ࡱ> RTMNOPQ3 bjbj ,BCl        ~4~4~484L5D LehR8:8(8888>89 ddddddd$f hdd 98899d=  88d@===9 8 8d=9d==b?!`  5d8F8 `c ^/~49vb&5dTeHLebj8i :|8i5d=         DEPARTMENT OF VETERANS AFFAIRS Veterans Benefits Administration Washington, D.C. 20420 April 6, 2009 Director (00/21) Training Letter 09-03 All VA Regional Offices and Centers In Reply Refer To: 211 SUBJ: Application of Revised Eye Sections of Rating Schedule Purpose The purpose of this training letter is to provide basic information about eye disabilities and how to apply the revised rating schedule provisions in evaluating them. Who to contact for additional information Questions concerning this letter should be emailed to VAVBAWAS/CO/21FL. /S/ Bradley G. Mayes Director Compensation and Pension Service Enclosure Eye Training letter A. Introduction There are numerous changes, both technical and substantive, in the revised eye sections of the rating schedule. Therefore, a careful reading of the revised schedule, the Fast Letter for the Final Rule: Schedule for Rating Disabilities; Eye (FL 09010), this training letter, and a comparison of the former schedule with the revised schedule, is advisable to assure correct application of the provisions of the changed schedule. Many of the changes are highly technical and represent revised methods of evaluating certain common eye disabilities. Two excellent sources of medical information on the Internet on eye conditions are:  HYPERLINK "http://www.mic.ki.se/Diseases/c11.html#C11.640.710" http://www.mic.ki.se/Diseases/c11.html#C11.640.710 (has countless links for all eye disorders from reliable sources, via the  HYPERLINK "mailto:info@kib.ki.se" Karolinska Institutet University Library, Stockholm, Sweden).  HYPERLINK "http://www.dmoz.org/Health/Conditions_and_Diseases/Eye_Disorders/" http://www.dmoz.org/Health/Conditions_and_Diseases/Eye_Disorders/ (has numerous links). B. Visual Impairment The term visual impairment is used often in the revised eye sections. It includes: impairment of visual acuity (excluding developmental errors of refraction), impairment of visual field, and impairment of muscle function. Therefore, when you see the term visual impairment, it is not a synonym for decreased visual acuity. The directions under a diagnostic code may direct to rate on visual impairment, on impairment of visual acuity, on loss of muscle function, on alternative criteria such as incapacitating episodes, on nonvisual impairment (which includes, for example, disfigurement), etc. Many eye disabilities can result in impairment of more than one eye function, so the criteria for many conditions are broad, and more than one type of assessment will be needed in some cases. Causes of visual impairment Visual impairment is the result of an eye disorder, rather than being the eye disorder or condition itself. Common causes include retinal degeneration (including macular degeneration), retinopathy, cataracts, glaucoma, muscle imbalance problems, corneal disorders, trauma, and infection. C. Rods and cones Rods - There are about 120 million rods in each eye, and their highest concentration is in the peripheral retina. Rods do not detect color, which is the main reason it is difficult to tell the color of an object at night or in the dark. However, rods detect movement out of the corner of the eye and operate in poor light or at night. Cones - There are about 6.5 to 7 million cones in each eye, and they are more concentrated in the center of the retina (macula). Cones are sensitive to bright light and to color and are used for color vision and for close work like reading. The macula lutea in the central portion of the retina provides the clearest, most distinct vision. It is about 2.5 to 3 mm in diameter. Exceptionally sharp vision may occur in an individual who has more cones per square millimeter of the macula than the average person. The center of the macula, called the fovea centralis, is about 0.3 mm in diameter. It contains all cones and no rods, and is therefore the point of sharpest, most acute visual acuity. Blind spot There are no rods or cones in the area where the optic nerve passes through the retina, and this results in the normal or physiologic blind spot. D. Measuring Visual Acuity 1. Examination requirements Uncorrected and corrected visual acuity for distance and near must be measured and recorded, as determined using Snellen's test type or its equivalent. Evaluation is based on corrected distance vision with central fixation, even when a central scotoma is present. 2. Distance visual acuity Visual acuity of 20/20 means a person can see on an eye chart (standard eye chart or Snellen letter chart) at 20 feet the smallest symbol that a person with normal visual acuity can see at that distance. Visual acuity of 20/40 means a person can see on an eye chart at 20 feet that which a person with normal visual acuity can see at 40 feet. In metric terms, 20/20 vision is 6/6, where the 6 refers to meters instead of feet, and 20/40 vision in metric terms is 6/12. Many people have visual acuity that is better than 20/20. For example, someone with 20/15 visual acuity can see on an eye chart at 20 feet that which a person with 20/20 visual acuity can see only at 15 feet. Visual acuity is based on the sharpness or clarity of central, rather than peripheral, vision. Section 4.76(b)(1) directs that central visual acuity be evaluated on the basis of corrected distance vision with central fixation, even if a central scotoma is present. This rule emphasizes that eccentric visual acuity (visual acuity other than central acuity) is not the basis of determining visual acuity. 3. Near visual acuity Measured by having a person read print samples of different sizes from a Jaeger card at a distance of 14 inches from the persons eye. Near visual acuity of 14/14 means that the person can read at 14 inches what someone with normal vision can read at 14 inches. 4. Pinhole test This is a quick screening test to differentiate vision problems due to refractive errors versus those due to non-refractive eye problems. Looking through a pinhole significantly reduces refractive errors of the cornea and lens. If vision improves with the use of a pinhole, decreased visual acuity is likely to be due to refractive error rather than eye disease. However, those with cataract and some other eye diseases may also have improved pinhole vision, so this is not a totally reliable test. 5. Refractive errors These are the most common cause of poor visual acuity and include myopia, or nearsightedness; hyperopia, or farsightedness; and astigmatism. Myopia is a reduced ability to see distant objects clearly, although near objects are seen clearly. It results when the visual image is focused in front of the retina rather than on it. Hyperopia at first causes difficulty in seeing near objects but later progresses to affect distance vision. It results when the visual image is focused behind the retina rather than on it. Astigmatism is blurred vision caused by abnormal curvature of the front surface of the cornea or of the lens. Presbyopia is a condition in which the focusing power of the eye is lost due to aging, as the elasticity of the lens diminishes with age (from around age 45 on), so that there is a gradual decrease in the ability to focus on near objects. 