•

Retinal Arterial Obstruction tn

Children and Young Adults
GARY C. BROWN, MD,* LARRY E. MAGARGAL, MD,* JERRY A. SHIELDS, MD,*
RICHARD E. GOLDBERG, MD,* PETER N. WALSH, MD, PhDt

Abstract: The records of 27 patients who developed retinal arterial

obstruction (RAO) prior to the age of 30 years were studied to ascertain
associated systemic and ocular findings as possible etiologic factors. A
history of migraine was found in approximately one third of the patients,
and coagulation abnormalities were also common. Trauma, sickle cell
hemoglobinopathies, cardiac disorders, use of oral contraceptives,
pregnancy, systemic lupus erythematosus and intravenous drug abuse
were less frequently encountered. Ocular abnormalities included in-
creased intraocular pressure, subtle buried drusen of the optic nerve
head and a congenital prepapillary arterial loop. In contrast to older
patients with RAO, there was no clinical evidence of atheromatous dis-
ease. In most patients, one or more systemic or ocular etiologic factors
could be discerned. Whereas etiologic relationships may be multifacto-
rial and generally differ from those commonly found in older patients with
RAO, the visual prognosis in younger and older patients appears to be
similar. [Key words: arterial loops, coagulation disorders, connective
tissue disorders, intravenous drug abuse, migraine headache, ocular
hypertension, optic nerve head drusen, oral contraceptives, retinal arte-
rial obstruction, rheumatic fever, sickle hemoglobinopathy, trauma.]
Ophthalmology 88:18-25, 1981

Blockage of the retinal arterial system may occur in
the form of central retinal arterial obstruction (CRAO),
branch retinal arterial obstruction (BRAO), cilioretinal
arterial obstruction (CAO), or combinations of the
aforementioned. 1 It may be related to known pre-
existing systemic disease or it may be the initial man-
ifestation of a previously undiagnosed systemic ab-
normality. Approximately 75% of patients over the age
of 40 with retinal arterial obstruction (RAO) have
clinical findings suggestive of emboli originating from
atheromatous disease of the carotid arteries. 2 In young
patients with RAO, atheromatous vascular disease is
rarely detected, and associated etiologic factors are
more often obscure and diverse.
While individual case reports dealing with RAO in
young patients are numerous, we are unaware of a
previous report analyzing a large series of young
people with this disorder. It was the purpose of this
retrospective study to more completely ascertain sys-
temic and ophthalmologic characteristics of patients
under the age of 30 years who present with RAO.

From the Retina Service, Wills Eye Hospital and Research Insti-
tute,* Philadelphia, and the Specialized Center on Thrombosis Re-
search and Department of Internal Medicine, t Temple University,
MATERIALS AND METHODS
Philadelphia.
Presented at the Eighty-Fifth Annual Meeting of the American
The Wills Eye Hospital Retina Service records of all
Academy of Ophthalmology, Chicago, November 2-7, 1980. patients under age 30 years who presented with either
central, branch, or cilioretinal arterial obstruction
Supported in part by the Retina Research and Development Foun-
dation, Philadelphia, and the Pennsylvania Lions Sight Conserva- were reviewed. The patients were evaluated between
tion and Eye Research Foundation, Inc. August 1967 and June 1979. Cases of questionable di-
Reprint requests to Larry E. Magargal, MD, Retinal Vascular Unit,
agnosis were excluded. With the exception of three
Wills Eye Hospital, Ninth and Walnut Streets, Philadelphia, PA patients in whom severe trauma was an obvious
19107. etiologic factor, all patients received a complete his-

