Eric Chen,
Allen C. Ho,
David R. Guyer
HISTORICAL PERSPECTIVE
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Key Features |
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Angioid streaks were first described by Doyne[1] in 1889, and in 1892 Knapp[2] coined the term as he thought that they resembled blood vessels. It was not until 1917 that Kofler[3] correctly stated that the main pathologic changes were at the level of Bruch's membrane. This finding was confirmed histopathologically in the late 1930s.[4]
The recognition of angioid streaks is important because they can be associated with choroidal neovascularization (CNV) and macular degeneration and can herald the presence of systemic disorders, such as pseudoxanthoma elasticum (PXE), Paget's disease, and the hemoglobinopathies (Table 152.1
TABLE 152.1 -- Common Systemic Findings Associated with Angioid Streaks
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Finding |
% |
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PXE |
34 |
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Paget's disease |
10 |
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Hemoglobinopathy |
6 |
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Idiopathic |
50 |
From Clarkson JG, Altman RD: Angioid streaks. Surv Ophthalmol 1982; 26:235-246.
CLINICAL FINDINGS
Angioid streaks are irregular, spoke-like, and curvilinear streaks that radiate outward from the peripapillary area in all directions (Fig. 152.1). They are not present at birth, but have been documented as early as childhood, with subsequent growth.[5] There is no gender or racial predilection. Angioid streaks are almost always bilateral, and can be distinguished from blood vessels as they lie beneath the retina and above the choroidal vasculature. Angioid streaks are within two disk diameters of the optic nerve in 27% of cases and are widespread in 73% of patients.[6] They usually taper as they extend away from the disk, and typically do not go past the equator. The streaks can be wide or narrow and can vary in number from one to many. There are often associated peripapillary chorioretinal changes.
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FIGURE 152.1 (a-c) Angioid streaks are irregular, curvilinear, reddish brown streaks that radiate outward from the peripapillary area. (d) Fluorescein angiography reveals hyperfluorescence of the streaks. |
The color of the streaks can range from red to dark brown, and is often dependent on the pigmentation of the fundus and degree of overlying retinal pigment epithelium (RPE) atrophy; they can appear gray if fibrovascular tissue is present. In one study,[6] 32% of streaks were gray, 23% were red, and 14% had pigment proliferation with a blackish coloration. Hyperpigmentation or atrophy of the RPE may occur at the margin of the streak.
The streaks themselves are usually asymptomatic, but associated complications may cause vision loss. Subretinal hemorrhage or choroidal rupture may occur even after minor trauma.[7] However, the main cause of vision loss in these patients is CNV, RPE detachment, and macular degeneration (Fig. 152.2). In one series,[6] macular degeneration was observed in 40 (72%) of 56 cases; 32 patients had exudative maculopathy, and eight patients had atrophic maculopathy. In another study of 110 cases, the occurrence of exudative macular degeneration was associated with the length of the streak, the distance of the streak from the fovea, and the diffuse or 'cracked eggshell' type of streak.[8] A case of an RPE tear associated with angioid streaks has also been reported.[9]
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FIGURE 152.2 This patient with angioid streaks (a) has the characteristic 'plucked-chicken' skin lesions (b) of PXE. (c) and (d) The patient experienced CNV, which occurs in a high percentage of patients with angioid streaks. |
Other associated findings include peau d'orange changes (Fig. 152.3), light margins along streaks, peripheral focal lesions (salmon spots), hemorrhage, paired red spots along the streaks, and disk drusen. In one series,[6] these lesions were observed in 61%, 57%, 44%, 35%, 19%, and 10% of patients, respectively. Some of these lesions, such as disk drusen, salmon spots, and peau d'orange changes, are exclusively or more commonly noted in cases of angioid streaks associated with PXE.