6. Diagnostic codes for loss of visual acuity There are now only 6, instead of 19, diagnostic codes that designate loss of visual acuity. 7. No light perception We removed the term "blindness" from the titles of diagnostic codes 6062 and 6064 because the term "blindness," as used in 38 U.S.C. 1114, "Rates of wartime disability compensation," has more than one meaning, and using it in the rating schedule to refer to only one level of visual impairment led to confusion. In evaluating visual acuity of one eye, no light perception is evaluated the same as light perception only. To avoid confusion, we have revised the titles of diagnostic codes 6062 to No more than light perception in both eyes and 6064 to No more than light perception in one eye. This term includes both vision with light perception only and vision with no light perception. E. Eye examinations for compensation and pension purposes Only licensed optometrists and ophthalmologists may conduct C&P eye examinations. A diagnosis is required when there are abnormal findings. A fundoscopic examination after dilation of the pupils is routine, unless medically contraindicated. Examinations of visual fields or muscle function are needed only when medically indicated (or when specially requested, such as on a BVA remand). F. Visual fields and perimetry 1. Goldmann kinetic perimetry The Goldmann kinetic perimeter or equivalent kinetic method remains an accepted method of measuring visual fields, but it is no longer the only accepted method. Instead of a 3mm. white test object, a standard target size and luminance (Goldmanns equivalent (III/4-e)) is now the requirement. 2. Automated perimetry Automated perimetry using Humphrey Model 750, Octopus Model 101, or later versions of these perimetric devices with simulated kinetic Goldmann testing capability is also an accepted method of measuring visual fields. 3. In all cases Results must be recorded on a standard Goldmann chart, which must beincluded with theexamination report. 4. Visual Fields Loss of lower half of visual field (inferior altitudinal field loss) is evaluated at 10 percent for the unilateral and 30 percent for the bilateral condition (or impaired visual acuity of 20/70 (6/21) for each affected eye). Loss of upper half of visual field (superior altitudinal field loss) is evaluated at 10 percent for both unilateral and bilateral conditions (or impaired visual acuity of 20/50 (6/15) for each affected eye). Altitudinal field loss may result from retinal, optic nerve, or brain lesions of many types. 5. Correction 10 percent (or impaired visual acuity of 20/50 (6/15) for each affected eye) is now the evaluation for unilateral or bilateral condition for both concentric contraction to 46 to 60 degrees and for loss of the nasal half of the visual field. This corrects the bilateral percentage evaluation of 20 percent formerly indicated for these conditions, because both bilateral and unilateral visual acuity of 20/50 warrant a 10 percent, not a 20 percent, evaluation. G. Evaluation of one eye Based on loss of visual acuity alone, the maximum evaluation for a single eye is 30 percent. With anatomic loss, the evaluation for a single eye is 40 percent. If there is anatomic loss and inability to wear a prosthesis, the evaluation for a single eye is 50 percent. If only one eye is service connected, consider the visual acuity of the non service connected (NSC) eye to be 20/40, subject to 3.383(a)(1). Computing aggravation. We removed former 4.78, which stated that aggravation of preexisting visual disability will be determined based upon the evaluation of vision in both eyes before and after suffering the aggravation, even if the impairment of vision in only one eye is service-connected, and that with subsequent increase in the disability of either eye due to intercurrent injury or disease not associated with service, the basis of compensation will be the condition of the eyes before suffering the subsequent increase. Under the new regulations, if visual impairment of only one eye is incurred or aggravated in service, only the visual impairment of that eye will be evaluated for compensation purposes. The visual acuity of the other (NSC) eye will be considered to be 20/40 for evaluation purposes, subject, of course, to the provisions of 38 CFR 3.383(a). Example #1 (aggravation): Pre-service, veteran had visual acuity of 20/50 of each eye due to scarring from an old injury. His left eye was re-injured in combat. Post-service, visual acuity of his right eye was 20/50, and of his left eye was 10/200. His evaluation is 20 percent for left eye aggravation (30 percent for 10/200 (current left eye) minus 10 percent for 20/50 (left eye on entrance)). Since his right eye is NSC, it is considered to have normal vision (20/40) for purposes of this calculation. Example #2 (incurrence): Pre-service, veteran had visual acuity of right eye of 20/70 and of left eye of 20/20, with history of bilateral inactive chorioretinitis. She developed a cataract in her left eye in service. Post-service, the visual acuity of her right eye was 20/70, and of her left eye was 10/200. Evaluation is 30 percent for her left eye cataract incurred in service based on visual acuity of 10/200. Since her right eye is NSC, it is considered to have normal vision (20/40) for purposes of this calculation. H. Other special provisions 1. Difference of more than three diopters in corrective lenses When the lens required to correct distance vision in the poorer eye differs by more than three diopters from the lens required to correct distance vision in the better eye (and the difference is not due to a congenital/developmental refractive error), and either the poorer eye or both eyes are service connected, evaluate the visual acuity of the poorer eye using either its uncorrected or corrected visual acuity, whichever results in better combined visual acuity. Note that this represents a change from the former schedule, which used a four-diopter, rather than a three-diopter, difference, and which limited this provision only to spherical correction. 