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Chorioretinal Vascular Abnormalities Associated with Angioid Streaks and Pseudoxanthoma Elasticum

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Chorioretinal Vascular Abnormalities Associated with Angioid Streaks and Pseudoxanthoma Elasticum CLINICAL SCIENCES Chorioretinal Vascular Abnormalities Associated With Angioid Streaks and Pseudoxanthoma Elasticum Michel Secre´tan, MD; Le´onidas Zografos, MD; David Guggisberg, MD; Bertrand Piguet, MD Objective: To analyze the retinal and choroidal a large vascular loop corresponding to an arteriovenous vascular abnormalities in eyes with angioid streaks communication between retina and choroid in 3 eyes (6%) (AS) associated with pseudoxanthoma elasticum and an anastomosis between 2 retinal arteries in 1 eye (PXE). (2%). Methods: Color photographs and fluorescein angio- Conclusion: Analysis of the vascular network in these grams of 54 eyes of 27 consecutive patients with AS and eyes showed several vascular abnormalities, among which PXE were examined retrospectively. chorioretinal arteriovenous communications appear to be the most dramatic. Results: Four (7%) of the 54 eyes had a major vascular abnormality at the level of the disc; this took the form of Arch Ophthalmol. 1998;116:1333-1336 HAT CAME to be than several decades cannot be consid- known as angioid ered valid, as it appears that AS will even- streaks (AS) were tually develop in virtually all patients with first described by long-standing PXE.8 Doyne1 in 1889, but Pseudoxanthoma elasticum is a rare this designation was not used until 1892, disease of the connective tissue with a W2 when Knapp applied the term to reflect prevalence of 1:160 000 and autosomal the supposed vascular nature of these recessive and dominant inheritance pat- streaks. terns. Both forms of PXE were recently Angioid streaks are broad, irregu- mapped to chromosome 16p13.1.9 It is a lar, reddish-brown or gray lines that multisystem disorder that can involve ar- radiate from the area around the optic terial walls, cardiac valves, skin, gastroin- nerve head and whose number, extent, testinal tract, and eyes (Bruch membrane, and age at onset are variable. Their lamina cribrosa).10 In time, all patients tend clinical significance lies in that they are to manifest a classic phenotype that in- related to breaks in the elastic layer of volves all of these systems, with consider- Bruch membrane,3 due to an abnormal able variation in extent of involvement. fragility of the lamina basalis4 caused by Due to calcification of the internal a degenerative process combined with elastic lamina, which results in second- calcium deposits.5 ary narrowing of vessel lumina, coro- Angioid streaks have been de- nary11 and peripheral vascular dis- scribed in association with numerous sys- eases12,13 are the most common and severe temic disorders involving elastic tissue, in- complications. Other as yet unexplained cluding pseudoxanthoma elasticum (PXE). vascular manifestations have been re- From the Department of The association of AS with PXE was first ported to be associated with PXE; these in- Ophthalmology, Hoˆpital Jules reported by Groenblad6 and Strandberg7 clude intracranial arteriovenous malfor- Gonin (Drs Secre´tan, Zografos, in 1929. The reported incidences of PXE- mations14 and orbital varices.15 and Piguet), and the Department of Dermatology, associated AS vary tremendously, depend- Based on the known systemic vas- Centre Hospitalier ing on the diligence with which patients cular changes associated with PXE, we Universitaire Vaudois are scrutinized. Therefore, an incidence wished to define the incidence of retinal (Dr Guggisberg), Lausanne, based on the observation of patients with or chorioretinal vascular abnormalities Switzerland. PXE who have been followed up for less in patients with PXE. ARCH OPHTHALMOL / VOL 116, OCT 1998 1333 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 SUBJECTS AND METHODS Among all eyes with AS that were examined from July 30, 1980, to February 2, 1997, at the Hoˆpital Jules Gonin, Lausanne, Switzerland, only those that met the following criteria were included in this study: (1) AS documented with color photographs and fluorescein angiography; (2) diagnosis of PXE based on results of clinical examination (yellow macules, papules, or plaques in commonly affected sites such as the neck, axillae, popliteal fossae, and antecubital areas) and/or skin biopsy (fragmenta- tion and calcification of elastic fibers in the middle and lower third of the dermis)16; and (3) no con- A comitant intraocular or intraorbital disease except choroidal neovascularization. We analyzed characteristics of the vessels and their embranchments. The level (prelaminar or post- laminar) of the first division of the central retinal ar- tery was noted, as were the numbers of multiple branchings and abnormal arteriovenous communi- cations. We recorded the numbers of additional ar- teries and abnormally coursing arteries (crossing over the raphe). Using the Fisher exact test, we per- formed a statistical comparison of the incidence of these vascular abnormalities with a control group. The control group consisted