACAINE (0.5% solution and 0.5% solution in combination with 0.25% sodium fluorescein.) CORNEAL ANESTHESIA Onset: 10 seconds Duration: 10 minutes CLINICAL USES: General purpose: contact tonometry, gonioscopy, suture removal, nasolacrimal duct probing, pachymetry and a scan ultrasonography. TOXICITY: Ocular - Corneal stippling, transient corneal edema, conjunctival hyperemia, lacrimation, allergic conjunctivitis, edematous lids. Systemic - Non reported. CONTRAINDICATIONS: Known allergy to any component. TETRACAINE (0.5% solution and ointment) CORNEAL ANESTHESIA Onset: 10-20 seconds Duration: 10 minutes Ointment provides longer anesthesia due to increase tissue contact time. CLINICAL USES: Solution - contact tonometry, gonioscopy, suture removal, nasolacrimal duct probing. Ointment: ERG testing, cauterization of corneal or conjunctival lesions. TOXICITY: Ocular - Transient stinging, corneal epithelial compromise, allergic conjunctivitis. Systemic - none reported but possible central nervous system involvement with doses over 1.5 mg/kg body weight.

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CONTRAINDICATIONS: Known allergy to tetracaine TOPICAL ANTIINFLAMMATORY AGENTS NON STEROIDAL ANTI-INFLAMMATORY AGENTS (NSAIDS) MECHANISM OF ACTION Block formation of inflammatory and pain mediators by inhibition of the cyclooxygenase pathway, which ultimately blocks the production of prostaglandins. They DO NOT inhibit the production of lipoxygenase-catalyzed production of leukotrienes. They have analgesic, antipyretic and anti-inflammatory activity. TOPICAL NSAIDS DICLOFENAC SODIUM 0.1% (VOLTAREN 0.1%) CLINICAL USES Treatment of postoperative inflammation following cataract extraction and photophobia following refractive surgery (Off labeled use: anti-inflammatory following ALT, treatment of seasonal allergic conjunctivitis, and pain associated with RK and PRK). Clinical trial concluded that the decrease in corneal sensitivity in normal human corneas was more pronounced and longer lasting with Voltaren 0.1% compared to ketorolac. DOSAGE: 1 drop 4 times a day TOXICITY Burning and stinging upon instillation, keratitis (mostly after cataract surgery), elevated IOP (mostly after surgery), dry eye (mostly after refractive surgery), discharge, corneal deposits or lesions. KETOROLAC TROMETHAMINE 0.4% (ACULAR LS) Original formulation of Acular was 0.5% Solution CLINICAL USES FDA approved for the treatment of postoperative inflammation as well as ocular allergy Off-label uses: GPC,VKC Approved for age 3 and older DOSAGE: 1 drop 4 times per day

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TOXICITY Burning and stinging upon instillation ( however much more tolerable compared to 0.5% formulation) BROMFENAC 0.09% (XIBROM) CLINICAL USES FDA approved for treatment of postoperative inflammation. DOSAGE: BID DOSING Well tolerated. NEPAFENAC 0.1% ( NEVANAC) New topical NSAID manufactured by ALCON. Nevanac is enzymatically converted to amfenac sodium which inhibits cycloxygenase. DOSAGE: TID dosing CLINICAL USES FDA approved for the treatment of postoperative inflammation Off-label use for treatment of diabetic macular edema with great future potential. FLURBIPROFEN (OCUFEN 0.03%) CLINICAL USES Inhibition of intraocular miosis, (off-label uses: treatment of inflammation following cataract or glaucoma laser surgery and uveitis syndromes.) DOSAGE: 1 drop every 30 minutes beginning 2 hrs. before surgery TOXICITY Burning and stinging upon instillation ALWAYS EXERCISE CAUTION WHEN THERE IS PREEXISTING CORNEAL EPITHELIAL COMPROMISE DUE TO THE RISK OF CORNEAL TOXICITY AND MELTING.

CORTICOSTEROIDS
Corticosteroids suppress pain, redness, and swelling associated with inflammation of tissue. They also demonstrate the ability to reduce collagen formation which can lead to scarring. Corticosteroids have the ability to reduce inflammation from various causes including radiation, mechanical, chemical, immunologic, and infectious. It is important to remember that corticosteroids suppress inflammation without affecting the underlying disease process. This can cause a subjective and objective clinical improvement in the presence of advancing disease. Steroids are usually contraindicated in infectious processes

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because they are not bactericidal, anti-viral or anti-fungal. Where the benefits outweigh the risks, steroids can be used concomitantly with anti-infective agents to decrease the inflammatory response while the anti-infective agent treats the condition. MECHANISM OF ACTION: Block the cyclooxygenase and lipo-oxygenase pathways in the inflammatory cascade inhibiting prostaglandins and leukotrienes. CLINICAL INDICATIONS: Topical: Used for a variety of inflammatory conditions which affect the lid, bulbar and palpebral conjunctiva, cornea, and anterior segment disease: Allergic conjunctivitis Superficial punctate keratitis Anterior uveitis Steroids reduce inflammation, pain, and reduce risk of synechial formation. Herpes Zoster keratitis Herpes Simplex Keratitis - Controversial. Do not use if epithelium is involved. Usually used only when the corneal stroma has been involved and the epithelium is intact but must also use antiviral agents concurrently. Infective conjunctivitis only if benefits outweigh risks Episcleritis - Reduces ocular discomfort Chemical, radiation, or thermal burns to the cornea- reduces scarring. Phylctenular keratoconjunctivitis with appropriate antimicrobial agent. To prevent graft rejection after keratoplasty Postoperative inflammation Periocular/intravitreal injections Uses include treatment of anterior/ posterior uveitis, endophthalmitis, chorioretinitis, and optic neuritis. New indications for the treatment of diabetic macular edema. Systemic Uses include treatment of posterior uveitis, endophthalmitis, optic neuritis, chorioretinitis and sympathetic ophthalmia. Principles of Therapy Appropriate route of administration must be determined (topical, periocular, systemic, or multiple routes). Treat immediately and with high enough dosage to suppress inflammation. Refine dosage with frequent reevaluation of clinical course of the process. Long term, high dose therapy needs to be discontinued with a gradual taper. Generally, inflammation of lids, conjunctiva, cornea, iris, and ciliary body can be suppressed with topical application. Topical steroid therapy is preferred for anterior segment disease, as opposed to systemic or periocular. More severe uveitis may need periocular steroids, in addition to topical therapy, administered by route of subconjunctival, sub-Tenon's, or retrobulbar injections. Studies have shown that retrobulbar injection provide high steroids concentrations for the sclera, choroid, retina, and vitreous for at least one week. Limitations of this mode of therapy include pain on injection and

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risk for acute increased intraocular pressure.

TOPICAL CORTICOSTERIODS DEXAMETHASONE SODIUM PHOSPHATE - SOLUTION 0.1%, OINTMENT 0.05% DEXAMETHASONE SUSPENSION - 0.1% (MAXIDEX) Less effective than prednisolone Tend to elevate IOP more than other topical steroids Used to treat mild to moderate inflammation Rarely used in clinical care FLUOROMETHALONE ACETATE - SUSPENSION 0.1% Flarex/eFlone Less likely to increase IOP than prednisolone Greater intraocular penetration and efficacy than fluoromethalone alcohol Used to treat moderate ocular inflammation FLUOROMETHALONE ALCOHOL - SUSPENSION 0.1%, 0.25% OINTMENT 0.1% FML Less likely to increase IOP than prednisolone Used to treat mild to moderate ocular inflammation LOTEPREDNOL ETABONATE- 0.5% SUSPENSION (LOTEMAX) Similar efficacy and does not tend to elevate IOP as much as pred acetate Approved for postsurgical ocular inflammation Site specific; Clinically potent, but quickly metabolized; therefore, fewer side effects (less likely to increase IOP); ester based steroid. Used to treat moderate to severe ocular inflammation LOTEPREDNOL ETABONATE- 0.2% SUSPENSION (ALREX) Site specific; Clinically potent, but quickly metabolized; therefore, fewer side effects (less likely to increase IOP) Used to treat allergic conjunctivitis PREDNISOLONE ACETATE SUSPENSION 0.12%, 0.125%, 1.0% Prednisolone acetate 1%=Pred Forte 0.12%= Pred Mild Has the greatest anti-inflammatory efficacy of all topical ophthalmic steroids Penetrates cornea and anterior chamber; used to treat anterior chamber reactions and Moderate to severe ocular inflammation (1%) PREDNISOLONE SODIUM PHOSPHATE SOLUTION 0.125%, 1.0% Does not penetrate cornea as well as acetate