18 0161-6420/81/0100/0018/$00.90 ©American Academy of Ophthalmology

 BROWN, et al • RETINAL ARTERIAL OBSTRUCTION

tory and general physical examination by an internist, Table 3). The headaches were usually unilateral, but
and whenever possible, referral to various subspe- with regard to the RAO, there was no preferential lat-
cialists as deemed necessary. All patients underwent erality. All eight patients with migraines had histories
complete ophthalmic examination and intravenous of previous scintillating scotomas associated with the
fluorescein angiography when patient cooperation and headaches, while five usually experienced nausea, and
clarity of ocular media permitted. Complete blood cell four, photophobia. One patient (patient 17, Table 1)
count, erythrocyte sedimentation rate, routine blood occasionally experienced ipsilateral hemihypesthesia
chemistries, cholesterol and triglyceride levels, and hemiparesis with his headaches. The headaches
antinuclear-antibody level, coagulation screening were usually present for many years, but in only one
studies, hemoglobin electrophoresis, serology, chest patient (patient 7, Table 1) did the headache occur si-
radiographs, skull radiographs, and other miscellane- multaneously with the arterial obstruction. In this pa-
ous blood and radiologic studies were obtained, when tient, the headache was ipsilateral to the obstruction.
indicated according to the history and physical exami-
nation. COAGULATION DISORDERS
The most recent nine patients in the series also
Coagulation disorders were comparable to a history
underwent specialized coagulation studies. Among
of migraine in frequency of association with RAO in
these, in addition to one-stage prothrombin time (PT),
young patients. However, while most patients re-
activated partial thromboplastin time (PTT) and
ceived routine coagulation screening tests, such as
platelet counts, were assays for fibrin degradation
prothrombin time, partial thromboplastin time, and
products, fibrinogen, and factors V and VIII. Using
total platelet count, only 9 of 27 received specialized
methods previously described, 3 platelet aggregation
platelet activity and coagulation factor studies, as
and serotonin release were done with ADP, epineph-
these were unavailable when earlier patients in the
rine, and collagen. Platelet coagulant activity assays in-
group were studied. Eight of these nine patients had at
cluded: (1) collagen-induced coagulant activity, the
least one abnormal result (Tables 4A,B) when com-
capacity of platelets stimulated by collagen to partake
pared with a large control group previously studied. 4
in intrinsic coagulation initiation in the apparent ab-
Only three patients were found to have an abnor-
sence of factor VIII; (2) platelet factor III activity, in
mality in routine coagulation screening tests (patients
which phospholipoproteins of platelet membranes, in
6, 17, and 22, Table 4A), and these patients were
the presence of calcium, promote interactions of fac-
among the group that underwent platelet coagulant ac-
tors V and Xa to activate prothrombin; (3) intrinsic
tivity and coagulation factor assays. Patient 6 also had
factor-Xa forming activity, in which membrane com-
a positive family history in that his mother died at age
ponents of platelets, in the presence of calcium, induce
24 following a pulmonary embolus. Extensive coagu-
interactions of factors VIII, IX, and Xla to activate
lation work-up of a female sibling was normal.
factor X; and (4) contact product forming activity, the
In eight of nine patients who had a detectable in-
ability of platelets to respond to ADP and partake in
crease in coagulation factor activity and/or platelet
factor XII activation. These assay methods have also
function activity, one demonstrated increased factor V
been previously described. 3 No patients had taken
activity and five had an increased factor VIII activity
medication within two weeks prior to the studies that
(Table 4A). One demonstrated a slight decrease in the
might affect platelet function and all coagulation and
level of fibrinogen and fibrin split products were nor-
platelet assays were matched against controls.
mal in all nine subjects. In three of nine patients, in-
creased collagen-induced coagulant activity was pres-
ent (Table 4B), while in three patients, there was in-
RESULTS
creased platelet factor III activity and one patient had
an increased intrinsic factor- Xa forming activity. In
There were 27 patients on file who developed RAO
the patient (patient 23) with the most abnormal coagu-
prior to age 30. Patient age ranged from 9 to 29 years,
lation factor and platelet activities, there was a severe
with a mean age of21.4 years. There were 14 males, 13
concomitant venous stasis retinopathy component in
females, 20 whites and 7 blacks. Nine patients pre-
addition to a macular branch arterial obstruction
sented with CRAO, 13 had BRAO, and cilioretinal arte- (Fig 1).
rial obstruction was present in five patients. The right
Platelets numbers were very slightly elevated in two
eye alone was involved in 14 cases, the left eye alone
patients and platelet aggregation and serotonin release
in 12 cases, and one patient, a drug abuser, had bilat-
were normal in all nine patients with collagen and
eral involvement with numerous branch arterial occlu-
ADP, and slightly increased with epinephrine in only
sions in each eye.
one patient.
Abnormalities associated with the patients in the
series are listed in Tables 1 and 2.
INTRAOCULAR DISORDERS
MIGRAINE
Six patients presented with localized intraocular
The most commonly associated finding was that of a disorders. Three patients (patients 1, 14, and 19) had
history of migrainous headaches (8 of 27 patients, elevated intraocular pressures greater than 35 mm Hg,