2. Difference of two or more steps between near and distance corrected vision Former 4.84 stated that when there is a substantial difference between the near and distant corrected vision, the case should be referred to the Director of the Compensation and Pension Service. The new regulation (4.76(b)(3)) states that when there is a difference equal to two or more scheduled steps between near and distance corrected vision, with the near vision being worse, the examination must include at least two recordings of near and distance corrected vision and an explanation of the reason for the difference, and Evaluations will be made in these cases as if distance vision were one step poorer than measured. There is no longer a need to send such cases to the Director of the Compensation and Pension Service. Example: Distance vision of the SC right eye is 20/40 and near vision is 20/100. This was confirmed on 2 readings and the examiner explained the reason for the difference. The left eye is NSC. The right eye visual acuity is rated at one step poorer than the measured distance vision, or 20/50. The left eye is considered 20/40, so the evaluation is 10 percent. 3. Evaluation when there is loss of both visual acuity and visual field in the same eye Raters must determine for each eye the percentage evaluation for visual acuity and for visual field loss (expressed as a level of visual acuity) and combine them under 38 CFR 4.25. The combined eye evaluation can then be combined with any other disabilities that are present. Formerly, such cases were referred to the Director of the Compensation and Pension Service for evaluation, but this is no longer necessary. Example: Corrected visual acuity of right eye is 20/40 and of left eye is 20/70, warranting a 10 percent evaluation. Visual field loss in right eye is remaining field 38 degrees (equivalent to visual acuity 20/70) and loss in left eye is remaining field 28 degrees (equivalent to visual acuity 20/100), warranting a 30 percent evaluation. The combined eye evaluation under 4.25 is 30 percent combined with 10 percent for a combined evaluation of 40 percent. I. Changes in evaluation of diplopia (double vision) 1. Diplopia plus decreased visual acuity or visual field loss Formerly, an evaluation for diplopia was applied to only one eye and was not combined with an evaluation for decreased visual acuity or visual field loss in the same eye. Also, when both diplopia and decreased visual acuity or visual field loss were present in both eyes, the evaluation for diplopia was assigned to the poorer eye, and the evaluation for either corrected visual acuity or contraction of visual field to the better eye. Now, when both diplopia and either unilateral or bilateral impaired visual acuity or visual field loss are present, the evaluation of the poorer eye (or the affected eye, if only one eye is service-connected) is made by assigning a level of visual acuity (for decreased visual acuity or visual field defect expressed as a level of visual acuity) that is: one step poorer than it would be otherwise, if the evaluation for diplopia under diagnostic code 6090 is 20/70 or 20/100. two steps poorer if the evaluation for diplopia is 20/200 or 15/200. three steps poorer if the evaluation for diplopia is 5/200. This results in the adjusted visual acuity. The adjusted level, however, cannot exceed 5/200. The percentage evaluation is then determined under diagnostic codes 6064 through 6066, using the adjusted visual acuity for the poorer eye (or the affected eye), and the corrected visual acuity for the better eye. Example: Veteran is service-connected for diplopia and decreased visual acuity of both eyes. Visual acuity of right eye is 20/100; left eye is 20/200. Diplopia is in the 31 to 40 degree range of upward vision, meaning it is the equivalent of 20/40 visual acuity. Therefore, the diplopia measurement has no effect on the overall evaluation, which would be 60 percent. If the diplopia had been in the 21 to 30 degree range of downward vision, it would have been equivalent to 15/200, and the evaluation for the left eye would have been 2 steps poorer than 20/200, or 10/200. The 10/200 combined with visual acuity of 20/100 for the right eye would still result in a combined evaluation of 60 percent. 2. Diplopia extending beyond more than one quadrant or range of degrees When the affected field with diplopia extends beyond more than one quadrant or range of degrees, diplopia will still be evaluated based on the quadrant and degree range that provides the higher (or highest) evaluation. 3. Diplopia in 2 separate areas of same eye When diplopia exists in two separate areas of the same eye, the equivalent visual acuity under diagnostic code 6090 shall be increased to the next poorer level of visual acuity, but not to exceed 5/200. Example: Veteran is service-connected for diplopia. Diplopia in both eyes is in the 31 to 40 degree range of upward vision and in the 31 to 40 degree range of lateral vision. The diplopia in the upward vision is equivalent to visual acuity of 20/40, while the diplopia in the lateral vision is equivalent to visual acuity of 20/70. Based on 4.78 (b) (2) and (3), the overall equivalent visual acuity for diplopia is 20/100, which is one step poorer than the diplopia (in this case, the lateral) that provides the higher evaluation. The overall evaluation for diplopia is therefore 10 percent (based on visual acuity of 20/100 for one eye and 20/40 for the other eye (diplopia is only taken into consideration for one eye). 4. Removal of 6092 We removed diagnostic code 6092 because diplopia due to limited muscle function is not functionally distinct from diplopia (double vision) and does not warrant a separate code. 5. Diplopia that is occasional or correctable Former 4.77 (Examination of muscle function.) included a statement that diplopia that is occasional or correctable is not considered a disability. We have removed that statement and instead placed a note under diagnostic code 6090 stating that, in accordance with 38 CFR 4.31, diplopia that is occasional or that is correctable with spectacles is evaluated at 0 percent. J. Keratoconus Keratoconus is an almost always bilateral corneal degenerative disorder in which there is progressive central thinning of the cornea, changing it from dome-shaped to cone-shaped. Eventually, there is significant visual impairment (often severe myopia and irregular astigmatism, as well as significant photophobia and glare). Its progression can halt at any stage. Early, eyeglasses may provide good vision. Later, rigid gas permeable (RGP) contact lenses are usually required to flatten the anterior corneal surfaces and obtain optimal visual acuity, but lens fitting can be difficult. About 10% of patients will require corneal transplantation, but contact lenses and/or eyeglasses may still be required. Rating keratoconus and other corneal conditions associated with severe astigmatism Section 4.76(b)(2) describes the method of evaluation for any corneal disorder that results in severe, irregular astigmatism where contact lenses are more useful for correction than eyeglasses. Keratoconus is such a corneal disorder. Evaluation of keratoconus under diagnostic