II. Epidemiology

  1. Leading cause of central Vision Loss over age 65 years
  2. Age-related changes
    1. Onset after age 50 years (mean onset: 75 years old)
    2. Prevalence
      1. Sixth decade: 1%
      2. Ninth decade: 15%

III. Risk Factors

  1. Advancing age (see epidemiology above)
  2. Female sex
  3. White race
  4. Tobacco abuse (increases risk 3 fold)
  5. Diet low in caretenoids
  6. Increased sunlight exposure
  7. Cardiovascular disease
  8. Aspirin (weak evidence only by observation study)
    1. Neovascular Macular Degeneration (wet AMD) was observed more with Aspirin use for >10 years
    2. Klein (2012) JAMA 308(23):2469-78 [PubMed]

IV. Pathophysiology

  1. Idiopathic
  2. Retinal photoreceptor dysfunction
    1. Results from atrophy or Choroidal neovascularization
    2. Mediators
      1. Subretinal Hemorrhage
      2. Subretinal Fibrosis (disciform scarring)
      3. Subretinal Fluid and lipid accumulation
    3. Outcome: Decreased Visual Acuity to blindness

V. Types

  1. Non-neovascular, dry, non-exudative ARMD
    1. Accounts for 80% of all diagnosed cases
    2. Geographic atrophy and drusen (yellow lipid deposits) predominate
  2. Neovascular, wet, exudative ARMD
    1. Accounts for 80% of significant visual Disability
    2. Choroidal neovascularization (CNV)
    3. Complicated by Macular Hemorrhage

VI. Symptoms

  1. Slow, insidious central Vision Loss
  2. Decreased dark adaptation

VII. Exam

  1. Amsler Grid
    1. Identifies central visual defects
    2. While staring at a central black dot, patients report missing or distorted grid lines

VIII. Signs: Bilateral eye involvement

  1. Drusen
    1. Yellow lipid deposits, debris within Retinal pigment basement membrane
    2. Appear with ophthalmoscope as yellow spots on Retina
    3. Small drusen <63 microns are seen on aging Macula
    4. Numerous, large drusen are consistent with ARMD
  2. Geographic atrophy
  3. Serous Retinal pigment epithelial detachment
  4. Choroidal neovascularization

IX. Management: General

  1. See Prevention and Nutritional Supplements and Antioxidants as below
  2. Vascular Endothelial Growth Factor Inhibitors
    1. Indicated for neovascular ARMD
    2. Intravitreal injection monthly for 4 months (or until stable), then every 3 months
    3. Monoclonal antibodies available for intravitreal injection
      1. Bevacizumab (Avastin)
        1. Not FDA approved, but $150/dose compared to >$1500 for other agents
      2. Ranibizumab (Lucentis)
      3. Pagaptanib (Macugen)
      4. Aflibercept (Eylea)

X. Management: Slow progression of CNV-related Vision Loss

  1. Laser photocoagulation of subretinal blood vessels
  2. Photodynamic therapy
    1. Give IV photosensitive drug (wavelength specific)
    2. Expose eye to targeted wavelength to activate drug
  3. Experimental
    1. External beam radiotherapy
    2. Vitreoretinal microsurgery

XI. Management: Visual aids

  1. Magnifiers
  2. Increased light sources in home environment

XII. Prevention: General measures

  1. Tobacco Cessation is key
  2. Control Hypertension
  3. Decrease UV Light exposure (Sunglasses)
  4. Nutrition
    1. See Nutritional Supplements below
    2. Decrease Dietary Fat intake
    3. Increase grean leafy vegetable intake

XIII. Prevention: Nutritional Supplements and Antioxidants

  1. Vitamin C, Vitamin E, Beta Carotene, and Zinc
    1. Indications
      1. Intermediate or advanced ARMD
    2. Efficacy
      1. May reduce progression from intermediate to advanced ARMD (NNT 22 over 5 years)
      2. May protect the other eye when ARMD is present in one eye
      3. Does not prevent development of ARMD
    3. Components (based on AREDS Study)
      1. Vitamin C 500 mg
      2. Vitamin E 400 IU
      3. Zinc 80 mg (25 mg in AREDS2)
      4. Copper 2 mg
      5. Beta Carotene 15 mg (removed in new formulations and replaced by Lutein and Zeaxanthin)
        1. No benefit per AREDS 2 study (and increased Lung Cancer risk in smokers)
    4. Components (based on AREDS2 Study)
      1. Includes components of the original AREDS Study without Beta Carotene
      2. Lutein 10 mg daily
        1. Improves vision in men with Macular Degeneration
        2. Richer (2004) Optometry 75:216-30 [PubMed]
      3. Zeaxanthin 2 mg
      4. Zinc 25 mg (reduced from 80 mg in AREDS formulation)
    5. References
      1. (2013) Presc Lett 20(8):45
      2. (2001) Arch Ophthalmol 119:1417-36 [PubMed]
  2. Components not found useful in ARMD prevention
    1. Beta Carotene (and Lung Cancer risk in smokers)
    2. Omega-3 Fatty Acids

XIV. Screening

  1. Initiate at age 45 years with dilated exam

XV. References

Images: Related links to external sites (from Bing)

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Ontology: Macular degeneration (C0024437)

Definition (CHV) deterioration of the eye part called macula lutea of the retina
Definition (CHV) deterioration of the eye part called macula lutea of the retina
Definition (CHV) deterioration of the eye part called macula lutea of the retina
Definition (CHV) deterioration of the eye part called macula lutea of the retina
Definition (MEDLINEPLUS)

Macular degeneration, or age-related macular degeneration (AMD), is a leading cause of vision loss in Americans 60 and older. It is a disease that destroys your sharp, central vision. You need central vision to see objects clearly and to do tasks such as reading and driving.