19
 OPHTHALMOLOGY • JANUARY 1981 • VOLUME 88 • NUMBER 1

Table 1. Systemic and Ocular Associations of Patients with Retinal Arterial Obstruction*

Abnormalities
Type of
Patient/ Age/Sex Race Eye(s) Obstruction Systemic Ocular
9 M Negro OS CRAO Hgb AS Trauma, increased
intraocular pressure
2 13 M Negro OD CRAO None Trauma
3 14 F Negro OS CRAO Hgb SS None
4 14 M Negro OS CRAO Trauma
5 15 M Caucasian OS BAO Trauma
6 16 M Caucasian OD BAO Increased factor VIII None
activity, decreased PT
and PTT
7 18 F Caucasian OD CAO Increased factor VIII None
activity, migraine,
pregnancy
8 19 M Caucasian OS CRAO Trauma
9 19 F Caucasian OD CRAO ? None
10 19 M Negro OD BAO Increased factor VIII Prepapillary arterial loop
activity
11 20 M Caucasian OS CRAO Increased platelet None
coagulant activity
12 22 F Caucasian OS BAO Migraine, BCP Drusen of the optic
nerve head
13 22 M Caucasian OS CRAO ? None
14 23 F Caucasian OD CAO Migraine, BCP Increase intraocular
pressure
15 24 M Caucasian OD BAO ? None
16 24 F Negro OD CRAO Hgb AS, SLE, None
rheumatic heart disease
17 24 M Caucasian OD CAO Migraine, decreased None
PTT, increased platelet
coagulant activity
18 25 F Caucasian OD BAO Migraine, pregnancy None
19 25 F Caucasian OS CAO Increased platelet Increased intraocular
coagulant activity pressure
20 25 M Caucasian OD BAO Ventricular septal None
defect
21 26 F Caucasian OD BAO BCP Possible drusen of the
optic nerve head
22 26 F Caucasian OS BAO Migraine, increased None
factor VIII activity
23 26 F Caucasian OS BAO Increased factor V None
and VIII activities,
increased platelet
coagulant activity,
decreased fibrinogen
24 27 M Negro ou BAO Drug abuse None
25 27 M Caucasian OD CAO ? None
26 27 F Caucasian OS BAO Migraine None
27 29 F Caucasian OD BAO Migraine None
* Blanks indicate no evaluation; ? indicates no association found; BCP-birth control pills; SLE-systemic lupus erythematosus.

although none had this finding alone as a predisposing
factor to the obstruction. Sickle cell trait and a history
of ocular trauma were present in patient 1, while in
patient 14 there was a history of migraine and oral
contraceptive use, and in patient 19, platelet coagulant
hyperactivity. Among the patients with normal in-
traocular pressures, two had subtle buried optic nerve
head drusen (patients 12 and 21) and both were using
oral contraceptives, while one also had a history of
migraine. One patient had a prepapillary arterial loop
and has been described elsewhere. 5
Intra-arterial emboli were observed in only two pa-
tients (patients 5 and 24). Patient 5 had a presumed
fibrin embolus after a retrobulbar hemorrhage and pa-
tient 24 showed bilateral multiple small white emboli
secondary to intravenous drug injections.

