COMITANT CONVERGENT STRABISMUS W I T H ACUTE ONSET*

HERMANN M. BURIAN, M.D.

Iowa City, Iozva
AND

JAMES E. MILLER, M.D.

Saint Louis, Missouri

Comitant convergent strabismus arises not young girl whose one eye had been bandaged
only in infancy and early childhood. It may for 10 days for a corneal foreign body. It
make its first appearance in older children, is noteworthy that this girl had not only a
in adults, and even in old people. It occurs vision of 1.0 in each eye, but was also em-
then more or less acutely, accompanied by metropic. It is easier to understand the oc­
diplopia. Strabismus of late onset has inter­ currence of an esotropia if the patient who
esting theoretical and practical implications. underwent the patching had an uncorrected
W e shall discuss in this paper the various hypermetropia, as did one of the patients cited
forms of acute comitant esotropia which by Swan, a nine-year-old boy. Both Pau­
have been described. fique's and Swan's patients required surgery
to obtain a full functional recovery.
I . A C U T E ONSET OF C O M I T A N T CONVERGENT
Surgery was not required in a patient seen
STRABISMUS F O L L O W I N G A R T I F I C I A L
and treated by one of us ( H . M. B.) and
I N T E R R U P T I O N OF F U S I O N
reported on by Bielschowsky. 3 This was a
The best known type of acute strabismus truly remarkable observation in that a large
is the one in which a deviation occurs follow­ manifest deviation with diplopia established
ing patching of one eye or loss of vision in itself following bandaging of one eye for
one eye from disease or injury. I n adults it two days in a 14-year-old boy with undected
usually takes the form of exotropia. In chil­ and uncorrected hypermetropia of + 5 . 0 D . ,
dren and young adults esotropia predomi­ O.U. T h e deviation disappeared within a few
nates. Many ophthalmologists have had, for days of intermittent wearing of a correction
example, the experience that a child, whose incorporating prisms. It remained latent over
eyes were originally straight, developed a some years of observation even though the
manifest deviation with diplopia during ther­ patient refused to wear his glasses once he
apeutic patching for amblyopia ex anopsia. had regained binocular vision. It was of in­
Swan 1 has reported on two such cases; in a terest in more than one respect that the pa­
third, a 27-year-old man, there was no am­ tient's older brother had had surgery for
blyopia. T h e occlusion had been prescribed esotropia two years prior to the incident re­
for diagnostic purposes by a nonmedical re- ferred to.
fractionist.
Occlusion of one eye represents a periph­
In the presence of an amblyopia such an eral obstacle to binocular vision. The seat
occurrence is not entirely unexpected, but it of the disturbance may also be of central
may also take place in cases in which it was nervous orgin. T h u s one of us 4 observed a
not foreseen, as in the cases reported by Pau- 17-year-old girl who, immediately following
fique2 and Swan. 1 Paufique's patient was a a slight concussion of the brain, developed a
manifest exotropia with spontaneous diplo­
* From the Department of Ophthalmology, Col­
lege of Medicine, State University of Iowa, and pia but without any signs of paralysis of the
from the Department of Ophthalmology, Wash­ extraocular muscles. This exotropia reduced
ington University School of Medicine. This work
was done during the tenure by one of us (J. E. M.) itself to an exophoria as the patient's general
of a Heed Fellowship in ocular motility. condition improved.
55
56 HERMANN M. BURIAN AND JAMES E. MILLER

DISCUSSION sion of one eye does not elicit a manifest

Particularly the last two examples show deviation. These are the ones who are said
very plainly the influence which a well-de­ to have orthophoria. If such a person loses
veloped sensory machinery has on the motor the vision in one eye to a great extent, he
system of the eyes. Sensorimotor fusion and may nevertheless develop an exotropia, in­
binocular vision result when there is a bal­ creasing as time goes on. Here the exotropia
ance between those forces which tend to keep must be caused not by pre-existing factors
the eyes properly aligned and those which but by new factors which have become oper­
tend to disrupt the alignment of the eyes. ative.
The forces keeping the eyes properly aligned We do not know what these new factors
are very strong in some individuals, so much are. It is unlikely that "diplopia-phobia" is
so that they can overcome remarkably great the responsible mechanism in all the cases,
obstacles. Even in the presence of an am- since in many of them the remaining visual
blyopia ex anopsia the peripheral fusional acuity in the diseased or injured eye is too
mechanism can prevent a manifest deviation. low for a disturbing diplopia to be present.
People who have such a strong sensory One could theorize that one factor might be
fusion mechanism have good binocular co­ a divergence impulse not held in check by
operation under all ordinary conditions of other optomotor impulses. This, together
seeing, but will develop a manifest deviation with the lateroversion impulse which would
under extremely abnormal conditions, for then be required to keep the normal eye fix­
example, if there occurs an artificial inter­ ating, and which would be additive to the
ruption of binocular vision of some duration. divergence impulse in the eye with poor
In other people the forces keeping the eyes acuity, would drive that eye even farther
aligned are barely able to overcome those outward. Over the months or years an actual
forces which tend to drive the eyes apart. contracture of the lateral rectus muscle of
Such individuals will develop a manifest de­ the deviated eye may add itself and help to
viation at the slightest provocation, even if keep the eye in an exotropic position. It is
only for a short period of time. The extent to be hoped that electromyography may be
to which the "fusion faculty," so much ma­ of assistance in deciding whether or not such
ligned by some in the past 20 years, is or is a sequence of events actually does occur.
not capable of overcoming the misalignment
of the eyes, is surely an important factor in I I . COMITANT CONVERGENT STRABISMUS
the etiology of manifest strabismus. OF THE "FRANCESCHETTI TYPE"