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Used to treat moderate to severe ocular inflammation RIMEXALONE - 1.% SUSPENSION (VEXOL) Similar efficacy and does not tend to elevate IOP as much as prednisolone acetate. Approved for postsurgical ocular inflammation. Used to treat moderate to severe ocular inflammation. Due to differences in corneal penetration, suspensions (acetate and alcohol derivatives) penetrate the cornea better and are typically more potent than solutions (phosphate derivatives). Duration of therapy is dependent on type of lesion and extent of condition. The therapy may need to be continued at reduced levels for at least several days following resolution of symptoms. Tapering therapy may prevent relapses. Empirically, the dosage of topical steroid recommended for severe inflammation is 1 drop every 1 to 2 hours for 1 to 2 days. After that time, 3 to 4 times a day is usually sufficient to suppress the inflammation. Ointments are typically administered in a thin coat 3-4 times per day. STEROIDS IN REVIEW TOXICITY: Ocular Posterior subcapsular cataracts - Mostly seen following systemic therapy, although some reports of topical steroid induced PSC's exist. Chance of developing PSC seems to be dose related. Adults will not develop PSC until after 1 year of steroid use, although childhood PSC can develop sooner. Diabetics are predisposed to develop PSC and Hispanics are more likely to develop steroid induced PSC than Caucasians or blacks. The PSC will not reverse after therapy. Intraocular pressure elevation. Can occur with topical, periocular, or systemic administration. Systemic therapy causes less IOP elevation probably because the ocular concentration is reduced. The IOP will usually return to pre-treatment levels within three weeks of termination of therapy. Pressure rise can occur in healthy eyes, but the elevation is normally greater in patients with open angle glaucoma and their children. Infection - There is an increased susceptibility to infectious agents with corticosteroid therapy. Secondary infections can occur. An exacerbation of fungal or viral infections can occur. The risk of superinfection is lower if simultaneous anti-infective agents are used. Cornea and sclera - corticosteroid therapy can lead to corneal and scleral thinning with possible perforation of the globe. CONTRAINDICATIONS/Cautions: Use caution and consider concomitant anti-infectives with infections such as: herpes simplex (extreme caution), fungal diseases of ocular structures, and viral diseases of the cornea and conjunctiva (vaccinia and varicella), mycobacterial infections, bacterial infections (especially corneal ulcerations) and after uncomplicated removal of a superficial foreign body. DO NOT prescribe topical steroids in the patient with EPITHELIAL HSV keratitis. Steroids may be indicated in the treatment of STROMAL HSV keratitis per the HEDS Study Group.

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ANTIBACTERIAL DRUGS Ocular indications: Prophylaxis or treatment of infections caused by bacteria. Infections may involve intraocular structures and/or superficial structures of the eye such as the cornea and conjunctiva. Considering the likely pathogens, choose an antibiotic to treat the infection whose activity spectrum and mechanism of action would be effective against the likely pathogen. A variety of antibiotic agents can be used to treat intraocular infections through subconjuctival or intravitreal injections or through intravenous use. The following is a summary primarily focusing on the topical antibiotics and their uses. Common topical indications include: conjunctivitis, blepharitis, keratoconjunctivitis, corneal ulcers, dacryocystitis, and prophylaxis against infection after epithelial defect. Recommended dosage of topical agents: mild to moderate infections 1 to 2 drops of topical solution every 4 hours OR 1/4" ribbon of topical ointment, BID-TID for 7 to 10 days severe infections up to 2 drops every hr and inclusion conjunctivitis oral dosage for adults or older child: 250mg or 333 mg QID for 2 to 3 weeks FLUOROQUINOLONES (QUINOLONES) MECHANISM OF ACTION inhibit DNA synthesis during bacterial replication by preventing the action of DNA gyrase SPECTRUM broad spectrum antibiotic with great potency active against most gram(-) bacteria including pseudomonas and many gram (+) bacteria including staphylococcus and some anaerobes bacterial resistance is low Superficial white precipitates can develop in the cornea during treatment for corneal ulcers with topical Ciloxan. (These precipitates have not been reported to effect corneal healing and resolve after discontinuation of ciprofloxacin). To decrease the chance of resistance developing, not typically used to treat mild conjunctival or corneal infections. CIPROFLOXACIN 0.3% (CILOXAN) solution and ointment CLINICAL USE Used to treat bacterial corneal ulcers, moderate to severe conjunctivitis. Indicated as a prophylactic antibiotic against moderate to severe ocular infection.

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DOSAGE: Ulcer: 2 gtt q15 min for 6H, 2gtt q 30 min for 18H, 2 gtt q 1 H for 24H Conjunctivitis: 1-2 gt q 2h for 2 days then q 4h or ung tid x 2d then bid x 5d. LEVOFLOXACIN 0.5% ophthalmic solution (Quixin) CLINICAL USE: Bacterial conjunctivitis Dosage: 1-2 drops q 2-4 h. OFLOXACIN 0.3% (Ocuflox) CLINICAL USE: bacterial conjunctivitis, bacterial corneal ulcer. DOSAGE: Ulcer: 1- 2gtt Q 30 min for 2 d, 2 gtt Q 1 H for 3-7 d Conjunctivitis: 1-2 gt q 2h for 2 days then q 4h. Fourth Generation Fluoroquinolones: Offer the advantage of better gram-positive coverage and less resistance development. GATIFLOXACIN 0.3%-ZYMAR-PRESERVED MOXIFLOXACIN 0.5% SELF PRESERVED-VIGAMOX/higher concentration/more physiological pH (6.8) Vigamox effective in the treatment of bacterial conjunctivitis. Dosage: 1gt TID for 7 days. Vigamox has been shown to achieve greater ocular penetration compared to Zymar. Typically, most corneal specialists will treat infectious keratitis with fortified antibiotics, Cephazolin and Tobramycin. However, monotherapy with topical fluoroquinonlones has been shown to be nearly as effective in the treatment of infectious keratitis, and is commercially available. This is important for early treatment while fortified antibiotics are being formulated ( takes several hours). Most large, central vision threatening corneal ulcers should be referred to a corneal subspecialist immediately! AMINOGLYCOSIDES Gentamicin/Tobramycin/Neomycin highly effective against infections caused by gram-negative bacilli and some gram-positive organisms bacterial resistance to the aminoglycosides by gram-negative bacilli exist cross-resistance exists aminoglycosides must be administered separately from penicillins to avoid inactivation Less expensive thean fluoroquinolones Effective for most garden variety cases of bacterial conjunctivitis Tobramycin exhibits less corneotoxicity compared to Gentamicin GENTAMICIN Topical formulations: solution 3mg/ml and ointment 3mg/g Genoptic

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Gentacidin Gentak Generic preparation Broad spectrum of activity against gram positive and negative organisms Active against Pseudomonas Not effective against some forms of Strep Treatment of bacterial corneal ulcers Usually used in combination with a fluroquinolone Intraocular or subconjuctival injections used as initial treatment for endophthalmitis Used in combination with a cephalosporin or a penicillinase producing staphylococci resistant penicillin (systemically, topically, subconjunctivally, or intravitreally) SIDE EFFECTS: minor irritation punctate epithelial keratopathy delayed would healing pseudomembranous conjunctivitis bulbar conjunctival hyperemia periocular skin and conjunctival paresthesia sensitization (~ 50% of patients allergic to neomycin become allergic to gentamicin) NEOMYCIN OCULAR USE: Used topically in combination with other agents as a solution or ointment.(AK-Spore, Neosporin, Maxitrol, Generic) most toxic aminoglycoside antibiotic topical ocular application in combination only sensitization occurs frequently; 4% develop contact dermatitis TOBRAMYCIN available as a topical ophthalmic solution or topical ophthalmic ointment Tobrex 0.3% Generic antibacterial activity and pharmacokinetic properties essentially identical to gentamycin, except tobramycin is not effective against N. gonorrhea. Broad spectrum antibiotic treatment of bacterial corneal ulcers usually used in combination with a fluroquinolone SIDE EFFECTS: Topical administration tearing burning

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photophobia eyelid edema conjunctival hyperemia and chemosis punctate epithelial erosions

BACITRACIN Ointment form available for topical use (AK-Tracin) MECHANISM OF ACTION: inhibits bacterial cell wall synthesis bactericidal against gram positive organisms staphylococci streptococci Clostridium difficile inactive against gram negative organisms except active against Neisseria AVAILABILITY: available as a single entity product (ointment only) or as a component of a fixedcombination product with polymyxin B (Polysporin) and neomycin (Neosporin) INDICATIONS primarily used topically to treat skin mucous membrane infections caused by gram positive bacteria Drug of choice for treating bacterial blepharitis Very effective against staphylococcal bacteria; main pathogen leading to blepharitis ERYTHROMYCIN Ilotycin, Generic 0.5% ointment MECHANISM OF ACTION: inhibits bacterial protein synthesis bacteriostatic SPECTRUM OF ACTIVITY effective primarily against gram-positive organisms prophylaxis with N. gonorrhea and Chlamydia trachomatis OCULAR USES: staphylococcal infections of the eyelid 1\4 ribbon of erythromycin ointment Q hs or more often if severity warrants adjunctive therapy: warm compresses and lid scrub with dilute baby shampoo prophylaxis of neonatal neonatorum due to chlamydia trachomatis and Neisseria gonorrhoeae alternative to silver nitrate recommended dosage: 0.5 to 1.0 cm ribbon of erythromycin ointment applied to each conjunctival sac