20
 BROWN, et al • RETINAL ARTERIAL OBSTRUCTION

Table 2. Summary of Systemic and Ocular Associations of tient also had sickle cell trait as a complicating factor.
Patients with Retinal Arterial Obstruction The fourth patient (patient 8) had a CRAO secondary
to severance of the central retinal artery by a metallic
Association Number of Patients
foreign body, while the fifth (patient 2) sustained a
Migraine 8 CRAO following blunt trauma, although the patho-
Coagulation abnormalities genesis of the CRAO is unclear.
Platelet
Factor 8 HEMOGLOBINOPA THY
Both
Intraocular abnormalities One patient (patient 3) with sickle cell disease (Hgb
Increased intraocular pressure SS), but without other contributing factors, experi-
(>35 mm Hg) 3 enced a CRAO obstruction during a hemolytic crisis.
Optic nerve head drusen,
6
Two patients (patients 1 and 16) presented with hemo-
buried 2 globin AS, however both had additional complicating
Prepapillary arterial loop 1 factors. Patient 1 had ocular trauma described above
Trauma and patient 16 also had rheumatic valvular heart dis-
Non-surgical 5 ease and was discovered on evaluation after the CRAO
Surgical
Hemoglobinopathy
to have systemic lupus erythematosus (SLE).
Hgb SS 3
Hgb AS ORAL CONTRACEPTIVES AND PREGNANCY
Oral contraceptives 3 Three women were using oral contraceptives at the
Pregnancy 2
Cardiac disorders
time of RAO. Of these, two (patients 12 and 21) also
Rheumatic heart disease had subtle buried optic nerve head drusen, and two
2 (patients 12 and 14) had a history of migraine. One
Ventricular septal defect
Miscellaneous (patient 14) presented with an intraocular pressure of
Systemic lupus erythematosus 40 mm Hg in the affected eye secondary to suspected
2
Talc emboli (drug abuse) primary open angle glaucoma. None of those on oral
No associated abnormalities found 4 contraceptives were able to return for coagulation
Evidence of atheromatous disease 0 factor or platelet studies.
Two women were pregnant at the time of obstruc-
tion, both in the first trimester. Each (patients 7 and
TRAUMA 18) had a history of migraine as a complicating factor,
while one of the two who was tested for coagulation
A history of ocular trauma was present in five pa- factor and platelet disorders also had an increased
tients. All in this group were males under 20 years of factor VIII activity level.
age. One patient (patient 5) experienced sudden loss of
vision after retrobulbar hemorrhage secondary to a CARDIAC DISORDERS
surgical repair of an ipsilateral blowout fracture of the
orbit. A second patient (patient 4), who sustained loss One patient (patient 16) had sickle cell trait and a
of both retinal and posterior ciliary circulations to the history of rheumatic fever at age 16. Her RAO oc-
eye from a knife wound, has been reported previ- curred at age 24, at which time the diagnosis of sys-
ously. 6 One child (patient 1) developed a hyphema temic lupus erythematosus (SLE) was made. Nine
with an elevated intraocular pressure of 36 mm Hg years later, the diagnosis of mitral and aortic valvulitis
after blunt trauma to the globe. While most patients due to rheumatic heart disease was confirmed at
with ocular contusion injury and increased intraocular autopsy. The second patient (patient 20) had a ven-
pressure do not develop vascular obstruction, this pa- tricular septal defect, but no evidence of endocarditis.

Table 3. History of Symptoms in Migrainous Patients with Retinal Arterial Obstruction

Unilateral Previous Scintillating Nausea and/or Headaches at Time of
Patient• Headaches Scotoma Vomiting Photophobia Arterial Obstruction

7 + + + + +
12 + + + +
14 + +
17 + + + +
18 + + +
22 + + +
26 + +
27 + + +
• See Table 1.

