Visual Field Changes in Branch Retinal 'Vein' Occlusion Colin H. Birchall, FRCS(Edin); Gordon S. Harris, FRCS(C); The visual field defects in 20 consecutive patients who had branch retinal "vein" occlusions included arcuate nerve fiber bundle scotomas, central scotomas, paracentral scotomas, and segmental peripheral constriction. The role of retinal arteriolar insufficiency in the production of these field defects is discussed. The available evidence suggests that the term "branch retinal vascular occlusion" is a preferable term to describe such clinical
findings. (Arch Ophthalmol 94:747-754, 1976)
visual field defects in branch retinal "vein" occlusion have been described in several texts,' "' and brief references have been made in other papers,"" indicating the types of defect that may be found. The most commonly described field changes are central scotomas, paracentral scoto¬ mas, and sector-shaped defects in the
The
corresponding quadrants. Peripheral
constriction of the visual field has been noted in central retinal vein occlusion. Harrington3 noted "sector or arcuate defects" and enlargement of angioscotomatous defects around the blind spot in the central type of venous occlusion. An authoritative paper by Aulhorn" indicated that when venous vascular occlusions were combined with ar¬ terial occlusions, perimetry showed absolute defects with horizontal or sector-shaped borders. She also illus¬ trated the characteristic patterns of
Accepted
for publication Feb 12, 1974. From the Department of Ophthalmology, University of British Columbia, and the Vancouver General Hospital, Vancouver, British Columbia, Canada. Reprint requests to Department of Ophthalmology, University of British Columbia, 2550 Willow St, Vancouver V5Z 3N9, British Columbia, Canada (Dr Harris).
Stephen
M.
Drance; FRCS; Iain S. Begg, FRCS(Edin)
suppression of macular sensitivity on profile perimetry, both in central and
in branch retinal vein occlusions. This study presents the findings of detailed examinations of visual fields of patients with fundus appearances of classical branch retinal vein occlu¬ sion. The results lend support to the hypothesis that relative retinal is¬ chemia is an important accompani¬ ment to the clinical signs of "venous" occlusion, and thus we prefer the use of the term "branch retinal vascular occlusion." The degree of retinal ischemia present may be minimal in the early stages of branch vascular
occlusion.
SUBJECTS AND METHODS
Twenty
consecutive patients with branch retinal vascular occlusions were fully investigated. All the patients studied had a provisional diagnosis of branch retinal vein occlusion made by the refer¬ ring ophthalmologists, and in all these patients the temporal vessels were in¬ volved. Because of the differing severity of symptoms and the nature of the onset of the disease process in the patients studied, there was a wide variation in the period of time between patients first noticing symp¬ toms and their presentation for examina¬ tion. This time varied between seven days and eight months. The visual field tests were performed as part of a detailed clinical examination and evaluation of these patients. Care was taken to exclude any patients who may have had other causes for their visual field defects, with particular reference to the many other lesions causing arcuate scotomas.10 None of the patients studied had chronic simple glaucoma or secondary glau¬ coma.
in an age studied. The central visual acuities ranged from 6/4.5
Fifteen
women
and five
range of 31 to 81 years
men
were
(20/15) (Snellen) to counting fingers at one
meter at the initial visit. Before patients with poor central visual acuity were included in the study, an assessment was
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made of their ability to maintain adequate central fixation. Patients were initially screened with the use of the Goldmann perimeter with the modifications of the Armaly method of detecting nerve fiber bundle defects." Patients with relative or absolute field defects subsequently had been plotted on the Tübingen (Oculus) perimeter. All patients also had profile perimetry of their maculae carried out on this instrument. White ten-minute arc test objects were used with a white background illumination of ten apostilbs (asb) using a 30-minute arc red fixation target.
RESULTS A number of different field changes were detected in the patients studied. They were grouped into the following
categories.
Arcuate Nerve Fiber Bundle Scotomas
showed fiber bundle defects (Fig 1). One example of such a defect in the inferior field with an absolute nucleus shown in the profile of the 315° meridian is seen (Fig 2). The fundus photographs (Fig 3 through 5) show a typical hemorrhagic retinopathy with evidence of arterial disease and a venous occlusion at the site of an arteriovenous crossing (arrow). The patient's visual acuity in this affected eye was 6/7.6 (20/25) with correction. Three of the patients with such inferior arcuate defects also showed field changes in the adjacent superior temporal field (Fig 6). The superior field defects exhibited sharp cut-off at the 180° meridian and had nuclei of absolute scotomas. Thus they also suggested nerve fiber bundle origins. There were distinct nasal steps across the 180° meridian. The fundus photo¬ graphs of one of these patients show the severe hemorrhagic retinopathy Nine of the 20
typical
arcuate
patients
nerve
Fig 1.—Large arcuate scotoma with absolute nucleus in patient superior temporal branch retinal vascular occlusion.
with
Fig 2.—Nerve fiber bundle defect with patient with superior temporal branch
two absolute nuclei in retinal vascular occlu¬
sion.