code 6035 is therefore, per 4.76(b)(2), based on corrected visual acuity, using contact lenses rather than eyeglass lenses if contact lenses provide the best corrected visual acuity and are customarily worn by the individual. However, if eyeglass lenses can best correct the visual acuity, the usual method of determining corrected visual acuity is the basis of evaluation. The former minimum evaluation was 30 percent when contact lenses were medically required. There is now no minimum evaluation. K. Cataract A cataract is an opacity or cloudiness in the lens of the eye. More than 50% of people over the age of 60 have some form of a cataract. Risk factors include aging, sunlight exposure, smoking, poor nutrition, eye trauma, systemic diseases, and certain medications, such as steroids. Cataracts usually lead to blurred vision, and may cause glare, halos, and dim color vision. Cataracts are not reversible. However, the rate and extent of progression is highly variable, and some cataracts become stationary. In cataract surgery, most or all of the lens is removed and replaced with an artificial intraocular lens or lens implant. Until the 1980's, complete lens removal, or intracapsular cataract extraction, in which the implant lens was placed in front of the iris because the lens capsule was removed, was the rule. Since then, removal of the lens nucleus and cortex, or extracapsular cataract extraction (mainly by phacoemulsification, which is breaking up of the lens by ultrasound, with removal of the pieces by suctioning), with retention of the lens capsule, is routine. The extracapsular surgery is less prone to complications. Rating cataracts: Diagnostic code 6027 now encompasses cataracts of all types. Evaluation preoperatively is based on visual impairment. Evaluation postoperatively is based on visual impairment if a replacement lens is present, and on aphakia (diagnostic code 6029) if there is no replacement lens. Example: Veteran is service-connected for postoperative cataract removal from left eye with a replacement lens present. Visual acuity of left eye is 20/70, with no other visual impairment. Right eye is NSC. Evaluation is 10 percent based on visual acuity of 20/70 for left eye and visual acuity of 20/40 (for the NSC right eye). L. Aphakia, Pseudophakia, and Pseudoaphakia Aphakia is absence of the lens of the eye, usually as a result of cataract extraction, but it may also be congenital or due to perforating wound or ulcer. Complications include detachment of the vitreous or retina, and glaucoma. The term "pseudophakia" means a condition in which the lens has been replaced by an artificial lens after cataract removal. The very similar term, "pseudoaphakia," refers to a membranous cataract. At times these almost identical terms with different meanings have been incorrectly used interchangeably. To avoid any possible confusion, instead of including the term "pseudophakia" in the evaluation criteria for post-operative cataract under diagnostic code 6027, we used unambiguous language concerning the post-operative evaluation of cataracts, with and without a replacement lens. Aphakia and dislocation of lens are now both evaluated under diagnostic code 6029. Rating aphakia: Evaluation is based on visual impairment, elevated by one step, with a minimum evaluation of 30 percent for unilateral or bilateral aphakia because there are additional effects of aphakia, such as severe hyperopia, magnification of the image, reduced peripheral vision, difficulty with image fusion, glare, photophobia, and possible ring scotomas, that are not encompassed by the impairment of visual acuity. Aphakia is treated by a strong convex lens prescription, usually by glasses, but sometimes by contact lenses. Example: The veteran is service-connected for postoperative cataract removal with aphakia of left eye. Corrected visual acuity is 20/70 for the left eye. Right eye is NSC. Veteran also has complaints of photophobia and occasional glare. His calculated evaluation would be 20/70 combined with 20/40, for 10 percent, elevated one step to 20 percent. However, there is a minimum evaluation of 30 percent for unilateral or bilateral aphakia, so the correct evaluation would be 30 percent. M. Diagnostic codes 6000 through 6009 These conditions were formerly evaluated at levels of 10 to 100 percent based on impairment of visual acuity or field loss, pain, rest-requirements, or episodic incapacity, combining an additional rating of 10 percent during continuance of active pathology. Many conditions have been renamed, some deleted, and some combined under a broader title. See the Fast Letter for the Final Rule: Schedule for Rating Disabilities; Eye (FL 09010) for details. They are now evaluated under a general rating formula, based either on visual impairment or on incapacitating episodes, whichever results in a higher evaluation. We defined an incapacitating episode as one requiring bedrest and treatment by a physician or other healthcare provider and provided evaluation levels of 10, 20, 40, and 60 percent based on incapacitating episodes. N. Uveal tract The iris is the most anterior portion of the uvea or uveal tract. Behind the iris are the ciliary body (with the ciliary muscle) and the choroid (beneath the retina). Any part of the uveal tract, including the choroid/retina complex, may become inflamed (uveitis). Symptoms are pain, tearing, light sensitivity, and blurred vision. The course of uveitis is usually 6-8 weeks. Decreased vision may result without treatment. Complications of uveitis or its treatment include cataracts, glaucoma, corneal changes, and retinitis. Uveitis = inflammation of entire uveal tract Iritis = iris inflammation Cyclitis = ciliary body inflammation Iridocyclitis = iris and ciliary body inflammation Choroiditis = choroid inflammation Chorioretinitis = choroid and retina inflammation (The retina frequently becomes inflamed when the choroid does, although it is not part of the uveal tract.) Uveitis may be associated with many systemic diseases, including rheumatoid arthritis, tuberculosis, syphilis, ankylosing spondylitis, Reiters syndrome, toxoplasmosis, histoplasmosis, cytomegalovirus disease, sarcoidosis, toxocariasis, and disorders of the immune system. It may also result from infections of the tonsils, sinuses, kidney, gallbladder, and teeth. O. Epiphora Epiphora is excessive tearing due to insufficient drainage of the tear film from the eyes. The acute type often results from corneal foreign bodies or allergic conjunctivitis, and usually resolves. Causes of chronic epiphora include ectropion and entropion, obstruction in the lacrimal system, and neurogenic disorders. Rating epiphora: Epiphora is rated under diagnostic code 6025 at 10 percent if it is unilateral and at 20 percent if it is bilateral. Example: Veteran had unilateral epiphora due to acquired nasolacrimal