0. Ocular Manifestations of Systemic Disease

1. OCULAR MANIFESTATIONS
Introduction
OCULAR MANIFESTATIONS
SYSTEMIC DISEASES

2. CATEGORIES OF SYSTEMIC DISEASE
Introduction
CATEGORIES OF SYSTEMIC DISEASE
Congenital
Traumatic
Vascular
Neoplastic
Autoimmune
Idiopathic
Infectious
Metabolic/Endocrine
Drugs/Toxins

3. EYE EXAMINATION Vision External Pupils Motility Anterior segment exam
Introduction
EYE EXAMINATION
Vision
External
Pupils
Relative afferent pupillary defect
Motility
Anterior segment exam
Dilated ophthalmoscopy
Visual fields

4. CONGENITAL DISORDERS Down syndrome Marfan syndrome Myotonic dystrophy
Tuberous sclerosis
Neurofibromatosis
Congenital metabolic disorders
Lysosomal storage
Carbohydrate metabolism

5. NEUROFIBROMATOSIS NF1 Café-au-lait spots Iris Lisch nodules
Congenital Disorders
NEUROFIBROMATOSIS NF1
Café-au-lait spots
Iris Lisch nodules

6. Shaken baby syndrome: multiple retinal hemorrhages
Traumatic Disorders
Shaken baby syndrome: multiple retinal hemorrhages

7. SYSTEMIC HYPERTENSION: FACTORS AFFECTING RETINAL ARTERIOLES
Vascular Disorders
SYSTEMIC HYPERTENSION: FACTORS AFFECTING RETINAL ARTERIOLES
Severity
Duration

8. Malignant hypertension
Vascular Disorders
Malignant hypertension

9. ophthalmic circulation
Vascular Disorders
Embolic Disease
Sites of emboli to the
ophthalmic circulation
Cholesterol embolus
(Hollenhurst plaque)

10. Left eye with superotemporal branch retinal artery occlusion
Vascular Disorders
Left eye with superotemporal branch retinal artery occlusion

11. “Cherry red spot” in CRAO
Vascular Disorders
CRAO
“Cherry red spot” in CRAO
Normal fundus

12. AMAUROSIS FUGAX "Fleeting Blindness"
Vascular Disorders
AMAUROSIS FUGAX "Fleeting Blindness"
Monocular dimming of vision
Temporary arterial obstruction
Sudden, transient, painless visual loss

13. AMAUROSIS FUGAX: A MULTIDISCIPLINARY EVALUATION
Vascular Disorders
AMAUROSIS FUGAX: A MULTIDISCIPLINARY EVALUATION
Cardiovascular
Cerebrovascular
Ophthalmologic

14. Talc embol in IV drug abuser
Vascular Disorders
Talc embol in IV drug abuser

15. Central retinal vein occlusion (CRVO)
Vascular Disorders
Central retinal vein occlusion (CRVO)

16. MIGRAINE: VISUAL SYMPTOMS
Vascular Disorders
MIGRAINE: VISUAL SYMPTOMS
Left eye
Right eye
Transient homonymous hemianopia
Scintillations
Transient obscuration of vision
Transient homonymous hemifield loss
Scintillations

17. MIGRAINE: TYPES With headache Without headache Classic Common
Vascular Disorders
MIGRAINE: TYPES
With headache
Classic
Common
Complicated
Without headache
Acephalgic

18. MIGRAINE: TREATMENT Prevent frequent attacks Examine for visual loss
Vascular Disorders
MIGRAINE: TREATMENT
Prevent frequent attacks
Examine for visual loss
Consider discontinuing oral contraceptives

19. BLOOD DYSCRASIAS WITH OCULAR MANIFESTATIONS
Vascular Disorders
BLOOD DYSCRASIAS WITH OCULAR MANIFESTATIONS
Hyperviscosity syndromes
Thrombocytopenia
Anemia, including sickle cell anemia

20. HYPERVISCOSITY SYNDROMES: OCULAR SYMPTOMS
Vascular Disorders
HYPERVISCOSITY SYNDROMES: OCULAR SYMPTOMS
Amaurosis fugax
Permanent visual loss

21. Vascular Disorders
Hyperviscosity dilated retinal veins, retinal hemorrhages, and disc edema

22. Vascular Disorders
Leukemia

23. Leukemic infiltration of the optic nerve
Vascular Disorders
Leukemic infiltration of the optic nerve

24. SICKLE CELL RETINOPATHY
Vascular Disorders
SICKLE CELL RETINOPATHY
HbSC disease (most common form)
HbSS disease
Sickle thalassemia

25. Retinal arterial occlusions “Sea fan” neovascularization
Vascular Disorders
SICKLE CELL ANEMIA
Retinal arterial occlusions
“Sea fan” neovascularization

26. MALIGNANT NEOPLASMS INVOLVING THE EYE
Neoplastic Disorders
MALIGNANT NEOPLASMS INVOLVING THE EYE
Primary ocular melanoma
Large cell lymphoma
Metastatic carcinoma

27. METASTATIC CARCINOMA Most common intraocular malignancy in adults
Neoplastic Disorders
METASTATIC CARCINOMA
Most common intraocular malignancy in adults
May be asymptomatic
May produce decreased or distorted vision