Fig 3.—Same patient as in Fig 2. Hemorrhagic retinopathy with blot hem¬ orrhage in macular area and macular
Fig 4—Same patient as in Fig 2 and 3. Arrow indicates presumed site of venous occlusion at arteriovenous crossover.
Fig 5.—Same patient as in Fig 2 through 4. Severe hemorrhagic retinopathy of branch vascular occlusion.
field is shown in
paracentral defect inferiorly in a patient with a superior temporal branch occlusion. The fundus photo¬ graph of this patient is shown in Fig 14. A severe hemorrhage extends over
edema.
with well-defined areas of retinal infarction (Fig 7 and 8, arrows). A year later, there was almost a complete resolution of the severe reti¬ nopathy with only a few blot hem¬ orrhages remaining (Fig 9 and 10), but the nerve fiber bundle defects remained almost unchanged (Fig 11). Patients with arcuate scotomatas show evidence of retinal infarction with areas of loss of retinal capillaries, illustrated by the fluorescein angiography film of a patient whose visual
Fig 11. This midveshows extensive ar¬ angiogram eas of nonperfused retina indicated by nous
arrow
(Fig 12).
Central Scotomas
All of the 20 patients studied showed central scotomas. In each case there was a relative scotoma, but two patients had absolute nuclei centrally. A typical example of the group is shown in Fig 13. There was a dense central scotoma with a less dense
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the neuroretinal rim of the disc, and there are parallel lines between the arrows that appear to run almost at right angles to the nerve fiber bundles. The origin of these lines is obscure; possibly they are due to edema fluid. Macular profile perimetry showed
Fig 6.—Visual field of patient with superior temporal branch retinal vascular occlusion. Absolute arcuate defect in inferior nasal
7.—Patient whose visual field is shown in Fig 6, taken just prior to visual field. There is superior temporal branch retinal vascular occlusion. Arrow indicates cot¬ ton-wool spot.
quadrant with absolute "cap" defect in adjacent superior nasal quadrant. 225° profile confirms absolute nature of scotoma.
Fig
Fig 8.—Same patient as in Arrow indicates second
6 and 7. cotton-wool
Fig
spot.
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Fig 9.—Same patient as in Fig 6 through 8, taken 13 months later. Most of retinal hemorrhages have cleared, as well as cotton-wool spots. Photograph frames do not exactly correspond.
quantitatively
the degree of suppres¬ sion of macular function in relative central scotomas. A normal macular profile of a 54-year-old woman is shown at the top right of Fig 15. The macular edema that was pres¬ ent gave a characteristic paracentral trough at 2° or 3° from the fovea, with a rise in retinal sensitivity usually around 5° from fixation and then the usual gradual fall of retinal sensitivity towards the periphery of the visual field (Fig 15, A through C). These characteristic profiles may be regular
Fig 10.—Same patient
in
Fig 6 through 9, taken at same time as Fig 9. This also shows clearing of hemorrhagic retinopa¬ thy. as
(Fig 15, A) or irregular (Fig 15, and C, and Fig 16, A and B), depending on
the distribution of the macular edema, and may be called "doughnut" scoto¬ mas when symmetrical (Fig 16, C). Paracentral Scotomas
Fig 12.—Intravenous fluorescein angiogram, venous phase, of same patient whose fields and fundus photographs are shown in Fig 6 through Fig 11. Arrow indicates large area of retina with noncapillary perfusion. Large dilated central vessel is arterial in origin.
Eight patients showed paracentral
scotomas. These scotomas
were either extensions of relative central scoto¬ mas beyond the central area, that is,
Fig 11.—Same patient as in Fig 6 through 10. Visual field plotted after photographs Figs 9 and 10 taken. Original field defects
soon
have enlarged remains.
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slightly. Superior
nerve
fiber bundle "cap" defect
13.—Patient with superior temporal branch retinal vascular occlusion showing relative central scotoma.
Fig
Fig 14—Patient whose visual field is shown in Fig 13. Severe hemorrhagic reti¬ nopathy with hemorrhage on superior and nasal edges of disc. Veins are dilated and arteries show evidence of arteriosclerosis. Between arrows are seen number of nearly parallel lines that run almost at right angles to nerve fiber bundles, but they are not concentric with optic nerve head. Origin of these lines is obscure; they may be caused by retinal edema.
becoming paracentral relative
scoto¬
mas, or dense nuclei of paracentral scotomas independent of any central scotoma present. An example is illus¬ trated by Fig 17. This patient had an occlusion of a tributary of the superior temporal vein at an arteriovenous crossing, and the fundus appearance is shown in Fig 18. There is hemorrhage distributed segmentally along the affected vein that is almost totally obscured by the hemorrhage. A little hemorrhage appears at the edge of the disc temporally.