AMD affects the macula, the part of the eye that allows you to see fine detail. It does not hurt, but it causes cells in the macula to die. There are two types: wet and dry. Wet AMD happens when abnormal blood vessels grow under the macula. These new blood vessels often leak blood and fluid. Wet AMD damages the macula quickly. Blurred vision is a common early symptom. Dry AMD happens when the light-sensitive cells in the macula slowly break down. Your gradually lose your central vision. A common early symptom is that straight lines appear crooked.

Regular comprehensive eye exams can detect macular degeneration before the disease causes vision loss. Treatment can slow vision loss. It does not restore vision.

NIH: National Eye Institute

Definition (CSP) deterioration of the macula lutea in the retina; may be inherited, drug induced, or due to aging; leads to a severe loss of central vision while peripheral vision is retained.
Concepts Disease or Syndrome (T047)
SnomedCT 422338006, 302891003
LNC MTHU020821
English MACULA LUTEA DEGENERATION, MACULAR DEGENERATION, Macular degeneration, Degenerative disorder of macula (disorder), Degenerative disorder of macula, macular degeneration (physical finding), macular degeneration, degeneration of macula, Macula lutea degeneration, Retinal macula lutea degeneration, Macular luteal degeneration, Degeneration macular, macular degenerations, Degeneration;macular, degeneration macular, macula lutea degeneration, Macular Degeneration, AMD, macular degeneration (diagnosis), Macular degeneration (disorder), degenerative disorder of macula (diagnosis), Macular degeneration of retina, unspecified
German MAKULADEGENERATION, Degeneration der Makula, retinale Macula lutea Degeneration, Macula lutea Degeneration, MAKULA LUTEA DEGENERATION, Makuladegeneration
Italian Degenerazione maculare, Degenerazione della macula lutea retinica
Spanish trastorno degenerativo de la mácula (trastorno), trastorno degenerativo de la mácula, Degeneración de la mácula lútea, Degeneración de la mancha amarilla de la retina, MACULA LUTEA, DEGENERACION, degeneración macular (trastorno), degeneración macular, Degeneración macular
Dutch macula lutea retina degeneratie, degeneratie macula, macula lutea degeneratie, Maculadegeneratie, macula degeneratie
French Dégénérescence de la tache jaune rétinienne, Dégénérescence maculaire lutéale, Dégénérescence de la macula lutea, DEGENERESCENCE DE LA MACULA LUTEA, DEGENERESCENCE MACULAIRE, Dégénérescence maculaire
Portuguese Degenerescência da mácula lútea, Degenerescência da mácula lútea retiniana, DEGENERESCENCIA DA MACULA LUTEA, DEGENERESCENCIA MACULAR, Degenerescência macular
Japanese 網膜黄斑変性, 黄斑変性, モウマクオウハンヘンセイ, オウハンヘンセイ
Czech Makulární degenerace, Degenerace sítnice v místě macula lutea, Degenerace macula lutea, Degenerace makula lutea
Hungarian Macula lutea degeneratio, Macula degeneratio

Ontology: Age related macular degeneration (C0242383)