28. Iris metastasis from lung carcinoma
Neoplastic Disorders
Iris metastasis from lung carcinoma

29. Choroidal metastasis from breast cancer
Neoplastic Disorders
Choroidal metastasis from breast cancer

30. Neoplastic Disorders
Ophthalmologists should monitor ocular metastases at regular intervals.

31. AUTOIMMUNE DISORDERS Connective tissue diseases Thyroid eye disease
Myasthenia gravis

32. CONNECTIVE TISSUE DISORDERS
Autoimmune Disorders
CONNECTIVE TISSUE DISORDERS
Dry eyes are the most common manifestation (keratoconjunctivitis sicca)
Symptoms
Burning
Foreign-body sensation
Photophobia

33. SJÖGREN’S SYNDROME Dry eyes Dry mouth  Connective tissue disorder
Autoimmune Disorders
SJÖGREN’S SYNDROME
Dry eyes
Dry mouth
 Connective tissue disorder
Specific antibodies

34. DRY EYES: TREATMENT Artificial tears Lubricating ointment at night
Autoimmune Disorders
DRY EYES: TREATMENT
Artificial tears
Lubricating ointment at night
Punctual occlusion
Environmental modification
Topical anti-inflammatory agents

35. Severe tear deficiency: corneal ulceration and perforation
Autoimmune Disorders
Severe tear deficiency: corneal ulceration and perforation

36. ANKYLOSING SPONDYLITIS: OCULAR SYMPTOMS
Autoimmune Disorders
ANKYLOSING SPONDYLITIS: OCULAR SYMPTOMS
Photophobia
Redness
Decreased vision
If iritis, refer for evaluation and treatment.

37. Topical corticosteroids may lead to serious ocular complications.
Autoimmune Disorders
Topical corticosteroids may lead to serious ocular complications.

38. RHEUMATOID ARTHRITIS: OCULAR MANIFESTATIONS
Autoimmune Disorders
RHEUMATOID ARTHRITIS: OCULAR MANIFESTATIONS
Dry eyes
Episcleritis
Scleritis
Corneal ulcers
Uveitis

39. Autoimmune Disorders
Episcleritis

40. Autoimmune Disorders
Scleritis

41. Scleritis Necrotizing scleritis
Autoimmune Disorders
Scleritis Necrotizing scleritis

42. Scleromalacia perforans
Autoimmune Disorders
Scleromalacia perforans

43. Peripheral corneal ulceration
Autoimmune Disorders
Peripheral corneal ulceration

44. Juvenile rheumatoid arthritis: band keratopathy
Autoimmune Disorders
Juvenile rheumatoid arthritis: band keratopathy

45. IRITIS IN JUVENILE RHEUMATOID ARTHRITIS
Autoimmune Disorders
IRITIS IN JUVENILE RHEUMATOID ARTHRITIS
Few symptoms or signs
Usually chronic
Secondary cataract and glaucoma
Refer for periodic ophthalmic screening.

46. SYSTEMIC LUPUS ERYTHEMATOSUS: OCULAR MANIFESTATIONS
Autoimmune Disorders
SYSTEMIC LUPUS ERYTHEMATOSUS: OCULAR MANIFESTATIONS
Dry eyes
Scleritis
Peripheral corneal ulcers
Retinopathy and optic neuropathy

47. Autoimmune Disorders
Systemic lupus erythematosis: Cotton wool spots and hypertensive retinopathy

48. Systemic lupus erythematosis: optic nerve involvement
Autoimmune Disorders
Systemic lupus erythematosis: optic nerve involvement

49. POLYARTERITIS NODOSA: OCULAR MANIFESTATIONS
Autoimmune Disorders
POLYARTERITIS NODOSA: OCULAR MANIFESTATIONS
Dry eyes
Corneal ulcers
Scleritis
Hypertensive retinopathy
Retinal vasculitis

50. SARCOIDOSIS More commonly affects African-Americans and Hispanics
Autoimmune Disorders
SARCOIDOSIS
More commonly affects African-Americans and Hispanics
Ocular involvement in about 25% of patients

51. SARCOIDOSIS Keratic precipitates in granulomatous uveitis
Autoimmune Disorders
SARCOIDOSIS
Keratic precipitates in granulomatous uveitis
“Candlewax drippings” in choroiditis

52. Autoimmune Disorders
Refer patients with sarcoidosis if ocular involvement is suspected. Treatment may include corticosteroids.