One cannot, however, assume that the eti­ The considerations regarding the genesis
ology is the same in all cases in which a man­ of the strabismus following the artificial in­
ifest deviation results from temporary or terruption of fusion may also be applicable to
permanent suspension of fusion. While in the form of acute convergent strabismus
many cases this suspension causes a pre­ which has been designated by Moutinho5 as
existing condition, a latent deviation, to man­ the "Franceschetti type."
ifest itself, in others this is certainly not the The strabismus in these patients is charac­
case. The number of adults who develop a terized by an acute onset with diplopia, a
gross exotropia following partial or total loss relatively large angle of squint, an absence of
of vision in one eye is too great to permit signs of paralysis, and good potential binocu­
one to conclude that they all had a potential lar co-operation. Occasionally the diplopia
exotropia of sizable degree. and the strabismus are at first intermittent
There are a number of persons in whom for a longer or shorter period of time before
the obstacles to binocular vision happen to be they become constant. The refractive error
so insignificant that even prolonged occlu­ is as a rule in the low hypermetropic range
 COMITANT CONVERGENT STRABISMUS 57

and the accommodative element is minimal. In 1956, two further publications appeared
In most of the patients no immediate cause on the subject of acute esotropia. In one of
for the onset of the strabismus can be ascer­ them Shukla13 described briefly the case of
tained, although in some of them a debilitat­ an 18-year-old girl with acute esotropia and
ing illness or a physical or psychic shock may diplopia which responded well to orthoptic
precede the onset. treatment. The other publication, by Norbis
A few cases of esotropia with acute onset and Malbran, 14 was especially interesting,
have been recorded in former years, but it is since these authors observed four siblings in
not always clear from the reports whether all of whom an esotropia of rather large de­
they belong in this category or not. For ex­ gree arose with diplopia at a relatively late
ample, in Bailliart's case6 he was able to cor­ age (from six to nine years). All were
rect the strabismus by a novocaine injection operated upon, but the functional result was
into the right medial rectus muscle of an not very good, although all had normal ret­
eight-year-old girl who had suddenly ac­ inal correspondence. Only one of the four
quired an intermittent esotropia after an developed small fusional amplitudes. It is
emotional upset. The effectiveness of the possible that the poor functional result was
novocaine injection would speak for a paretic due to the fact that three of the four chil­
rather than a comitant esotropia. dren came to treatment at a late date: two
In 1945 Burian 7 published four cases of six years after the onset and one four years
esotropia with acute onset with diplopia. after the onset. Also, there was a consider­
The patients were from 11 to 72 years. In able residual deviation after surgery in three
three of them the onset was intermittent. No of them.
signs of paralysis of an extraocular muscle We shall now report on some cases of
were present in any of the patients; they all acute convergent strabismus which we have
had low hypermetropic refractive errors and seen more recently.
a visual acuity of 20/20 or better in each eye.
CASE REPORTS
The angle of squint ranged from 20 to 60
degrees. All patients showed good binocular CASE 1
co-operation and surgery resulted in an ex­ A white boy was first seen on July 31, 1951,
when he was six and one-half years of age. There
cellent functional result. was no family history of strabismus. Turning in
The importance of this type of esotropia of the right eye of the boy occurred rather sud­
denly in December, 1950, shortly after he had en­
has been particularly stressed by Frances- tered school. At the same time the child complained
chetti and his collaborators8"10 who have re­ of diplopia which gradually disappeared. For a
ported on a number of patients, all of whom time prior to the turning in of the right eye, the
child had had some pains in his legs but had had
had the characteristics described. The case no fever or any other manifestations of illness.
contributed by Meunier11 was rather inter­ At the first visit it was found that the patient
esting in that it concerned an emmetropic had an uncorrected visual acuity of: O.D., 6/21—1;
girl, four years of age, who had a vision of O.S., 6/12—2 and that there was, without glasses,
an esotropia of 35 arc degrees for distance and
10/10 in each eye. In contrast to this, Mou- near, with preference for fixation with the left eye.
tinho's patient,5 a 16-year-old girl, had a stra­ The retinal correspondence was normal in all tests.
bismus which was definitely accommodative There were no signs of paralysis of an extraocular
muscle. Cycloplegic refraction revealed: O.D.,
in nature. Accordingly, the deviation was re­ +2.0D. sph. C +2.0D. cyl. ax. 90° ; O.D., +2.25D.
lieved by nonsurgical means. Schlossman12 sph. C +1.0D. cyl. ax. 85°. These glasses were
also observed three patients who acquired an prescribed and occlusion of the left eye was or­
dered.
acute strabismus with diplopia. Their ages With this correction the angle of squint was re­
ranged from 50 to 57 years, but only one of duced ; fusional amplitudes were noted on the major
them had an esotropia, the other two were amblyoscope and a slight amblyopia of the right
eye (6/12) was corrected by occlusion. On Decem­
exotropic. ber 6, 1951, the angle of squint with correction was
58 H E R M A N N M. B U R I A N A N D J A M E S E. M I L L E R