duct obstruction. He would be rated at 10 percent. P. Dacryocystitis An inflammation of the lacrimal sac (tear sac), which is often due to an obstruction of the nasolacrimal duct (tear duct). Causes are unknown. Usually causes swelling, redness, and pain below the eye, and there may be a mucous discharge at the nasal corner of the eye. Rating dacryocystitis All disorders of the lacrimal apparatus are rated under diagnostic code 6025 at 10 percent if unilateral and at 20 percent if bilateral. Q. Retina 1. Retinopathy Refers to diseases that affect the retina. Common underlying causes include diabetes and hypertension. Can cause partial or complete loss of vision and can occur slowly or suddenly. Can resolve spontaneously or cause permanent damage, depending on the underlying illness. See TL 00-06 for a discussion of diabetic retinopathy and other eye complications of diabetes. Hypertensive retinopathy is arteriosclerotic damage to the blood vessels of the retina caused by hypertension. It causes blockage (infarcts) or hemorrhage of the blood vessels. The degree of retinopathy is graded on a scale of I to IV. At Grade I, no symptoms may be present. Grade IV hypertensive retinopathy includes swelling of the optic nerve and macula, which can cause decreased vision. Central serous retinopathy (CSR) is a collection of fluid under the retina that may cause blurry vision, distortion of shapes, and sometimes a change in the refractive error towards hyperopia. Retinal detachment may be present. It has been linked to chronic stress and steroid use. Most resolve spontaneously in 3-6 months, but many will recur. 2. Maculopathy (macular retinopathy) A group of diseases resulting in the destruction and dysfunction of cells in the macular area of the retina. Loss of central or detail vision results, with blurred central vision, but intact peripheral vision. Drusen, tiny yellow deposits deep to the central retina, are a common early sign. Age-related macular degeneration (ARMD) or maculopathy is the leading cause of irreversible blindness among Americans 65 and older. Causes are unclear but may include family history, ultraviolet light exposure, advanced age, hyperopia, smoking, and hypertension. Forms of maculopathy are dry ARMD (90% of cases) and wet ARMD. In dry ARMD, vision loss is more gradual and less severe than in wet ARMD. Only 10% of cases are wet ARMD, but it accounts for 90% of all severe vision loss from ARMD. In wet ARMD, new, very fragile blood vessels behind the retina grow toward the macula. These vessels often leak blood and fluid under the macula and can quickly lead to the loss of central vision. Dry ARMD requires no specific treatment, while wet ARMD may require laser surgery to seal leaking blood vessels. Cystoid macular edema (CME) is a painless, cyst-like swelling of the macula that results in blurred, distorted central vision, usually temporary. It may be associated with retinal vein occlusion, uveitis, diabetes, or cataract surgery. 3. Retinal detachment and tear As part of the normal aging process, in most people between age 50 and 70, the vitreous gel becomes liquid and pulls on and detaches from the retina (posterior vitreous detachment). Occasionally, the vitreous membrane pulls enough to create a tear in the retina with a shower of floaters due to blood cells from a broken blood vessel. The tear progress to a detachment of the retina with partial or complete loss of vision, which often resembles a curtain or veil in front of the eye or in the periphery. Nearsighted individuals are at increased risk, as are those who have had cataract surgery or a head or eye injury. If the detachment is not repaired as soon as possible by an argon laser, cryotherapy, or a tiny belt or scleral buckle around the equator of the eye, permanent vision loss can result. 4. Retinitis pigmentosa Retinitis pigmentosa (RP) constitutes a group of hereditary diseases of the retina. Although it is hereditary, as with other hereditary diseases, it may be service-connected. See, for example, VAOPGCPREC 11-1999. Most commonly, the rods of the retina gradually degenerate, resulting in poor night vision and eventually, loss of all peripheral vision to the point that only central or tunnel vision remains. The cones may later degenerate as well. However, some people have primarily cone cell degeneration, and therefore first lose central visual acuity and color discrimination. The majority of people with RP are legally blind by the age of 40. Rating retinitis pigmentosa There is no diagnostic code for retinitis pigmentosa. Evaluation is based on visual impairment. Often this is loss of peripheral visual fields, but there may also be loss of central visual acuity and widespread field loss. R. Optic nerve and disc 1. Optic atrophy Due to acquired or congenital degeneration of the nerve fibers of the optic nerve and optic tract. Produces decreased clarity, visual field changes, or both. The cause is sometimes unknown, but there are many known causes, including central retinal vein or artery occlusions, degenerative retinal disease, glaucoma, certain metabolic diseases (such as diabetes), and toxicity (methanol, tobacco-alcohol, lead, etc.). Degeneration and atrophy of optic nerve fibers is irreversible. Rating optic atrophy: Evaluate based on visual impairment. 2. Optic Neuritis A unilateral inflammation of the optic nerve. Results in blurred or distorted vision, reduced color vision, or a blind spot. Pain on eye movement may precede the visual loss. Most vision is recovered within 6 months, although some loss of vision may be permanent. Causes include demyelinating diseases such as multiple sclerosis or postinfectious encephalomyelitis; systemic viral or bacterial infections such as Lyme disease or tuberculosis; nutritional and metabolic diseases; Lebers Hereditary Optic Neuropathy; complications of sinusitis, meningitis, syphilis, chorioretinitis, etc.; and toxic reactions to tobacco, methanol, and other substances. Treatment often includes steroids. Rating optic neuritis: Evaluate based on visual impairment. 