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FIGURE 152.3 A PED with irregular epithelial borders and without stromal infiltrate was noted in a patient with systemic vitamin A deficiency due to chronic alcoholism. There were deep stromal folds and corneal edema. Peripheral corneal vascularization was also noted. The PED was refractory to antibiotics and corticosteroids. The PED and night blindness resolved after using systemic vitamin A and nutrition supplements. |
ANCILLARY TEST FINDINGS
Many authors[6,10-13] have observed early hyperfluorescence of the streaks with late staining (see Fig. 152.1). Others,[6,13,14] however, have reported hypofluorescence of the streaks with hyperfluorescence at the margins, which stain late; hypofluorescence is caused by separation of the underlying choriocapillaris and subsequent nonperfusion.[13] In some cases, fluorescein angiography and red-free images may reveal angioid streaks not observed clinically.[10,15] The classic findings of associated CNV, RPE detachments, and serous or hemorrhagic detachments may be observed on fluorescein angiography or indocyanine green angiography (ICGA), which may be superior in visualizing occult CNV.[16] The peau d'orange changes, which may represent focal defects of Bruch's membrane and the choriocapillaris, produce hypofluorescent areas on fluorescein angiography and a speckled pattern in the midperiphery on ICGA.[13]
Usually, visual fields, color testing, ERG, EOG, and dark adaptation tests are all normal.
NATURAL HISTORY
Although patients with angioid streaks are usually asymptomatic early in the course of the condition, visual loss usually occurs with time. In one report,[17] vision of 20/200 or worse was noted in most eyes after 50 years of age, while in another series,[18] greater than 50% of patients had an initial visual acuity of 20/40 or better, but more than half of the patients were legally blind at an average follow-up of 3.6 years. In another study of 29 cases, 66% of patients had a visual acuity of 20/200 or worse.
The cause of this vision loss is macular degeneration or CNV, or both (see Fig. 152.2). Three separate studies have reported macular degeneration in ~70% of patients with angioid streaks.[6,19,20] Macular degeneration has even been observed in a 14-year-old patient.[19]
Unlike age-related macular degeneration, the exudative form is more common than the atrophic type of maculopathy in patients with angioid streaks.[18,21] However, the exudative form is found less frequently in patients with angioid streaks and sickle cell disease than in patients with other associated systemic conditions.[22] Macular degeneration is not always associated with a foveal angioid streak, and it does not occur in all patients with a streak through the fovea.[10]
Piro and associates[18] studied 62 patients with angioid streaks and found that 86% had CNV in at least one eye. Bilateral CNV was noted in 49% of patients, and 37% of patients had unilateral CNV. Five fellow eyes of 22 patients with unilateral CNV developed CNV during a mean follow-up period of 18 months. Mansour and co-workers[8] reported that the CNV was associated with streak length, streak distance from the fovea, and the diffuse type of streak in their series.
Minor trauma may cause subretinal hemorrhage, often with macular involvement (Fig. 152.3).[4,7,10,22] Patients should be warned to avoid trauma and contact sports and to monitor their vision regularly with an Amsler grid for the onset of CNV.
SYSTEMIC ASSOCIATIONS
Clarkson and Altman[23] found an associated systemic disease in 50% of the 50 patients in their series. PXE, Paget's disease, and hemoglobinopathy were diagnosed in 34%, 10%, and 6% of the cases, respectively (see Table 152.1). Piro and associates[18] noted PXE in 61% of their patients and no systemic disease in 35% of the patients. Federman and colleagues[13] reported PXE in 30 (54%) of their 56 patients. In 1941, Scholz[20] reported that 59% of 131 patients with angioid streaks had PXE. Many other systemic disorders have been associated with angioid streaks, but some of these associations may be coincidental (Table 152.2). PXE, Paget's disease, and the hemoglobinopathies remain the most commonly found associated systemic diseases.
