Ординатура / Офтальмология / Учебные материалы / Retinal Vascular Disease Joussen Springer

21 III

Fig. 21.5.10. Fluorescein angiogram at 56 s after injection in the ocular ischemic syndrome eye shown in Fig. 21.5.9. There is filling of the choroidal vasculature superotemporally, but a marked delay in choroidal filling elsewhere (star). Leading edges of fluorescein dye, distinctly abnormal phenomenona, can be seen within the retinal arteries (arrow)

Fig. 21.5.12. Fluorescein angiography at 90 s after injection in an ocular ischemic syndrome eye reveals numerous, small punctate areas of hyperfluorescence which correspond to retinal microaneurysms. The retinal vessels are beginning to leak as well

Fig. 21.5.11. Histopathology of the poster segment in an eye with the ocular ischemic syndrome. Both the inner and outer retina are attenuated due to retinal vascular and choroidal ischemia, respectively. Note that the retinal pigment epithelium (arrow) appears to be intact. Retinal pigment epithelial changes are not a prominent feature of the ocular ischemic syndrome. (Courtesy of W. Richard Green, MD). H&E, ×20

Fig. 21.5.13. Histopathologic specimen of a retinal microaneurysm in an eye with the ocular ischemic syndrome. Rupture (arrow) of these anomalies and damaged small retinal vessels result in the retinal hemorrhages seen in ocular ischemic syndrome eyes (Fig. 8)

Fig. 21.5.15. Hyperfluorescent foci due to neovascularization of the retina occurring secondary to ocular ischemia in the eye of a non-diabetic man with severe ipsilateral carotid artery obstruction. Retinal capillary non-perfusion (arrow) can be seen immediately to the left of the largest area of retinal neovascularization

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Fig. 21.5.16. Trypsin digest of a region of the retina with capillary non-perfusion in an ocular ischemic syndrome eye. The vessels are acellular tubules, thus explaining why reperfusion does not occur in non-perfused areas. (Courtesy of W. Richard Green, MD). ×160

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Fig. 21.5.18. Fluorescein angiogram at over 10 min after injection reveals staining of the larger retinal vessels, especially the retinal arteries. (Courtesy of Dr. Neal Atebara)

Fig. 21.5.19. Normal electroretinographic pattern (upper tracing in the normal right eye) and diminution of the amplitude of the a- and b-waves in the left eye (lower tracing) with the ocular ischemic syndrome. The a-wave is diminished due to choroidal vascular insufficiency and outer retinal ischemia, and the b- wave is diminished due to hypoperfusion within the retinal vessels supplying the inner retina

A. Intravenous fluorescein angiography [6] – listed in order of specificity

Delayed choroidal filling – 60 % (Fig. 21.5.10) Most specific

>Five seconds from the first appearance of dye until complete choroidal filling

Late arterial staining – 85 % (Fig. 21.5.18) Delayed arteriovenous transit time – 95 %

Least specific

> Eleven seconds from the first appearance of retinal arterial dye until complete retinal venous filling

Macular edema – 17 % (Fig. 21.5.17) Additional signs

Retinal capillary non-perfusion (Fig. 21.5.15) Microaneurysmal hyperfluorescence (Fig. 21.5.17) Optic nerve head hyperfluorescence (Fig. 21.5.17)

B. Electroretinography [6, 7] (Fig. 21.5.19)

Diminished a-wave amplitude due to outer retinal ischemia Diminished b-wave amplitude due to inner retinal ischemia

C.Ophthalmodynamometry

Positive in unilateral cases

Light digital pressure is a good substitute. Retinal arterial pulsations, if not already present, can be induced with light digital pressure on the lid

D.Color Doppler ultrasonography [18]

Diminished choroidal flow

Reversal of flow in the ophthalmic artery

E. Carotid non-invasive studies

Duplex ultrasonography and oculoplethysmography have an 88 – 95 % chance of detecting a carotid stenosis of > 75 % [9, 18, 32]

MRA angiography is similar to Doppler ultrasonography in diagnosing carotid stenosis [10]

A. Diseases

Systemic arterial hypertension – 73 % Diabetes mellitus – 56 % Atherosclerotic cardiac disease – 50 % Previous stroke – 25 %