Segmental Peripheral Constriction In one patient there was a little nasal peripheral constriction (Fig 19) associated with a large arcuate nerve fiber bundle defect in the
sponding quadrant and
nerve
Fig 15.—Macular profiles. At top right, Normal profile of 54-year-old normal female patient. At bottom; Characteristic profile seen in macular edema where depression is uniform in two meridians (A); irregular profiles are shown of macular edema where edema is distributed unequally around fovea ( and C). NORMAt Nasal Color:
Light
Int.:
Target:
^ W
250
Background:
10
Temporal
Size:
¿± 10 30
W
100
320
135°
45°
MACULA OD
corre¬
3.2
fiber
bundle type scotoma in the
adjacent superior quadrant (compare Fig 6 and
10
-32
20).
-ìoo
COMMENT
The severe hemorrhagic retinopa¬ thy that has previously been described as the classical feature of central and
°D MACULA
-320 1000 °
°
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/16/2015
320 1000
i
io°
3.2 10
32
100
320 1000
NORMAL Nasal
Color:
Light
Int,
Target
^
250
Background;
10
Temporal
Size: m¡n
10
W
Fixation Pt.:
OD MACULA
30 W
225
180°
1000
1000
Fig 16.—Macular profiles in macular edema. At bottom, Macular edema on nasal side of fovea only gives depression of profile at 3° and 0° temporal meridian (A); irregular profile of more severe macular edema giving more severe relative scotoma (B); symmetrical profile that may be called "doughnut" scotoma (C).
Fig
17.—Visual field of scotoma.
patient
with
tributary
vein occlusion
causing paracentral
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branch retinal vein occlusion is now thought to occur when there is also some degree of arterial insufficiency present. The work of Hayreh" on Rhesus monkeys demonstrated that, in order to produce the florid retinop¬ athy experimentally, it was necessary to compromise the central retinal artery as well as the central retinal vein. Occlusion of the central retinal vein alone produced tortuosity and dilatation of the veins with mild hyperemia of the disc, all these changes regressing in a period of three to four weeks. The clinical study by Paton et al' of patients with venous occlusion stressed the importance of retinal ischemia in the etiology of this condi¬ tion. Histopathologic studies1- have also demonstrated severe structural changes in the walls and lumina of arterioles in quadrants that show the retinopathy of branch occlusions. The association of quadrantal hem¬ orrhagic retinopathy with arterial disease, arteriolar disease, and retinal infarction, as well as venous changes, is thus well established. The absolute arcuate scotomas found in this study are produced by arteriolar disease. It is possible to consider that in cases of branch retinal "vein" occlusion, relative scoto¬ mas are caused by severe retinal hemorrhage and edema alone, but our findings demonstrate that the hem¬ orrhages and edema are not respon¬ sible for all the visual field changes found. The absolute arcuate scotomas in our patients did not improve during the period of observation, which extended to 15 months. The hem¬ orrhages and edema had cleared in the interim period, indicating that they were not responsible for the field changes but that the latter were caused by arteriolar insufficiency. The striking feature of this study is the high prevalence (45%) of patients having arcuate nerve fiber bundle defects with very dense relative or absolute nuclei, even though there was no selection of patients on the basis of the severity of their retinopathy. The fundus findings range from very mild blot hemorrhages with venous tor¬ tuosity to very severe hemorrhages with extensive retinal infarcts. The
Fig
18.—Patient whose field is
seen
in
Fig
17, showing mild hemorrhagic retinopathy
and little disc.
hemorrhage
at
temporal edge
of
Fig 19.—Peripheral visual field constricted nasally
occlusion.
Fig 20.—Central visual field of patient whose peripheral field is shown in Fig inferior arcuate scotoma with absolute "cap" defect in adjacent superior
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in
patient with
19. Large absolute nasal quadrant.
branch vascular
patients whose visual field is shown (Fig 1) subsequently developed a branch vascular occlusion in the inferotemporal quadrant some weeks after a fundus photograph of this area was taken. The preocclusive fundus photograph (Fig 21) showed clear evidence of arteriovenous nipping, silver wiring, and slight irregularity of the lumen of the inferior temporal retinal artery. Thus, in this case scle¬ rotic changes of the retinal arteries were present prior to the onset of the retinopathy. After the second branch vascular occlusion, the fundus ap¬ pearance is as shown in Fig 22. An interesting and previously undescribed finding is the involvement of the temporal retinal quadrant above or below the horizontal nerve fiber bundle raphe, causing an addi¬ tional absolute nerve fiber scotoma adjacent to the main nerve fiber bundle defect corresponding to the arteriolar involvement. This produces the "cap" defect that we have described in this study. The most likely explanation of this feature is that the arteriolar supply to the temporal retina has crossed the hori¬ zontal raphe, and the retinal arteriolar and capillary insufficiency in this adjacent area has caused the absolute nerve fiber bundle cap defect. Clinical observations support this hypothesis since several patients in this study had hemorrhagic retinopathy and infarction below the horizontal raphe in the midtemporal periphery in superior temporal vascular occlusions. The affected superior temporal ves¬ sels in these cases cross the horizontal raphe and pass into the inferior our
Fig 21 .—Superior temporal vascular occlu¬ sion in patient whose visual field is shown in Fig 1. Inferotemporal arteries and arterioles show silver wiring; there is also arteriovenous nipping in inferotemporal quadrant.
