Angioid streaks

1. ANGIOID STREAKS
Dr. Asim wasim

2. Angiod Streaks
 Irregular,
Spoke like,
curvilinear
streaks that
radiate outward
from the
peripapillary
area in all
directions

3. HISTORICAL PERSPECTIVE
First described by Dr. Robert W. Doyne, a
British ophthalmologist, in 1889.
1892, Knapp thought that they
resembled vessels and used the term
angioid streaks
 1917 that Kofler correctly stated that the
main pathologic changes were at the
level of Bruch's membrane.
This finding was confirmed
histopathologically in the late 1930s.

5. CNVM

6. Important
Recognition is important because
can be associated with
(CNVM)
Macular degeneration
Pseudoxanthoma elasticum (PXE),
Paget's disease and the
hemoglobinopathies

7. Clinical features:
Symptoms:
– Usually
asymptomatic,
but may affect
vision over time
due to
progression of
streaks towards
the fovea

8. They lie beneath
the retina and
above the
choroidal
vasculature and
thus can be
distinguished
from blood
vessels.

9. These lesions are almost always
bilateral.

10. Angioid streaks are within 2 disc
diameters of the optic nerve in 27
percent of cases and are
widespread in 73 percent of
patients.
They usually do not go past the
equator. The streaks can be wide or
narrow and can vary in number
from one to many.
 There is usually associated
peripapillary chorioretinal changes.

11. Fluorescein angiographic findings:
Irregular hyperfluorescence of
the streaks during early phases
and late staining
Can be seen as
hypofluorescence of the streaks
outlined by hyperfluorescence
margins, which stain in the late
phases
Some clinically invisible streaks
may be observed during
fluorescein angiography

12. Cause of vision Loss
Macular
degeneration
or CNVM, or
both

13. Unlike AMD, the exudative form
is more common than atrophic
form.
Macular degeneration is not
always associated with angioid
streak passing through the
fovea

14. Subretinal Hemorrhage,
Minor trauma may
cause subretinal
hemorrhage, often
with macular
involvement.

15. Macular scarring after CNV

16. Most common systemic associations
Idiopathic 50%
Pseudoxanthoma elasticum (PXE) 34%
Paget's disease 10%
Sickle cell disease 5%
Ehler's- Danlos Syndrome 1-2%

17. Other systemic diseases
–Abetalipoproteinemia
–Acromegaly
–Dwarfism
–Diabetes mellitus
–Hemochromatosis
–Facial angiomatosis
–Idiopathic
thrombocytic purpura
–Chronic familial
hyperphosphatemia
–Hypercalcinosis
–Diffuse lipomatosis
–Acquired hemolytic
anemia
–Myopia
–Neurofibromatosis
–Epilepsy
–Senile elastosis
–Sturge-Weber
syndrome
–Trauma
–Tuberous sclerosis

18. Pseudoxanthoma Elasticum (PXE)
Systemic elastorrhexis that affects
mainly the skin, eyes,
gastrointestinal (GI) system, and
heart.
Female: male= 2:1.
Patients usually diagnosed in the
third to fourth decades of life.
Inheritance is usually A/R but may
be A/D.

19. Condition is caused by elastic fiber
abnormalities with secondary calcification.
 Recently, accumulation of polyanions in
the dermis has been reported as an early
finding.
The polyanions attract calcium, which
causes mineralization. Thus, the basic
defect in PXE may not be one of calcium or
elastin metabolism but rather of
glycosaminoglycans and glycoproteins.
These glycosylated molecules may attach
to elastic fibers, mineralize, and cause
abnormal collagen synthesis.

20. Clinical features
The characteristic
skin change in PXE
is a redundant waxy,
yellow, papule-like
lesion, which
commonly affects the
neck, face, abdomen,
axillary areas,
inguinal regions,
periumbilical area,
and oral mucosa

21. This skin lesion
looks like a
“plucked
chicken.”

22. Other systemic findings
Cerebral ischemia, cerebrovascular
accidents, intracranial aneurysms,
claudication, hypertension,
myocardial infarction, and GI
hemorrhage with or without
ulceration. The GI hemorrhage may
be life-threatening, can occur in up
to 15 percent of patients, and may
occur before the skin or eye
findings.

23. The peau d'orange
changes represent a
diffuse mottling of the
RPE in the temporal
midperiphery and
consist of multiple
yellowish RPE lesions
that have the
appearance of the skin
of an orange (peau
d'orange)

24. The salmon spot is a multiple focal,
yellow, atrophic peripheral RPE
lesion that appears “punched-out”
like a histoplasmosis syndrome
spot. Macular drusen and atypical
drusen may be present in up to 75
percent of cases.
Optic disc drusen are commonly
associated with PXE and angioid
streaks.

25. Paget's Disease
Paget's disease or osteitis
deformans is a chronic,
progressive connective tissue
disorder that involves the collagen
matrix of bone.
The condition may be due to a slow
virus related to measles or may be
transmitted in an autosomal
dominant manner. Males and
females are equally affected.

26. Ocular Features
Occasionally patients with Paget's
disease may have peau d'orange lesions
and other RPE changes.
Optic atrophy, sometimes secondary to
bony compression, may also occur.