TABLE 152.2 -- Other Systemic Disorders Associated with Angioid Streaks
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Acromegaly |
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Ehlers-Danlos syndrome |
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Facial angiomatosis |
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Heterotopic calcification with hyperphosphatemia |
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Idiopathic thrombocytic purpura |
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Multiple hamartoma syndrome with uterine cancer |
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Ocular melanocytosis |
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Lead poisoning |
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Familial polyposis |
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Hypo- and abetalipoproteinemia |
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Chronic familial hyperphosphatemia |
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Diabetes |
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Hemochromatosis |
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Acquired hemolytic anemia |
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Hypercalcinosis |
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Hyperphosphatemia |
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Myopia |
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Neurofibromatosis |
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Senile elastosis |
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Tuberous sclerosis |
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Diffuse lipomatosis |
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Dwarfism |
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Epilepsy |
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Nephrolithiasis |
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Congenital dyserythropoietic anemia (CDA III) |
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Trauma |
PSEUDOXANTHOMA ELASTICUM
PXE is a form of systemic elastorrhexis that mainly affects the skin, eyes, gastrointestinal system, and heart.[23] Females are affected twice as often as males. Patients usually are diagnosed in the third to fourth decades of life. The inheritance of this rare disorder is usually autosomal recessive but may be autosomal dominant, and the underlying genetic defect has been localized to chromosome 16 and a mutation in the transport protein ABC-C6.[24,25] It has been thought that the condition is caused by elastic fiber abnormalities with secondary calcification. However, the earliest finding in PXE was determined by cytoimmunochemistry and X-ray analysis to be an accumulation of polyanions in the dermis.[26] These authors speculate that 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.
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 (see Fig. 152.2). This skin lesion looks like a 'plucked chicken'. Skin biopsy results reveal elastic tissue staining of the deep dermis, often with calcification. Lebwohl and associates[27] performed biopsies on scar and normal flexural skin in patients suspected of having PXE without the typical clinical skin findings. Six of 10 scar biopsy results showed fragmentation of elastic tissue, and three normal flexural skin biopsy results showed signs of PXE. These authors concluded that scar biopsy is useful in suspected cases of PXE without the characteristic clinical skin lesions.
Other systemic findings in PXE include cerebral ischemia, cerebrovascular accidents, intracranial aneurysms, claudication, hypertension, myocardial infarction, and gastrointestinal hemorrhage with or without ulceration. The gastrointestinal hemorrhage may be life-threatening, can occur in up to 15% of patients, and may occur before the skin or eye findings.
The first ocular findings in PXE were reported in 1903.[28] The first report of the association between PXE and angioid streaks was in 1929 by Groenblad[29] and Strandberg.[30] The ocular and cutaneous findings of PXE are referred to as the Groenblad-Strandberg syndrome in their honor. Angioid streaks are present in ~85% of patients with PXE.[19,23] The streaks usually occur in early adulthood but can occur in patients as young as age 8 years.[5]
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' (see Fig. 152.4). These lesions may be observed before the occurrence of the angioid streaks.[31] They are usually seen in association with PXE but occasionally can be present in cases of Paget's disease or sickle hemoglobinopathy.[23]
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FIGURE 152.4 (a and b The peau d'orange lesion is a diffuse mottling of the RPE in the temporal midperiphery. These changes appear as multiple, yellowish retinal pigment epithelial lesions that have the appearance of an orange skin (peau d'orange). |
The salmon spot[31] is a multifocal, yellow, atrophic, and peripheral RPE lesion that appears 'punched-out' like a histoplasmosis syndrome spot. Macular drusen and atypical drusen may be present in up to 75% of cases.[21] The presence of all five varieties of pattern dystrophy in patients with PXE and angioid streaks has been reported by Agarwal,[32] and Kadri and colleagues[15] described intraretinal bands from the optic disk, peripheral retinal degeneration, and bilateral retinal detachments in one case. Multiple small crystalline bodies may be present in 75% of patients in the midperipheral or juxtapapillary regions.[10] These crystalline bodies are associated with atrophic RPE changes and may resemble a comet when RPE atrophy occurs in a tail-like configuration next to it. Vascular abnormalities, including chorioretinal arteriovenous communications, have also been described.[33]
Optic disk drusen are found clinically or echographically in up to a quarter of patients with PXE and angioid streaks. Coleman and co-workers[34] stated that the incidence of disk drusen is 20-50 times greater in patients with PXE than in normal individuals. Shields and associates[6] found disk drusen in 10% of the patients in their series, and Pierro and colleagues[35] observed them in 21.6% of their 58 patients. Calcium-containing macromolecules may attach to the elastic fibers at the cribriform plate, disrupt axonal flow, and produce disk drusen, which may be the first manifestation of ocular PXE.[34] The peau d'orange and other pigmentary lesions may precede angioid streaks, which may not develop until the third to fourth decades of life. The angioid streaks then lead to CNV, disciform scarring, and macular degeneration.