20A of esotropia with 6 4 R H T for distance and an attempt to minimize the accommodative factor
30 4 of esotropia with 3 4 R H T for near. On March and to stabilize the deviation.
12, 1952, a five-mm. recession of the right medial This was achieved, and on February 2, 1957, the
rectus muscle was performed which reduced the measurements were as follows: With glasses (O.D.,
angle of squint for distance to 8A and to 104 for +3.0D. sph.; O.S., +3.75D. sph.; D F P discon­
near with correction. In the ensuing years the boy tinued on November 23, 1956) 30A for distance and
continued to develop good amplitudes, but some 40 4 for near; without glasses 60 4 for distance, 80 4
exotropia appeared. The spherical correction was for near. The child now preferred the left eye for
reduced in power and energetic orthoptic treatment fixation and there was considerable suppression of
was instituted. the right eye in the Worth four-dot test both for
On February 24, 1956, he was again wearing a distance and near vision. The diplopia had virtually
full correction for his refractive error (O.D., disappeared by this time, but the retinal correspond­
+2.0D. sph. C +2.0D. cyl. ax. 90°; O.S., +2.0D. ence had remained normal. No fusional movements
sph. G +2.0D. cyl. ax. 87.5°). His visual acuity was could be elicited on the troposcope.
6/6 in each eye. With refractive correction there was The adduction was excessive in both eyes and
no shift for distance; for near there were 3 4 eso- it was decided to perform a recession of both
phoria. H e had good fusional amplitudes and 100- medial rectus muscles. This was done on February
percent stereopsis (Keystone DB 6 card), in spite of 24, 1957. When examined again on April 2, 1957,
the fact that there was a slight incomitance due, no it was found that the child had a vision of 6/6 in
doubt, to the asymmetric operation (primary posi­ each eye with her correction; with glasses there
tion 3 4 E, levoversion 104 X, dextroversion 0 ) . was no shift in the prism and cover test either for
The excellent result has been maintained. When distance or near. The patient still suppressed the
he was last seen on May 27, 1957, the amplitude right eye in the Worth four-dot test for distance,
of fusion was even larger than before and the but fused in near fixation. On the troposcope the
condition otherwise as found on previous visits. subjective and objective angle was at 124 (proximal
For distance there was no shift with correction, for convergence), but there were fusional amplitudes
near 4 4 of esophoria. However, without correction of 144 of convergence and 5 4 of divergence. Dur­
the boy had an esotropia of 124 for distance and ing April, 1957, the child was given a series of
15 4 for near. orthoptic treatments, followed by home exercises
to reinforce her binocular co-operation.
CASE 2 When last seen on July 3, 1957, the child showed
A six and one-half-year-old white girl, first seen no shift for distance or near fixation with the
on March 26, 1956, complained of diplopia of two glasses. Without glasses there was an esophoria of
weeks' duration. The mother had noted at the same 6 4 for distance and 124 for near; there was fusion
time a turning of the left eye. There was no his­ in distance and near vision on the Worth four-dot
tory of ocular difficulties prior to this episode. How­ test; the fusional amplitudes, measured with rotary
ever, she had had frequent colds and tonsillitis prisms, were 124 of convergence and 4 4 of diver­
throughout the winter, with some hearing loss, and gence; N.P.C., measured objectively, nine cm.;
an acute suppurative otitis media for which a measured subjectively with a red filter,15 12 cm.;
myringotomy was done. 100-percent stereopsis with DB„ Keystone card.
Except for a left esotropia, both eyes were nor­
mal in every respect. The uncorrected vision was CASE 3
6/6, R.E., and 6/9—3, L.E. Without correction the This patient, a 14-year-old white girl, was seen
angle of squint was about 25 4 for distance and 45 4 at Washington University. On her first visit she
for near. An orthoptic check revealed normal reti­ complained of left esotropia and diplopia of six
nal correspondence in all tests, some suppression, months' duration. The diplopia was intermittent for
O.S., for distance and near in the Worth four-dot one week after which it became constant. Examina­
test, and small fusional amplitudes on the tropo- tion of the eyes revealed no abonormalities except
scope. for the strabismus. The vision was 20/20 in each
There was no evidence of a paralysis of the sixth eye with —5.5D. sph., O.U. Without correction
nerve on either the right side or the left. Based on there was a left esotropia of 50 4 for distance,
a noncycloplegic refraction, the child was given a with correction 70 4 . The rotations were normal
correction of: O.D., +1.75D. sph. C —0.37 D. cyl. and there were no signs of a paralysis of an
ax. 42°; O.S., +2.75D. sph. extraocular muscle. The orthoptic evaluation
She was seen again on August 3, 1956, at which showed normal correspondence and fusional am­
time she reported that she still had diplopia off and plitudes with some alternating suppression. No
on. With her correction the angle of squint was systemic or neurologic abnormalities were found
in a physical examination which included a spinal
164 for distance and 25 4 for near. Repeated re-
lap.
examinations in October, November, and Decem­
ber, 1956, revealed an increase and instability of On October 23, 1956, a five-mm. recession of
the angle of squint; D F P drops, and later a the left medial rectus muscle with a six-mm. re­
stronger correction for hypermetropia, based on section of the left lateral rectus muscle was done.
refraction in atropine cycloplegia, were ordered in Postoperatively the patient demonstrated fusion
 COMITANT CONVERGENT STRABISMUS 59