27. Systemic findings
Enlarged bone mass affecting the
pelvis, skull, spine, humeri, and
femora, extraskeletal calcifications
of the skin and arteries, pain,
secondary osteoarthritis,
neurologic damage, cardiac
disease, decreased hearing,
hyperparathyroidism, and
claudication.

28. Hemoglobinopathies that
occasionally are associated
–Homozygous sickle cell disease (Hb-SS)
–Sickle cell trait (Hb-AS)
–Sickle cell thalassemia (Hb-thal)
–Sickle cell hemoglobin (Hb-SC)
–Hemoglobin H (Hb-H)
–Homozygous B-thalassemia major
–Intermedia
–Minor and hereditary spherocytosis

29. Hemoglobinopathies
Complications, such as CNVM or
macular degeneration, are
uncommon.
The frequency of angioid streaks
appears to be higher in elderly
patients

30. PATHOPHYSIOLOGY
The pathogenesis of angioid streaks is
controversial,
In PXE and Paget's disease, Bruch's
membrane may become calcified and
brittle,
Cracks may occur in a brittle Bruch's
membrane because of the ocular
muscles pulling the eye around a fixed
origin.
Calcification is observed in other parts
of the body in both PXE and Paget's
disease, and thus this mineralization
may be a common mechanism.

31. The cause of angioid
streaks in sickle cell
disease is even more
confusing.
Calcification is not
common in this
disorder.

32. PATHOLOGIC FEATURES
It is believed that the pathology
may be a combination of diffuse
elastic degeneration of the Bruch
membrane, iron deposition in
elastic fibers from hemolysis with
secondary mineralization, and
impairment of nutrition because of
sickling, stasis, and small vessel
occlusion

33. Work up
Lab Studies:
50% of patients with angioid
streaks have systemic association.
General workup is important to
diagnose and treat other aspects of
the disease that may be life
threatening, such as GI
hemorrhage, heart disease, anemia,
and pathological fractures.

34. Pagets disease
The diagnosis is made by finding
an elevated serum alkaline
phosphatase level, increased
urinary total peptide
hydroxyproline levels, and
characteristic radiographic
findings.

35. Imaging Studies:
Fluorescein angiography
Indocyanine green angiography
Radiology studies: X-rays of head,
abdomen, and lower extremities are
helpful to show bone involvement
in Paget disease of the bone and
premature calcification of arteries
in PXE.

36. Other Tests:
GI studies: GI hemorrhage is
common in patients with PXE.
Dermatologic: Skin biopsy may
provide important diagnostic clues
in cases of PXE with angioid
streaks.

37. TREATMENT
Observation and patient counseling
Asymptomatic Patients need to be
monitored closely
–Amsler grids and FA.
Safety glasses should be considered for
these patients with angioid streaks
because trauma precipitates
hemorrhage.
Should not engage in contact sports.

38. TREATMENT
in some cases genetic counseling
should be considered.
Prophylactic treatment of angioid
streaks should not be performed,
as it may induce CNVM.

39. Laser photocoagulation
Argon laser for lesions that are located
at least 100 mm from the center of foveal
avascular zone (FAZ).
Many patients need multiple treatments
because of persistent leakage and
recurrence that occurs during the first 3
months. 77%
Careful setup of laser power and spot
size is important to prevent further
damage to the brittle and mineralized
Bruch membrane.

40. Photodynamic therapy
PDT is used for treatment of subfoveal
CNV.
 It uses a light activated drug (eg,
verteporfin [Visudyne]) and applying a
nonthermal red light in the range of 689
nanometers. The total energy delivered
is 50 J/cm2 over a period of 83 seconds.
The power of laser output can be
adjusted according to size of CNV and
ophthalmic lens magnification.
Long-term effects of PDT are unknown.
Patients with angioid streaks are at
higher risk of recurrent CNV.

41. Submacular surgery
Patients with classic subfoveal
CNVM are not candidates for laser
photocoagulation therapy. In the
past, they were left without
treatment. However, recent
advances in instrumentation and
vitreoretinal surgical techniques
made it possible to remove CNVM
without significant damage to RPE
and neurosensory retina.

42. Low-vision aids
may be useful

43. D/D
Age-related macular degeneration.
Other disorders that are confused with the
angioid streaks include
Choroidal sclerosis,
Myopia and lacquer cracks,
Histoplasmosis,
Toxoplasmosis,
Retinal vasculitis
Papilledema,
Traumatic hemorrhage.
Choroidal rupture and choroidal folds

44. CONCLUSIONS
Angioid streaks are distinctive
fundus lesions that are associated
with systemic disorders in 50
percent of cases. The most
commonly associated diseases
include PXE, Paget's disease, and
the hemoglobinopathies.

45. Angioid streaks themselves are usually
asymptomatic, but the occurrence of
CNVM and macular degeneration can
cause devastating visual loss. The
streaks represent breaks in a calcified
and thickened Bruch's membrane. Laser
photocoagulation may be beneficial to
treat CNVM associated with angioid
streaks; however, the recurrence rate is
high.

46. THANKYOU