PAGET'S DISEASE
Paget's disease or osteitis deformans is a chronic, progressive connective tissue disorder that involves the collagen matrix of bone.[23] Osteoclastic activity with an osteoblastic reaction occurs. The condition may be due to a slow virus related to measles or to the respiratory syncytial virus[36] or may be transmitted in an autosomal dominant manner. Males and females are equally affected. Systemic findings include an 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. The diagnosis is made by finding an elevated serum alkaline phosphatase level, increased urinary calcium and total peptide hydroxyproline levels, and characteristic radiographic findings.
The first associations between Paget's disease and angioid streaks were made by Verhoeff in 1928[37] and Rowland in 1929.[38] Angioid streaks are found in 8-15% of patients with Paget's disease.[20,23]However, others have suggested that the association may be less common. Dabbs and Skjodt[39] found only one (1.4%) of 70 patients with angioid streaks to also have Paget's disease.
Clarkson and Altman[23] studied 50 patients with active Paget's disease. Angioid streaks were found in seven patients. Those with angioid streaks were found to have had a longer duration of Paget's disease, a high alkaline phosphatase level, more disease sites seen on X-ray film, and increased urinary hydroxyproline excretion. In addition, six of the seven patients had skull involvement.
Occasionally, patients with Paget's disease may have peau d'orange lesions and other RPE changes.[10,23] Visual loss is usually caused by CNV, but optic atrophy, sometimes secondary to bony compression, may also occur.
HEMOGLOBINOPATHIES
The first association of angioid streaks with sickle cell disease was made in 1959 by Lieb and co-workers.[40] In a selected population of patients with sickle cell disease, five (6%) of 69 patients were found to have streaks.[41] Condon and Serjeant[42] did not find any patients with angioid streaks in their study of 76 patients with homozygous sickle cell disease (sickle cell anememia or hemoglobin SS disease). In another study of 124 patients with hemoglobin SS disease, no patients with streaks were observed.[43] However, five (1.4%) of 356 patients in another series[44] had angioid streaks, and in Clarkson and Altman's report[23] six patients with hemoglobin SS disease and one patient with hemoglobin SC disease, had angioid streaks. The frequency of angioid streaks appears to be higher in elderly patients[45]; of 60 elderly patients with hemoglobin SS disease, 13 (22%) had streaks, whereas only three (2%) of 150 younger patients were observed to have angioid streaks.
Hamilton and associates[46] reported 21 of 242 patients with hemoglobin SS disease to have angioid streaks. Their patients had good prognoses, as only two individuals had macular disease.
Angioid streaks have also been reported in association with other hemoglobinopathies besides hemoglobin SS disease, such as hemoglobin SC disease[47]; hereditary spherocytosis[48]; sickle trait (hemoglobin AS)[49]; ?-thalassemia major, minor, and intermedia[50]; hemoglobin H disease[51]; and sickle cell thalassemia.[52]
Overall, angioid streaks appear in 1-2% of patients with hemoglobinopathies, with the incidence increasing with age. Complications, such as CNV or macular degeneration, are uncommon with angioid streaks associated with sickle cell disease.