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*Oral drug: treating chlamydia trachomatis infections in infants and children safe use for: pregnant women nursing mothers children under 8 years of age *chlamydial ophthalmia neonatorum oral dosage: 25 mg/Kg body weight every 12 hours for at least 2 weeks *alternative drug to tetracycline for the treatment of chlamydial venereal disease, trachoma and inclusion conjunctivitis oral dosage for adults or older child: 250mg or 333 mg QID for 2 to 3 weeks SIDE EFFECTS: *one of the safest antibiotics *topical side effects mild allergic reactions manifested as urticaria and other rashes; fever GRAMICIDIN similar to polymyxin B and colistin in that it alters the permeability characteristics of the bacteria cell membrane, killing the cell. Unlike polymyxin B and colistin, gramicidin is effective against gram-positive bacteria and available in solution. Available only as commercial preparation with polymyxin B and neomycin (Neosporin eyedrops not ointment). PENCILLINS Mechanism of Action Inhibit the synthesis of bacterial cell wall by preventing the terminal step in the process from taking place. Due to the cell walls abnormal development, death of the organism results. The greatest bactericidal effect is on actively dividing cells. SPECTRUM: Penicillins highly effective against gram-positive cocci (such as streptococcus pneumoniae, s. pyogenes, other streptococci), some bacilli and some gram-negative cocci and bacilli. Ineffective against or resistant to pseudomonas (gram negative rod shaped) and staphylococcus aureus (gram positive). OCULAR USE Not used topically due to high incidence of allergic reactions to drug; reserved for major/serious ocular infections and used for subconjunctival and intravenous injection Side Effects of Drugs Affecting Cell Wall 1) essentially non-toxic

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2) hypersensitivity a) contact dermatitis b) Steven-Johnson syndrome (type IV reaction) intradermal skin tests to predict hypersensitivity 3) superinfection with resistant organisms after long-term treatment AMPICILLIN Broader spectra of activity however less effective against bacteria that are sensitive to penicillin G. Range of antimicrobial activity includes gram(-) bacteria such as Hemophilus influenzae, Escherichia coli, Proteus mirabilis. Ineffective against most staphylococci destroyed by pencillinase. Used in treatment of gonococcal conjunctivitis. CARBENICILLIN Active against pseudomonas aeruginosa and certain proteus, enterobacter, and Acinetobacter species METHICILLIN (STAPHCILLIN) Resistant to pencillinase so indicated in the treatment of infections caused by strains of that produce penicillinase such as staphylococcus aureus and S. epidermidis PENICILLIN G POLYMYXIN B AND COLISTIN (POLMYXIN E) MECHANISM OF ACTION: interact with the phospholipids of the cells membrane, disrupting the osmotic integrity of the cell. Ultimately, this increases the bacterial cells permeability and causes leakage of intracellular molecules bactericidal poor systemic absorption does not penetrate blood-brain barrier CLINICAL USES: highly active against gram-negative bacteria, but limited use due to toxicity Availability in combination with other antibiotics in preparation or with steroids (ointment or suspension) OCULAR INDICATIONS: effective for the treatment of common bacterial infections of the conjunctiva and lids PYRIMETHAMINE DARAPRIM AND TRIMETHOPRIM (BACTRIM, SEPTRA)

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PHARMACOLOGY: *inhibit dihydrofolate reductase, which catalyzes the reduction of dihydrofolic acid to tetrahydrofolic acid *work in synergy with sulfonamides to increase the range of activity against microorganisms - sulfonamides inhibit the enzyme that converts PABA to dihydrofolic acid while - pyrimethamine and trimethoprim inhibit the enzyme that converts dihydrofolic acid to tetrahydrofolic acid CLINICAL USES: PYRIMETHAMINE No topical agent used in combination with sulfadiazine or with triple sulfonamides (sulfadiazine, sulfamerazine, and sulfamethazine). It is useful in treatment of toxoplasmosis. TRIMETHOPRIM Topically only available in solution form in combination with polymyxin B (Polytrim). Broad spectrum, bacteriostatic. Not effective against pseudomonas Useful in pediatric eye infections. useful for patients allergic to sulfonamides (no cross sensitivity) SULFONAMIDES SODIUM SULFACETAMIDE (SULFAMYD, BLEPH-10, AK-SULF, SULF-10) Topically applied Sulfonamides 10%, 15% and 30% solutions and 10% ointment also available in combination with steroids prednisolone acetate and prednisolone phosphate MECHANISM OF ACTION: Affects intermediary metabolism of bacteria; Bacterial cells are impermeable to folic and must synthesize this chemical internally from paraminobenzoic acid (PABA) to survive. Bacteriostatic CLINICAL USES: Broad spectrum but WIDESPREAD resistance Not effective against Neisseria or Pseudomonas species Poor efficacy against staph now (33%) but still good for strep and haemophylus kids Ineffective against very purulent infections (PABA in pus). *adult inclusion conjunctivitis (1st/2nd-tetracycline or erythromycin; 3rd-sulfonamides) *trachoma (1st tetracycline; 2nd erythromycin; 3rd oral sulfamethoxazole and oral or topical sulfacetamide) *chlamydial venereal disease (1st/2nd-tetracycline or erythromycin; 3rd-sulfonamides) *toxoplasmic retinochoroiditis (1st-sulfadiazine and trisulfapyriimidines) SIDE EFFECTS:

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hypersensitivity reactions (systemic or topical route) urticaria and rashes (accompanied by pruritis and fever) malaise serum sickness-like syndrome contact dermatitis (topical administration) erythema multiforme (Steven-Johnson syndrome) exfoliative dermatitis multiple small wine concretions of sulfadiazine within cysts in the palpebral conjunctiva (with topical sulfadiazine ointment) white plaque formation on the cornea (with topical sulfacetamide) decrease in corneal sensitivity (with 30% topical sulfacetamide) adverse drug interactions with hypoglycemic drug tolbutamide chlorpropamide coumadin anti-coagulants PABA-containing compounds and PABA analogs Procaine NOTE: Rarely prescribed in clinical optometric practice. TETRACYCLINES: Most commonly prescribed by optometrists in the treatment of recalcitrant lid disease, ocular rosacea, and in several corneal disease. MECHANISM OF ACTION inhibit protein synthesis in human cells as well as in microorganisms so antimicrobial activity works against normal bacterial flora in addition to pathogenic microorganisms - greater degree of protein inhibition is produced in microorganisms CLINICAL INDICATIONS Topical: Prophylaxis or treatment of superficial infections Recommended by CDC as an effective alternative to silver nitrate for prophylaxis of gonococcal ophthalmia neonatorum ( ointment form). Oral: Doxycycline: Most commonly prescribed for treatment of following ocular conditions: Blepharitis Meibomitis Recalcitrant evaporative dry eye syndrome Ocular manifestations of acne rosacea 100 mg BID reduced by 1 daily dose after every month of therapy followed by discontinuation or lowest maintenance dose

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Chlamydial infections Tetracyclines have been the mainstay of anti-chlamydial therapy for over 20 years. The current Centers for Disease Control and Prevention (CDC) recommendation is oral doxycycline 100 mg twice a day for seven days. recalcitrant cases of non-tuberculous phycetenular keratoconjunctivitis

CONTRAINDICATIONS: CATEGORY D: Contraindicated in pregnant woman in lactating women in children under 8 years of age depresses bone growth and discolors teeth MIXED-COMBINATION TOPICAL ANTIBIOTICS Combines spectrum of activity of antibiotics in preparation NEOSPORIN (OINTMENT) polymyxin B-bacitracin-neomycin dosage: apply Q 3-6 hrs for 7 to 10 days ocular indications: superficial external ocular infections NEOSPORIN (SUSPENSION) polymyxin B-neomycin-gramicidin dosage: instill 1-2 drops BID to QID for 7 to 10 days POLYSPORIN (OINTMENT) polymyxin B-bacitracin dosage: apply Q3-6 hrs for 7 to 10 days mixed combination ointment of choice when steroid is not needed POLYTRIM (SOLUTION) trimethoprim sulfate-polymyxin B sulfate dosage: instill 1-2 drops BID to QID for 7 to 10 days drug of choice for children because highly effective against Haemophilus and Strep. Pneumonia MIXED-COMBINATION TOPICAL ANTIBIOTICS AND CORTICOSTERIODS Ocular indication: Indicated for steroid responsive inflammatory ocular conditions for which a corticosteroid is indicated and where superficial bacterial ocular infection or a risk of bacterial ocular infection exists. Consider spectrum of activity of antibiotic and strength of steroid required. BLEPHAMIDE//VASOCIDIN (SUSPENSION//SOLUTION OR OINTMENT)

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sulfacetamide-prednisolone

CORTISPORIN (SUSPENSION, OINTMENT) polymyxin B-bacitracin-neomycin-hydrocortisone indications: ophthalmic infections MAXITROL (OINTMENT/SUSPENSION) neomycin sulfate-polymyxin B-dexamethasone indications: ophthalmic infections TOBRADEX (OINTMENT/SUSPENSION) 0.1% dexamethasone and 0.3% tobramycin Clinically used often indications: ophthalmic infections ZYLET (SUSPENSION) 0.5% loteprednol and 0.3% tobramycin Indications: ophthalmic infections Excellent antibiosis with the safety and potency of loteprednol

ANTIVIRAL DRUGS CIDOFOVIR (Vistide) Oral and IV forms available Topical and intraocular injectable forms not currently available INDICTIONS: Treatment of CMV retinitis MECHANISM OF ACTION Inhibition of viral DNA synthesis SIDE EFFECTS: Renal impairment Granulocytopenia FOSCARNET SODIUM (Foscavir) IV form only INDICATIONS: Treatment of CMV in patients with AIDS May be used alone or in combination with gancyclovir Treatment of acyclovir resistant mucocutaneous HSV infections in immunocompromised patients MECHANISM OF ACTION:

