Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from Brit. J. Ophthal. (I975) 59, 257

Angioid streaks

I. Ophthalmoscopic variations and diagnostic problems

JERRY A. SHIELDS, JAY L. FEDERMAN, TERRANCE L. TOMER, AND WILLIAM H. ANNESLEY, JR. From the Service, Wills Hospital, Philadelphia

The clinical significance and ophthalmoscopic Although it was not our primary purpose to deter- features of angioid streaks have received considerable mine the incidence and type of associated systemic attention in the ophthalmic literature (Paton, I962; diseases, we listed those in which the data were Paton, 1972; Connor, Juergens, Perry, Hollenhorst, readily available, and in other cases we made the and Edwards, I96I). Angioid streaks usually have a medical diagnosis after the angioid streaks were characteristic ophthalmoscopic appearance and recognized in our clinic. A diagnosis of pseudo- diagnosis is easy. In some instances, however, they xanthoma elasticum (PXE) was made on the basis may be subtle or may simulate other more common of clinical examination or by skin biopsy in 30 fundus conditions and the diagnosis may be more cases (54 per cent). Two patients had sickle cell difficult. The ophthalmoscopic variations are dis- disease. None was known to have Paget's disease. A cussed in this report with some of the more common i6-year-old white male with angioid streaks and a diagnostic problems which occurred in a large series confirmed diagnosis of of patients with angioid streaks. These problems may had physical features compatible with Marfan's be overcome by recognizing the variable clinical syndrome. A few had mild cardiovascular disease. manifestations of this condition. The remainder either had no evidence of medical disease, or the medical data were insufficient. http://bjo.bmj.com/ Methods The visual acuities ranged from 6/6 to hand movements. No patient was completely blind, despite All patients with angioid streaks of the ocular fundus who extensive macular involvement in some. One had were evaluated and photographed on the Retina Service of chronic open-angle glaucoma. No significant ab- Wills Eye Hospital between March I967 and February normalities were present in refractive errors, ocular I 974 were included in this study. Most patients had repeat- adnexae, anterior segment, or vitreous. ed evaluations by one or more of the authors during a follow-up period of 6 months to 7 years. The clinical The fundus findings of the angioid streaks were on October 3, 2021 by guest. Protected copyright. records, fundus photographs, and fluorescein angiograms variable (Table I). The streaks demonstrated were reviewed. The ophthalmoscopic variations and variations in distribution and colour. In addition, diagnostic problems which occurred were evaluated in several associated fundus findings were frequently detail. present. In each case there was some degree ofperipapillary Results chorio-retinal degeneration. The angioid streaks radiated from the peripapillary region as irregular From March I967 until February 1974, 56 patients lines of variable size and shape, lying deep to the with angioid streaks were evaluated and photo- retinal vessels. They were confined to within two disc graphed. The diagnosis in each case was established diameters of the optic nerve head (peripapillary by observing the typical fundus features of this region) in I5 of the 56 cases (27 per cent) (Fig. i). condition (Paton, I962; Paton, 1972; Connor and In 41 cases (73 per cent), they were more widespread, others, 196I). All cases were bilateral. radiating in an irregular manner from the The ages of the patients ranged from i 6 to 76 for variable distances into the fundus (Fig. 2). In years (mean 49). There were so whites and 6 blacks; all cases, they tapered to an end before reaching the 32 women and 24 men. equator. There was a tendency for the streaks to involve the macular area. Address for reprints: J. A. Shields, MD, Retina Service, Wills Eye .H_nspital, 1601 Spring Garden Street, Philadelphia, Penna. 19130 The colour of the angioid streaks was grey in I8 Presented in part at the annual Meeting of the Section on Ophthalmo- cases (32 per cent), and red in I3 (23 per cent). logy of the American Medical Association, Chicago, Illinois, 24 June, Usually, however, they were an in-between colour 1974. Supported in part by the Retina Research and Development Foundation, Philadelphia, and the Lions Club of Pennsylvania. such as red-brown, or red-grey. In eight instances Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from 258 British Journal of