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 OPHTHALMOLOGY • JANUARY 1981 • VOLUME 88 • NUMBER 1

Table 4A. Coagulation Studies in Young Patients with Retinal Arterial Obstruction
Factor Assay Fibrin
Patient PT PTT v VIII Fibrinogen Split Products
Normal (10.2-12.9 sec) (25.1-35.9 sec) (50-150%) (50-150%) (150-300 mg/dl) (neg)
6 9.7 17.3 nl 205% nl neg
7 nl nl nl 220% nl neg
10 nl nl nl 215% nl neg
11 nl nl nl nl nl neg
13 nl nl nl nl nl neg
17 nl 21.9 nl nl nl neg
19 nl nl nl nl nl neg
22 9.4 nl nl 185% nl neg
23 nl nl 350% 175% 140 neg

Table 48. Platelet Coagulant Activities

Collagen-Induced
Coagulant Factor 3 Activity Intrinsic Factor-Xa Contact-Product
Patient Activity Collagen Kaolin Forming Activity Forming Activity
Normal (62-154) (40-160) (39-167) (30-170) (42-158)
6 nl nl nl nl nl
7 nl nl nl nl nl
10 nl nl nl nl nl
11 430 nl 225 nl nl
13 nl nl nl nl nl
17 nl 190 190 nl nl
19 265 nl nl 185 nl
22 nl nl nl nl nl
23 220 235 nl nl nl

While only three patients underwent echocardiog-
raphy, no patients were found to have symptoms or
signs of the midsystolic click syndrome or an atrial
myxoma.
MISCELLANEOUS
Rheumatologic work-up was posttlve in only one
patient (patient 16), who also had rheumatic heart dis-
ease and sickle cell trait. Previously undiagnosed sys-
temic lupus erythematosus was discovered with a
positive antinuclear antibody test to a dilution of
1: 1280. The only patient with bilateral arterial
obstructions was an intravenous drug abuser with
numerous simultaneous branch arteriolar obstructions
in each eye. In four patients, no evidence of associated
systemic or ocular abnormalities could be found. At-
tempts to schedule three of these patients for more
thorough coagulation factor and platelet studies were
unsuccessful.
In 17 of the 27 patients in whom 14-hour fasting
cholesterol and triglyceride levels were obtained, all
were found to be within normal limits. Histories and
physical examinations disclosed no evidence of cardio-
vascular, cerebrovascular, or peripheral vascular
atherosclerotic disease and no patients were found to
have systemic arterial hypertension, syphilis, or dia-
betes mellitus.
Carotid angiography was performed in only one pa-
tient (patient 27), a 29-year-old female. The study was
normal in this oldest patient in the series.
MORTALITY
Follow-up of this group of patients ranged from one
month to 98 months, with an average of 26.2 months.
One patient (patient 16) in the series died 98 months
after obstruction and autopsy revealed severe lupus
nephritis and acute staphylococcal endocarditis
superimposed upon old mitral and aortic valvulitis due
to rheumatic heart disease.
VISUAL MORBIDITY
Visual prognosis for the involved eyes is shown in
Table 5. Relatively minor improvement occurred in the
CRAO group except in one patient in whom a cilioreti-
nal artery spared the foveola, enabling the visual
acuity to improve from 6/60 to 6/5. In both the branch
and cilioretinal arterial groups, visual acuities often
improved several lines, usually within the first two
weeks after obstruction.
Goldmann visual fields disclosed scotomas, often
with sloping borders, corresponding to the extent of
retinal ischemia in the distribution of the obstructed
retinal artery.

22
 BROWN, et al • RETINAL ARTERIAL OBSTRUCTION

Table 5. Visual Prognosis in Involved Eyes of Children and Young Adults with Retinal Arterial Obstruction
and Adequate Visual Follow-up
Type of Obstruction Initial Acuity: No. of Patients Follow-up Acuity: No. of Patients
Central 6/5 1
(n = 9) 6/60 1 CF 2
HM 3 HM 2
LP 3 LP 1
NLP 2 NLP 3
Branch 6/6 4 6/6 6
(n = 10) 6/9 2 6/9 1
6/120 1 6/12 1
CF 1 CF 2
HM 2
Cilioretinal 6/6 6/6 3
(n = 4) 6/12 6/9
6/15
1/21
NLP = no light perception; LP = light perception; HM = hand motion; CF = counting fingers; n = number of patients