Peripheral arterial disease requiring bypass surgery – 20 % Stroke rate – 4 %/year

B. 5-year mortality (Fig. 21.5.20)

40 %

Cardiovascular disease is the leading cause of death

Referral to primary care physician or cardiologist is important

Fig. 21.5.20. Survival in patients with the ocular ischemic syndrome. The 5-year mortality is 40 %, primarily due to cardiovascular death (OIS ocular ischemic syndrome)

21.5.6Therapeutic Modalities

A.Ocular [36]

Panretinal photocoagulation if there is iris neovascularization and the anterior chamber angle is open or a glaucoma filtering procedure is under consideration (Fig. 21.5.21)

36 % success in eradicating iris neovascularization

B. Systemic

Endarterectomy [19, 21, 36] (Figs. 21.5.22, 21.5.23) Improves or maintains vision in 1/3 of cases without a 100 %

ipsilateral obstruction

100 % carotid obstruction: endarterectomy ineffective since clot propagation distally [39]

C. Carotid endarterectomy indications in general

a)Beneficial if 70 % stenosis and symptomatic (mild stroke, TIA and/or amaurosis fugax) [33]

i)Endarterectomy 2-year stroke rate: 9 %

ii)Aspirin only 2-year stroke rate: 26 %

b)Aspirin is the preferred treatment over surgery if the carotid stenosis is < 70 % [15, 1]

c)Angioplasty and stenting will have an increasing role [29]

21.5 The Ocular Ischemic Syndrome 525

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Fig. 21.5.22. Carotid endarterectomy surgery. The bifurcation of the common carotid artery into the internal carotid and external carotid arteries is shown by the arrow

Fig. 21.5.21. Argon laser panretinal photocoagulation in an eye with iris neovascularization due to the ocular ischemic syndrome

Fig. 21.5.23. Atherosclerotic plaque removed from a severely obstructed carotid artery. Fibrous, calcific and lipid components are present

References

1.Barnett HJ, Taylor DW, Eliasziw M, Fox AJ, Ferguson GG, Haynes RB, Rankin RN, Clagett GP, Hachinski VC, Sackett DL, Thorpe KE, Meldrum HE, Spence JD (1998) Benefit of carotid endarterectomy in patients with symptomatic moderate or severe stenosis. North American Symptomatic Carotid Endarterectomy Trial Collaborators. N Engl J Med 339:1415 – 25

2.Bolling JP, Buettner H (1990) Acquired retinal arteriovenous communications in occlusive disease of the carotid artery. Ophthalmology 97:1148 – 1152

3.Bosley TM (1986) The role of carotid noninvasive tests in stroke prevention. Semin Neurol 6:194 – 203

4.Brown GC (1986) Anterior ischemic optic neuropathy occurring in association with carotid artery obstruction. J Clin Neuro-ophthalmol 6:39 – 42

5.Brown GC (1986) Macular edema in association with severe carotid artery obstruction. Am J Ophthalmol 102:442 – 448

6.Brown GC, Magargal LE (1988) The ocular ischemic syndrome. Clinical, fluorescein angiographic and carotid angiographic features. Int Ophthalmol 11:239 – 251

7.Brown GC, Magargal LE, Simeone FA, Goldberg RE, Federman JL, Benson WE (1982) Arterial obstruction and ocular neovascularization. Ophthalmology 89:139 – 146

8.Bullock J, Falter RT, Downing JE, Snyder H (1972) Ischemic ophthalmia secondary to an ophthalmic artery occlusion. Am J Ophthalmol 74:486 – 493

9.Castaldo JE, Nicholas GG, Gee W, Reed JF (1989) Duplex ultrasound and ocular pneumoplethysmography concordance in detecting severe carotid stenosis. Arch Neurol 46:518 – 522

10.D’Onofrio M, Mansueto G, Faccioli N, Guarise A, Tamellini P, Bogina G, Pozzi Mucelli R (2006) Doppler ultrasound and contrast-enhanced magnetic resonance angiography in assessing carotid artery stenosis. Radiol Med (Torino) 111: 93 – 103

