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Edited by James Eckman, M.D. and Allan Platt, PA-C
Eye Inflamation and Retinopathy
Glaucoma, retinal detachment, and neovascularization with hemorrhage occur with increased frequency in patients with sickle syndromes. These complications may be more frequent in patients with Hb SS and high hemoglobin level, Hb SC, and Hb S beta thalassemia. Symptoms related to the eye must be carefully evaluated. Routine annual ophthalmology examinations are essential for preventive care.
Clinical Findings
- Present Illness. Note onset of symptoms, duration, exposure to chemicals/make-up, morning matting, purulent discharge, pruritus, sneezing, coryza, seasonal symptoms, tearing, cough, pharyngitis, or exposure to conjunctivitis. Eye pain, visual change, photophobia, foreign body, trauma, or chemical exposure (Obtain an emergency Ophthalmology consult).
- Past Medical History. Determine date of last eye examination. Document history of retinal detachment, hemorrhage, LASER treatments, allergic history, present medications.
- Eye Examination. Determine visual acuity and fields.
- Inspection. Foreign bodies, periorbital edema, pupillary constriction, ciliary injection, tearing, entropion, subconjunctival hemorrhage, hordeolum (sty), pupillary reaction to light and accommodation, clarity and depth of anterior chamber.
- Extra-ocular movements. Determine range of muscle motion, convergence.
- Fundiscopic. Increased cup/disk ratio, hemorrhage, scarring.
- Fluorescein stain. Corneal abrasion, ulceration, dendritic etching.
- Lymphatics. Preauricular adenopathy.
- Nose/Sinus. Congestion, nasal polyps, rhinorrhea, sinus tenderness.
- Mouth. Pharyngitis.
- Skin. Vesicular, erythematous rash over face/scalp.
Laboratory- Minimum Lab. Gram stain and Culture for purulent conjunctivitis and orbital cellulitis. CBC with reticulocyte count, blood cultures, urinalysis and culture for orbital or periorbital cellulitis.
- Conjunctivitis. Morning matting, non-purulent discharge, URI symptoms, exposure or atopic history, chemical exposure, herpes zoster, make-up use, normal conjunctiva, and negative fluorescein study favor allergic or viral conjunctivitis. Purulent discharge suggests bacterial conjunctivitis, herpes simplex, or foreign body.
- Hordeolum, chalazion. Localized swelling of lid with acute inflammation - hordeolum, without acute inflammation - chalazion.
- Iritis, Iridocyclitis. Photophobia, consensual photophobia, pupil constriction and decreased reactivity, ciliary injection, clouding in anterior chamber.
- Trauma. Corneal abrasion, foreign body, entropion with conjunctivitis suggest traumatic conjunctivitis.
- Herpes simplex. Severe pain, photophobia, irritation, conjunctivitis, and dendritic etching or ulceration on fluorescein examination supports this diagnosis.
- Dacrocystitis. Purulent conjunctivitis with inflammation of the tear duct with swelling of the medial and inferior lid are found.
- Orbital or periorbital cellulitis. Swelling and redness of the eye lids and sinus tenderness are seen with periorbital cellulitis. Loss of vision, chemosis, proptosis, or ophthalmoplegia suggest orbital cellulitis.
- Retinal hemorrhage. Acute loss of vision blood in vitreous and failure to visualize fundus support this diagnosis.
- Retinal detachment. Visual changes with spots in front of eye, flashes of light, curtain in front of eye with gray haziness on ophthalmoscopic exam are classic findings.
- Glaucoma. Shallow anterior chamber, clouding of the cornea, dilated pupil, increased cup/disk ratio, decreased venous pulsation are seen with acute angle-closure glaucoma .
Criteria for Admission and Ophthalmology referral.
- Because of the potential for serious complications in patients with sickle syndrome, all patients except those with non-purulent conjunctivitis, simple corneal abrasion, and foreign body which has been removed should be referred to an ophthalmologist. Simple blunt trauma to the eye should always be referred because of the potential for retinal hemorrhage and detachment.
- Patients with periorbital or orbital cellulitis should be admitted for I.V. antibiotics, further diagnostic evaluation, and observation. All patients with retinal detachment and most with acute angle-closure glaucoma should have therapy initiated in the hospital.