Fig 22.—Same patient as in Fig 21, six weeks later. Second branch vascular occlusion has occurred, this time in infero¬ temporal region. There are large cotton¬ wool spots in both temporal quadrants.
relationship between the severity of retinopathy and degree of field loss was not always closely correlated. However, all patients with arcuate
fiber bundle scotomas had cotton-wool spots in the affected quadrant, although in some cases these were very small. The severity of hemorrhages in patients having ar¬ cuate nerve fiber defects varied from mild to very severe. Aulhorn found extensive absolute scotomas of "arterial origin" with horizontal borders in 8 out of 44 cases of branch vein occlusion studied. She wrote that areas of arteriolar occlu¬ sions could be seen in these cases. This view is confirmed by our study. One of nerve
quadrants.
The central and paracentral scoto¬ have been previously described and are entirely predictable findings, as determined from the associated macular and paramacular hem¬ orrhages and edema. Profile perimetry appeared to be a very sensitive test of macular edema, mas
presenting characteristic, doughnutshaped scotomas. Asymmetrical varia¬ tions in the shape of this doughnut scotoma occurred when only mild edema was present or when the edema was distributed unevenly around the fovea. There was evidence of macular
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edema in all cases, although in the mildest cases it was not always detect¬ able on slit-lamp examination using a three-mirror Goldmann gonioscopic lens. This edema was always detected, however, on the late films (30 minutes of more after injection) following intravenous fluorescein angiography. The manner in which the perifoveal edema was distributed and its degree of severity were noted on the late fluorescein films. It was found by exploring the macula profiles in vari¬ ous meridians (0°, 45°, 135°, 180°, 225°, and 315°) that the scotomas
corresponded quantitatively accurate¬ ly with the edema as seen on the films.
Thus, in addition to intravenous fluo¬ rescein angiography, macular profile perimetry may be used as a speedy and accurate method for the detection of macular edema.
Dr Birchall was the recipient of a Retinal from the British Columbia Medical Services Foundation and a traveling grant from Trustees of the Keeler Awards.
Fellowship
Key Words.—Branch retinal vascular oc¬ clusion; arcuate nerve fiber bundle scoto¬
ma; central scotoma; paracentral scotoma; peripheral constriction; arteriolar insuffi¬ ciency; nerve fiber bundle "cap" defect.
References 1. Paton A, Rubinstein K, Smith VH: Arterial insufficiency in retinal venous occlusion: A short
symposium.
Trans
Ophthalmol
Soc UK 84:559\x=req-\
586, 1964.
2. Dubois-Poulson A: Le Champ Visuel, Topographie Normale et Pathologique de Ses Sensibilit\l=e'\s.Paris, Masson et Cie, 1952, p 650. 3. Reed H, Drance SM: The Essentials of Perimetry, Static and Kinetic, ed 2. London, Oxford University Press, 1972, p 93. 4. Traquair HM: An Introduction to Clinical Perimetry, ed 4. St Louis, CV Mosby Co, 1943, pp
105-106. 5. Harrington DO: The Visual Fields, a Textbook and Atlas of Clinical Perimetry, ed 3. St Louis, CV MosbyCo, 1971, pp 171-172. 6. Hayreh SS: Occlusion of central retinal vessels. Br J Ophthalmol 49:626, 1965. 7. Hill DW, Griffiths JD: The prognosis in retinal vein thrombosis. Trans Ophthalmol Soc UK 90:309-322, 1970. 8. Krill AE, Archer D, Newell FW: Photocoagulation in complications secondary to branch vein occlusion. Arch Ophthalmol 85:48-60, 1971. 9. Aulhorn E: Gesichtsfeldver\l=a"\nderungenbei netzhautgef\l=a"\bverschl\l=u"\ssen.Klin Monatsbl Augenheilkd 143:234-247, 1963. 10. Harrington DO: The Bjerrum scotoma. Am J Ophthalmol 59:646, 1965. 11. Rock WJ, Drance SM, Morgan RW: A modification of the Armaly visual field screening technique for glaucoma. Can J Ophthalmol 6:283\x=req-\ 292, 1971. 12. Rabinowicz IM, Litman S, Michaelson IC: Branch venous thrombosis: A pathological report. Trans Ophthalmol Soc UK 88:191-208, 1968.