OTHER SYSTEMIC ASSOCIATIONS
PXE, Paget's disease, and the hemoglobinopathies are most commonly associated with angioid streaks. However, many other associated systemic conditions have been reported.
Angioid streaks have been found in patients with the Ehlers-Danlos syndrome,[53] a rare autosomal dominant connective tissue disorder with hyperextensible skin and hyperflexible joints caused by an abnormality in the synthesis and metabolism of collagen. Ocular findings include high myopia, keratoconus, blue sclera, ectopis lentis, and retinal detachment, while systemic findings include cardiovascular disease, diaphragmatic hernias, and diverticuli of the gastrointestinal and respiratory tracts.
The other systemic conditions associated with angioid streaks probably represent coincidental findings. These disorders include acromegaly,[54] facial angiomatosis,[55] heterotopic calcification with hyperphosphatemia,[56] idiopathic thrombocytic purpura,[57] multiple hamartoma syndrome with uterine cancer,[58] ocular melanocytosis,[59] lead poisoning,[60] familial polyposis,[61] hypo- and abetalipoproteinemia,[62] chronic familial hyperphosphatemia,[63] diabetes, hemochromatosis, acquired hemolytic anemia, myopia, senile elastosis, tuberous sclerosis, hypercalcinosis,[23] hyperphosphatemia,[56] neurofibromatosis,[64] diffuse lipomatosis,[65] dwarfism,[66] epilepsy,[67] nephrolithiasis,[68] congenital dyserythropoietic anemia (CDA III),[69] and trauma[1] (see Table 152.2).
PATHOPHYSIOLOGY
Although the pathogenesis of angioid streaks remains controversial, the elastic lamina of Bruch's membrane seems to be primarily affected, with secondary degeneration of the choriocapillaris and RPE with disease progression. In PXE and Paget's disease, Bruch's membrane may become calcified and brittle. Adelung[70] studied the lines of force in the eye resulting from traction of the ocular muscles pulling the eye around the fixed site of the optic nerve, leading to the peripapillary origin and radial extension of cracks occurring in a brittle Bruch's membrane. 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.
The cause of angioid streaks in sickle cell disease is even more confusing. Calcification is not common in this disorder; studies have conflicted as to whether a diffuse elastic degeneration is present in patients with sickle cell disease. Another theory is that iron deposition in Bruch's membrane may occur from hemolysis. This iron deposition could cause mineralization of Bruch's membrane. However, only one study[4] documented iron staining of Bruch's membrane. Other studies could not confirm these findings. Jampol and associates[71] stated that calcification may be more important than iron deposition. Another hypothesis for the occurrence of angioid streaks in patients with sickle cell disease stated that the lesions occur from impaired nutrition caused by sickling and stasis.[72] Small-vessel occlusion could also be important in the pathogenesis.[46]
PATHOLOGIC FEATURES
The first histopathologic studies of angioid streaks were reported in the late 1930s.[4] Basophilia and calcification of a thickened Bruch's membrane were observed in both reports. Breaks in Bruch's membrane correlated with the clinical sites of the streaks. Elastic degeneration was noted, and some breaks were invaded by fibrovascular tissue from the choroid. Thus, angioid streaks appear to be linear cracks in a thickened, degenerated, and calcified Bruch's membrane.
It appears that the histopathologic appearance is similar regardless of the associated systemic condition.[23,71] Iron deposition has not been found in cases of associated sickle cell disease except for one report.[4,46,71,73] Dreyer and Green[73] studied the histopathologic features of 32 eyes from 21 cases. Two patients had PXE, five patients had Paget's disease, and 14 patients had no systemic disorder. These authors confirmed that angioid streaks are defects in the thickened, calcified elastic layer of Bruch's membrane. They found that the earliest change was a break in the elastic and collagenous layers of Bruch's membrane. Fibrovascular ingrowth occurred in some of the breaks. Secondary changes included thickening of the basement membrane of the RPE, RPE atrophy, choriocapillaris damage, photoreceptor loss, RPE hypertrophy or hyperplasia, serous retinal detachment, and disciform scar formation. CNV and serous retinal detachment were observed in two cases, and disciform scars were present in eight eyes. Salmon spots were found to be isolated breaks in Bruch's membrane with fibrovascular ingrowth.