our institution over the same time period, it can be

Fig 1. Macular branch arterial obstruction in a 26-year-old woman.
The area of nerve fiber layer edema is outlined by the black arrows.
Superimposed is a severe venous stasis retinopathy, characterized
by dilated veins and retinal hemorrhages (white arrows). Work-up
revealed abnormal coagulation factor and platelet activities.
DISCUSSION
Retinal arterial obstructions are rare in people under
the age of 30 years. Of 338 patients with RAO seen at
Wills Eye Hospital from July 1967 to June 1979, the
average age was 58.5 years. Twenty-seven (8%) were
under the age of 30. When the number of patients in
this series is compared with the total number seen at
estimated that less than 1 in 50,000 outpatients pre-
senting to the ophthalmologist will be a person under 30
years with retinal arterial obstruction. And even this
figure may be falsely high since some patients in the
series were referred from outlying areas.
Without clinicopathologic correlation at the time of
RAO, it is most difficult to establish the exact under-
lying cause. However, associated systemic and ocular
abnormalities should be fully investigated to facilitate
appropriate management. From this study, it is clear
that cause may be multifactorial and multiple organ
systems may be involved. Therefore, the discovery of
one abnormality should not preclude a thorough
search for additional contributing factors.
A history of migraine was present in almost one
third of patients in this series. When contrasted with a
10% incidence in the general population/ this figure is
substantially higher. Migraine has been described in
the literature in association with RAO phenomena in
young people 8 - 10 and presumably has been thought to
exert its deleterious effect by poorly understood vaso-
spastic mechanisms. From this series and the liter-
ature/-10 it appears that similar concurrent cerebral
arterial obstructions must be quite rare, if they do
occur.
To our knowledge, coagulation abnormalities have
not been previously reported specifically in associa-
tion with RAO in young people. Increased platelet
coagulant activity has, however, been related to RAO
in a general age population in those patients with ab-
sence of other predisposing factors to obstruction such
as hypertension and type IV hyperlipoproteinemia. 4
While increased levels of factor V activity, factor VIII
activity, and platelet coagulant activity have been
demonstrated in our series of patients, these abnor-
malities are very diverse and the clinical application of
these findings at present is uncertain and merits further