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Inhibition of binding site on viral DNA polymerase MAJOR SIDE EFFECTS: * renal impairment, seizure Some patients do not tolerate Foscarnet as well as gancyclovir. ADVERSE DRUG REACTIONS Nephrotoxic drugs and pentamine GANCICLOVIR SODIUM (Cytovene) Modes of administration: intravenous, intravitreal, intravitreal implant, oral INDICATIONS: Prevention and treatment of CMV disease and CMV retinitis in immunocompromised patients (such as AIDS patients and transplant recipients). Active CMV retinitis initial treatment (induction) with IV form of drug. To prevent reoccurrence patients must be on maintenance therapy either through IV form, intravitreal form (injection or implant) or oral form. Those taking the oral agent typically have more rapid reoccurrence rate of the condition than IV form. Those too ill for IV therapy or unwilling to have the intravitreal implant surgically inserted may opt for intravitreal injections. Vitreal implant=Vitrasert MAJOR SIDE EFFECTS: Granulocytopenia, anemia, neutropenia and thrombocytopenia ADVERSE DRUG REACTIONS: Zidovudine, cytotoxic drugs, nephrotoxic drugs, imipenemcilastatin, probenecid, and didanosine. ZIDOVUDINE (Retrovir) AZT First drug approved for the treatment of HIV Nucleoside reverse transcriptase inhibitor Best used now in combination with the newer class of anti-HIV drugs

Drugs for HIV. The biggest treatment breakthrough is highly active antiretroviral therapy (HAART), a combination of drugs that suppress the human immunodeficiency virus. HAART allows your immune system to recover and fight off infections like CMV retinitis.

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IDOXURIDINE (IDU) 0.1% solution MECHANISM OF ACTION Alters normal DNA synthesis Efficacy limited to DNA viruses; herpes virus group INDICATIONS: 1) Herpes Simplex Virus infections dendritic/geographic epithelial ulcers 1 drop Q 1 hr during the day, Q 2 hrs during the night with improvement: 1 drop Q 2 hrs during the day, Q 4 hrs at night -continue 3-7 days after corneal healing -treat maximum 21 days -75% of patients cured in 2 weeks but does not eradicate latent virus in trigeminal ganglion -no effect on recurrence rate of herpes keratitis primary herpetic keratitis -same as above -administers until conjunctivitis/periocular skin lesions resolve Epithelial disease responds better to this treatment than stromal disease. *treatment of herpes stromal keratitis with high dose steroids require prophylactic (simultaneous) use of IDU SIDE EFFECTS: Irritation, pain, puritis, chemosis, allergic reactions, photophobia, corneal punctate defects, corneal clouding TRIFLURIDINE (Viroptic) 1% solution Most commonly prescribed for herpetic eye disease. It is available in generic formulation. GENERAL FEATURES: *analog of thymidine antiviral mechanism not completely known inhibits DNA synthesis in both virus-infected and normal host cells INDICATIONS: Primary keratoconjunctivitis and recurrent epithelial keratitis secondary to herpes simplex. 1 drop every 2 waking hours max. 9 drops per day until re-epithelialization

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following re-epithelialization, taper to 1 drop q 4hrs (awake) for additional 7 days if no improvement after 7 days or if re-epithelialization hasnt occurred in 14 days consider alternate treatment. avoid administering for more than 21 days

SIDE EFFECTS: *mild,. transient burning or stinging on instillation *lid edema VIDARABINE (Vira-A) NOTE: NO LONGER COMMERCIALLY AVAILABLE; HOWEVER THE TMOD EXAM MAY STILL HAVE QUESTIONS? 3% ointment GENERAL FEATURES: Mechanism of action not established Analog of the purine nucleoside adenosine Active against vaccinia virus, Herpes simplex (type 1 and 2), and Varicella zoster virus INDICATIONS: Acute keratoconjunctivitis, recurrent epithelial keratitis and superficial keratitis(which has not resolved after treatment with idoxuridine) due to herpes simplex virus. ADMINISTRATION: Apply 5 times per day up to every 3 hours for a maximum of 21 days If no improvement after 7 days or complete reepithelialization after 21 days consider alternate treatment. -Treatment should continue at a reduced dose for 7 days after corneal resolution SIDE EFFECTS: stinging, burning, irritation, lacrimation, and injection follicular conjunctivitis, marked punctate keratitis, corneal erosion, and trophic epithelial defects (may be disease related). CONTRAINDICATION: Sterile trophic ulcers. ORAL ANTI-VIRALS For Herpetic Eye and Varicella Zoster Disease Primarily prescribed for the treatment of herpetic eye disease (HSV) and herpes zoster ophthalmicus (HZO) ( Varicella Zoster) Disease. ACYCLOVIR (ZOVIRAX)

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HSV: Shown to be effective in reducing the recurrence rate of all froms of herpetic eye disease, but most significantly it reduced the recurrence of stromal disease by 51%. HZO: 800 mg po 5 x daily x one week

VALACYCLOVIR ( VALTREX) HSV: 500mg TID x one week HZO: 1000mg TID x one week FAMCICLOVIR (FAMVIR) HZO 500mg TID x one week HSV 250 mg TID x one week ANTIFUNGAL DRUGS NATAMYCIN (Pimaricin) Natacyn 5% suspension only FDA-approved drug for topical treatment of ocular fungal infections Standard of care intitial therapy May be used in combination with Amphotericin B MECHANISM OF ACTION Binds to fungal cell membrane, alters cell membrane permeability. All anti-fungals penetrate an intact epithelium poorly INDICATIONS: Fungal blepharitis, conjunctivitis, and keratitis. Treatment of fungal corneal ulcers PRECAUTIONS: Effectivity as a single agent treatment for fungal endophthalmitis has not been established. Topical use only Resistance AMPHOTERICIN B 0.15% Second topical choice Must be compounded by a pharmacist GLAUCOMA MEDICATIONS BETA-ADRENERGIC ANTAGONISTS (BETA-BLOCKERS) Beta-receptors are localized throughout the body, including vascular and respiratory tissues. These receptors are divided into two main types: beta 1 and beta 2.

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Beta 1 receptors are present in blood vessel walls and cardiac muscle. When beta 1 receptors are stimulated, tachycardia and increased cardiac output results. When these receptors are blocked, bradycardia and decreased cardiac output results. Beta 2 receptors are present in bronchial musculature. Stimulation of the Beta 2 receptors in bronchial musculature leads to bronchial dilation. Blockage of beta 2 receptors leads to bronchospasm/constriction. There is some mild cross sensitivity of these receptor functions in each tissue, but the basic division holds.

MECHANISM OF ACTION: Decreasing aqueous production produces depression of intraocular pressure. The exact mechanism is unknown but aqueous humor production may be reduced up to 50%. Some agents have been shown to also slightly increase outflow. There is no affect on pupil size or accommodation. Systemic absorption can occur with topical administration. An approximate 30% reduction in intraocular pressure has been noted with oral beta-blockers. Use caution in prescribing topical beta-blockers in combination with patients taking oral beta-blockers. The predominant beta-receptor within the anterior segment of the eye is beta2 which is identical to that found in the respiratory system. Beta1 receptors are found within the myocardium. Non-selective beta blockers block both beta1 and beta2 receptors while selective beta blockers block only beta1 receptors. Decrease of IOP (10-15%) can manifest in the contralateral eye in uniocular trials. Responsiveness to some of the beta-blockers decreases over time (escape) especially early in the treatment phase. Long term drift can occur over time with the use of topical betablockers. There is no cross sensitivity to Beta-blockers, so one can switch to another if desired effect is no longer reached. INDICATIONS: Traditionally, first drug of choice to treat most forms of glaucoma. In todays management, prostaglandins have quickly become the drug class of choice for first-line therapy. TIMOLOL MALEATE (TIMOPTIC, TIMOPTIC-XE (gel formulation), Isatol): 0.25%, 0.5% Non-selective Beta-blocker. DOSAGE: Traditional dosage: 1 drop BID, or QD for gel-based Timopic-XE Best to instill gel formulation upon waking due to adrenergic system down-regulation during sleep Some studies have shown equal affect for 0.25% QD to 0.50% BID. May initiate treatment with 0.25% QD. IOP Reduction: Onset: 30 mins. Peak: 1-2 hrs. Duration: 12-24 hrs.