in all directions of gaze, with amplitudes of con­ Terson 1 6 ; M a l b r a n " ) , but the evidence for
vergence up to 704 and divergence to zero. A such an occurrence is incontrovertible. F u r ­
slight incomitance was present in gaze to the left
with overaction of the right medial and lag of the thermore, it is apparent from the literature
left lateral rectus muscles. and from the cases presented in this paper
When the patient was last seen on May 27, that a comitant esotropia of late and acute
1957, she had 15A of esophoria for distance and
A
10 of esophoria for near. onset may present any of the earmarks of
an esotropia beginning in infancy: it may be
CASE 4
familial, accommodative, nonaccommodative,
This patient, a nine-year-old boy, was also intermittent, constant, and occur in patients
seen at Washington University. On his first
visit on May 29, 1957, he reported diplopia of with widely varying refractive errors.
four months' duration. The patient could date the From a theoretical standpoint the cases
onset of the diplopia to a baseball game where
he was suddenly aware of two baseballs. His local with late onset of comitant esotropia gain
ophthalmologist referred the patient for neurologic particular significance, and it is of interest
and ophthalmologic consultation. to inquire into the genesis of the deviation.
There was no family history of strabismus or
other disease. The patient had developed normally T h e question arises: is the genesis essentially
and had had no significant illness. There was no different in cases of late onset from the gen­
evidence of pathologic changes in the external, esis in cases with onset in early infancy?
slitlamp, and ophthalmoscopic examination of the
eyes. The intraocular pressure was normal and T h e answer to this question depends to
the visual fields were full. The visual acuity was some extent on one's beliefs about the eti­
20/20 in each eye. ology of comitant strabismus. If one takes
Investigation of the extraocular motility showed
that the boy had a left esotropia of 3 ^ in all di­ the view that comitant strabismus is a con­
rections of gaze except in levoversion where the de­ dition which occurs in early infancy due to
viation measured 344. In the orthoptic analysis the an abnormal development of the optomotor
patient had normal correspondence and amplitudes
of fusion (15 a convergence, 5A divergence, distance reflexes based on retarded myelogenesis
and near). (Keiner 1 8 ) or due to a faulty development of
The patient was admitted for study to the Chil­ the binocular reflexes (Chavasse) then a
dren's Neurologic Section at Children's Hospital
strabismus of late onset must necessarily
for study. A re-examination of the eyes confirmed
the previous findings. Neurologic consultation in­ have a different etiology. If one believes,
dicated partial left sixth nerve paralysis on the however, that the normal sensorimotor co­
possible basis of nasal pharyngeal tumor with in- operation of the eyes is the result of a deli­
tracranial extension. However, this was not borne
out by further studies. Electromyography proved cate balance between the forces which tend
both lateral rectus muscles to be normally func­ to maintain this balance and the forces which
tioning, without change in pattern in response to tend to disrupt it,* as has been indicated in
Tensilon. Electro-encephalograms showed a pre­
dominantly slow dysrythmia with overlying fast the discussion of the strabismus following
activity, indefinite in relation to complaints. Skull artificial interruption of fusion, then it is
films and pneumoencephalograms were normal ex­
cept for evidence of sphenoidal sinusitis.
Accordingly, treatment for the sinusitis was in­ * Among the forces which work against the
stituted. Surgery for the strabismus (bimedial re­ proper alignment of the eyes are: a large "basic"
cession) was recommended, but the operation was deviation (Swan1"), weaknesses in the anlage of
postponed at the request of the parents. the sensory system (defective retinal rivalry and
readiness to suppress; low responsiveness to dis­
DISCUSSION
parate stimulation), particularly when combined
with obtacles of optical nature (anisometropia,
F r o m a practical standpoint it is important and so forth) ; abnormalities in the convergence
and divergence innervation and in the relation of
to realize that an esotropic deviation with convergence and accommodation, either intrinsic
diplopia may arise at any age without being or due to abnormal peripheral stimuli (uncorrected
due to the paralysis of a lateral rectus mus­ refractive errors, and so forth) ; mechanical and
anatomic anomalies of muscles and orbits. They
cle. It has been doubted in the past that this may also include a delayed development of the
is possible ( M o r a x , in his discussion with optomotor reflexes, as suggested by Keiner.
60 HERMANN M. BURIAN AND JAMES E. MILLER