findings. (Arch Ophthalmol 94:747-754, 1976)
visual field defects in branch retinal "vein" occlusion have been described in several texts,' "' and brief references have been made in other papers,"" indicating the types of defect that may be found. The most commonly described field changes are central scotomas, paracentral scoto¬ mas, and sector-shaped defects in the
The
corresponding quadrants. Peripheral
constriction of the visual field has been noted in central retinal vein occlusion. Harrington3 noted "sector or arcuate defects" and enlargement of angioscotomatous defects around the blind spot in the central type of venous occlusion. An authoritative paper by Aulhorn" indicated that when venous vascular occlusions were combined with ar¬ terial occlusions, perimetry showed absolute defects with horizontal or sector-shaped borders. She also illus¬ trated the characteristic patterns of
Accepted
for publication Feb 12, 1974. From the Department of Ophthalmology, University of British Columbia, and the Vancouver General Hospital, Vancouver, British Columbia, Canada. Reprint requests to Department of Ophthalmology, University of British Columbia, 2550 Willow St, Vancouver V5Z 3N9, British Columbia, Canada (Dr Harris).
Stephen
M.
Drance; FRCS; Iain S. Begg, FRCS(Edin)
suppression of macular sensitivity on profile perimetry, both in central and
in branch retinal vein occlusions. This study presents the findings of detailed examinations of visual fields of patients with fundus appearances of classical branch retinal vein occlu¬ sion. The results lend support to the hypothesis that relative retinal is¬ chemia is an important accompani¬ ment to the clinical signs of "venous" occlusion, and thus we prefer the use of the term "branch retinal vascular occlusion." The degree of retinal ischemia present may be minimal in the early stages of branch vascular
occlusion.
SUBJECTS AND METHODS
Twenty
consecutive patients with branch retinal vascular occlusions were fully investigated. All the patients studied had a provisional diagnosis of branch retinal vein occlusion made by the refer¬ ring ophthalmologists, and in all these patients the temporal vessels were in¬ volved. Because of the differing severity of symptoms and the nature of the onset of the disease process in the patients studied, there was a wide variation in the period of time between patients first noticing symp¬ toms and their presentation for examina¬ tion. This time varied between seven days and eight months. The visual field tests were performed as part of a detailed clinical examination and evaluation of these patients. Care was taken to exclude any patients who may have had other causes for their visual field defects, with particular reference to the many other lesions causing arcuate scotomas.10 None of the patients studied had chronic simple glaucoma or secondary glau¬ coma.
in an age studied. The central visual acuities ranged from 6/4.5
Fifteen
women
and five
range of 31 to 81 years
men
were
(20/15) (Snellen) to counting fingers at one
meter at the initial visit. Before patients with poor central visual acuity were included in the study, an assessment was
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/16/2015
made of their ability to maintain adequate central fixation. Patients were initially screened with the use of the Goldmann perimeter with the modifications of the Armaly method of detecting nerve fiber bundle defects." Patients with relative or absolute field defects subsequently had been plotted on the Tübingen (Oculus) perimeter. All patients also had profile perimetry of their maculae carried out on this instrument. White ten-minute arc test objects were used with a white background illumination of ten apostilbs (asb) using a 30-minute arc red fixation target.
RESULTS A number of different field changes were detected in the patients studied. They were grouped into the following
categories.
Arcuate Nerve Fiber Bundle Scotomas
showed fiber bundle defects (Fig 1). One example of such a defect in the inferior field with an absolute nucleus shown in the profile of the 315° meridian is seen (Fig 2). The fundus photographs (Fig 3 through 5) show a typical hemorrhagic retinopathy with evidence of arterial disease and a venous occlusion at the site of an arteriovenous crossing (arrow). The patient's visual acuity in this affected eye was 6/7.6 (20/25) with correction. Three of the patients with such inferior arcuate defects also showed field changes in the adjacent superior temporal field (Fig 6). The superior field defects exhibited sharp cut-off at the 180° meridian and had nuclei of absolute scotomas. Thus they also suggested nerve fiber bundle origins. There were distinct nasal steps across the 180° meridian. The fundus photo¬ graphs of one of these patients show the severe hemorrhagic retinopathy Nine of the 20
typical
arcuate
patients
nerve
Fig 1.—Large arcuate scotoma with absolute nucleus in patient superior temporal branch retinal vascular occlusion.
with
Fig 2.—Nerve fiber bundle defect with patient with superior temporal branch
two absolute nuclei in retinal vascular occlu¬
sion.