11.Dhobb M, Ammar F, Bensaid Y, Benjelloun A, Benabderra-

zik T, Benyahia B (1986) Arterial manifestations in Beh¸cet’s disease: four new cases. Ann Vasc Surg 1:249 – 252

12.Donnan GA, Sharbrough FW (1982) Carotid occlusive disease. Effect of bright light on visual evoked response. Arch Neurol 39:687 – 689

13.Duker JS, Belmont JB (1988) Ocular ischemic syndrome secondary to carotid artery dissection. Am J Ophthalmol 106:750 – 752

14.Effeney DJ, Krupski WC, Stoney RJ, Ehrenfeld WK (1983) Fibromuscular dysplasia of the carotid artery. Austral N Z J Surg 53:527 – 531

15.European Carotid Surgery Trialists’ Collaborative Group. MRC European Carotid Surgery Trial: interim results for symptomatic patients with severe (70 – 99 %) or with mild (0 – 29 %) carotid stenosis. European Carotid Surgery Trialists’ Collaborative Group. Lancet 337:1235 – 43

16.Hamed LM, Guy JR, Moster ML, Bosley T (1992) Giant cell arteritis in the ocular ischemic syndrome. Am J Ophthalmol 113:702 – 705

17.Hayreh SS (1976) So-called – “central retinal vein occlusion.” Venous-stasis retinopathy. Ophthalmologica 172:14 – 37

18.Ho AC, Lieb WE, Flaharty PM, Sergott RC, Brown GC, Bosley TM, Savino PJ (1992) Color Doppler imaging of the ocular ischemic syndrome. Ophthalmology 99:1453 – 62

19.Ishikawa K, Kimura I, Shinoda K, Eshita T, Kitamura S, Inoue M, Mashima Y (2002) In situ confirmation of retinal blood flow improvement after carotid endarterectomy in a patient with ocular ischemic syndrome. Am J Ophthalmol 134:295 – 7

20.Kahn M, Green WR, Knox DL, Miller NR (1986) Ocular features of carotid occlusive disease. Retina 6:239 – 252

21.Kawaguchi S, Okuno S, Sakaki T, Nishikawa N (2001) Effect of carotid endarterectomy on chronic ocular ischemic syndrome due to internal carotid artery stenosis. Neurosurgery 48:328 – 32

22.Kearns TP (1979) Ophthalmology and the carotid artery. Am J Ophthalmol 88:714 – 722

23.Kearns TP, Hollenhorst RW (1963) Venous stasis retinopa-

thy of occlusive disease of the carotid artery. Proc Mayo Clin 38:304 – 312

24.Knox DL (1965) Ischemic ocular inflammation. Am J Ophthalmol 60:995 – 1002

25.Kobayashi S, Hollenhorst RW, Sundt TM Jr (1971) Retinal arterial pressure before and after surgery for carotid artery stenosis. Stroke 2:569 – 575

26.Madsen PH (1965) Venous-stasis insufficiency of the ophthalmic artery. Acta Ophthalmol 40:940 – 947

27.Magargal LE, Sanborn GE, Zimmerman A (1982) Venous stasis retinopathy associated with embolic obstruction of the central retinal artery. J Clin Neuro-ophthalmol 2:113 – 118

28.Matonti F, Prost Magnin O, Galland F, Hoffart L, Coulibaly F, Conrath J, Ridings B (2006) Internal carotid artery dissection on arterial fibromuscular dysplasia causing a central retinal artery occlusion: a case report. J Fr Ophtalmol 29(7):e15

29.Mazighi M, Tanasescu R, Ducrocq X, Vicaut E, Bracard S, Houdart E, Woimant F (2006) Prospective study of symptomatic atherothrombotic intracranial stenoses: the GESICA study. Neurology 66:1187 – 91

30.Michelson PE, Knox DL, Green WR (1971) Ischemic ocular inflammation. A clinicopathologic case report. Arch Ophthalmol 86:274 – 280

31.Mizener JB, Podhajsky P, Hayreh SS (1997) Ocular ischemic syndrome. Ophthalmology 104(5):859 – 64

32.Neale ML, Chambers JL, Kelly AT, Connard S, et al. (1994) Reappraisal of duplex criteria to assess significant carotid artery stenosis with special reference to reports of the