FIG. 1 (a)

FIG. 2 AIore extensive angioid streaks. Note the light areas adjacent to the streaks and the paired red spots (arrows)

Table I Fundusfindings in 56 patients with angioid streaks* http://bjo.bmj.com/ Fundusfindings Number Percentage Distribution of streaks Peripapillary region only 15 27 Widespread, posterior pole 4I 73 Colour of streaks rkIG. I (0) Grey i8 32 on October 3, 2021 by guest. Protected copyright. FIG. I (a) Left eye of a 58-year-old white male in Red 13 23 March 1967, showing mild peripapillary angioid streaks Combination 25 45 (b) Same eye 7years later showing no change in Pigment proliferation into streaks 8 appearance 14 Macular degeneration (I 4 per cent), there was pigment proliferation Atrophic 8 40 72 32 J over the angioid streaks producing a black appearance Exudative (Fig. 3). Mottled fundus (Peau d'orange) 34 6i Several associated fundus changes were present Light margins along streaks 32 57 (Table I). Macular degeneration, usually bilateral, Peripheral focal lesions 24 44 was present in 40 of the 56 patients (72 per cent). Fresh haemorrhage Ig 35 Atrophic macular changes, characterized by pig- Paired red spots along streaks 10 I9 mentary mottling, but no haemorrhages or exudates, Drusen of optic disc 5 I0 were present in eight cases. Exudative maculopathy, * Since the fundus findings were usually bilateral and fairly sym- characterized by haemorrhage, oedema, or white scar metrical, this table gives the total cases, rather than the total tissue, was present in 32 patients. Close scrutiny number of . revealed an angioid streak close to the macula in each of these patients (Fig. 4). In several instances, the leaving a yellow fibrous scar (Fig. 5). Some patients patient first sought ophthalmic care as a result of had a fibrous tissue scar in the macula when first seen. sudden macular haemorrhage, often after mild In some instances, it was a localized mound (Fig. ocular trauma. This usually resolved in a few months, 4), and in most cases it was more diffuse (Fig. 6). Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from Angioid streaks 259

FIG. 5(a) FIG. 3 Pigment proliferation into angioid streaks producing a black appearance. Note the light areas adjacent to the streaks and the disciform macular lesion to the right http://bjo.bmj.com/

FIG. 5 (b) on October 3, 2021 by guest. Protected copyright. FIG. 5 (a) Acute macular haemorrhage in a patient with angioid streaks. Note the angioid streak inferior to the haemorrhage (b) Resolution of the haemorrhage 7 months FIG. 4 Angioid streaks associated with a disciform later. Only a small mound offibrous tissue remains and a macular scar, probably secondary to previous haemorrhage. streak is present in the area ofprevious haemorrhage (arrow) Note the angioid streak passing through the inferior portion of the lesion (arrow) plasmosis were observed in 20 cases (37 per cent). The number of such lesions in a single eye ranged A mottled appearance in the fundus (peau d'orange) from five to 30. They were often, but not always, was present in 34 patients (6 i per cent). This consisted located in close association with a streak. They varied of a granular reddish-brown speckled area which from small, slightly elevated glistening yellow nodules usually extended from the macula towards the temporal without pigment to well-delineated partially pig- equator (Fig. 7). In two cases it was more widespread, mented spots (Fig. 9). extending nasally to surround the optic disc. It Fresh haemorrhages were present in I9 patients appeared to be more common in patients with PXE. (34 per cent). They were most often located in the In 32 cases (57 per cent) there were light areas macular region, but could occur anywhere in the along the margins of the streaks (Figs 2, 3, and 8). fundus along the course ofthe angioid streaks (Fig. 5a). These areas extended equidistant from either edge In about ten cases (I9 per cent), poorly delineated, of the streak to not more than three times the width of focal reddish-brown spots less than one-half disc the streak (Fig. 8). Focal chorio-retinal lesions diameter in size, were present just outside the light- resembling those seen in presumed ocular histo- coloured area along the margins of the angioid Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from 260 British Journal of Ophthalmology

FIG. 6 Difuse disciform macular lesion in patient with angioid streaks. Note the extensive fibrous tissue and pigment proliferation FIG. 8 Light coloured areas along the margins of angioid streaks. Note that they extend equidistant from the streak on either side http://bjo.bmj.com/ on October 3, 2021 by guest. Protected copyright.