Fig 3.—Same patient as in Fig 2. Hemorrhagic retinopathy with blot hem¬ orrhage in macular area and macular
Fig 4—Same patient as in Fig 2 and 3. Arrow indicates presumed site of venous occlusion at arteriovenous crossover.
Fig 5.—Same patient as in Fig 2 through 4. Severe hemorrhagic retinopathy of branch vascular occlusion.
field is shown in
paracentral defect inferiorly in a patient with a superior temporal branch occlusion. The fundus photo¬ graph of this patient is shown in Fig 14. A severe hemorrhage extends over
edema.
with well-defined areas of retinal infarction (Fig 7 and 8, arrows). A year later, there was almost a complete resolution of the severe reti¬ nopathy with only a few blot hem¬ orrhages remaining (Fig 9 and 10), but the nerve fiber bundle defects remained almost unchanged (Fig 11). Patients with arcuate scotomatas show evidence of retinal infarction with areas of loss of retinal capillaries, illustrated by the fluorescein angiography film of a patient whose visual
Fig 11. This midveshows extensive ar¬ angiogram eas of nonperfused retina indicated by nous
arrow
(Fig 12).
Central Scotomas
All of the 20 patients studied showed central scotomas. In each case there was a relative scotoma, but two patients had absolute nuclei centrally. A typical example of the group is shown in Fig 13. There was a dense central scotoma with a less dense
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the neuroretinal rim of the disc, and there are parallel lines between the arrows that appear to run almost at right angles to the nerve fiber bundles. The origin of these lines is obscure; possibly they are due to edema fluid. Macular profile perimetry showed
Fig 6.—Visual field of patient with superior temporal branch retinal vascular occlusion. Absolute arcuate defect in inferior nasal
7.—Patient whose visual field is shown in Fig 6, taken just prior to visual field. There is superior temporal branch retinal vascular occlusion. Arrow indicates cot¬ ton-wool spot.
quadrant with absolute "cap" defect in adjacent superior nasal quadrant. 225° profile confirms absolute nature of scotoma.
Fig
Fig 8.—Same patient as in Arrow indicates second
6 and 7. cotton-wool
Fig
spot.
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Fig 9.—Same patient as in Fig 6 through 8, taken 13 months later. Most of retinal hemorrhages have cleared, as well as cotton-wool spots. Photograph frames do not exactly correspond.
quantitatively
the degree of suppres¬ sion of macular function in relative central scotomas. A normal macular profile of a 54-year-old woman is shown at the top right of Fig 15. The macular edema that was pres¬ ent gave a characteristic paracentral trough at 2° or 3° from the fovea, with a rise in retinal sensitivity usually around 5° from fixation and then the usual gradual fall of retinal sensitivity towards the periphery of the visual field (Fig 15, A through C). These characteristic profiles may be regular
Fig 10.—Same patient
in
Fig 6 through 9, taken at same time as Fig 9. This also shows clearing of hemorrhagic retinopa¬ thy. as
(Fig 15, A) or irregular (Fig 15, and C, and Fig 16, A and B), depending on
the distribution of the macular edema, and may be called "doughnut" scoto¬ mas when symmetrical (Fig 16, C). Paracentral Scotomas
Fig 12.—Intravenous fluorescein angiogram, venous phase, of same patient whose fields and fundus photographs are shown in Fig 6 through Fig 11. Arrow indicates large area of retina with noncapillary perfusion. Large dilated central vessel is arterial in origin.
Eight patients showed paracentral
scotomas. These scotomas
were either extensions of relative central scoto¬ mas beyond the central area, that is,
Fig 11.—Same patient as in Fig 6 through 10. Visual field plotted after photographs Figs 9 and 10 taken. Original field defects
soon
have enlarged remains.
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slightly. Superior
nerve
fiber bundle "cap" defect
13.—Patient with superior temporal branch retinal vascular occlusion showing relative central scotoma.
Fig
Fig 14—Patient whose visual field is shown in Fig 13. Severe hemorrhagic reti¬ nopathy with hemorrhage on superior and nasal edges of disc. Veins are dilated and arteries show evidence of arteriosclerosis. Between arrows are seen number of nearly parallel lines that run almost at right angles to nerve fiber bundles, but they are not concentric with optic nerve head. Origin of these lines is obscure; they may be caused by retinal edema.
becoming paracentral relative
scoto¬
mas, or dense nuclei of paracentral scotomas independent of any central scotoma present. An example is illus¬ trated by Fig 17. This patient had an occlusion of a tributary of the superior temporal vein at an arteriovenous crossing, and the fundus appearance is shown in Fig 18. There is hemorrhage distributed segmentally along the affected vein that is almost totally obscured by the hemorrhage. A little hemorrhage appears at the edge of the disc temporally.