53. GIANT CELL ARTERITIS: SYMPTOMS
Autoimmune Disorders
GIANT CELL ARTERITIS: SYMPTOMS
Jaw claudication
Headache
Scalp tenderness
Fever
Weight loss
Fatigue
Myalgias
Acute visual loss

54. Giant cell arteritis: ischemic optic neuropathy
Autoimmune Disorders
Giant cell arteritis: ischemic optic neuropathy

55. Giant cell arteritis: third cranial nerve involvement
Autoimmune Disorders
Giant cell arteritis: third cranial nerve involvement

56. GIANT CELL ARTERITIS: DIAGNOSIS
Autoimmune Disorders
GIANT CELL ARTERITIS: DIAGNOSIS
Clinical history
STAT ESR and CRP
Temporal artery biopsy
Fluorescein angiogram

57. GIANT CELL ARTERITIS: MANAGEMENT
Autoimmune Disorders
GIANT CELL ARTERITIS: MANAGEMENT
Begin treatment immediately with high-dose corticosteroids daily
Do not wait for results of temporal artery biopsy
Ophthalmologist, PCP, and rheumatologist manage jointly

58. THYROID OPHTHALMOPATHY
Autoimmune Disorders
THYROID OPHTHALMOPATHY
Not always correlated with serum thyroid levels
Can progress after thyroid function is normal

59. SPECTRUM OF THYROID OPHTHALMOPATHY
Autoimmune Disorders
SPECTRUM OF THYROID OPHTHALMOPATHY
Eyelid retraction
Exophthalmos (proptosis)
Extraocular muscle dysfunction
Corneal exposure
Conjunctival erythema
Optic nerve dysfunction

60. THYROID OPHTHALMOPATHY: OCULAR INVOLVEMENT
Autoimmune Disorders
THYROID OPHTHALMOPATHY: OCULAR INVOLVEMENT
Widened palpebral fissure
Soft tissue involvement

61. THYROID OPHTHALMOPATHY: ADVANCED
Autoimmune Disorders
THYROID OPHTHALMOPATHY: ADVANCED
Proptosis
Inferior rectus muscle restriction

62. THYROID OPHTHALMOPATHY: SEVERE
Autoimmune Disorders
THYROID OPHTHALMOPATHY: SEVERE
Corneal damage
Swollen optic disc

63. THYROID OPHTHALMOPATHY: COMPUTED TOMOGRAPHY OF THE ORBIT
Autoimmune Disorders
THYROID OPHTHALMOPATHY: COMPUTED TOMOGRAPHY OF THE ORBIT
Proptosis
Enlarged extraocular muscles

64. THYROID OPHTHALMOPATHY: TREATMENT OF CONGESTIVE PHASE
Autoimmune Disorders
THYROID OPHTHALMOPATHY: TREATMENT OF CONGESTIVE PHASE
Tear substitutes
Corticosteroids
Orbital irradiation or surgical decompression

65. THYROID OPHTHALMOPATHY: TREATMENT OF CICATRICIAL PHASE
Autoimmune Disorders
THYROID OPHTHALMOPATHY: TREATMENT OF CICATRICIAL PHASE
Lid surgery
Muscle surgery
Orbital surgery

66. MYASTHENIA GRAVIS: SYSTEMIC INVOLVEMENT
Autoimmune Disorders
MYASTHENIA GRAVIS: SYSTEMIC INVOLVEMENT
Refer suspects for neurologic evaluation.

67. Idiopathic Disorders
Papilledema

68. INTRACRANIAL HYPERTENSION: CAUSES
Idiopathic Disorders
INTRACRANIAL HYPERTENSION: CAUSES
Brain tumor
Meningitis
Venous sinus thrombosis
Hydrocephalus
Idiopathic intracranial hypertension (pseudotumor cerebri)

69. OPTIC NEURITIS May be initial manifestation of MS
Idiopathic Disorders
OPTIC NEURITIS
May be initial manifestation of MS
Acute, painful vision loss in one or both eyes
Treat initial episode with intravenous methylprednisolone
Interferon beta-1a may be of benefit

70. AIDS: OCULAR MANIFESTATIONS
Infectious Disorders
AIDS: OCULAR MANIFESTATIONS
Dry eye
Retinal cotton-wool spots
CMV retinitis
Kaposi’s sarcoma (eyelid or conjunctiva)

71. AIDS: Cotton wool patches
Infectious Disorders
AIDS: Cotton wool patches

72. AIDS: cytomegalovirus (CMV) retinitis
Infectious Disorders
AIDS: cytomegalovirus (CMV) retinitis

73. CMV RETINITIS: THERAPY
Infectious Disorders
CMV RETINITIS: THERAPY
HAART (Highly Active Anti-Retroviral) Therapy
IV ganciclovir
IV foscarnet
Intravitreal ganciclovir
Implantable pellets of sustained-release ganciclovir into the vitreous

74. Infectious Disorders
CD4 COUNTS
< 100 cells/ml: Opportunistic infections (eg, CMV retinitis)
> 100 cells/ml: Other ocular infections (eg, syphilis)

75. Metabolic/Endocrine Disorders
Diabetic retinopathy

76. SYSTEMIC MEDICATIONS WITH SIGNIFICANT OCULAR EFFECTS
Drugs/Toxins
SYSTEMIC MEDICATIONS WITH SIGNIFICANT OCULAR EFFECTS
Toxic Retinopathy
Thioridazine
Chloroquine
Hydroxychloroquine
Tamoxifen
Toxic Optic Neuropathy
Ethambutol
Isoniazid
Fluoroquinolones

77. OCULAR MANIFESTATIONS
Conclusion
OCULAR MANIFESTATIONS
SYSTEMIC DISEASES