conceivable that this balance may be upset I I I . COMITANT CONVERGENT STRABISMUS

at any age. OF THE "BlELSCHOWSKY TYPE*'
We believe this latter more general view Most authors recognize a third form of
to be the correct one. The genesis of the stra­ acute esotropia and refer to it as "Bielschow-
bismus of late onset would then be essentially sky's type."
the same as the genesis of the strabismus of In 1922, Bielschowsky20 reported on pa­
early onset and there would be little reason tients with acute comitant convergent stra­
to differentiate the cases in which the mani­ bismus of a special type which had previous­
fest deviation occurred following artificial ly been described by von Graefe21 and others
interruption of fusion from those in which and who had these characteristics: myopic
there is no such obvious cause for the ap­ refractive error of 5.0D. or less; late, more
pearance of the strabismus. or less acute onset of the deviation following
Whenever the proper alignment of the physical or psychic shock or exhaustion; un­
eyes has given way, there is nothing to pre­ crossed diplopia for distance, fusion for
vent the forces tending to increase the devia­ near; no evidence of paralysis of the extra-
tion from becoming ever more active, and ocular muscles; slight restriction of abduc­
the diplopia-phobia postulated by van der tion, but no excess of adduction; neuropathic
constitution.
Hoeve may well add its share to it.
While the genesis of the deviation is thus Bielschowsky had no doubt that myopia
likely to be the same, regardless of the age at played a role in the etiology of this form of
onset and of the immediate precipitating strabismus. He accepted von Graefe's view
cause, the course of the disease is very much that in uncorrected myopes there developed
dependent on it. If the deviation occurs while an increased tonus, a "dynamic preponder­
ance" of the medial rectus muscles. This in­
the patient is in a highly plastic stage, in in­
creased tonus was explained by the tendency
fancy or early childhood, there will be, in­
of uncorrected myopes to hold print or sew­
deed, an abnormal development of the bin­
ing excessively close to the eyes and by the
ocular reflexes and all the well-known sen-
fact that the compensatory innervation—or
sorimotor symptoms of strabismus will be relaxation of convergence—necessary to
found. At the same time, a functional cure overcome the esophoria would be the less the
will meet with considerable difficulties. On nearer the habitual fixation distance in close
the other hand, the clinical signs and the work lay to the eyes. Ordinarily, the com­
course in a strabismus of late onset are easily pensatory fusional (divergence) innervation
explained on the basis of the fact that these would keep the increased tonus of the medial
patients have enjoyed more or less normal rectus muscles in check, but under adverse
binocular vision prior to acquiring the mani­ conditions it would not be able to do so, with
fest deviation. They all exhibit, therefore, a resulting manifest esotropia.
normal retinal correspondence and frequent­ Von Graefe's description of the condition
ly also good binocular co-operation when differed from Bielschowsky's only in that the
tested with a major amblyoscope. Once the former found an excessive adduction as well
proper alignment of the eyes is restored to as a deficient adduction. Bielschowsky added
the patients by nonsurgical or surgical the important observation that all patients
means, they quickly regain full binocular co­ in that category which he had observed
operation. The diplopia accompanying the showed signs of a neuropathic constitution,
appearance of the deviation is no more sur­ and he postulated furthermore a pre-existing
prising than is the diplopia occurring in pa­ esophoria and a generally excessive conver­
tients with acute paralysis of an extraocular gence tonus.
muscle. In the early literature, an excellent casuis-
 COMITANT CONVERGENT STRABISMUS 61