FIG. 9 Focal chorio-retinal lesions associated with FIG . 7 Typical mottledfundus or 'peau d'orange' angioid streaks and PXE. Note that the more inferior temporal to the macula. Note the angioid streaks within lesion is non pigmented, whereas the more superior one is the area of involvement partially pigmented streaks (Fig. 2). These always occurred in pairs, Intravenous was performed one on each side of the angioid streak. Each affected in 31 ofthe 56 patients. Contrary to most descriptions, eye had from one to ten pairs of such lesions. we found that the streaks themselves were hypo- Another characteristic finding, usually nasal to fluorescent during the arterio-venous phase, and most the optic disc, consisted of numerous short vertical of them remained hypofluorescent throughout the lines concentric to the disc margin. These resembled angiogram, even into the late phases. There was, a cracked egg shell (Fig. IO), and appeared to be however, early hyperfluorescence of the yellow tissue more common in blacks and young patients. along the margins of the angioid streaks. These areas Drusen of the optic disc were present in five cases showed progressive staining in the late angiograms (9 per cent), and were bilateral in each instance. One which often spilled over centrally to stain the region patient had been evaluated neurologically because the of the streak. Close scrutiny, however, revealed that drusen simulated papilloedema (Fig. i i). the streak remained hypofluorescent. The fluorescein Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from Angioid streaks 26I

Table II Referring diagnosis in 56 patients with angioid streaks

Diagnosis No. of cases Percentage Angioid streaks i6 29 Macular degeneration 13 23 Choroidal sclerosis 2 Myopia I Histoplasmosis 2 Toxoplasmosis I Retinal vasculitis and papilloedema I Traumatic haemorrhage I No diagnosis rendered I5

FIG. IO Cracked egg shell appearance nasal to the optic disc in a i6-year-old white male with PXE. On the nasal side, the streaks tend to develop concentric to the disc http://bjo.bmj.com/

FIG. I2 Peripapillary 'choroidal sclerosis' in a patient

with drusen of the optic disc. The angioid streaks are on October 3, 2021 by guest. Protected copyright. difficult to see (arrow) because of the extensive confluent ...e -Rss__peripapillary changes FIG. iI Drusen of the optic disc associated with angioid streaks in a 37-year-old whitefemale with PXE. called choroidal sclerosis, and myopia. In 13 cases The patient was referred with the diagnosis of 'papilloedema (23 per cent), the ophthalmologist had made the and retinal vasculitis' diagnosis of macular degeneration but had failed to consider angioid streaks as the underlying cause angiographic features of angioid streaks in this series (Fig. 4). In some cases, the peripapillary chorio- will be the subject of a separate report (Federman, retinal changes were so diffuse that the angioid Shields, and Tomer, I975). streaks were no longer detectable. Examination of a Of particular interest were some of the problems 55-year-old white female referred because of'choroidal which had occurred in the ophthalmoscopic diagnosis sclerosis', revealed no clearly detectable angioid of angioid streaks (Table II). In i6 patients (29 streaks, but prominent drusen of the optic discs per cent),w~~~~~~~~~.X-the diagnosis of angioid streaks had been (Fig. 12). Suspecting PXE, we arranged to examine made and the management of the case had been other family members, and her younger brother was directed accordingly. In other cases, angioid streaks found to have classical angioid streaks (Fig. I 3). were confused with more common degenerative, Both siblings were subsequently proved to have PXE. inflammatory, and traumatic conditions affecting the One patient was referred with a diagnosis of chorio- fundus. retinal degeneration probably because of myopia. Degenerative conditions simulated by angioid Examination revealed angioid streaks but no streaks included senile macular degeneration, so- significant myopia. Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from 262 British Journal of Ophthalmology