Segmental Peripheral Constriction In one patient there was a little nasal peripheral constriction (Fig 19) associated with a large arcuate nerve fiber bundle defect in the
sponding quadrant and
nerve
Fig 15.—Macular profiles. At top right, Normal profile of 54-year-old normal female patient. At bottom; Characteristic profile seen in macular edema where depression is uniform in two meridians (A); irregular profiles are shown of macular edema where edema is distributed unequally around fovea ( and C). NORMAt Nasal Color:
Light
Int.:
Target:
^ W
250
Background:
10
Temporal
Size:
¿± 10 30
W
100
320
135°
45°
MACULA OD
corre¬
3.2
fiber
bundle type scotoma in the
adjacent superior quadrant (compare Fig 6 and
10
-32
20).
-ìoo
COMMENT
The severe hemorrhagic retinopa¬ thy that has previously been described as the classical feature of central and
°D MACULA
-320 1000 °
°
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/16/2015
320 1000
i
io°
3.2 10
32
100
320 1000
NORMAL Nasal
Color:
Light
Int,
Target
^
250
Background;
10
Temporal
Size: m¡n
10
W
Fixation Pt.:
OD MACULA
30 W
225
180°
1000
1000
Fig 16.—Macular profiles in macular edema. At bottom, Macular edema on nasal side of fovea only gives depression of profile at 3° and 0° temporal meridian (A); irregular profile of more severe macular edema giving more severe relative scotoma (B); symmetrical profile that may be called "doughnut" scotoma (C).
Fig
17.—Visual field of scotoma.
patient
with
tributary
vein occlusion
causing paracentral
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branch retinal vein occlusion is now thought to occur when there is also some degree of arterial insufficiency present. The work of Hayreh" on Rhesus monkeys demonstrated that, in order to produce the florid retinop¬ athy experimentally, it was necessary to compromise the central retinal artery as well as the central retinal vein. Occlusion of the central retinal vein alone produced tortuosity and dilatation of the veins with mild hyperemia of the disc, all these changes regressing in a period of three to four weeks. The clinical study by Paton et al' of patients with venous occlusion stressed the importance of retinal ischemia in the etiology of this condi¬ tion. Histopathologic studies1- have also demonstrated severe structural changes in the walls and lumina of arterioles in quadrants that show the retinopathy of branch occlusions. The association of quadrantal hem¬ orrhagic retinopathy with arterial disease, arteriolar disease, and retinal infarction, as well as venous changes, is thus well established. The absolute arcuate scotomas found in this study are produced by arteriolar disease. It is possible to consider that in cases of branch retinal "vein" occlusion, relative scoto¬ mas are caused by severe retinal hemorrhage and edema alone, but our findings demonstrate that the hem¬ orrhages and edema are not respon¬ sible for all the visual field changes found. The absolute arcuate scotomas in our patients did not improve during the period of observation, which extended to 15 months. The hem¬ orrhages and edema had cleared in the interim period, indicating that they were not responsible for the field changes but that the latter were caused by arteriolar insufficiency. The striking feature of this study is the high prevalence (45%) of patients having arcuate nerve fiber bundle defects with very dense relative or absolute nuclei, even though there was no selection of patients on the basis of the severity of their retinopathy. The fundus findings range from very mild blot hemorrhages with venous tor¬ tuosity to very severe hemorrhages with extensive retinal infarcts. The
Fig
18.—Patient whose field is
seen
in
Fig
17, showing mild hemorrhagic retinopathy
and little disc.
hemorrhage
at
temporal edge
of
Fig 19.—Peripheral visual field constricted nasally
occlusion.
Fig 20.—Central visual field of patient whose peripheral field is shown in Fig inferior arcuate scotoma with absolute "cap" defect in adjacent superior
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in
patient with
19. Large absolute nasal quadrant.
branch vascular
patients whose visual field is shown (Fig 1) subsequently developed a branch vascular occlusion in the inferotemporal quadrant some weeks after a fundus photograph of this area was taken. The preocclusive fundus photograph (Fig 21) showed clear evidence of arteriovenous nipping, silver wiring, and slight irregularity of the lumen of the inferior temporal retinal artery. Thus, in this case scle¬ rotic changes of the retinal arteries were present prior to the onset of the retinopathy. After the second branch vascular occlusion, the fundus ap¬ pearance is as shown in Fig 22. An interesting and previously undescribed finding is the involvement of the temporal retinal quadrant above or below the horizontal nerve fiber bundle raphe, causing an addi¬ tional absolute nerve fiber scotoma adjacent to the main nerve fiber bundle defect corresponding to the arteriolar involvement. This produces the "cap" defect that we have described in this study. The most likely explanation of this feature is that the arteriolar supply to the temporal retina has crossed the hori¬ zontal raphe, and the retinal arteriolar and capillary insufficiency in this adjacent area has caused the absolute nerve fiber bundle cap defect. Clinical observations support this hypothesis since several patients in this study had hemorrhagic retinopathy and infarction below the horizontal raphe in the midtemporal periphery in superior temporal vascular occlusions. The affected superior temporal ves¬ sels in these cases cross the horizontal raphe and pass into the inferior our
Fig 21 .—Superior temporal vascular occlu¬ sion in patient whose visual field is shown in Fig 1. Inferotemporal arteries and arterioles show silver wiring; there is also arteriovenous nipping in inferotemporal quadrant.