of 50 consecutive eyes that sustained indirect traumatic choroidal rupture and in which fluorescein angiography was performed in our retina department; P,.05 was considered statis- tically significant. B We also recorded other clinical findings, such as cup-disc ratio, peau d’orange, and choroidal new vessels. To differentiate arteries from veins, and to de- termine the direction of blood flow in the case of ill- defined vascular abnormalities, indocyanine-green an- giography (ICG) was performed whenever possible. Patients were contacted and asked to return specifi- cally for this examination. All patients for whom in- formed consent was obtained underwent rapid- sequence ICG with emphasis on the choroidal and early arterial filling. To determine the presence or ab- sence of optic nerve head drusen, B-scan echogra- phy was also performed on all of these eyes. RESULTS C A total of 54 eyes of 27 patients were examined. Patients Figure 1. Color fundus photographs of the 3 eyes that showed papillary ranged in age from 24 to 68 years (median, 46 years); vascular loops (arrows). there were 11 men and 16 women, which is in accor- dance with the higher incidence of PXE in women. the control group. Using ICG in 2 of these 3 eyes, this Optic nerve head drusen were clinically apparent in loop was shown to be an arteriovenous communication 20 eyes (37%), but disc excavation was consistently ab- between the central retinal artery and a large choroidal sent. Mottling of peripheral retinal pigment epithelium vein. Both of these eyes had dilated and beaded choroi- was present in all eyes. Neovascularization was noted in dal vessels. In 1 eye (Figure 2, A), the drainage vein ap- 35 eyes (65%). Obstruction of the central retinal artery peared to divide, with 1 branch coursing toward the tem- was seen in 1 eye (2%). poral inferior vortex vein and other toward the nasal The most obvious vascular abnormality was a large inferior vortex vein. In the second eye (Figure 2, B), the vascular loop at the level of the optic disc, observed in 3 vein seemed to course around the optic nerve head. The (6%) of the 54 patient eyes (Figure 1), but in none of diagnosis of PXE had been confirmed by results of skin ARCH OPHTHALMOL / VOL 116, OCT 1998 1334 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 A Figure 3. Color fundus photograph of the left eye of a 44-year-old patient with angioid streaks and pseudoxanthoma elasticum showing a retinoretinal anastomosis (anastomosis between 2 retinal arteries) (arrows) between the nasal superior and temporal superior artery. Postlaminar division of the central retinal artery (arrowhead) and an artery crossing over the raphe (arrowheads) are seen. B Figure 2. A, Indocyanine-green angiography of the left eye of the 48-year-old patient shown in part A of Figure 1, confirming the arteriovenous retinochoroidal direction of the blood flow. The large beaded vessels are seen coursing toward the temporal inferior vortex vein and the nasal inferior vortex vein (arrows). B, Indocyanine-green angiography of the left eye of the 60-year-old patient shown in part B of Figure 1. biopsy in both patients. No evidence of drusen was seen on results of B-scan echography in these eyes. In the third Figure 4. Color fundus photograph of the left eye of a 32-year-old patient with angioid streaks and pseudoxanthoma elasticum showing an abnormal eye (patient unavailable for follow-up), ICG was not per- division of the central retinal artery (arrows) and an abnormal location of the formed, but a similar-appearing retinochoroidal anasto- upper temporal artery, which crosses over the raphe (arrowheads). mosis was partially visible on the color photographs (Fig- ure 1, C). COMMENT A retinoretinal arterial anastomosis (anastomosis be- tween 2 retinal arteries) was present in 1 eye (2%) Various types of congenital and acquired vascular anas- (Figure 3). In this case, the direction of the blood flow tomoses involving the retina, the choroid, or both have was not clear on results of ICG. Some irregularity of di- been described previously.17-23 ameter was present in the anastomotic segment of the ar- Acquired retinoretinal arteriovenous communications tery. As in the other cases, B-scan echography failed to are recognized manifestations of ischemic diseases such as show any drusen of the optic nerve head. diabetes, proliferative sickle cell retinopathy, and occlusive Minor vascular abnormalities were also recorded. disease of the carotid artery. Following ischemic obstruc- Forty-four eyes (81%) of patients with PXE and AS had tion or destruction of physiologic vessels, these communi- a postlaminar division of the central retinal artery, com- cations develop to allow blood to flow from obstructed ar- pared with only 12 eyes (24%) in the control group teries to patent veins.17 Acquired retinochoroidal arteriove- (P,.005) (Figure 3). The presence of arteries crossing nous communications have been described also in the late over the raphe in 5 patient eyes (9%) vs none in the con- stages of age-related macular degeneration,18 in vascularized trol group was also significant (Figure 3 and Figure 4).
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