tic contribution was given by Theobald22 who vision at one point at a certain distance from
summarized von Graefe's views clearly and the eyes; no diplopia at near, or crossed di­
succinctly and emphasized that the rarity of plopia if there is a secondary convergence
the occurrence was due to the fact that it insufficiency. Nevertheless, Bielschowsky and
depended upon the concatenation of circum­ the authors following him did not believe
stances seldom met with: uncorrected my­ that these cases belonged in the category of
opia, excessive close work, leading to "inter- paralysis of divergence, because they felt
ni of more than average strength, with lateral that the diagnosis of a paralysis of diver­
rectus muscles preternaturally lengthened gence, while theoretically justified, was not
and weakened from constant stretching." supported by clinical and pathologic evi­
His patient was a 39-year-old myopic dress­ dence, and because of the absence of signs
maker, who was also a constant reader, but of a severe central nervous disease in these
who used glasses only for distance vision. patients.
She had had a convergent strabismus and Closer consideration shows, however, that
annoying diplopia for some years; all signs there is no reason why cases of acute eso-
corresponded to those already described. tropia of the "Bielschowsky type" should not
Successive tenotomies, first of the right me­ be identified with a paralysis of divergence.
dial rectus, then of the left, restored binocular There is in these patients no excess of adduc­
vision.* tion, but a deficiency of abduction. The in­
In the more recent literature there are creased tonus of the convergence innervation
only few references to cases of strabismus and of the medial rectus muscles which has
of this form. Cords23 and Malbran24 mention been postulated should lead to a convergence
it, but only report on the presentations of excess. In cases of convergence excess the
von Graefe and Bielschowsky whose opin­ distance of the double images increases in­
ions they share. Weber 25 has reported two stead of decreases when the fixation object
cases in some detail and so has Frances- is approached to the eyes and at no point is
chetti.10 The cases described most recently it seen singly.1 Weber has also shown that
by Dubois-Poulsen, Benmansour, and At- while the patients possess good fusional am­
tane26 are of great interest in themselves, but plitudes, as measured by rotary prisms, these
do not seem to fit very well into this group. are displaced relative to the zero point, that
In discussing the differential diagnosis of is, the amplitudes are on the convergence side
this type of strabismus, Bielschowsky, as and do not even reach zero. The patients then
well as Cords and Weber, mentions the fact "lack relaxation," or, better, have no diver­
that it has all the earmarks of a paralysis of gence amplitudes. Weber has also achieved
divergence: uncrossed diplopia for distance; good therapeutic results with the use of base-
decrease in the distance of the double images out prisms which is the treatment of choice
as the fixation object approaches; single for paralysis of divergences, whereas Biels­
chowsky felt that prisms were not indicated
* The following remark of Theobald's is of some and advised instead bilateral advancement of
interest. "The presence of annoying diplopia in the lateral rectus muscles.
a convergent strabismus of some years' standing
struck me at once as anomalous (for I am a firm Since Bielschowsky's publication in 1922,
believer in the doctrine that amblyopia is soon
produced in a squinting eye through active sup­ much has been learned about paralysis of di­
pression of its retinal image) ; but the anomaly was vergence. It is clear from the literature and
explained, when it was discovered that in reading
the patient enjoyed binocular vision (author's t Cases of acute esotropia in myopia of moderate
italics). In this way the retina of the squinting degree with signs of convergence excess (increase
eye . . . was continually exercised. . . ." This was in distance of double images when the fixation ob­
written in 1886. It sounds much more up to date ject approached) were described by Berlin" in
than much that was published in the ensuing 40 or 1897. He pointed out that this feature clearly
SO years. separated his cases from those of von Graefe.
62 HERMANN M. BURIAN AND JAMES E. MILLER

from our own experience that paresis or The uncorrected vision was 6/6 in each eye, the
paralysis of divergence represents a definite refraction : O.D., +0.75D. sph.; O.S., +0.5D. sph.
There was no observable shift in the cover test
clinical entity. It is also clear that it may either for distance or for near, but there was
occur in people without serious central nerv­ homonymous diplopia at six m., the distance of
ous and systemic disease, although it has the double images being about 10 to 12 cm. As
the fixation object was approached to the eyes the
been recorded as a sequela of epidemic en­ double images approached each other and from
cephalitis, multiple sclerosis, pseudotumor three m. on there was no diplopia. On the tropo-
cerebri and brain tumor, intracranial hemor­ scope the fusional amplitudes measured 264 base-
out and 4 4 base-in; there was 60-percent single
rhage, head injury, lues, influenza, diphtheria, central binocular vision and 90-percent stereopsis;
and lead poisoning. the N.P.C. was at four cm. There was no evidence
It is conceivable that there may be an of paralysis on the Lancaster red-green test.
With prisms of 24 base-out in front of each eye
"organic" and a "functional" paralysis of the patient had single vision for all distances. She
divergence, just as there is an organic paral­ was given that correction and returned on March
ysis of convergence and a functional con­ 22, 1956, reporting that she had worn the glasses
with comfort and had experienced no diplopia
vergence insufficiency. O r else, the cause of when wearing them. She was asked to report again
the paralysis of divergence may remain sub- in six weeks but was not seen until November 11,
clinical in some cases and not detectable by 1956. At that time she informed us that she had had
no illness of any sort since she had last been seen
our diagnostic methods. However this may by us. She had experienced no diplopia while wear­
be, paralysis of divergence is a definite clin­ ing the glasses, but the diplopia would return after
ical entity and its occurrence is not neces­ she left the glasses off for 20 or 30 minutes. On
examination it was found that with her glasses there
sarily dependent upon severe neurologic or was no diplopia, but after she had been without
other systemic affections. T h u s there would glasses for 35 minutes there was again spontaneous
seem to be no reason why we should differ­ homonymous diplopia for distance, the separation of
entiate from it the cases of esotropia for dis­ the double images being approximately eight cm.
For near there was single vision. The patient
tance in patients with myopia which have was advised to wear the glasses if she felt the
been reported as cases of acute strabismus need for it and to come for a recheck in six months.
of the "Bielschowsky type." When last seen on June 14, 1957, the patient
reported that she had discarded the glasses shortly
In concluding this paper we shall report after her visit to us in November, 1956, and that
an example of what we consider to be a she had not seen double since. She had had no ill­
ness in the interim, had felt entirely well and had
typical, though mild, paralysis of divergence done very well in school. At this last visit she had
which occurred in an eight-year-old girl, the no spontaneous diplopia; she had 90-percent single
youngest patient in whom we have diagnosed central binocular vision and 100-percent stereopsis;
the fusional amplitudes had remained the same.
this condition. With the Maddox rod there was an esophoria of
44 to 5A for distance and of 5A to 6A for near
C A S E REPORT without refractive correction.
CASE S SUMMARY
The patient was first seen on February 28, 1956.
Her presenting complaint was diplopia for dis­ The three forms are described under
tance which had begun acutely two weeks pre­ which an acutely arising comitant esotropia
viously. She had had no headaches, fever, malaise, may be observed.
or any illness prior to or at the time of the onset
of the diplopia, but she had had a few falls which, It is concluded that the etiology of the
however, remained without consequence so far as acute esotropia following artificial interrup­
the patient knew. Her local physician found her tion of binocular vision does not differ
in excellent physical condition; a psychiatric ex­
amination was without any positive findings. She essentially from the etiology of the sponta­
was referred to us by a member of the Depart­ neously occurring forms, except that in the
ment of Pediatrics, who could uncover no physical former the precipitating cause is known.
abnormality of any sort.
External examination, slitlamp examination, and It is furthermore concluded that comitant
funduscopy revealed no abnormality in either eye. esotropia may arise at any age. Comitant eso-
 COMITANT CONVERGENT STRABISMUS 63