3. Papilledema Swelling of the optic disc (papilla). Most commonly due to increased intracranial pressure (often from a tumor), malignant hypertension, or thrombosis of the central retinal vein. Is also seen in idiopathic intracranial hypertension (pseudotumor cerebri), a condition in which there is elevated cerebrospinal fluid pressure but no space-occupying intracranial lesion. Usually bilateral. Vision is not affected initially, although the blind spot is enlarged. If untreated, secondary optic atrophy and permanent vision loss can occur. S. Glaucoma Glaucoma is a group of diseases that produce damage to the optic nerve and decreased peripheral vision. It may in advanced cases proceed to tunnel vision, in which only the central retina (macular area) is intact. Untreated, it leads to optic atrophy and blindness. Field loss is not measurable until 25% to 40% of the optic nerves axons have been destroyed. Major risk factors include elevated intraocular pressure, family history of glaucoma, advanced age, and being of African-American descent. Other risk factors include cardiovascular disease, diabetes mellitus, myopia, hypertension, and migraine headaches. Eye pressure or intraocular pressure (IOP) Measured by means of a tonometer. Normal range of IOP is 10 mm Hg to 21 mm Hg. IOP may be high due to too much fluid entering the eye or not enough fluid leaving the eye. IOP of 21 mm Hg or higher is ocular hypertension, making someone a glaucoma suspect and at increased risk for developing glaucoma. IOP that will cause glaucoma varies. One-third of glaucoma patients have normal IOP (low tension glaucoma). Loss of visual fields can therefore occur even when the pressure is not elevated. Many with elevated IOP have no evidence of glaucomatous damage. Some patients with ocular hypertension are treated to lower the pressure but many are just observed. In an examination for glaucoma, the examiner checks the intraocular pressure, does a visual field exam, and examines the optic nerve. Primary Open Angle Glaucoma is the most common type (90% of all glaucoma). It is asymptomatic early but shows elevated IOP in most cases, enlargement of the optic cup, and progressive visual field loss. It is treated primarily with medications. Angle Closure Glaucoma includes 2 types: acute and chronic (or primary) angle closure glaucoma. Acute angle closure glaucoma may be associated with blurred vision, colored halos, severe pain, red eye, and nausea or vomiting. Eye pressure usually goes up very fast, within hours, and may be 40 to 70 mm Hg. It requires emergency care to prevent substantial eye damage. Chronic angle closure glaucoma is often asymptomatic, and changes are more gradual. Treatment includes medications, laser procedures, and surgery. Secondary glaucoma may also occur. Steroid-induced glaucoma may result from either steroid eye-drops or systemically administered steroids. The eye pressure usually returns to normal in a few days or weeks after discontinuing the medication. Neovascular glaucoma is associated with proliferative diabetic retinopathy or central retinal vein occlusion. Diagnostic code 6012 is now "angle-closure glaucoma" instead of "glaucoma, congestive or inflammatory," and diagnostic code 6013 is now "open-angle glaucoma" instead of "glaucoma, simple, primary, noncongestive". Rating angle closure glaucoma: This was formerly evaluated either as iritis or by rating at 100 percent if there were "frequent attacks of considerable duration; during continuance of actual total disability." It is now evaluated the same as diagnostic codes 6000 through 6009, based either on visual impairment or on incapacitating episodes, whichever results in a higher evaluation. There is a 10 percent minimum evaluation if continuous medication is required, but no minimum evaluation if there is no visual impairment and no treatment is needed other than frequent observation. Rating open angle glaucoma: Formerly, this was evaluated based on impairment of visual acuity or field loss, with a minimum evaluation of 10 percent. Now, it is evaluated based on visual impairment, with a 10 percent minimum evaluation if continuous medication is required, but there is no minimum evaluation if there is no visual impairment and no treatment is needed other than frequent observation. T. Trachoma Trachoma is an infection caused by Chlamydia trachomatis. It spreads from person to person by flies feeding on ocular secretions. Endemic to North Africa, Southeast Asia, India, and the Middle East. First produces conjunctivitis, then infection of the cornea, and may progress to conjunctival scarring, corneal scarring, and blindness. Antibiotics are effective for treatment. Rating trachoma - Trachoma was formerly evaluated based on impairment of visual acuity, with a minimum evaluation of 30 percent for active pathology. There is still a 30 percent minimum evaluation for active trachoma. Evaluation for inactive trachoma is based on residuals, such as visual impairment and disfigurement. U. Toxoplasmosis Toxoplasmosis is an infection caused by the parasite Toxoplasma gondii. Most affected people have no symptoms, but it can affect the brain, eyes, and other organs. It is most serious in pregnant women and in people with compromised immune systems. The great majority of infections are congenital, due to transmission from an infected mother to the fetus. However, it can be acquired by eating contaminated raw or undercooked meat or dairy products or by ingesting food, water, or dirt contaminated with cat feces. Wild and domestic cats are hosts for the parasite. In the eye, it can result in retinitis and uveitis and may lead to severe visual loss (in about one-third). Treatment includes antimicrobial agents, and sometimes steroids. V. Amaurosis Fugax Amaurosis fugax is transient, unilateral, partial blindness that lasts from a few seconds to several hours. After an episode, vision returns to normal. It is a type of transient ischemic attack (TIA) caused by partial loss of blood flow to the eye from any of a number of causes, for example, an embolus temporarily blocking circulation to the retina, vasospasm, arteritis, or atherosclerotic vascular stenosis. Rating amaurosis fugax Amaurosis fugax is best rated analogous to TIAs of other types under the neurologic section of the rating schedule. Until the neurologic portion of the rating schedule is revised, diagnostic code 8046, (cerebral arteriosclerosis) is probably the most appropriate code, and evaluation at 10 percent might be considered when there are repeated episodes. W. Eye vascular disorders Behcet's Disease An auto-immune disease that causes inflammation of the blood vessels (occlusive vasculitis). Characterized by recurrent uveitis, retinitis, and oral and genital ulceration. May also cause arthritic, gastrointestinal, and central nervous system symptoms. Many patients with ocular involvement will eventually be legally blind. Branch Retinal Artery Occlusion Usually due to an embolus from a carotid artery. Presents with painless, sudden loss of peripheral, and sometimes central, vision. Approximately 90% will recover visual acuity of 20/40 or better. Branch Retinal Vein Occlusion Due to a localized thrombus in a branch retinal vein due to arteriosclerosis in an adjacent arteriole. Usually results in unilateral decreased central vision, but loss of peripheral vision, distortion of vision, or "blind spots" may occur. Among the common predisposing factors are hypertension, diabetes, and cardiovascular disease. Some require laser treatment, but many have resolution of the retinal hemorrhages and macular swelling over several months, with retention of