Jensen[74] studied Bruch's membrane in PXE using histochemical, ultrastructural, and X-ray microanalytic techniques. He found two types of calcification: hydroxyapatite and CaHPO4. He also observed a 'thready' material in the membrane and an increased amount of acid mucopolysaccharide. He stated that malformed collagen may be the underlying abnormality in PXE.
TREATMENT
As even trivial trauma can precipitate hemorrhage in patients with angioid streaks, safety glasses should be considered. In addition, these patients should not engage in contact sports. Low-vision aids may be useful, and in some cases, genetic counseling should be considered. Prophylactic treatment of angioid streaks should not be performed, as patients are generally asymptomatic and treatment may induce CNV, but patients should be advised to use an Amsler grid regularly to allow early detection of CNV.
Although there have been no prospective randomized controlled trials of different treatment options for CNV associated with angioid streaks, it seems reasonable to consider treatment of CNV to slow down progression of the CNV, decrease size of the central scotoma, or reduce a patient's metamorphopsia.
Successful outcomes with laser photocoagulation have been reported with well-defined juxta- or extrafoveal CNV, although the recurrence rate may be higher with CNV associated with angioid streaks than with CNV associated with other macular disorders. Singerman and Hatem[75] treated eight eyes with extrafoveal CNV with laser photocoagulation. Improved or stabilized vision was noted in seven of the eight cases. Recurrences occurred in four eyes. Piro and associates[18] found a poor response to laser photocoagulation in their series because of recurrences. Brancato and colleagues[76] treated 13 such eyes and stated that laser photocoagulation should be performed. Van Eijk and Oosterhuis[77] successfully treated seven of 15 eyes. Eight eyes had subfoveal recurrences. Gelisken and associates[78] treated 30 such eyes. Sixteen of the eyes had stable or improved vision postoperatively. Twelve of the remaining 14 cases retained 20/200 vision or better during a mean follow-up period of 3.4 years. Eleven untreated fellow eyes developed macular degeneration and the loss of central vision.
In the largest series, Pece and co-workers[79] treated 66 eyes and found a significant decrease in vision in the first year, but no significant change thereafter. They concluded laser treatment could end CNV and help stabilize visual acuity or slow down visual loss, although a high frequency of recurrences (77% of eyes) demanded intense clinical and angiographic follow-up, mostly in the first 3 months after treatment.
The lack of treatment for subfoveal CNV led to developments in the surgical treatment of CNV in angioid streaks. Thomas and associates[80] first reported four eyes that underwent surgical removal of subfoveal membranes, with a mean loss of one line of vision and visual outcome at 20/200 or worse at 7 months; recurrent neovascularization occurred in one eye. They postulated that diffuse abnormalities in Bruch's membrane seen in angioid streaks made it difficult to preserve underlying RPE after CNV removal. Adelberg and colleagues[81] reported only one eye out of five improving after surgery, with no recurrences at follow-up up to 6 months. Fujii et al[82] described inferior limited macular translocation for angioid streak-associated subfoveal CNV; two of four eyes had a final vision of 20/80 or better, with one of these suffering from a retinal detachment, while another eye had extrafoveal and subfoveal recurrence at 8 weeks and 9 months, respectively.
Photodynamic therapy (PDT), using an intravenous photosensitive drug (verteporfin) that is activated with infrared laser, is another therapeutic option with mixed results in treating angioid streak-associated CNV. Karacorlu et al[83] reported eight eyes treated successfully, with short-term cessation of leakage on fluorescein angiogram and no deterioration in visual acuity over a mean of 8.7 months, while Shaikh and associates[84] treated 11 eyes and found conversion of a CNV to a fibrous disciform lesion in nine eyes and lesion enlargement in seven of these eyes.