Fig 22.—Same patient as in Fig 21, six weeks later. Second branch vascular occlusion has occurred, this time in infero¬ temporal region. There are large cotton¬ wool spots in both temporal quadrants.
relationship between the severity of retinopathy and degree of field loss was not always closely correlated. However, all patients with arcuate
fiber bundle scotomas had cotton-wool spots in the affected quadrant, although in some cases these were very small. The severity of hemorrhages in patients having ar¬ cuate nerve fiber defects varied from mild to very severe. Aulhorn found extensive absolute scotomas of "arterial origin" with horizontal borders in 8 out of 44 cases of branch vein occlusion studied. She wrote that areas of arteriolar occlu¬ sions could be seen in these cases. This view is confirmed by our study. One of nerve
quadrants.
The central and paracentral scoto¬ have been previously described and are entirely predictable findings, as determined from the associated macular and paramacular hem¬ orrhages and edema. Profile perimetry appeared to be a very sensitive test of macular edema, mas
presenting characteristic, doughnutshaped scotomas. Asymmetrical varia¬ tions in the shape of this doughnut scotoma occurred when only mild edema was present or when the edema was distributed unevenly around the fovea. There was evidence of macular
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edema in all cases, although in the mildest cases it was not always detect¬ able on slit-lamp examination using a three-mirror Goldmann gonioscopic lens. This edema was always detected, however, on the late films (30 minutes of more after injection) following intravenous fluorescein angiography. The manner in which the perifoveal edema was distributed and its degree of severity were noted on the late fluorescein films. It was found by exploring the macula profiles in vari¬ ous meridians (0°, 45°, 135°, 180°, 225°, and 315°) that the scotomas
corresponded quantitatively accurate¬ ly with the edema as seen on the films.
Thus, in addition to intravenous fluo¬ rescein angiography, macular profile perimetry may be used as a speedy and accurate method for the detection of macular edema.
Dr Birchall was the recipient of a Retinal from the British Columbia Medical Services Foundation and a traveling grant from Trustees of the Keeler Awards.
Fellowship
Key Words.—Branch retinal vascular oc¬ clusion; arcuate nerve fiber bundle scoto¬
ma; central scotoma; paracentral scotoma; peripheral constriction; arteriolar insuffi¬ ciency; nerve fiber bundle "cap" defect.
References 1. Paton A, Rubinstein K, Smith VH: Arterial insufficiency in retinal venous occlusion: A short
symposium.
Trans
Ophthalmol
Soc UK 84:559\x=req-\
586, 1964.
2. Dubois-Poulson A: Le Champ Visuel, Topographie Normale et Pathologique de Ses Sensibilit\l=e'\s.Paris, Masson et Cie, 1952, p 650. 3. Reed H, Drance SM: The Essentials of Perimetry, Static and Kinetic, ed 2. London, Oxford University Press, 1972, p 93. 4. Traquair HM: An Introduction to Clinical Perimetry, ed 4. St Louis, CV Mosby Co, 1943, pp
105-106. 5. Harrington DO: The Visual Fields, a Textbook and Atlas of Clinical Perimetry, ed 3. St Louis, CV MosbyCo, 1971, pp 171-172. 6. Hayreh SS: Occlusion of central retinal vessels. Br J Ophthalmol 49:626, 1965. 7. Hill DW, Griffiths JD: The prognosis in retinal vein thrombosis. Trans Ophthalmol Soc UK 90:309-322, 1970. 8. Krill AE, Archer D, Newell FW: Photocoagulation in complications secondary to branch vein occlusion. Arch Ophthalmol 85:48-60, 1971. 9. Aulhorn E: Gesichtsfeldver\l=a"\nderungenbei netzhautgef\l=a"\bverschl\l=u"\ssen.Klin Monatsbl Augenheilkd 143:234-247, 1963. 10. Harrington DO: The Bjerrum scotoma. Am J Ophthalmol 59:646, 1965. 11. Rock WJ, Drance SM, Morgan RW: A modification of the Armaly visual field screening technique for glaucoma. Can J Ophthalmol 6:283\x=req-\ 292, 1971. 12. Rabinowicz IM, Litman S, Michaelson IC: Branch venous thrombosis: A pathological report. Trans Ophthalmol Soc UK 88:191-208, 1968.