35.Schlaegel T (1983) Symptoms and signs of uveitis. In: Duane TD (ed) Clinical ophthalmology, vol 4. Harper & Row, Hagerstown, pp 1 – 7

36.Sivalingam A, Brown GC, Magargal LE (1991) The ocular ischemic syndrome. III. Visual prognosis and the effect of treatment. Int Ophthalmol 15:15 – 20

37.Sivalingham A, Brown GC, Magargal LE, Menduke H (1989) The ocular ischemic syndrome II. Mortality and systemic morbidity. Int Ophthalmol 13:187 – 191

38.Sturrock GD, Mueller HR (1984) Chronic ocular ischaemia. Br J Ophthalmol 68:716 – 7123

39.The EC/IC Bypass Study Group (1985) Failure of extracra- nial-intracranial arterial bypass to reduce the risk of ischemic stroke. Results of an international randomized trial. N Engl J Med 313:1191 – 1200

40.Wiebers DO, Swanson JW, Cascino TL, Whisnant JP (1989) Bilateral loss of vision in bright light. Stroke 20:554 – 558

41.Young LHY, Appen RE (1981) Ischemic oculopathy, a manifestation of carotid artery disease. Arch Neurol 38:358 – 361

D. Pauleikhoff, B. Padge

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Core Messages

Retinal telangiectasis is a group of rare retinal vascular anomalies affecting the retinal capillaries

Characteristically irregular dilation, leakage and edema occur in the macula, rarely combined with vascular changes in the retinal periphery

22.1.1 History

Idiopathic juxtafoveolar retinal telangiectases (IJRT) were classified by Gass and Oyakawa in 1982 using biomicroscopic and fluorescein angiographic data [6]. This classification was updated by Gass and Blodi in 1993 [5], who subdivided IJRT into three groups (Table 22.1.1). Group 1 IJRT are unilateral in most cases and characterized by dilated retinal capillaries and abnormal leakage leading to an easily visible exudation. Group 2 IJRT are mostly bilateral and characterized by late staining on fluorescein angiography with minimal exudation. Later in the disease process retinal pigment epithelial proliferation or

Table 22.1.1. Summary of findings in idiopathic juxtafoveolar retinal telangiectasis [5]

Telangiectasis may develop unilaterally or bilaterally and is commonly characterized by a slow decrease in visual acuity in adulthood

The long-term prognosis for reading vision is usually good

The pathogenesis of these changes is unknown

secondary subretinal neovascularization may develop. In Group 3 changes are based on bilateral capillary occlusion. These changes lead to easily visible telangiectasis, parafoveolar capillary occlusion and minimal exudation.

22.1.2 Clinical Course of the Disease

Essentials

Group 1: Unilateral telangiectasis with abnormal leakage of capillaries

–Group 1A: Visible and exudative idiopathic juxtafoveolar retinal telangiectasis with lipid exudates, size > 1 disk diameter

–Group 1B: Visible and exudative focal idiopathic juxtafoveolar retinal telangiectasis, size < 1 disk diameter

Group 2: Bilateral idiopathic juxtafoveolar retinal telangiectasis

–Group 2A: Nonexudative bilateral idiopathic juxtafoveolar retinal telangiectasis

–Group 2B: Juvenile occult familial idiopathic juxtafoveolar retinal telangiectasis

Group 3: Occlusive idiopathic juxtafoveolar retinal telangiectasis

–Group 3A: Occlusive idiopathic juxtafoveolar retinal telangiectasis without central nervous system vasculopathy

–Group 3B: Occlusive idiopathic juxtafoveolar retinal telangiectasis with central nervous system vasculopathy

In Group 1A primarily male patients are affected. Commonly this group is characterized by a unilateral development of retinal telangiectasis and in most cases the vascular changes are easily visible. Patients with unilateral IJRT may be asymptomatic or may experience a mild reduction of visual acuity. Biomicroscopic findings are prominent telangiectatic retinal capillaries in the temporal half of the fovea with a 2 DD involvement of the macula, mostly associated with surrounding intraretinal lipid exudates (Fig. 22.1.1a).