tropia of late onset has no particular distin- with normal binocular vision for near seen in
guishing features; all the well-known classes myopes and described by von Graefe, Biels-
of comitant esotropia are represented. The chowsky, and others are clinically indistin-
diplopia accompanying it is an expression of guishable from cases of divergence paralysis.
the fact that the patient had binocular vision W e could find no reason why they should be
prior to the deviation; its explanation does classified into a separate clinical entity.
not differ from that which applies to the rT . ., r , ... ,
,. , . . , . ,. University Hospitals.
dtplopta in acute paralytic strab.smus. m ^ ^ R. % / j (W)
1 he genesis of a deviation appearing in
older children and adults is likely to be iden- NOTE: While this paper was in print, an addi-
tical with the genesis of a deviation mani- tional paper on convergent strabismus with acute
r ,■ ■, „ • • c ,., , JJ-.- i onset came to our attention (Alajmo, A.: Arch.
festing itself in infancy, although additional 0 t t a L > 6V2U lgs?) The author ^ ^ ^ as
factors may be present in the latter case. w e do, that the only distinguishing feature of this
However, the course of the disease is mate- form of strabismus is the late onset and that it
d o e s not
rially influenced by the age of onset. f ^fTer otherwise in its genesis or clinical
J
J a ^ picture from the convergent strabismus arising in
The cases of acute esotropia for distance e a r i y childhood.
REFERENCES

1. Swan, K. C.: Esotropia following occlusion. Arch. Ophth., 37:444-451, 1947.

2. Paufique, L.: Quoted from Franceschetti: Ophthalmologica, 123:224, 1952.
3. Bielschowsky, A.: Lectures on Motor Anomalies. Hanover, N. H., Dartmouth College Publications,
1956, pp. 73-74.
4. Burian, H. M.: Etiology of heterophoria and heterotropia in strabismus. In Ophthalmic Symposium
(I), J. H. Allen, ed., St. Louis, Mosby, 1950.
5. Moutinho, H. Strabisme convergent concomitant aigu "type Franceschetti." Modern Problems in
Ophthalmology. Basle/New York, Karger, 1957, v. 1, pp. 410-416.
6. Bailliart, P.: Quoted from Franceschetti: Ophthalmologica, 123 :224, 1952.
7. Burian, H. M.: Motility clinic: Sudden onset of convergent strabismus. Am. T. Ophth., 28 :407-410,
1945.
8. Franceschetti, A., and Bischler, V.: Strabisme convergent concomitant aigu chez l'adulte. Confinia
neurol., 8:380-382, 1947/1948.
9. Franceschetti, A., and Moutinho, H.: Strabisme convergent concomitant aigu. Bull. Soc. beige
Ophtal., 98:269-275, 1951.
10. Franceschetti, A.: Le strabisme concomitant aigu. Ophthalmologica, 123 :219-226, 1952.
11. Meunier, M. A.: Strabisme concomitant aigu. Bull. Soc. beige Ophtal., 101:429-432, 1952.
12. Schlossman, A.: Sudden onset of concomitant strabismus in adults. EENT Monthly, 34:677-678,
1955.
13. Shukla, K. N.: Acute concomitant squint with diplopia simulating bilateral lateral rectus paresis.
Am. J. Ophth., 41:121-124, 1956.
14. Norbis, A. L., and Malbran, E.: Concomitant esotropia of late onset. Brit. J. Ophth., 40 :378-380,
1956.
15. Capobianco, N. M.: The subjective measurement of the near point of convergence and its signifi­
cance for the diagnosis of convergence insufficiency. Am. Orthoptic. J., 2 :40-42, 1952.
16. Terson, A.: Un Strabisme d'origine insolite avec guerison medicate. Bull. Soc. d'ophtal. (Paris),
1935, pp. 22-25.
17. Malbran, J.: Estrabismo y Paralisis. Clinica y Terapeutica. Editorial in Oftal. Argentina, Buenos
Aires, p. 332.
18. Keiner, G. B. J.: New Viewpoints on the Origin of Squint: A Clinical and Statistical Study on Its
Nature, Cause, and Therapy. The Hague, Martinus Nijhoff, 1951.
19. Swan, K. C.: The sensory phvsiology of binocular vision. In Strabismus: Ophthalmic Symposium
(I), J. H. Allen, ed. St. Louis, Mosby, 1950.
20. Bielschowsky, A.: Das Einwartsschielen der Myopen. Ber. DeutscheOphth. Gesell., 43:245-248, 1922.
21. von firaefe, A.: Ueber die von Myopie abhangige Form convergierenden Schielens und deren
Heilung. Arch. f. Ophth., 10:156-175 (pt. I) 1864.
22. Theobald, S.: A case of convergent squint associated with myopia of high grade: Tenotomy of both
recti interni muscles: Restoration of binocular vision: The divided muscles still retaining a preponderance
of power. Arch. Ophth., 15 :191-195, 1886.
64 HERMANN M. BURIAN AND JAMES E. MILLER