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Mean decrease in IOP from BID therapy is approximately 20-33% in patients with untreated pressures greater than 22 mm Hg. See INDICATIONS for Beta-blockers. CONTRAINDICATIONS: Chronic Obstructive Pulmonary Disease Bronchial asthma or history of asthma Congestive heart failure Second or third degree atrioventricular block First degree heart block with a PR-segment interval > 0.24 secs History of myocardial infarct or heart failure Decreased myocardial contractility Known allergies to beta-blockers (orals included) USE WITH CAUTION Sinus Bradycardia Pulse rate (<55 beats/min) Hypotension (<100/60) Labile diabetes Thyroid in pts prone to develop thyrotoxicosis Congenital or juvenile glaucoma safety and efficacy not established in children Narrow angles General anesthesia controversial to withdraw prior to surgery Medications: Patients using calcium channel blockers, digitalis preparations, and oral beta-blockers should be monitored. SIDE EFFECTS: Topical: 1) Allergic conjunctivitis 2) Burning irritation 3) Dry eye 4) Corneal hypesthesia 5) Lacrimation 6) Ocular myasthenia gravis ptosis, diplopia, weakness 7) Superficial punctate keratitis 8) Orbital pain 9) Hyperemia 10) Persistent hypotony Systemic: 1) Mask symptoms of hypoglycemia in diabetics 2) Prevention of usually rebound of plasma glucose concentration in diabetics 3) Bradycardia 4) Hypotension, decreased cardiac output 5) Arrhythmias, palpitations 6) Asthma exacerbated

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7) Increased airway resistance, bronchospasm, dyspnea, edema 8) CNS effects lethargy, confusion, hallucinations, depression, sleep disturbances, memory loss 9) Decreased exercise tolerance 10) Decreased libido 11) Syncope 12) Skin rash 13) May mask signs of hyperthyroidism 14) Nausea, emesis, diarrhea 15) Joint pain 16) Sudden death 17) Decrease HDL cholesterol (by 8% to 9%), thereby raising ratio of total to HDL cholesterol. BETAXOLOL HYDROCHLORIDE ( BETOPTIC-S 0.25%) Cardioselective Beta-blocker DOSAGE: Typical dosage: 1 drop BID Onset:30 mins. Peak:2 hrs Duration: 12 hrs. Betoptic is not as efficacious as Timolol or Levobunolol in its hypotensive effects (although clinically equivalent), but may do an equal or better job of preserving the visual field??) INDICATIONS: Betoptic being selective for beta-one receptors, it is the B-blocker of choice for individuals with a compromised respiratory system i.e. COPD, asthma etc. The drug is not absolutely selective, it is just less likely to induce respiratory complications. With the introduction of the prostaglandin class, beta-blockers should not be used in patients with pulmonary disease. Administer to these patients with caution. Nonselective beta-blockers appear to cause vasoconstriction at the laminar level. Betoptic does not appear to cause this vasoconstriction, so may be more appropriate for patients with normotension glaucoma.

CONTRAINDICATIONS: Same as Timolol with the exception of Betaxolol being safer for patients with respiratory disease (COPD, asthma). Administer to these patients with caution. Consider alternate therapy for those with severe respiratory disease. SIDE EFFECTS: Same as Timolol except with Betoptic-S, patients report less irritation after instillation to the anterior segment than with other beta-blockers.

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CARTEOLOL (OCUPRESS) 1% Non-selective Beta-blocker possessing intrinsic Sympathomimetic activity (ISA). DOSAGE: Typical dosage: 1 drop BID IOP Reduction: Onset and Peak: not determined Duration: 12 hrs. Decrease in IOP similar to Timolol 22-25% INDICATIONS: Same as Timolol. CONTRAINDICATIONS: Same as Timolol with exception that is does not aggravate cholesterol or lipid profiles as much. May be more appropriate for those with coronary disease, arteriosclerosis or hypercholesteremia. SIDE EFFECTS: Same as Timolol, but does not cross blood-brain barrier as easily as other non-selective B-Blockers so may protect against depression. NOTE: Recent literature review by Dr. Paul Lama revealed that beta-blockers do not cause depression, although b-blocker induced depression has been reported for years. LEVOBUNOLOL (BETAGAN) HYDROCHLORIDE 0.25%, 0.5% Non-selective Beta-blockers DOSAGE: Typical dosage: QD or BID Durasite 0.1% IOP reduction: Onset: 1 hr. Peak: 2 hrs. 12-24 hrs, 0.5% concentration is FDA-approved for oncedaily dosing but .25% works well when only administered QD. IOP reduction is similar to Timolol. Levobunolol is less expensive. INDICATIONS: Same as Timolol CONTRAINDICAITONS: Same as Timolol SIDE EFFECTS: Same as Timolol METIPRANOLOL (OPTIPRANOLOL) 0.3%

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Non-selective Beta-blockers DOSAGE : Typical dosage: 1 drop BID IOP Reduction: Onset 30 mins. Peak: 2 hrs. Duration: 12 24 hrs. Expected decrease in IOP similar to Timolol 20-30% Is less expensive than Timolol INDICATIONS: Same as Timolol. CONTRAINDICATIONS: Same as Timolol. SIDE EFFECTS: Same as Timolol COSOPT Combination of timolol maleate and dorzolamide (Trusopt) Dosage: BID INDICATION, CONTRAINDICATIONS and SIDE EFFECTS Same as for Timolol and Trusopt TIMPILO Combination of timolol maleate and pilocarpine 2/4%. Not used clinically in the the US More commercially available in other countries ( Canada) INDICATION, CONTRAINDICATIONS and SIDE EFFECTS Same as for Timolol and Pilocarpine

PROSTAGLANDIN ANALOGs Mechanism of action: enhances uveoscleral outflow; therefore, IOP can be lowered below episcleral venous pressure Quickly becoming first choice for initial therapy

CAUTION

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Aphakia, pseudophakia, history of intraocular inflammation, risk of macular edema SIDE EFFECTS: Ocular Darkening of irides, mostly in patients with hazel and mixed-color irides. Mild punctate staining. Conjunctival hyperemia. Foreign body sensation Cystoid macular edema in aphakic individuals Uveitis in those with previous history of intraocular inflammation Eye lash growth Periorbital skin darkening Systemic Rare BIMATOPROST 0.03% (LUMIGAN) Amide based Dosage: QD Tends to cause most local ocular side effects Most studies show Lumigan to be slightly more efficacious in lowering IOP, however this is offset by its disproportionate ocular side effect profile LATANOPROST .005% (XALATAN) Ester based Dosage: QD before bed XLT study reported Xalatan to be most tolerated drug within the prostaglandin class TRAVOPROST 0.004% (TRAVATAN) Ester based Dosage: QD before bed May have greater efficacy in African Americans than the other drugs in this group DOCOSANOID COMPOUNDS UNOPROSTONE (0.15%) RESCULA REMOVED FROM THE US MARKET MECHANISM OF ACTION: unknown; increase aquesous humor outflow Dosage: BID Currently not indicated for first line therapy

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EPINEPHRINES . DIPIVERFRIN HYDROCHLORIDE (DIPVALYL HCl, PROPINE, AKPro 0.1%) Note: Not typically prescribed intodays glaucoma management MECHANISM OF ACTION: Mainly enhances aqueous outflow, but also decreases production of aqueous. Dipivefrin is a prodrug of epinephrine. The addition of two pivalyl groups allows the prodrug to be lipophilic, thus allowing for increased intraocular penetration. Once through the cornea, the molecule become epinephrine and stimulates the target receptors. It has the same therapeutic effect as epinephrine. Causes mydriasis. DOSAGE: Typical dosage: 1 drop BID IOP reduction: Onset: 0.5-1 hrs Peak: 2-4 hrs Duration 12 hrs Range of decrease in IOP is 15-26%. Takes about 3 months to achieve full therapeutic efficacy INDICATIONS: 1) Treatment of primary and secondary glaucomas 2) Can be used concomitantly with topical B-blockers CONTRAINDICATIONS: Same as Epinephrine. SIDE EFFECTS: 1) Allergic blepharitis 2) Mydriasis 3) Follicular conjunctivitis 4) Cystoid Macular edema in aphakes: Probable TMOD/board question 5) Corneal edema Less likely to develop systemic and extraocular side effects due to the nature of the drug, but intraocular effects still expected because active drugs is still epinephrine. Less likely to discolor lenses. EPINEPHRINE (Epinephrine HCL 0.1%, Epifrin 0.5%, 1% and 2%, Glaucon 1% and 2% and Epinal 1.5%, 1%) Note: Clinically, not prescribed in todays glaucoma management. MECHANISM OF ACTION: Direct-acting sympathomimetic agents which, act on alpha and beta-receptors. They cause conjunctival vasoconstriction, transient mydriasis and reduce IOP when applied

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topically. They are believed to decrease IOP by increasing aqueous outflow and decreased aqueous production. DOSAGE: Typical dosages; 1 drop BID Hydrochloride and borate solutions are equally effective. Unstable if exposed to sunlight. IOP Reduction Onset 2 hrs Duration: 12 hrs. INDICATIONS:. Treatment of primary and secondary open angle glaucomas. May be used in combination with miotics, beta blockers, hyperosmotics or carbonic anhydrase inhibitors. Consider in patients who may not tolerate miosis or browache. Not the primary choice of drug since it has less IOP reduction than other hypotensive medications .Vasoconstriction of small vessels during ocular surgery. CONTRAINDICATIONS: Narrow angles without a patent iridectomy Plateau iris syndrome Aphakia (ICCE) Soft contact lens wearer Discontinue prior to general anesthesia USE WITH CAUTION Cardiac arrhythmias Hypertension Diabetes Hyperthyroidism Ischemic heart disease Asthma Concurrent therapy of Tricyclic antidepressants and/or MAO inhibitors. SIDE EFFECTS: Cystic Macular edema with approximately 20-30% occurrence in aphakes. Flame hemorrhages on some occasions. Vasoconstriction with rebound conjunctival hyperemia Stinging burning, irritation Punctal stenosis Adrenochrome deposits in the conjunctiva Soft contact lens discoloration Allergic blepharitis Follicular conjunctivitis Corneal epithelial edema Mydriasis Intolerable effects occur in 20-50% of cases. Just not prescribed in US!