F I G. I 5 Focal haemorrhagic macular nodule simulating histoplasmosis in a 57-year-old whitefemale with angioid FIG . I 3 Fundus of asymptomatic brother ofpatient streaks and PXE. Note the angioid streak deep to the shown in Fig. I2, illustrating typical angioid streaks papillomacular bundle (arrow)

Inflammatory conditions simulated by angioid of PXE was subsequently confirmed, and the streaks included histoplasmosis, toxoplasmosis, and evaluation for histoplasmosis was negative. In several retinal vasculitis. In two patients, the peripapillary, cases, a fresh haemorrhagic nodule in the macula macular, and peripheral changes had led to the was highly suggestive of histoplasmosis (Fig. 15). erroneous diagnosis of ocular histoplasmosis. One was In one case angioid streaks were mistaken for a 46-year-old woman who had been diagnosed at a ocular toxoplasmosis. A 30-year-old black female had major eye institute as having ocular histoplasmosis; been followed-up for 6 years with a diagnosis of toxo- http://bjo.bmj.com/ she had been treated periodically for 2 years with plasmosis because of a large pigmented macular systemic and retrobulbar steroids with no improve- lesion and several pigmented lesions in the adjacent ment. Fundus examination in our clinic revealed fundus. Examination in our clinic revealed that each angioid streaks with peripapillary, macular, and peripheral lesions in both eyes (Fig. 14). The diagnosis on October 3, 2021 by guest. Protected copyright.

FIG. 14 Perzpapillary angioid streaks and macular _ changes in a 46-year-old whitefemale treated at another FIG . I6 Pigmented macular lesion simulating toxo- institution for ocular histoplasmosis. There were numerous plasmosis in a 30-year-old blackfemale with PXE. peripheral focal lesions as well. The true diagnosis was Thepatient had beenfollowed-upfor 6years with a diagnosis PXE of toxoplasmosis before the angioid streaks were detected Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from Angioid streaks 263

pigmented lesion was closely associated with an Paton, 1959; Geeraets and DuPont, I 960) and angioid streak and the diagnosis of PXE was sub- possibly other conditions (Green, Friedman-Kien, and sequently confirmed (Fig. I6). Banfield, I 966; Howard, I 963; Wright, I 964; As the name implies, angioid streaks may super- McPhaul and Engel, I96I; Percival, I968; Scheie ficially resemble blood vessels. One 37-year-old and Hogan, 1957; Yatzkan, I957; Kalina, 1970). white female was referred with the diagnosis of Fluorescein angiography has been found useful in their 'papilloedema and retinal vasculitis'. The true recognition (Smith, Gass, and Justice, I964; Patnaik diagnosis was drusen of the optic disc and angioid and Malik, 197 ). Of great importance to the streaks (Fig. i i). Skin biopsy subsequently proved that ophthalmologist are the ophthalmoscopic variations the patient had PXE. In some cases haemorrhage of angioid streaks. Previous clinical observations are following mild ocular trauma was the initial clinical confirmed in this report, relative incidence of the manifestation of angioid streaks and the diagnosis various fundus features are elaborated upon, and of traumatic retinal haemorrhage was made without diagnostic problems which the ophthalmologist may recognizing the angioid streaks. An i8-year-old white encounter are discussed. female had blunt trauma to the right eye and was In those patients having widespread angioid streaks found to have numerous retinal haemorrhages. Close (72 per cent), recurrent haemorrhages and exudative examination revealed that the haemorrhages had macular changes were frequent. In those with only occurred along the course of angioid streaks (Fig. I 7). mild peripapillary streaks (28 per cent), there were The patient was subsequently proved to have PXE. usually no associated fundus abnormalities, macular lesions did not occur, and good vision was maintained. An example of this was the patient who showed no change over a 7-year period (Fig. i). He remained asymptomatic with perfect vision. No systemic disease could be diagnosed, despite extensive evalu- ation and skin biopsy. It is tempting to speculate that angioid streaks with this appearance have a benign course and offer a good visual prognosis. On the other hand, patients with associated findings such as a mottled fundus or drusen, are more likely to have PXE or other systemic disease and have a http://bjo.bmj.com/ tendency to develop macular changes and visual loss. The colour of the angioid streaks appeared to be unrelated to systemic disease, associated fundus changes, or prognosis. Paton (1972) relates the colour of the angioid streaks to the pigmentation of the