23. Cords, R.: Die Pathologie der Augenbewegungen: In Schieck-Briickner, Kurzes Handb. Ophth.,
Berlin, Springer, 1930, v. 3, pp. S41-S42.
24. Malbran, J.: See ref. ", p. 247.
25. Weber, E.: Mit Diplopie einhergehendes Konvergenzschielen Myoper (Bielschowsky). Ophthal-
mologica, 114:320-324. 1947.
26. Dubois-Poulsen, A., Benmansour, and Attane: Deux cas de strabisme concomitant convergent
apparus chez l'adulte. Bull. Soc. beige Ophtal., 109 :60-73, (Fasc. II) 1953.
27. Berlin: Ueber eine eigentiimliche Form von Strabismus Convergens bei Myopie und deren Behand-
lung durch Konkavglaser. Festschr. Stuttgart arztl. Verein 1897. Rev. in Zentralbl. f. Augenh., 21:217,
1897.

SPONTANEOUS I N T R A - E P I T H E L I A L CYSTS O F IRIS

AND CILIARY BODY W I T H GLAUCOMA*
PAUL A. CHANDLER, M.D.
Boston, Massachusetts
AND
H A R R Y E . BRACONIER, M.D.
Brookline, Massachusetts

Spontaneous intra-epithelial cysts of the cysts found in this eye also. The pupil was
iris and ciliary body have been described dilated widely and gonioscopic examination
many times in the literature. For the most carried out, using the Koeppe contact lens,
part the diagnosis has been suspected by ob­ the microscope from a Comberg slitlamp,
serving a localized forward bulge of the and a Barkan hand-lamp for illumination.
iris 1-12 and/or a dark brown tumor present­ Most of the cysts were on the ciliary pro­
ing in the pupillary space, usually with the cesses, but some were evidently on the pos­
pupil dilated, but sometimes with the pupil terior surface of the iris. Some of the cysts
small.13"23 were clear and transparent, whereas others
FranQois,25 in 1948, was the first to report were heavily pigmented. In some cases only
the observation by gonioscopy of cysts of the one cyst was observed, in others several were
ciliary processes. In his case, after successful seen, and in one case there were 12 cysts.
iridencleisis for acute glaucoma, by gonio- A heavily pigmented tumor mass present­
scopic examination two cysts of the ciliary ing between the iris and lens led to a diag­
processes were observed in the coloboma. nosis of uveal sarcoma in several reported
Except for two anterior synechias below, cases, 2 ' 4 " 6 ' 11 ' 16 '" and the eye was enucleated.
the angle was evidently of good width below Pathologic examination of these eyes in each
and nasally. On the temporal side the angle case revealed multiple cysts of the iris and
was very narrow and the trabecular zone was ciliary processes. In the case reported by
not visible. Scheie,12 in 1953, reported the Bickerton and Clarke,3 the tumor was re­
diagnosis of cysts of the iris and ciliary pro­ moved by iridectomy and proved to be an
cesses by gonioscopic examination in 20 eyes iris cyst. In several other cases a diagnosis
of 16 patients. The presence of cysts was of iris cyst was made clinically, and the cyst
suspected in most cases by noting localized removed by iridectomy. 7 ' 13,14 ' 18,19 ' 21 ~ 23 In a
bulging in the periphery of the iris. In some few cases a clinical diagnosis of iris cyst was
cases, after finding cysts in one eye, exami­ made, but no operative treatment was carried
nation of the other eye was carried out and out. 1 ' 8 ' 9 ' 15 Several authors have reported on
the pathology of spontaneous epithelial
* From the Massachusetts Eye and Ear In­ cvsts 2> 4_7 ' n ' 14 ' 14_19 ' 2 1 > 2 2 ' 2 4 > 2S
firmary. In most of the reported cases there was no