- Conjunctivitis. Bacterial. Sulfonamide ophthalmic ointment Q.I.D. for 7 days. Return to Ophthalmology if worse or no better in 3 days.
Viral. Sulamyd drops, iii (3) drops in each eye T.I.D. Good hand washing and single wash cloth.
Atopic. Vasocon drops, i (1) drop in each eye Q.I.D., Chlortrimeton PA 12mg, i Q 12 hours., discontinue make-up, hair sprays and preparations, perfumes.
- Hordeolum, chalazion. Warm compresses to eye and topical antibiotics.
- Iritis, Iridocyclitis. Refer for urgent ophthalmology evaluation and consider evaluation for systemic disease.
- Trauma. Simple corneal abrasion may be treated with topical antibiotics, eye patch, with or without homatropine. With foreign body and abrasion, remove foreign body, instill topical antibiotic ointment, and have ophthalmology see in 24 hours. Entropion, refer to ophthalmology. Presence of hyphemia should prompt emergent evaluation and treatment by an ophthalmologist because of the risk for acute glaucoma.
- Herpes simplex. Refer for immediate ophthalmology evaluation and treatment with idoxiuridine, vidarabine, or acyclovir.
- Dacrocystitis. Initiate topical and oral antibiotics based on gram smear and culture. Patient should be evaluated by ophthalmology in follow-up.
- Orbital or periorbital cellulitis. Patient needs treatment with I.V. antibiotics after meningitis, sepsis, sinusitis, and CNS complications are excluded. CT scan and LP are indicated in patients with orbital cellulitis. Common organisms H. influenza, S. pneumoniae, S. aureus, and S. pyrogenes can all cause life-threatening infections in patients with sickle syndromes.
- Retinal hemorrhage. Emergent ophthalmology referral.
- Retinal detachment. Emergent ophthalmology referral.
- Glaucoma. Emergent ophthalmology referral.
Nursing Considerations
:Instruct patient/parent that most complications are preventable and treatable with early detection so reporting any visual changes, eye pain, redness, discharge, or drainage is important.
Instruct patient how to instill drops, use clean hand washing techniques, warn patient not to touch tip of the dropper to eye or surrounding tissue.
Teach the importance of compliance with recommended therapy
Suggest dark glasses for phobophobia, and teach the patient to watch for and report adverse reaction to ocular medication.
Encourage patient to rest during the acute illness.
Stress the importance of yearly eye exams.
Patients should have ophthalmology examinations on a regular basis for detection of neovascularization, early glaucoma, and other pathology. This should be done on an annual basis in early childhood and increased in frequency with onset of pathology. Early detection and Laser photocoagulation of neovascularization has been associated with a decrease incidence of retinal hemorrhage. Salicylates and NSAIDs should be avoided, if possible, in patients with neovascularization.
Partial exchange transfusion is recommended in patients requiring eye surgery. All trauma to the globe or sudden change in visual acuity requires emergent evaluation by an ophthalmologist because of the risk of acute glaucoma, retinal detachment, or hemorrhage.
Information about the frequency of complications which may result in loss of vision must be presented to patients and parents. Education must stress that most complications are preventable and treatable with early detection and treatment reinforcing the need for regular eye examination by an ophthalmologist familiar with retinal disease in sickle cell syndromes. They must also be instructed to present for immediate evaluation for trauma, eye pain, or visual changes.
References
McCormick AQ Eye Disorders - The Red Eye in Handbook of Pediatric Emergencies Baldwin GA editor, Lippencott Williams Wilkins, 1994.
Goldberg MF. Classification and pathogenesis of proliferative sickle retinopathy. Amer. J. Ophthalmol. 71:649-665, 1971.
Armaly MF. Ocular manifestations in sickle cell disease. Arch. Intern. Med. 132:670-679, 1974.
Nagpal KC, Goldberg MF, Rabb MF. Ocular manifestations of hemoglobinopathies. Survey of Ophthalmol. 21:391-411, 1977.
Jampol LM. Condon P, Farber M, Rabb M, Ford S, Serjeant G. A randomized clinical trial of feeder vessel photocoagulation of proliferative sickle cell retinopathy. Ophthalmology 90:540-545, 1983.
Cohen SB, Fletcher MF, Goldberg MF, Jednock N. Diagnosis and management of ocular complications of sickle hemoglobinopathies. Ophthalmic. Surg. Part I. 17:57-374, 1986.