individual eye. on October 3, 2021 by guest. Protected copyright. It is not uncommon for angioid streaks to be associated with extensive proliferation and migration

FIG . 17 Haemorrhages along the course of angioid of the retinal pigment epithelium (Figs 3 and i6). streaksfollowing blunt ocular trauma in an i8-year-old This is particularly true in black patients and may whitefemale who was previously asymptomatic. Subsequent lead to diagnostic confusion (Fig. i6). evaluation confirmed the diagnosis ofPXE In this series, 72 per cent of the patients had some degree of macular degeneration, which was usually Discussion bilateral (Figs 4-6). This agrees with the series of Connor and others (I96I) in which the incidence was The clinical significance of angioid streaks has re- 73 per cent. The macular lesion usually consisted of ceived considerable attention (Paton, I962; Paton, haemorrhage and scar tissue probably secondary to 1972; Connor and others, I96I). Its relationship fibrovascular tissue growing into the streak from the to pseudoxanthoma elasticum (PXE) is well . With recurrent haemorrhages, the macular documented (Connor and others, I96I; Bischler, involvement becomes more extensive. Since patients 1956; Cowper, I954; Erbakan, I96I; Gills and with macular involvement are normally apprehensive, Paton, I965; Hagedoorn, 1939; Klien, 1949; it is important to inform them that this condition Scholz, 1941; Voisin and Lombard, '955; Clay, does not usually cause complete blindness, but that 1932; Rosen, I968; Shimizu, I96I). It appears to their central vision may be permanently impaired. be significantly related to Paget's disease, (Paton, The so-called mottled fundus (peau d'orange) seen in I962; Paton, I972; Terry, I934; Gass and Clarkson, 6I per cent of our cases, is a highly characteristic 1973) , (Paton, I962; Paton, I972; finding in patients with PXE (Bischler, I956; Gills Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from 264 British Journal of Ophthalmology