Definition (MSH) Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (CHV) A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness
Definition (NCI_NCI-GLOSS) A condition in which there is a slow breakdown of cells in the center of the retina (the light-sensitive layers of nerve tissue at the back of the eye). This blocks vision in the center of the eye and can cause problems with activities such as reading and driving. Age-related macular degeneration is most often seen in people who are over the age of 50.
Definition (NCI) Age-related loss of vision in the central portion of the retina (macula), secondary to retinal degeneration.
Concepts Disease or Syndrome (T047)
MSH D008268
ICD9 362.50
ICD10 H35.30
SnomedCT 267718000, 155112007, 18222007, 302891003, 193385004, 193384000
LNC LA16307-3
English Degeneration, Macular, Degenerations, Macular, Macular Degeneration, Macular Degenerations, SMD - Senile macular degen, AAMD - Age related macular degeneration, Senile macular degen.unspecif., Unspecified senile macular degeneration, ARMD (age related macular degeneration), Age related macular degeneration, AMD-Age-related macular degen, ARMD-Age-related macular degen, Age-related macular degeneratn, Macular degeneration NOS, Maculopathy, Age-Related, Macular Dystrophies, Maculopathies, Age Related, Age-Related Macular Degeneration, AGE RELAT MACULOPATHY, Age Related Maculopathies, AGE RELAT MACULOPATHIES, MACULOPATHY AGE RELAT, Macular Degenerations, Age-Related, Age Related Macular Degeneration, Age-Related Macular Degenerations, Degeneration, Age-Related Macular, Degenerations, Age-Related Macular, Age-Related Maculopathy, Macular Dystrophy, Dystrophies, Macular, Maculopathy, Age Related, MACULOPATHIES AGE RELAT, Age-Related Maculopathies, Dystrophy, Macular, Maculopathies, Age-Related, Age Related Maculopathy, Macular Degeneration [Disease/Finding], age related macular degeneration (ARMD), age-related maculopathy, age related maculopathy, senile macular degeneration, Macular degeneration (senile) of retina, unspecified, Macular degeneration senile, age related macular degeneration, Macular degeneration (senile), unspecified, Macular Degeneration, Age-Related, Age related macular degeneration (disorder), Unspecified senile macular degeneration (disorder), Age-related macular degeneration (diagnosis), macular degeneration age-related, Macular degeneration, age-related, age-related macular degeneration, AMD, macular degeneration, ARMD, Macular degeneration, SMD - Senile macular degeneration, Senile macular degeneration, AMD - Age-related macular degeneration, ARMD - Age-related macular degeneration, Age-related macular degeneration, Age-related macular degeneration (disorder), Senile macular retinal degeneration, Senile macular retinal degeneration, NOS, Age related macular degeneration (disorder) [Ambiguous], Senile macular degeneration of retina, unspecified
Swedish Makuladegeneration
Czech makulární degenerace, Senilní makulární degenerace, Degenerace makuly spojená s věkem, Makulární (senilní) degenerace sítnice, blíže neurčená, degenerace makuly, věkem podmíněná degenerace makuly, věkem podmíněná makulární degenerace
Finnish Makularappeuma
Russian MAKULIARNAIA DEGENERATSIIA, ZHELTOGO PIATNA DISTROFIIA, VMD, ВОЗРАСТНАЯ МАКУЛЯРНАЯ ДЕГЕНЕРАЦИЯ, VOZRASTNAIA MAKULIARNAIA DEGENERATSIIA, ВМД, ЖЕЛТОГО ПЯТНА ДИСТРОФИЯ, МАКУЛЯРНАЯ ДЕГЕНЕРАЦИЯ
Japanese 加齢黄斑変性, 黄斑症-加齢関連, 黄斑変性, 黄斑部変性, 黄斑部変性症, 黄斑変性症, 加齢関連黄斑症, 網膜黄斑変性症, 加令関連黄斑症, カレイオウハンヘンセイ, ロウジンセイオウハンヘンセイ, 老人性黄斑変性, 老人性黄斑変性、詳細不明, ロウジンセイオウハンヘンセイショウサイフメイ, 網膜黄斑変性, 黄斑症-加令関連
French DMLA, Dégénérescence maculaire liée à l'âge, Maculopathie liée à l'âge, Dystrophie maculaire, Dégénérescence maculaire (sénile) de la rétine, non précisée, Dégénérescence maculaire sénile, Dégénérescence maculaire, Dégénérescence de la macula
Italian DMLE, Degenerazione maculare legata all'età, Distrofia maculare, AMD, Degenerazione maculare senile, Degenerazioni maculari legate all'età, Degenerazione maculare correlata all'età, Degenerazione senile della macula, Degenerazione maculare (senile) della retina, non specificata, Degenerazione maculare
Portuguese Maculopatia Relacionada a Idade, Degenerescência macular relacionada com a idade, Degenerescência macular senil, Degenerescência senil da mácula, Degenerescência macular (senil) da retina NE, Degeneração Macular
Spanish Maculopatía Relacionada con la Edad, Degeneración macular senil, Degeneración macular asociada a la edad, Degeneración macular (senil) de la retina, no especificada, degeneración retiniana macular senil (trastorno), degeneración senil de la mácula retiniana, degeneración macular relacionada con la edad (trastorno), degeneración macular relacionada con la edad, degeneración macular senil, degeneración retiniana macular senil (concepto no activo), degeneración retinal macular senil, degeneración retiniana macular senil, degeneración senil de la mácula retinal, Degeneración Macular
Croatian ŽUTA PJEGA, DEGENERACIJA
Polish AMD, Zwyrodnienie plamki żółtej, Zwyrodnienie plamki żółtej związane z wiekiem
German Makuladegeneration (senil) der Retina, unspezifisch, Makuladegeneration senil, senile Makuladegeneration, altersbedingte Makuladegeneration, Makuladegeneration
Dutch macula degeneratie seniel, seniele macula degeneratie, niet-gespecificeerde macula degeneratie (seniel) van de retina, leeftijdsgebonden maculadegeneratie, Degeneratie, macula-, Maculadegeneratie
Hungarian Macula degeneratio (senilis), nem meghatározott, Életkorhoz kötött macula degeneratio, Senilis macularis degeneratio, Senilis macula degeneratio
Norwegian Makuladegenerasjon, Aldersrelatert makuladegenerasjon, Maculadegenerasjon, Senil makuladegenerasjon