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SYSTEMIC: Severe headache, heart palpitations, hypertensive crisis, tachycardia, premature ventricular contractions, angina, anxiety, faintness, trembling, pallor.

ALPHA-2 ADRENERGIC AGONIST APRACLONIDINE HYDROCHLORIDE (IOPIDINE 0.5% and 1.0%) MECHANISM OF ACTION: Apraclonidine is a selective alpha2 adrenergic agonist. The mechanism of action not completely established. Stimulation of alpha-adrenergic receptors may result in decreased aqueous formation. DOSAGE: Typical dosage: 1%: 1 drop 1 hour before laser procedure, 1 drop immediately upon completion of procedure., 0.5%: 1 or 2 drops TID IOP reduction: Onset: 1 hr. Peak: 3-5 hrs Duration 7-12 hrs.. NOTE: Tachyphylaxis (loss of effect) of 0.5% over time in some patients. Monitor patients. Benefits for most patients is <3 months. INDICATIONS: 1) 1%: Treatment/prophylaxis of IOP spikes following anterior segment laser procedures. 2) 0.5%: Short term adjunctive Tx in patients on maximally tolerated Tx who require lower IOP. CONTRAINDICATIONS: Do not use in patients taking MAO inhibitors. Use with caution in patients with severe cardiovascular disease. Use with caution in patients with a history of vasovagal syncope. SIDE EFFECTS: Ocular 0.5%: Hyperemia, pruritis, discomfort, tearing, lid edema, blurred vision, foreign body sensation, dry eye, conjunctivitis, discharge and blanching. 1.0%: Conjunctival blanching, lid retraction, mydriasis, foreign body sensation, dry eye irritation, burning, discomfort, hypotony, corneal microhemorrhage. Overall discontinuation rate is 15%. Most common events leading to discontinuation are related to allergic reaction. Systemic with 0.5%/1.0%:

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Dry mouth, dry nose, fatigue, decreased libido, paresthesia, pruiritus, abdominal pain, GI upset, emesis, Bradycardia, vasovagal attack. BRIMONIDINE 0.2% (ALPHAGAN) Also available in generic formulation MECHANISM OF ACTION: A selective alpha2 adrenergic agonist. Bidual mechanism: Reduces IOP by reducing aqueous humor production and increasing uveoscleral outflow. DOSAGE: Two or three times daily (typically BID dosing in practice) IOP reduction: Peak 2 hrs Duration 6-8 hours. INDICATIONS: Treatment of glaucoma and ocular hypertension CONTRAINDICATIONS: Do not use in patients taking MAO inhibitors SIDE EFFECTS: Ocular Hyperemia, burning, stinging, blurring, foreign body sensation, conjunctival follicles, allergic reaction Systemic Oral dryness, headache BRIMONIDINE (ALPHAGAN P) 0.15 AND 0.1% Enhanced tolerability profile compared to Iopidine and Alphagan 0.2% Typically prescribed as second line agent Typically presecribed as BID dosing, however are FDA approved for TID dosing Reduces IOP by approximately 25% Clinically shown to be fairly equivalent in lowering IOP as compared to Alphagan 0.2% with significantly less allergic reaction Preserved with Purite CHOLINERGIC AGONISTS DIRECT-ACTING MIOTICS MECHANISM OF ACTION: Direct-acting cholinergic (parasympathomimetic) drug which reduces IOP by increasing aqueous outflow facility. This natural alkaloids molecules stimulate muscarinic receptors in the ciliary body and cause contraction of the longitudinal muscles. Subsequent force upon the adjacent structures pull the trabecular meshwork (TM) space open.

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CARBACHOL (0.75%, 1.5%, 2.25%, 3% topical solution and 0.01% intraocular solution (Miostat)) Topical: Action onsets in 10 to 30 minutes with its maximum effect at 2-4 hours. Duration is 4-8 hours. Topical Dosage: Up to TID INDICATIONS: Topical: Similar to pilocarpine for various forms of glaucoma Used in patients allergic or refractory to pilocarpine. Intraocular: Used to induce miosis during surgery. CONTRAINDICATIONS: Similar to pilocarpine Relatively contraindicated in patients with peptic ulcers, GI spasms, and urinary tract obstructions. SIDE EFFECTS: Similar to pilocarpine but worse in shallowing the anterior chamber angle and increasing vascular permeability. Corneal clouding Bullous keratopathy Intraocular: postoperative iritis SYSTEMIC: Similar to pilocarpine but effects are greater. PILOCARPINE AND EPINEPHRINE (combination 1-6% pilocarpine with 1% epinephrine) Additive effect lowering IOP. Marked pupillary miosis typical of pilocarpine not present.

PILOCARPINE HYDROCHLORIDE (0.25% - 10%) Note: Practitioners are prescribing pilocarpine less with the introduction of newer medications with fewer side effects. Pilocarpine HCL is less expensive than most anti-glaucoma medications and is commercially available. Reduction of intraocular pressure in ocular hypertensives and glaucomatous eyes is 1040%. There is a significant effect upon diurnal variation.

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Onset: 10- 30 minutes with a peak at 75 minutes.. Duration of 4-8 hrs. Administration and Dosage: 1 drop three to four times a day. Dependent on patient response. Individuals with heavily pigmented irides may require higher strengths. Concentrations greater than 4% are typically only used in patients with dark irides. INDICATIONS: Primary open angle glaucoma Acute angle closure glaucoma Non-inflammatory secondary glaucomas Pre-operative and Post-operative: to lower IOP. .125% conc. used for Adies tonic pupil diagnosis Differential diagnosis of dilated, fixed pupil (IIIrd nerve vs. pharmacologic dilation with 1% pillocarpine) SIDE EFFECTS: Miosis Ciliary spasm/Accommodative myopia Decreased night vision Decreased vision in the presence of PSC, cortical cataract Induction of lenticular nuclear sclerosis Browache, eyepain Myokymia Visual field constriction Congestion and hyperemia Shallowing of Ant. Chamber of 8-12% Follicular conjunctivitis Stinging and burning Rare: Retinal detachment SYSTEMIC: Rare with routine use. Nausea, vomiting, salivation, lacrimation, vasodilatation (hypotension) with rebound hypertension, Bradycardia with rebound tachycardia, Diaphoresis reported with repeat doses, Bronchospasm, and pulmonary edema. *Cholinergic toxicity reversible with parental atropine. CONTRAINDICATIONS: Neovascular glaucoma Prolonged used in chronic angle closure glaucoma/narrow angles Acute and chronic inflammatory glaucoma Malignant glaucoma Use with caution: Younger patients (prone to ciliary spasm) Patients with predisposition to retinal tears and detachments Cataracts Aphakes

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Asthmatics Vitreous hemorrhage

PILOCARPINE OCULAR THERAPEUTIC SYSTEM (Ocusert 20, 40) Time release form which releases of 20 or 40 micrograms per hour; One week replacement 20 is approximately equivalent to 0.5-1 % drops QID, 40 is approximately equivalent to 2-3% drops QID Less miosis and visual disturbances than traditional form but foreign body sensation Good for pre-presbyopes and patients with poor compliance.

PILOPINE HS GEL 4% Ophthalmic ointment (acrylic-based vehicle) which may be used with younger patients, with poor compliance, or those with less tolerance for side effects (decreased vision, browache). Pilopine HS is used in lieu of pilocarpine solution. Use a ribbon at bedtime. Side effects occur at night while the patient is asleep. Pupillary miosis escapes the following day. Patients may complain of early morning blur and may develop focal punctate opacities in Bowmans and the corneal stroma after 9 months of treatment. TIMPILO Fixed concentration in combination of pilocarpine and timolol maleate. PHYSOSTIGMINE (Eserine sulfate 0.25% ointment) Know for TMOD-not prescribed in practice. ACTIVITY Miosis onset 20-30 minutes. Duration of 12-36 hours. IOP reduction: Peak 4-6 hrs. Duration of 12-36 hours. INDICATIONS: Treatment of open angle glaucoma

CONTRAINDICATIONS: Active uveal inflammation History of quiescent uveitis Same as pilocarpine Discontinue use several weeks before intraocular surgery Drug Interactions: Carbamate/Organophosphate insecticides, pesticides, Succinylcholine, systemic anticholinesterases.