and Paton, i965; Gass, I970). This gives a speckled diffuse, often involving the papillomacular bundle appearance to the fundus, usually most pronounced (Fig. 6). Finally, careful fundus examination and temporal to the macula (Fig. 7). This finding may fluorescein angiography will often reveal angioid precede the development of angioid streaks (Bischler, streaks in the vicinity of the macular lesion (Figs 4 I956; Gass, I970). We have observed angioid and 5). streaks develop in areas with such involvement There have been some reports of an association (Fig. 7). between so-called choroidal sclerosis and angioid About 57 per cent of the patients in this series streaks with PXE (Britten, I 966). One of our patients demonstrated light areas coursing along the margins was initially thought to have this entity, but examina- of the angioid streaks (Figs 2, 3, 8, and 9). There is tion of the family revealed a sibling with typical little discussion in the literature as to their histological angioid streaks (Figs i 2 and I3). It is possible that counterpart. Gass and Clarkson (1973); Gass (1970) the degeneration of the retinal pigment epithelium feel that they may represent both thinning of the and the choriocapillaris may become wider and retinal pigment epithelium and a fibrovascular finally coalesce (Paton, I972), so that the streaks membrane growing through the angioid streak into become difficult or impossible to recognize, even with the space beneath the retinal pigment epithelium. the aid of fluorescein angiography. In such instances, Focal chorio-retinal lesions were present in 44 per the fundus picture may be identical to so-called cent of our cases. These have been called 'salmon choroidal sclerosis. spots' (Smith and others, I964; Krill, Klien, and The peripapillary and macular changes seen with Archer, I973) and are similar to the 'punched-out' angioid streaks may also simulate myopic chorio- lesions seen with ocular histoplasmosis (Paton, 1972). retinal degeneration. One of the patients in this series Their relationship to angioid streaks and PXE is was referred for a retinal detachment originally obscure. thought to be related to myopia. Examination, Fresh haemorrhages were noted in the fundus of however, revealed no significant myopia, but angioid 35 per cent of the patients (Figs 5a, Is, and I 7). streaks were present. Further examination revealed These occurred along the course of the angioid that the patient had signs of PXE. We have also had streaks, most commonly in the macular area. Recur- occasion to examine a patient with myopia who was rent haemorrhages in this region are the principal referred with the erroneous diagnosis of angioid cause of visual loss in patients with angioid streaks. streaks. In most instances, however, the differentiation

There appears to be a relationship between ocular between angioid streaks and myopia should not be http://bjo.bmj.com/ trauma and retinal haemorrhage in patients with difficult, because the large disc and temporal crescent angioid streaks (Britten, I966), although a history of of myopia is characteristic. In addition, the macular trauma may be difficult to elicit (Paton, I972). lesion (Fuchs's spot) in myopia is usually small and Paired reddish-brown spots occurred along the discrete as opposed to the larger diffuse macular margins of the angioid streaks in I9 per cent of our degeneration seen in angioid streaks. In high myopia, cases (Fig. 2). To our knowledge, these have not linear 'lacquer cracks' have been observed in the been thoroughly discussed in previous articles, posterior pole. Although they may represent a on October 3, 2021 by guest. Protected copyright. perhaps because they are subtle and may be easily similar process, they are usually isolated to the macular overlooked unless the fundus photographs are care- region and do not radiate from a peripapillary fully examined. We do not know the significance of location. Of course, the refractive error of myopia this finding. They appear to be different from the would also be helpful in making the differentiation. dark spots within the areas ofpeau d'orange, mentioned Angioid streaks may occasionally simulate certain by Krill and others (I 973). inflammatory conditions of the fundus. The peri- It is known that drusen of the optic disc are occa- papillary, macular, and peripheral changes may sionally found in eyes with angioid streaks (Paton, resemble those of ocular histoplasmosis; this occurred I972; Krill and others, I973). This association in two of our cases (Figs 14 and I5). In our series, occurred in five of our cases (IO per cent), and was some degree of peripapillary change was present in found in patients with PXE in every instance. The I 00 per cent, macular degeneration in 72 per cent, and significance of this association is uncertain. focal chorio-retinal lesions in 37 per cent. Most reports The associated fundus findings in this series of in the literature fail to note the close similarity between patients were responsible for some of the problems in these two conditions, although Paton (1972) has diagnosis. The most common problem was in making mentioned that the peripheral 'punched-out' lesions the diagnosis of senile macular degeneration and seen associated with angioid streaks are similar to failing to recognize that angioid streaks were the those seen in histoplasmosis. In 22 of our patients underlying cause. The differentiation should not be (40 per cent), peripapillary, macular, and peripheral difficult because the macular changes usually occur focal lesions were present in the same eye simulating at a younger age in patients with angioid streaks. the histoplasmosis triad. It is important to make the In addition, the degenerative process is usually more proper differentiation in these cases because ocular Br J Ophthalmol: first published as 10.1136/bjo.59.5.257 on 1 May 1975. Downloaded from Angioid streaks 265