Menchini and colleagues[85] evaluated 48 eyes with baseline vision of 20/200 or greater and found that most had no or limited vision loss after 1 year and suggested PDT could be used to limit or delay visual loss, while another report by Ladas et al[86] with 24 eyes found unsatisfactory anatomic and functional results with PDT, even when re-treatments were performed earlier than the conventional time of 3 months. Nineteen of the 24 eyes had a final best-corrected visual acuity of 20/400 or worse. Browning and co-workers[87] found PDT seemed to limit visual loss in most patients through the first 12 months of follow-up; in 16 eyes with subfoveal CNV, 12 lost fewer than eight letters and 14 of the 16 lost fewer than 15 letters on ETDRS charts.
Costa et al[88] also described a novel technique of selective occlusion of subfoveal CNV in five patients with photothrombosis of the neovascular ingrowth site with large-spot, lower-intensity 810-nm laser to ICG concentrated in vascular lesions. They demonstrated rapid induction of CNV hypoperfusion within 1 h, and improvement of visual acuity and partial restoration of retinal architecture by optical coherence tomography up to 12 months after treatment.
DIFFERENTIAL DIAGNOSIS
Only 39% of cases in one series[6] were correctly diagnosed as having angioid streaks before referral. Twenty-three percent of these cases were misdiagnosed as age-related macular degeneration. Other disorders that were confused with the angioid streaks included choroidal sclerosis, myopia and lacquer cracks, histoplasmosis, toxoplasmosis, retinal vasculitis and papilledema, and traumatic hemorrhage. Other entities to consider in the differential diagnosis include choroidal rupture and choroidal folds (Table 152.3).
TABLE 152.3 -- Differential Diagnosis of Angioid Streaks
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Age-related macular degeneration |
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Choroidal sclerosis |
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Myopic lacquer cracks |
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Histoplasmosis |
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Toxoplasmosis |
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Retinal vasculitis and papilledema |
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Traumatic hemorrhage |
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Choroidal rupture |
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Choroidal folds |
CONCLUSIONS (OR OVERVIEW)
Angioid streaks are distinctive fundus lesions that represent breaks in a calcified and thickened Bruch's membrane. They are an important diagnosis for the ophthalmologist to make, as they are associated with systemic disorders and their resultant complications in 50% of cases; the most common diseases seen include PXE, Paget's disease, and the hemoglobinopathies. Angioid streaks themselves are usually asymptomatic, but trivial trauma can cause significant subretinal hemorrhage, and the occurrence of CNV and macular degeneration can cause devastating visual loss. Treatment options for angioid streaks have shown mixed results. While laser photocoagulation, submacular surgery, and PDT may all be attempted in an effort to slow down progression of the CNV, decrease size of the central scotoma, or reduce a patient's metamorphopsia, long-term prognosis remains guarded, as the recurrence rate with any treatment is high.
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Pseudoxanthoma Elasticum
Paget's Disease
Hemoglobinopathies
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REFERENCES
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2. Knapp H: On the formation of dark angioid streaks as an unusual metamorphosis of retinal hemorrhage. Arch Ophthalmol 1892; 21:289-292.
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18. Piro PA, Scheraga D, Fine SL: Angioid streaks. Natural history and visual prognosis. In: Fine SL, Owens S, ed. Management of retinal vascular and macular disorders, Baltimore: Williams & Wilkins; 1983:136-139.
19. Connor PJ, Juergens JL, Perry HO, et al: Pseudoxanthoma elasticum and angioid streaks. A review of 106 cases. Am J Med 1961; 30:537-543.
20. Scholz RO: Angioid streaks. Arch Ophthalmol 1941; 26:677-695.
21. Verhoeff FH: Histological findings in a case of angioid streaks. Br J Ophthalmol 1948; 32:531-544.
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