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SIDE EFFECTS: Ocular Accommodative spasm, Stinging, Hyperemia, Induced myopia, Browache, Lid twitch, Follicular conjunctivitis, Iris cysts at pupillary border, Activation of latent iritis or uveitis, Lenticular opacities, Punctal stenosis, Retinal Detachment, Conjunctival thickening, Posterior synechia, Pupillary block with subsequent rise IOP Systemic: Nausea, vomiting, abdominal cramps, diarrhea, urinary incontinence, fainting, sweating, salivation, difficulty breathing, cardiac irregularities. CARBONIC ANHYDRASE INHIBITORS (CAI) MECHANISM OF ACTION: Inhibits the enzyme carbonic anhydrase which is active in the ciliary processes. In the presence of a carbonic anhydrase inhibitor, the transfer of the sodium and bicarbonate ions from the plasma of the ciliary processes to the aqueous is depressed. In turn, the osmotic fluid movement which usually follows the ion flow is reduced, thus less aqueous is formed. All commercially available carbonic anhydrase inhibitors are unsubstituted, aromatic sulfonamide compounds. SYSTEMIC AGENTS ACETAZOLAMIDE (DIAMOX) (125 mg. 250 mg tablets, 500 mg time-released capsules (SEQUELS), 500 mg IV solution) DOSAGE: Adults: 250 mg 1 gram per day BID to QID P.O. Acute cases: 500 mg( not sequels) followed by 125 or 250 mg QID Children: 5-10 mg/kg/dose, IM or IV QID 8-30 mg/kg/day divided doses TID QID P.O. Maximum effective dose: 1000 mg/24hrs. IOP reduction: Tablets: Onset - .5-1.5 hrs. Peak: 2-4 hrs. Duration: 8-12 hrs. Parental (IV): Onset 1-2 minutes Peak: 3=20-30 mins. Duration: 4 hrs. INDICATIONS: 1) Additive therapy to maximally tolerated topical glaucoma medications a. Primary open angle glaucoma b. Secondary open angle glaucoma 2) Acute glaucoma secondary to hyphema, inflammation ( Most common) 3) Acute/chronic angle closure glaucoma 4) Intraoperative/post-operative pressure spikes

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CONTRAINDICATIONS: 1) Glaucoma with extensive PAS formation causing scarring 2) Patients with history of sulfonamide allergies 3) Patient with history of idiosyncratic reactions to sulfonamides 4) Diabetics cause metabolic acidosis 5) History of renal disease (calculi) 6) History of liver disease (cirrhosis) 7) Sickle cell anemia can induce sickling 8) Aspirin therapy cause acidosis 9) Diuretic or digitalis therapy cause hypokalemia 10) Addisons disease cause hypokalemia 11) Cyclosporine cause nephrotoxicity 12) Pregnant females 13) Elderly poor tolerance 14) Chronic obstructive pulmonary disease exacerbate pre-existing respiratory acidosis ADVERSE DRUG INTERACTIONS: Acetazolamide, Salicylates, and Difunisal SIDE EFFECTS OF SYSTEMIC CAIS: 1) Paresthesia of the digits and perioral area 2) SYSTEM COMPLEX Malaise Fatigue Depression Weight loss Decreased libido 3) Metallic taste 4) Renal calculi formation 5) Metabolic Acidosis 6) Blood dyscrasias 7) Abdominal cramps, GI irritation 8) Breathing difficulty excess CO2 formation 9) hypokalemia 10) Dermatitis 11) Idiosyncratic reaction (sulfonamide allergy) 12) Transient myopia 13) Renal calculi formation in predisposed pts. *Reports of 30 80% of patients on maximal system CAI therapy develop intolerable side effects. *Many side effects are dose related. *Consider baseline tests for sickle cell and serum levels of creatinine, potassium, CAI levels, and CO2 combining power to monitor electrolytes.

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DICHLORPHENAMIDE (DARANIDE) (50 mg tablets) Typical dosage: 25 100 mg BID TID PO Maximum effective dose is 200 mg/24/hrs. IOP Reduction: Onset: 0.5 1 hr. Peak: 2-4 hrs. Duration: 6-12 hrs. IOP reduction is equivalent to acetazolamide. INDICATIONS: Adjunctive therapy for the treatment of primary and secondary glaucoma for patients intolerant of acetazolamide and methazolamide. Acute Glaucoma due to quick onset. CONTRAINIDCATIONS: See Contraindications for Acetazolamide SIDE EFFECTS: See side effects for Acetazolamide 1) Causes more confusion and anorexia in patients. Patients report a higher frequency and severity of side effects. Believed to deplete potassium more than other CAIs 2) Only 20% of patients can tolerate Daramide beyond 6 wks, limiting its usefulness. METHAZOLAMIDE (NEPTAZANE) DOSAGE: Tablets: 25 or 50 mg. Typical dosage: 25 100 mg BID or TID Initial dosage: 25 mg BID Maximum effective dose is 300 mg/24 hrs. Methazolamide has a plasma half-life almost three times that of acetazolamide. Approximately 55% of the drug is plasma bound so less amount of the drug is required to produce a hypotensive effect. IOP reduction: Onset: 2-4 hrs. Peak: 6-8 hrs. Duration: 10-18 hrs. INDICATIONS: See Indications for Acetazolamide CONTRAINDICATIONS: See Contraindications for Acetazolamide SIDE EFFECTS: See Side Effects for Acetazolamide, however:

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Less than acetazolamide paresthesia, acidosis Produces less effect upon urinary citrate levels thus less likely to develop kidney stone formation.

TOPICAL AGENTS INDICATIONS: chronic treatment of 1o and 2o open angle GLC and ocular hypertension. SIDE EFFECTS: Stinging, burning, hyperemia, blurred vision, headaches, and bitter taste. May induce changes to the corneal endothelium due to high dose volume of CAI in a topical solution. Avoid in patients with true sulfa allergies, caution in those with renal impairment BRINZOLAMIDE OPHTHALMIC 1.0% (AZOPT) DOSAGE: 1 gt tid May cause less stinging than Trusopt DORZOLAMIDE HYDROCHLORIDE 2.0% (TRUSOPT) In a 2% solution, Dorzolamide is additive to beta-blockers and has been shown to decrease IOP 15-22%. DOSAGE: -2% solution TID reduces IOP 22% - 26% -Administered BID, it decreases IOP 21% - 24% -When added to Timolol, Trusopt will lower IOP at additional 13% - 21% -Recommended to instill B-blocker in eye first then Trusopt. -CAIs reduce nocturnal flow rate by 25%, compared to B-blockers which do not suppress aqueous formation during sleep. COSOPT Combination of timolol maleate and dorzolamide (Trusopt) Dosage: BID INDICATION, CONTRAINDICATIONS and SIDE EFFECTS Same as for Timolol and Trusopt HYPEROSMOTIC AGENTS TOPICAL AGENTS Topically applied, hypertonic solution or ointment acts to draw water out of the cornea. Ability to affect hydration in corneas with traumatized epithelium seems limited. TOPICAL GLUCOSE (GLUCOSE-40)

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DOSAGE: Typical dosage: UNG applied 2-6 times daily. INDICATIONS: Treatment of corneal edema CONTRAINDICATIONS: Hypersensitivity TOPICAL GLYCERIN (OPHTHALGAN) Topical hyperosmotic agent used to resolve corneal edema for diagnosis purposes. Action is transient so for short term usage. DOSAGE: 1-2 drops after instillation of topical anesthetic Painful and extremely irritating if no anesthetic Onset: 1-2 minutes INDICATIONS: 1) Reduction of corneal edema Bullous keratopathy, Fuchs dystrophy, angle closure glaucoma. Needed for ophthalmic and gonioscopic evaluation. CONTRAINDICATIONS: Hypersensitivity to its components SIDE EFFECTS: 1) Significant irritation, can be painful, so use anesthetic prior to instillation. SODIUM CHLORIDE (ADSORBANAC, MURO-128)( 2%, 5% Solution, 5% Ointment) DOSAGE: Typical dosage: 1-2 drops or small amount for ointment, every 3-4 hrs or as directed. INDICATIONS: 1) Temporary resolution of corneal edema: Fuchs, Bullous Keratopathy, Hydrops, chronic increased IOP 2) Moderate to Severe Epithelial Basement Membrane Disorder 3) Recurrent Corneal Erosions CONTRAINDICATIONS: Hypersensitivity to its components. SIDE EFFECTS: Burning irritation ORAL AGENTS GLYCERIN OR GLYCEROL (OSMOGLYN)

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INDICATIONS: Acute angle closure attacks Pre and post-operatively (intraocular surgery) DOSAGE: 1 to 2 g/kg 1.5g/kg body weight = 1.5-3.0 ml/kg body weight 50% lime-flavored solution to be served over cracked ice and with a straw Approximately 4-6 ounces per individual Preoperatively: 1.to 1.5 hrs prior to surgery IOP Reduction: Onset: 10 30 minutes Peak: 1-1.5 hrs. Duration: 4-5 hours CONTRAINDICATIONS: Anuria Severe cardiac decompensation Severe dehydration Severe cardiac decompensations Acute pulmonary edema USE WITH CAUTION DIABETICS Patients who are nauseated or vomiting Dehydrated individuals Cardiac, renal, or hepatic disease Confused mental state Hypersensitivity to its components SIDE EFFECTS: Nausea, emesis, headache, confusion, dehydration, hyperglycemia, ketoacidosis, cardiac arrhythmias ISOSORBIDE (ISMOTIC) INDICATIONS: Acute angle closure Pre and post-operative intraocular surgery *Oral agent of choice for Diabetics DOSAGE: Vanilla-mint flavor, serve over cracked ice with a straw 1.5g/kg body weight = 1.5 ml/kg body weight IOP Reduction: Onset: 10 30 minutes Peak: 1-1.5 hrs. Duration: 5-6 hours CONTRAINDICATIONS: Anuria

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Severe dehydration Acute pulmonary edema Severe cardiac decompensation Hypersensitivity to its components

SIDE EFFECTS: Less nausea and vomiting than glycerol. Headache, confusion, disorientation, gastric discomfort, hypermatremia, rash, syncope, lethargy, dizziness, vertigo, thirst, hiccups. INTRAVENOUS AGENT MANNITOL (MANNITOL, OSMITROL) 5%, 10%, 15%, 20%, 25% solutions INDICATIONS: Reduction of Intraocular Pressure DOSAGE: Intravenous only. 1.5-2 gms/kg body weight of 15-20% solution. Onset: 30-60 mins. Peak: 60 mins. Duration: 6-8 hours. CONTRAINDICATIONS: Anuria due to severe renal disease Severe pulmonary congestion Active intra-cranial bleeding Severe dehydration Hypersensitivity to its components Progressive heart failure Progressive renal failure SIDE EFFECTS: Severe headache, hypotension, hypertension, tachycardia, angina-like chest pains, nausea, emesis, urinary retention, urticaria, chills, dehydration, pain, hypokalemia, increased electrolyte secretion, blurred vision, coma, subdural hematoma.