good vision. Central Retinal Artery Occlusion Usually due to an embolus from the carotid artery that prevents blood flow to the retina. Presents with unilateral severe loss of vision. May be preceded by episodes of amaurosis fugax. Two-thirds are associated with underlying hypertension, and one-fourth have carotid artery arteriosclerosis, valvular heart disease, or diabetes. No treatment has conclusively been shown to be of value. The retina suffers irreversible injury after only 90 minutes of ischemia, so the great majority have severe and permanent visual loss. Central Retinal Vein Occlusion Due to a thrombus of the central retinal vein. Associated with hypertension, chronic open-angle glaucoma, or arteriosclerosis. Presents with sudden, painless, visual loss. The worse the vision initially, the worse the final visual acuity. Complications may require retinal laser treatment. X. Pterygium and pinguecula These are benign ocular surface growths caused by wind, dust, and ultraviolet light. Pinguecula (plural is pingueculae) A pinguecula is a callus-like benign growth of the conjunctiva. It appears as a small, yellow or white, thickened area of tissue that may contain calcium. It grows adjacent to the cornea, but not over it, usually on the nasal side. Pingueculae do not affect vision, but may cause irritation if elevated. Rating pingueculae Pingueculae are evaluated under diagnostic code 6037 based on disfigurement because most result in few, if any, symptoms. However, if chronic inflammation occurs, with findings such as swelling, redness, and irritation (sensation of foreign body), consider an evaluation analogous to chronic conjunctivitis (diagnostic code 6018). Pterygium (plural is pterygia) A pterygium is a benign connective tissue (fibrovascular) wing-shaped lesion extending from the conjunctiva onto the cornea. Pterygia are slightly elevated, white or reddish, and are most common on the nasal side of the cornea. Complications include irregular astigmatism; diplopia (if an extra-ocular muscle is affected); and other visual problems. Symptoms may include, in addition to visual problems, redness, itching, and irritation. However, some are only a cosmetic problem. Rating pterygia Pterygia were formerly evaluated based on loss of vision but are now evaluated under diagnostic code 6034 based on visual impairment, disfigurement, conjunctivitis, etc., depending on the particular findings. More than one evaluation may be needed if there are multiple separate disabling effects. Example: Veteran was diagnosed with asymptomatic pterygia of both eyes on separation exam. His visual examination was otherwise normal, with bilateral visual acuity of 20/20. He was service connected at 0 percent for bilateral pterygia. Five years later, he claimed an increase because the pterygium of the left eye had grown and was now affecting his vision. He also complained of progressive redness, irritation, and watering of his left eye. Examination showed a red, thick conjunctiva on the left with mucous secretion and a reddish pterygium partially covering the cornea. Visual acuity of the right eye was 20/20, but he had developed an astigmatism of the left eye because the pterygium had covered and distorted part of the cornea, resulting in a visual acuity of 20/50. Note that in this case, astigmatism, which is ordinarily a developmental error of refraction, is an acquired condition secondary to a service connected condition and is therefore evaluated as part of his service connected disability. His evaluation would be 10 percent for active chronic conjunctivitis of left eye (under diagnostic code 6018) and 10 percent for decreased visual acuity (under diagnostic code 6066), for a combined evaluation of 20 percent. Y. Symblepharon A symblepharon is an adhesion between the conjunctivae or between the conjunctiva and the cornea. Common causes are ocular pemphigoid (a rare inflammatory blistering condition that affects mainly the oral and ocular mucous membranes and sometimes the skin, resulting in adhesions and scarring), surgery for pterygia or other eye disorders, or chemical injury. Rating symblepharon: Symblepharon was formerly rated under the criteria for diagnostic code 6090 (diplopia). However, since it may also result in other types of impairments, it is now evaluated under diagnostic code 6091 based on the specific findings present, which may include visual impairment, lagophthalmos, disfigurement, etc. It may be evaluated under more than one set of criteria when appropriate. Example: Veteran is service-connected for symblepharon of right eye due to an accidental caustic chemical burn of her right eye. She has residuals of right lagophthalmos due to scarring with diplopia of right eye on upward vision in the area of 31 to 40 degrees, also attributed to symblepharon. Her left eye is normal. Evaluation is 10 percent under 6022 for unilateral lagophthalmos, and 0 percent under 6066 for diplopia, for a combined evaluation of 10 percent. Z. Nystagmus Nystagmus is a group of conditions with involuntary unilateral or bilateral repetitive, rhythmic eye movements. The movements may be slow or fast, horizontal, vertical, or rotary. It is often due to neurologic disease, such as multiple sclerosis, and can be caused temporarily by certain drugs, although nystagmus is congenital in many cases. Nystagmus may or may not be associated with visual impairment. Rating nystagmus: Evaluate under diagnostic code 6016 at 10 percent if it is central (due to neurologic disease). AA. Ectropion Sagging and outward turning of the lower eyelid and eyelashes. Can lead to excessive tearing, crusting of the eyelid, mucous discharge, and irritation of the eye. Most are due to relaxation of the tissues of the eyelid as a result of aging, but some result from scarring of the eyelid caused by chemical and thermal burns, trauma, skin cancers, or previous eyelid surgery. Rating ectropion: Evaluate under diagnostic code 6020 at 10 percent if unilateral and 20 percent if bilateral. BB. Entropion Entropion is a rolling inward of the lower eyelid and eyelashes towards the eye. The skin of the eyelid and the eyelashes rub against the cornea and conjunctiva. Can lead to excessive tearing, crusting of the eyelid, mucous discharge, a feeling that something is in the eye, irritation of the cornea, and impaired vision. Entropion is usually due to relaxation of the tissues of the eyelid as a result of aging, but may also result from scarring of the inner eyelid by chemical or thermal burns, inflammatory diseases such as ocular pemphigoid, or allergic reactions. Rating entropion: Evaluate under diagnostic code 6021 at 10 percent if unilateral and 20 percent if bilateral. CC. Lagophthalmos Inability or decreased ability to close the upper eyelid. Due to paralysis of the orbicularis muscle in the eyelid. May stem from Bell's Palsy, vascular