histoplasmosis, an inflammatory process, is often streaks should prevent such diagnostic difficulties. treated with corticosteroids, whereas angioid streaks This study has shown that the clinical characteristics are usually associated with PXE, a heredode- of angioid streaks may vary considerably. In some generative process, in which corticosteroids are cases they may be so subtle that they are not apparently not helpful. recognized, and in other instances they may be seen There are certain clinical features which should but interpreted as something else. An ophthalmo- differentiate angioid streaks from ocular histo- scopic differential diagnosis of angioi4 streaks is plasmosis. The macular lesion of histoplasmosis is presented in Table II. The macular changes which often unilateral, whereas the macular involvement in occur in over 70 per cent of the cases may be mis- angioid streaks is almost always bilateral. In histo- interpreted as 'senile macular degeneration'. Because plasmosis, the peripapillary chorio-retinal atrophy eyes with angioid streaks may have peripapillary, often follows the contour of the optic disc, whereas in macular, and peripheral lesions, they may occasion- angioid streaks it is more irregular. The macular ally simulate other conditions producing a similar lesion is usually a focal nodule in histoplasmosis, triad, such as histoplasmosis and myopia. Since most whereas in angioid streaks the macular lesion is patients with angioid streaks have pseudoxanthoma usually more diffuse. Careful examination will elasticum or other significant systemic diseases, the often reveal the angioid streak adjacent to the macular ophthalmologist should be familiar with their clinical lesion. We also feel that the peripheral focal lesions variations and differential diagnoses. are different in the two conditions. In histoplasmosis, they appear 'punched-out' and a dull yellow-grey in Synopsis colour, while in angioid streaks they are often slightly elevated and have a glistening appearance. In both Fifty-six patients with angioid streaks were evaluated instances, they may show variable pigmentation. In ophthalmologically. Most had repeated fundus angioid streaks, they may represent fibrous tissue photography and fluorescein angiography during a mounds at the sites of previous focal deep retinal follow-up period of 6 months to 7 years. The haemorrhages, rather than inflammatory foci as seen ophthalmoscopic variations and diagnostic difficulties in histoplasmosis. A detailed systemic investigation which occurred were noted. would be helpful in differentiating between histoplas- In most instances, the angioid streaks were not mosis and PXE or other diseases associated with initially recognized and the patient was referred with angioid streaks. another diagnosis. In several cases, the peripapillary, http://bjo.bmj.com/ In rare instances, angioid streaks may be confused macular, and peripheral changes seen with angioid with ocular toxoplasmosis. This is especially true in streaks were found to simulate other better known black patients where considerable pigment prolifera- fundus conditions, resulting in erroneous diagnosis tion may occur along the margins of the streaks. One and improper treatment. In some cases, the angioid of our patients was followed-up for 6 years with a streaks were so subtle that they were overlooked and diagnosis of toxoplasmosis because of a large partially in others they were observed, but initially interpreted pigmented macular lesion and several pigmented as something else. Because of the medical significance on October 3, 2021 by guest. Protected copyright. peripheral lesions. Careful examination revealed that of angioid streaks, ophthalmologists should be aware each lesion was closely associated with angioid of their variable features. These are discussed, with streaks (Fig. i6) and the diagnosis of PXE was emphasis upon those subtleties which differentiate subsequently confirmed. angioid streaks from other conditions which they may As their name implies, angioid streaks may resemble simulate. On the basis of these observations, an blood vessels. The light areas along the margins of the ophthalmoscopic differential diagnosis of angioid streaks may give the appearance of perivascular streaks is proposed. infiltrates or sheathing and the associated haemorr- hages may lead to further confusion. One of our We thank the members of the Retina Service and the patients who had drusen of the optic disc was thought Staff ofWills Eye Hospital for allowing us to evaluate their to have papilloedema and retinal vasculitis (Fig. i I). patients, and Mr Peter Breit, Ms Thea Fischer, and Ms Familiarity with the clinical appearance of angioid Jean Douglas for their assistance.

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