Group 1B telangiectasis differs primarily in the size of lesion from Group 1A. The extension of telangiectatic vessels is only 1 disk diameter temporal of the fovea and the changes are mostly not associated with lipid exudates (Fig. 22.1.2). Male predilection, unilaterality and biomicroscopic and fluorescein angiographic features are nearly the same. It may be a mild form of Group 1A telangiectasis [5].

Patients in Group 2A IJRT have the most common form of idiopathic juxtafoveolar retinal telangiecta-

sis, but the cause is still unknown. Unlike Group 1 there is no sex predilection found in Group 2A IJRT. Also the telangiectases are usually diagnosed in the late 5th life decade, nearly 20 years later than those in Group 1 (Table 22.1.1). Typically the changes demonstrate bilateral symmetry with telangiectatic vessels in the temporal half of the fovea, which are difficult

22 III to detect on biomicroscopy (Fig. 22.1.3a). Therefore the main diagnostic tool is fluorescein angiography (Fig. 22.1.3a, b). The biomicroscopic changes are microaneurysms, minimal exudation, increased reti-

nal thickness, small yellow crystalline exudate (Fig. 22.1.3a) and right-angled venules. In the course of the disease proliferation of retinal pigment epithelium and development of subretinal neovascularization may develop [5, 10].

The pattern on fluorescein angiography is characterized by symmetric bilateral rapidly fluorescein stained capillary walls (Fig. 22.1.3b). This staining is followed by a late diffuse staining of the middle and outer retina surrounding part but sparing the foveola itself (Fig. 22.1.3c). This disorder primarily affects

the deep or outer juxtafoveolar capillary network in a zone within 1 DD of the center of the fovea.

Group 2A is further subdivided into five stages of development [5].

Stages 1 – 3 are characterized by intraretinal changes (Fig. 22.1.3). These stages include increased visibility of telangiectasis primarily affecting the outer capillary network temporal to the fovea. In Stage 3 there is evidence of one or several slightly dilated and blunted retinal venules that extend at right angles into the depth of the parafoveolar retina. Biomicroscopically there are often crystalline deposits visible in the temporal foveolar area (Fig. 22.1.3a) [10].

In Stage 4 is seen a reactive pigment epithelial proliferation caused by the loss of outer receptor cells and consecutive retinal pigment epithelial proliferation (Fig. 22.1.3d). It leads to one or several foci of black plaques of retinal pigment epithelium within the retina, often beneath the blunted tips of the right angled venules. Sometimes these venules are enveloped by pigment epithelial cells.

Stage 5 is characterized by development of secondary subretinal neovascularization (Fig. 22.1.3e, f). This neovascularization usually develops in the temporal half of the fovea and in the vicinity of intraretinal pigment epithelial migration. The absence of pigment epithelial detachment and a limited size of the neovascular complex suggest the retinal rather than the choroidal vasculature is the primary source of the new vessels [5].

Group 2B IJRT are characterized by an early development of telangiectasis and subfoveal neovas-

cularization. It was described in two brothers, 9 and 12 years old, by Gass et al. [5]. There have been no further cases reported in the literature.

Group 3 IJRT are characterized by easily visible telangiectasis associated with parafoveolar capillary occlusion and minimal exudation. This group is further subdivided into two subgroups: Group 3A and Group 3B (Table 22.1.1).

The main characteristic in Group 3A IJRT is an extensive occlusion of the juxtafoveolar capillary network. This leads to a minimal exudation and to a remarkably good visual acuity despite marked enlargement of the capillary free zone. This zone can be greater than 1 DD in size [5]. The visual loss is primarily caused by capillary obstruction and occlusion but not by exudation. The usual range of visual acu-

Table 22.1.2. Group 2A IJRT – clinical findings

Stage Characteristics

1Occult, normal fundus, fluorescein staining, no exudation

2Loss of retinal transparency, minimal telangiectasis, no exudation

3Mild telangiectasis, blunted dilated right angle venules, capillary remodeling, and capillaries in the outer retina, no exudation

4Intraretinal migration of retinal pigment epithelium (RPE), superficial stellate RPE plaques, less loss of retinal transparency, no exudation

5Subretinal new vessels, exudation and hemorrhage