23
 OPHTHALMOLOGY • JANUARY 1981 • VOLUME 88 • NUMBER 1
investigation. It is probable that had all patients in the
series undergone factor and platelet coagulant assays,
the incidence of coagulation abnormalities within the
total group would have been higher.
Certain ocular disorders appear to play a role in the
cause of RAO in young patients. A significantly ele-
vated intraocular pressure (> 35 mm Hg) was present
in three patients. Although other possible etiologic
factors were found in each patient, increased in-
traocular pressure may be an important predisposing
factor. Optic nerve drusen have been described in as-
sociation with acute retinal arterial vascular obstruc-
tion, 11 as was the case with two of our patients. Since
drusen alone as complicating factors were not ob-
served, it is probably necessary to have additional ab-
normalities to cause obstruction. Whether the associ-
ation of RAO with optic nerve drusen is merely coinci-
dental is uncertain. Preretinal arterial loops have been
associated with RAO in young people. 5 These
obstructions usually involve the inferior arteries, and
kinking of the loop which supplies the affected vessel
has been proposed as an etiologic mechanism.
Trauma appears to be a prominent etiologic factor,
particularly in young males. Severance of the central
retinal artery and compression from retrobulbar
hematoma following blowout fracture repair 12 are
identifiable etiologic agents. It is possible that en-
dothelial cell damage with exposure of collagen, a po-
tent stimulus for platelet aggregation, may also be a
factor in these cases. It should be emphasized that
increased intraocular pressure alone following trauma
does not appear to cause arterial obstruction in young
people. In these cases, other associated abnormalities
should be considered.
Sickle cell disease is well known as a cause of vasa-
occlusive phenomena throughout the body, and hemo-
globin electrophoresis should be obtained in all young
black patients with RAO. From the two patients in this
series and other case reports, 13 - 15 it seems probable
that sickle trait alone usually does not cause RAO ex-
cept in combination with other pathologic processes.
Oral contraceptives have been reported to be as-
sociated with RAO in young people, 16 - 17 presumably
the estrogen content being implicated as a risk factor in
thrombo-embolic diseaseY However, in each of the
three patients in this series using oral contraceptive
agents, other possible contributory factors were also
present. When the two pregnant patients are included,
it is seen that four of these five patients also had a
strong history of migraine headaches, while two had
subtle optic nerve head drusen. One had an increased
factor VIII activity level and one, an increased in-
traocular pressure. The clinical significance of coagu-
lation factor abnormalities in this group of patients is
uncertain.
Cardiac disorders have been well recognized as
etiologic factors of retinal arterial obstruction in the
younger age group. Atrial myxoma is a known cause, 18
but is most often associated with other neurologic
24
signs. We found no symptoms or signs of atrial myx-
oma in our group, but only three patients underwent
echo cardiography. Mitral leaflet prolapse has also
been implicated as an etiologic factor, 19 '20 but none of
the patients in this series was found to have a midsys-
tolic click. Calcific, vegetative platelet and fibrin em-
boli have been reported as causes of RA0, 21 although
no emboli were seen in the fundi of our two patients
with cardiac disease. In these two patients with car-
diac abnormalities, it is uncertain whether these find-
ings were of primary etiologic importance. Even in the
presence of heart disease, other possible contributory
factors should be considered, as is evidenced by our
patient with rheumatic heart disease, systemic lupus
erythematosus, and sickle trait.
Connective tissue disorders should be included in
the differential diagnosis of diseases predisposing to
RAO in the young. While periarteritis nodosa and
systemic lupus erythematosus are both known causa-
tive diseases, 22 the etiologic incidence of these entities
is probably low, as evidenced in this series.
Talc and cornstarch emboli from filler mixed with
crushed methylphenidate tablets have been seen in the
retinal arterioles of drug abusers. 23 These emboli are
bilateral and usually glistening, and most often lodge in
multiple small arterioles around the macula. In these
cases, continued intravenous injection may produce
extensive pulmonary capillary obstruction. This leads
to arteriovenous shunt formation, possibly facilitating
passage of emboli into the arterial circulation.
Homocystinuria, 24 Fabry's disease, 25 loiasis, 26 ven-
triculography ,27 Sydenham's chorea28 and carotid an-
giography29 have also been associated with RAO in the
young. Additional entities have been described and
merit a more comprehensive review. While retinal
arterial obstruction secondary to atherosclerotic ca-
rotid arterial disease has been described at a relatively
young age, 30 we are unaware of reports of atheroma-
tous disease being associated as an etiologic factor with
RAO in those under the age of 30 years. Therefore, we
do not routinely recommend carotid angiography on
these patients. However, in the presence of a carotid
bruit, unexplained retinal arterial emboli, hyperlipo-
proteinemias, a strong family history of atherosclerosis,
positive non-invasive studies, or other factors sugges-
tive of carotid artery disease, this procedure may be
indicated.
Surprisingly, visual prognosis in these young people
appears to be similar to that in an older age group.1. 31
Those with sustained CRAO, resulting in extensive
retinal infarction, most often have a visual acuity
ranging from counting fingers to no light perception.
Those with BRAO or CAO often improve to 6/9 or
better as hypoxic retina recovers.
Of those four patients in the series without as-
sociated systemic abnormalities, only one received
comprehensive multisystem physical, laboratory, and
radiographic evaluations. However, from the findings
presented, it seems that most patients under the age of
 BROWN, et al • RETINAL ARTERIAL OBSTRUCTION
30 years with RAO have some form of presently de-
tectable systemic or ocular disorder which may be of
etiologic importance.
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