ANTIALLERGY AND DECONGESTANT AGENTS

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DECONGESTANT MECHANISM OF ACTION Upon instillation into the conjunctival fornix, these agents cause vessel constriction due to alpha adrenergic stimulation. Naphazoline, oxymetazoline and tetrahydrozoline are imidazole derivatives. Relieve hyperemia and chemosis without directly affecting the allergic cascade. NAPHAZOLINE (0.012%, 0.02%, 0.03%, and 0.1% solutions) INDICATION: To relieve hyperemia and chemosis due to minor ocular irritation. 0.012% through 0.1% solutions have been shown to produce greater conjunctival blanching than phenylephrine or tetrahydrozoline. DOSING: Every 3-4 hours Duration of action 3-4 hrs. SIDE EFFECTS: Ocular Stinging and burning upon instillation, blurred vision, pupil mydriasis, mild elevation of intraocular pressure, increased redness, irritation, discomfort, superficial punctate keratitis. Systemic Generally none due to low concentration. Possible headache, hypertension, nervousness, nausea, palpitation, tachycardia. CONTRAINDICATIONS: Contraindicated in patients with angle closure GLC, or potentially occludable angles. Caution in patients with compromised corneas as systemic vasopressor response may occur, in patients taking MAO inhibitors, Beta blockers and local anesthetics and in patients with cardiovascular disease, hyperthyroidism and diabetes. PHENYLEPHRINE See Phenylephrine Under Mydriatics and Cycloplegics 0.12% concentration used for vasoconstriction. 2.5% and 10% solutions available for pupillary dilation. DOSING: 4 times a day SIDE EFFECTS: Same as naphazoline except more likely to cause rebound congestion (resulting in conjunctivitis medicametosa) and pupillary dilation than the other decongestants; therefore not a decongestant of choice. CONTRAINDICATIONS:

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Same as naphazoline TETRAHYDROZOLINE (0.05%) INDICATIONS: For temporary relief of redness and burning/irritation. DOSING: 4 times a day Duration of action 1-4 hours TOXICITY: Same as naphazoline CONTRAINDICATION: Same as naphazoline TOPICAL ANTI-HISTAMINES INDICATIONS: Provide relief for the symptoms of allergic or inflammatory conjunctivitis. DOSAGE: 1 drop four times a day SIDE EFFECTS: Stinging and burning, headaches, visual disturbances, dry mouth, but usually no CNS effects from topical instillation. CONTRAINDICATIONS: Narrow anterior chamber angles (may cause pupillary dilation) EMEDASTINE DIFUMARATE (Emadine) (0.05% Solution) MECHANISM OF ACTION Relatively selective H1 receptor agonist LEVOCABASTINE (Livostin) (0.05% SUSPENSION) Note: As of December 2004, Novartis has stopped production of Livostin MECHANISM OF ACTION A potent selective H1 receptor agonist. MAST CELL STABILIZERS: MECHANISM OF ACTION: Act by stabilizing and inhibiting mast cell degranulation (release of histamine). Also inhibits eosinophil, neutrophil, and monocyte activation. INDICATIONS: Used for treatment of more chronic mast-cell related conditions. Poorly suited to treatment of acute conditions. Typically used for long term preventative or maintenance therapy.

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CONTRAINDICATIONS: Hypersensitivity to component. CROMOLYN SODIUM 4% (CROLOM, OPTICROM) DOSAGE: Used 4 to 6 times/day. Symptomatic relief takes several days to up to six weeks. INDICATIONS: 1) Vernal conjunctivitis 2) Giant papillary conjunctivitis (off-label use) OCULAR SIDE EFFECTS: Transient ocular stinging Conjunctival injection Watery or itchy eyes Styes Dryness around the eyes LODOXAMIDE TROMETHAMINE 0.1% (ALOMIDE ) DOSAGE: Used 4 times/day for up to 3 months. Symptomatic relief takes several days to up to six weeks. INDICATIONS: 1) Allergic conjunctivitis (off-label use) 2) Vernal conjunctivitis 3) Giant papillary conjunctivitis (off-label use) OCULAR SIDE EFFECTS: Transient ocular stinging NEDOCROMIL SODIUM 2% (ALOCRIL) DOSAGE: Used 2 times/day. Symptomatic relief takes several days to up to four weeks. INDICATIONS: Allergic conjunctivitis OCULAR SIDE EFFECTS: Transient ocular stinging SYSTEMIC SIDE EFFECTS unpleasant taste, headaches

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 PEMIROLAST POTASSIUM 0.1% (ALAMAST) DOSAGE: Used 4 times/day. Symptomatic relief takes several days to up to six weeks. INDICATIONS: Allergic conjunctivitis OCULAR SIDE EFFECTS: Transient ocular stinging SYSTEMIC SIDE EFFECTS Headaches ANTIHISTAMINE / MAST CELL STABILIZER COMBINATION: INDICATION: For the temporary prevention of signs and symptoms of allergic conjunctivitis. AZELASTINE HYDROCHLORIDE 0.05% (OPTIVAR) MECHANISM OF ACTION: antihistamine, mast-cell stabilizer, eosiniphil chemotaxis inhibitor DOSAGE: 1 drop 2 times per day OCULAR SIDE EFFECTS: Transient ocular stinging SYSTEMIC SIDE EFFECTS Unpleasant taste, headaches KETOTOTIFEN FUMARATE 0.025% (ZADITOR) MECHANISM OF ACTION: Antihistamine, mast-cell stabilizer, eosiniphil chemotaxis inhibitor DOSAGE: 1 drop 2 times per day OCULAR SIDE EFFECTS: Transient ocular stinging, conjunctivitis SYSTEMIC SIDE EFFECTS

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Headaches OLAPATADINE HYDROCHLORIDE 0.1% (PATANOL) MECHANISM OF ACTION: A relatively selective H1 receptor antagonist with mast cell stabilizer properties. FDA approved to treat ALL signs and symptoms of allergic conjunctivitis DOSAGE: 1 drop in each affected eye 2 times per day at an interval of 6-8 hrs. SIDE EFFECTS: Ocular Burning, stinging, dry eye, foreign body sensation, hyperemia, keratitis, lid edema, puritis Systemic Headache OPHTHALMIC DECONGESTANT/ ANTIHISTAMINE COMBINATIONS MECHANISM OF ACTION: H1 anti-histamine which blocks histamine receptors on cell membrane of the ocular tissue. Inhibits tissue edema, and itching. Produces localized antimuscarinic, anesthetic, and CNS effects. ANTAZOLINE PHOSPHATE PHENIRAMINE MALEATE Although these drugs may be used alone, they are commercially available with a vasoconstrictor to enhance the effects of both medications. Usually combined with naphazoline or phenylephrine. Examples: VASOCON-A OPCON-ANAPHCON-A Naphazoline HCL 0.05% + Antazoline Phosphate 0.5% Naphazoline HCL 0.027% + Pheniramine maleate 0.315% Naphazoline HCL 0.05% + Pheniramine Maleate 0.3%

Typical dosage is 1 drop every 3-4 hours. INDICATIONS: Temporary relief of minor symptoms such as itching and redness due to allergic conjunctivitis CONTRAINDICATIONS: Contraindicated in patients with angle closure GLC potentially occludable angles, or hypersensitivity to the components. Caution in patients taking MAO inhibitors, Beta

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blockers, local anesthetics and sedatives, tranquilizers, narcotic pain relievers, alcohol and antianxiety medications. Caution with elderly patients and children under age 12. SIDE EFFECTS: Ocular Stinging and burning upon instillation, blurred vision, pupil mydriasis, mild elevation of intraocular pressure, increased redness, irritation, discomfort, superficial punctate keratitis. Systemic Generally none due to low concentration. Possible headache, hypertension, nervousness, nausea, palpitation, tachycardia. TOPICAL ANTIINFLAMMATORY AGENTS NON STEROIDAL ANTI-INFLAMMATORY AGENTS KETOROLAC TROMETHAMINE (ACULAR) CORTICOSTEROIDS FLUOROMETHALONE (FML) LOTEPREDNOL ETABONATE 0.2% (ALREX) LOTEPREDNOL ETABONATE 0.5% (LOTEMAX) PREDNISOLONE SEE PREVIOUS SECTION

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