With the advent of digital cameras and video recorders, parents are taking more pictures of their children than ever. Many parents will bring their child in because of the way an eye looks in a photo. When this occurs, they most often state one eye looks white or looks like there is something inside the eye. Whenever a parent states this, it is to be taken seriously. A full optometric examination, including dilated fundus examination, should be performed. Relatively benign causes for leukocoria include anisometropia, congenital cataract, and strabismus (these can lead to amblyopia). There are causes, however, which can have a much more detrimental impact on a child's life, including retinoblastoma, ROP, and Coats' disease.

Differentials:

• Retinoblastoma
• Retinopathy of prematurity
• Coats' disease

	URL: http://www.opt.indiana.edu/ce/peddz/leukocor.htm Revised: November 18, 2006
	IU Optometry home page: http://www.opt.indiana.edu/ Comments (content): Dr. Don Lyon at dwlyon@indiana.edu Comments: Web Administrator Page design and coding: Terri Greene Copyright © 2006, The Trustees of Indiana University

Retinoblastoma Retinoblastoma (RB) is the most common type of pediatric tumor. Incidence is 1 in 20,000. Hereditary form (40 percent of RB cases) Autosomal dominant transmission (mutation in germline) 80-90 percent bilateral Presents around 1 year of age Fair chance of developing non-ocular tumors during life Non-hereditary form (60 percent of RB cases) Not transmissible (mutation in single retinal cell) Unilateral and unifocal disease Presents around 2 years of age Patients are not predisposed to having non-ocular tumors Clinical features: Leukocoria 80-85 percent of presenting patients Strabismus second most common finding Can mimic an anterior iritis Rubeosis Optometric examination may reveal elevated area of retina with a serous detachment Tumor can be whitish or yellow Mainly presents in the posterior areas of the retina, rarely anterior 66 percent of all RB tumors develop before 2 years; 95% develop before 5 years Diagnosis is made on appearance and neurological imaging (CT, MRI). Treatment is dependent upon tumor size, severity, and potential visual function of the affected eye(s). Enucleation Radiation (external and plaque) Chemotherapy Follow-Up/Monitoring: In cases of hereditary forms of retinoblastoma, siblings and offspring need to be closely monitored at birth and every 6 months until the age of 5 or 6 years. Retinoblastomas [Return to top]

Retinopathy of Prematurity Retinopathy of prematurity (ROP) usually affects infants whose birth weight is less than 1500 grams (3 lbs 5 oz). Gestational period can play a role as well. Infants born before 28 weeks can show signs of ROP. Infants who weighed between 1500 grams and 2000 grams with an unstable clinical course should also be suspect. Some children with ROP will also have additional medical/systemic health concerns, and the optometrist will be a member of their medical team There are five stages to ROP that can be identified through clinical examination. Stage1 ROP is a definite demarcation line, which is flat, between the vascularized and avascularized retina. Stage 2 ROP occurs when a ridge, with height and width, appears between the vascularized and avascularized areas. Stage 3 ROP is when there is neovascularization along the ridge which extends into the vitreous. Stage 4 ROP occurs when a partial retinal detachment develops. If the fovea is not involved it is termed stage 4A. If the fovea is off with a partial detachment, it is stage 4B. Stage 5 ROP indicates a complete retinal detachment has occurred. "Plus Disease" Along with the changes at the vascular-avascular interface, vessel dilation and tortuosity of the major arteries and veins in the posterior pole can develop. Dilation and tortuosity occurring in at least two of the four quadrants around the optic nerve is termed "plus disease" and a plus sign (+) is added to the notation; Stage 3 ROP with "plus disease" would be recorded as Stage 3+ ROP. ROP Stage 3 ROP "Plus Disease": Note the tortuosity of the blood vessels and posterior pole hemorrhages Status post laser treatment for ROP Treatment and examination schedule of infants with ROP Treatment of ROP will depend upon the stage of the condition and will be made by the physician monitoring the patient. Treatment options typically include cryoablation or laser photocoagulation in an attempt to minimize the development of neovascularization and retinal detachments. A percentage of infants with ROP will require simple monitoring with dilated fundus examinations every week or two until the physician thinks the ROP is regressing. Once treatment is completed or regression is noted, the infant is released from the care of the pediatric ophthalmologist. Initially these patients are seen 4-6 months after they have been released from post-operative care or once the clinician feels the ROP has regressed fully. The schedule for future examinations will be dependent upon the results of the post-ROP baseline examination. It will also be important to communicate findings with the rest of the patient's medical team to ensure that the patient is receiving the best care possible. Sequella can include: High myopia Glaucoma Amblyopia Strabismus Nystagmus [Return to top]

Coats' Disease Coats' Disease is a congenital abnormality of retinal blood vessels usually seen in males in late childhood, but it can present at a much younger age. Children who exhibit the condition earlier seem to have a quicker progression of the disease. This condition is typically unilateral but can be bilateral with asymmetrical presentation. Males are affected more than females and there does not appear to be any ethnic or racial factors associated with the condition. The exact cause of Coats' disease is unclear at this time given the disease is considered rare and has sporadic presentation. The following clinical signs can be seen in patients with Coats' disease. Strabismus Reduced visual acuity Leukocoria Retinal telangiectasis and massive exudates with vessel dilation The lesions are most often seen in the superior temporal quadrant affecting the periphery more than the posterior pole. Advanced cases of Coats' disease can cause retinal detachments. Coats' can be easily mistaken for retinoblastoma. At times a CT scan is necessary to look for calcification that is typically seen in retinoblastoma but not in Coats'. Coats' Disease Coats' Disease Arteriovenous phase of FA demonstrating hyperfluorescent retinal telangiectasias Late phase of FA demonstrating leakage of the dye from vascular abnormalities Treatment of Coats' disease: Cryotherapy and/or photocoagulation are typically performed to contain/stop the exudative changes. The child may require multiple treatments over his/her lifespan. In rare cases, enucleation may be necessary secondary to advanced neovascular glaucoma. The child may have continued low vision in the affected eye(s) even with successful treatment. [Return to top]

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