lesions, trauma, neurosurgery, and other causes, including thyroid eye disease. May be cosmetic but may also include dry eye, discomfort, corneal ulceration, and decreased vision. Rating lagophthalmos Lagophthalmos is rated at 10 percent if unilateral and 20 percent if bilateral, under diagnostic code 6022. Example: Veteran has bilateral lagophthalmos as a residual of thyroid disease. Evaluation is 20 percent. DD. Corneal transplant Corneal transplant, or keratoplasty, is used to treat such conditions as corneal opacification due to trauma or infection of the cornea, keratoconus, corneal dystrophy, and corneal scarring from previous eye surgery. Complications include infection, bleeding, glaucoma, retinal detachment, cataract formation, uveitis, and rejection of the graft. Rejection occurs in 5 to 30%. Rating corneal transplant Corneal transplant is evaluated under diagnostic code 6036, with evaluation based on visual impairment. There is a minimum evaluation of 10 percent if there is pain, photophobia, and glare sensitivity, which may be signs of rejection of the transplant. Other complications, such as retinal detachment or glaucoma, may require separate ratings if they result in additional disabilities. Example: Veteran had a bilateral corneal transplant 16 months ago for severe service connected keratoconus. His initial visual acuity after surgery was 20/40 in right eye, 20/40 in left eye, but recently has been 20/40 in right eye, 20/70 in left eye, with blurring at times in right eye, plus excessive glare, redness, and occasional pain. He has been undergoing treatment for transplant rejection. He is rated at 10 percent under 6066. Although he would be entitled to the minimum 10 percent evaluation for his symptoms if his visual acuity were normal, he does not warrant an additional 10 percent for those symptoms because he can be evaluated based on visual acuity. Since he is undergoing treatment, a future examination is warranted. EE. Hemianopsia Hemianopsia means loss of half of a visual field. Certain brain lesions almost always take away the right or left halves of the field rather than the top or the bottom. Hemianopsia may occur in one eye or both, according to where the lesion is. A lesion that results in the loss of the nasal half of the visual field of one eye and loss of the temporal half of the visual field in the other eye is called homonymous hemianopsia. Common causes are strokes, tumors, and brain injuries. Rating hemianopsia All types of hemianopsia and all other visual field defects are evaluated under diagnostic code 6080. Homonymous hemianopsia is evaluated at 30 percent. Hemianopsia due to bilateral loss of the nasal field is 10 percent and due to bilateral loss of the temporal field is 30 percent. Hemianopsia is a distinct entity that would always be evaluated at 10 or 30 percent. If a field loss is not clearly hemianopsia, the usual visual field measurements would be needed and would be the basis of evaluation. FF. Scotoma Scotoma is a loss of vision in a defined area or island in one or both eyes, and is often called a blind spot (as opposed to "the" blind spot, which is the physiologic blind spot that everyone has due to the optic nerve passing through the retina). It may be due to demyelinating diseases, such as multiple sclerosis; macular degeneration; glaucoma; choroiditis, and many other conditions. Scotoma of various types can be a prodromal symptom of migraine, where it is often scintillating, but may also be due to a lesion within the eyeball, choroiditis, hemorrhage, or a lesion in the visual system in the brain. Rating scotoma - Scotoma is evaluated under diagnostic code 6081. Formerly, the criteria called for a minimum 10 percent evaluation for a large or centrally located scotoma. Now, the minimum evaluation (for each eye) for a scotoma affecting at least one-quarter of the visual field (quadrantanopsia) or that is centrally located and of any size is 10 percent. Otherwise, evaluation is based on visual impairment. GG. Benign and malignant neoplasms of the eye Benign eye neoplasms are evaluated under diagnostic code 6015 based on visual impairment combined with a rating for any nonvisual impairment, such as disfigurement. There is no longer a minimum evaluation. Malignant eye neoplasms are evaluated under diagnostic code 6014, and there are 2 possible methods of evaluation. If a malignant neoplasm of the eyeball requires therapy that is comparable to that used for systemic malignancies, i.e., systemic chemotherapy, X-ray therapy more extensive than to the eye, or surgery more extensive than enucleation, a 100 percent evaluation would be assigned and continued beyond the cessation of treatment, with a mandatory VA examination six months following the completion of such treatment, with any change in evaluation subject to 3.105(e). If there has been no local recurrence or metastasis, evaluation is then made on residuals. However, not all malignant neoplasms of the eye (for example, iris melanoma and choroid melanoma) are totally disabling or require treatment that is totally disabling for a period of time; some require no treatment other than observation. If a malignant neoplasm of the eyeball does not require therapy that is comparable to that for systemic malignancies, that is, treatment is confined to the eye, evaluate based on visual impairment and nonvisual impairment, such as disfigurement (diagnostic code 7800). Residuals are also evaluated in this way. HH. Ptosis Ptosis means a droopy eyelid (blepharoptosis). Can affect vision if it covers part or all of the pupil. May be congenital or acquired. May be due to muscle (levator) or nerve (oculomotor nerve) impairment associated with aging, injury, neoplasm, diabetes, myasthenia gravis, aneurysm, stroke, post-cataract surgery, etc. May be a cosmetic problem and may also result in loss of the superior field of vision. Rating ptosis Formerly, ptosis (diagnostic code 6019) was evaluated equivalent to visual acuity of 5/200 whenever the pupil was wholly obscured, equivalent to 20/100 if the pupil was one-half or more obscured, and on disfigurement if less than one-half of the pupil is obscured. Now, because the extent to which a pupil is obscured can be difficult to determine reliably, it is evaluated based on visual impairment or, in the absence of visual impairment, on disfigurement. II. Loss of portion of the eyelids Loss of portion of the eyelids (diagnostic code 6032) was formerly based on disfigurement. Now it is based on visual impairment, combined with an evaluation for nonvisual impairment, such as disfigurement. JJ. SMC footnotes The footnotes referring to entitlement to SMC have been revised. See the Fast Letter for the Final Rule: Schedule for Rating Disabilities; Eye, for details of the changes.     Page  PAGE 26. 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