Retinoschisis

1. Retinoschisis Presented By: Robin Reyna Charlotte Tompkins Valerie Hyde

2. Topics Covered In Presentation • Description of Condition • Causes of Retinoschisis • Parts of the Visual System Affected • The Effects of Retinoschisis • How it is Diagnosed • Common Treatments • Is it Congenital or Acquired? Is it Progressive or Stable? • Anticipated Functional Implications • Case Study • References

3. Description of Condition • Retinoschisis is the splitting of the retina into layers. There are two forms of this disorder. The acquired form of this disease affects both men and women, where as the congenital form of this disease mostly affects boys and young men. The congenital form of this disease is referred to as X-linked Retinoschisis, meaning that it is an inherited disease. This disease is characterized by a slow, progressive loss of vision that corresponds with the splitting of the layers of the retina.

4. Causes of Retinoschisis • There are two forms of this disease. • The “senile” form of this disease, which affects both men and women and is a result of aging. This form is not inherited. • The “juvenile” form of this disease is inherited on the X chromosome, meaning that it is passed from mother to son. (Must have one copy of the defective gene for it to be expressed in boys because they only have one X chromosome. Girls are thought to be only carriers of this disease.)

5. Parts of the Visual System affected • This disease causes the retina to split into layers. This splitting causes the vision loss. When the retina splits, another condition called Cystoid Macular Edema can present itself. • Cystoid Macular Edema is a swelling of the macula. Fluid collects within the layers of the macula producing distorted vision and or vision loss.

6. The Effects of Retinoschisis • Central Vision can be impaired. Visual Acuity can range from 20/30 to 20/200. • The vision loss in Retinoschisis is caused by the formation of tiny cysts in the retina. These cysts often form a "spoke-wheel" pattern which is frequently very subtle and is usually detected only by a trained clinician. • Second, peripheral vision can be lost due to the splitting of the inner layer of nerve cells from the outer layer of cells.

7. How it is Diagnosed • The electroretinogram (ERG) is a test used in assessing the function of the retina. The eye is stimulated with light after either dark or light adaptation. Contact lenses, embedded with an electrode, are worn by the patient. The reaction of the eye is recorded and evaluated. • Children who show schisis of the peripheral retina need more frequent examinations.

8. Common Treatments • There are no medical or Surgical Treatments available to treat Retinoschisis. • Retinoschisis is a disease of the nerve tissue, glasses will not "repair" this nerve tissue damage. • Some Eye Doctors do prescribe glasses for protection of the eyes or because of photophobia. (Transition type lenses would then be prescribed to protect the eyes from UV damage and to lessen the light sensitivity that is sometimes problematic for the patient.)

9. IS it congenital or acquired? Is it progressive or stable? • Retinoschisis is both congenital and acquired. • Senile form of this disease is the acquired form. • Juvenile or X-linked Retinoschisis is the congenital form of this disease. Meaning that one is born with it. • In the juvenile form of this disease, the rate of vision loss is progressive to some point and then begins to stabilize.

10. Anticipated Functional Implications of Retinoschisis • Materials may have to be adapted by enlarging the print size. • Need to eliminate visual clutter. • May need to tactually enhance worksheets. • Pre-Braille skills may need to be introduced. • Preferential seating may be needed. • Hard copies of lessons presented on the overhead or board. • May need direct task lighting. • May wear cap or shades outside or inside when eyes are being bothersome. • Safety issues may arise due to the inability to detect objects/drop offs in his pathway. An O&M Evaluation might be needed to address these issues if the need arises.

11. Case Study C.M is a 5 yr old little boy, who was born 12 weeks premature. He was in and out of the hospital during the first year of his life. He has had no lasting effects of being 12 weeks premature. He lives with his mom, aunt and several cousins. C.M. first complained about his eyes hurting during the Summer of 2005. He was taken to the Pediatrician. Then in January of 2006 he was referred to Baylor Ophthalmology for a complete exam. During this time, he was diagnosed with Juvenile X-Linked Retinoschisis and prescribed glasses with tinted (Transition) polycarbonate lenses for protection and possibly reduce the fatigue and pain from lights. Doctor states that C.M. is legally blind. The Doctor also states that his near acuities with glasses are 3/400 (right eye) and 20/200 (left eye). His symptoms that led to the diagnosis of Retinoschisis were: -Headaches -Eyes hurt -Intermittent periods of total blindness for up to 10 minutes -Light sensitivity -Retinal Detachment -Cystoid Macular Edema.

12. Case Study Continued The CTVI evaluated C.M. using observation, Parent Interview, Child Interview and Project IVEY, Carolina Holmgren Color Vision Test, and Visual Field Testing. Her results are reported below: -Severity of Condition *C.M. has a progressive medical condition that will result in no vision or a serious visual loss even after correction. He is not impaired in any other way. *C.M. is loosing his vision at an accelerated rate. His visual acuity in August 2007 was 20/80 in his better eye and in December 2007 his acuity was 20/200 in the better eye and 3/400 in other eye. As of February 2008, his visual acuity was 20/250 in the better eye and he was only able to count fingers in the other eye.

13. Case Study Continued -Carolina Holmgren Color Vision Test *Color vision seems to be intact. -Observation of Eyes *C.M. demonstrated positive pupillary response to pen light. *He demonstrated blink to threat reflex. *Normal eye muscle test (confrontational) indicated that he probably uses both eyes together. -Visual Field Testing *Brought object from behind his head in to visual field, C.M. may have significant field limitation of 45-60 degrees on the right with most effective visual field being on the left.

14. Case Study Continued • Modifications: -Extended time on assignments -Materials adapted by enlarging print size -Eliminate Visual Clutter -Tactually enhance worksheets -Pre-Braille skills should be introduced since his eye condition is progressive. -Preferential seating may be needed to obtain best field of vision. -Hard copies of lesson presented on the overhead or on the board. -May need direct task lighting. -May wear cap or shades when outside or inside when eyes are bothering him.

15. Case Study Continued • Recommendations -C.M. should register with TEA as a student with a Visual Impairment. -Receive direct services by CTVI minimum of 35 sessions for 20 minutes each per semester. -C.M. should not be identified as functionally blind. -Receive an Orientation and Mobility Evaluation by an Orientation and Mobility Specialist. -Receive Low Vision Assessment by a Low Vision Specialist.

16. Case Study Continued--Present Level of Performance • C.M. was evaluated by the Orientation and Mobility Specialist. She determined that he needed instruction in using a cane for safety purposes when traveling. • He is learning to recognize letters in Braille and write in Braille. • He can identify all textures: hard, soft, rough and smooth. • He can write his first, middle, and last name, all the letters of the alphabet, and can draw a face with four parts. • He is independent in all self-help skills. • He can add double digit numbers.

17. References • Retinoschisis. (n.d.) Retrived July 21, 2008, from The University of Michigan Kellogg Eye Center Web site: http://www.kellogg.umich.edu/patientcare/conditions/retinoschisis.html • Retinoschisis. (n.d.) Retrieved July 21, 2008, from Foundation Fighting Blindness Web site: http://www.blindness.org/visiondisorders/causes.asp?type=12 • Dictionary of Eye Terminology 5th Ed., Barbara Cassin and Melin L. Rubin, MD, Editor, 2006. Gainesville: FL, pg 231. • Retinoschisis. (n.d.) July 21, 2008, from TSBVI Web site: http://www.tsbvi.edu/Education/anomalies/Retinoschisis.htm • Retinoschisis. (May 1, 2006) Retrived July 21, 2008 from AOL Health Web site: http://www.aolhealth.com/conditions/x-linked-juvenile-retinoschisis