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Optic nerve dysfunction in thyroid T HYROID eye disease-also known compression of the optic nerve by
eye disease is thought to be due to as Gmaves ophthalmopathy, thy- enlarged extnaoculam muscles near
compression of the optic nerve by noid ophthalmopathy, dysthynoid eye the orbital apex (2-6). We used high-
enlarged extraocular muscles near disease, and thyroid-type endocrine resolution CT to evaluate the extent
the orbital apex. High-resolution ophthalmopathy-is the most com- of extmaocular muscle enlargement
computed tomography (CT) scans of mon cause of pmoptosis in adults. The and impingement on the optic nerve
78 orbits of 31 patients with thyroid diagnosis is based on characteristic in patients with and without clinical
eye disease were reviewed. Axial signs, such as exophthalmos, lid me- evidence of optic nerve disease. We
scans alone were inadequate for traction, lid lag, peniombital edema, devised a quantitative muscular in-
demonstrating compression of the and ocular motility disturbances. Re- dex that is easily measured from a co-
optic nerve. With a coronal refor- suits of laboratory tests for thyroid monal reformatted image of the orbit,
matted scan from the axial scans, a function may or may not be abnor- which can assist the radiologist in de-
muscular index was devised and mal. These findings are often supple- termining whether the extraoculam
measured to reflect extraocular mus- mented with computed tomography muscle enlargement appears signifi-
cle impingement on the optic nerve. (CT) to exclude apical mass lesions cant enough to cause optic nerve dys-
Orbits with optic nerve dysfunction and to demonstrate the characteristic function on the basis of compres-
had significantly higher muscular extraoculan muscle enlargement seen sion.
indices than those without optic in thyroid eye disease.
nerve dysfunction, supporting the Thyroid eye disease commonly oc-
PATIENTS AND METHODS
hypothesis that optic nerve dys- curs in patients with hyperthyroid-
function is usually secondary to ism, although orbital abnormalities
Sample Selection and CT
compression by enlarged extraocu- may occur before, during, on after the
lar muscles. Muscular indices of 67% phase of systemic hypenthynoidism. Retrospective analysis was performed
or greater in patients with optic The disease often occurs in patients on all high-resolution CT scans of pa-
nerve dysfunction were diagnostic without known thyroid abnormali- tients with thyroid eye disease who were
of compressive optic neuropathy, ties and namely occurs in patients with being followed up by the Neuro-Oph-
thalmology Consult Office at Washington
while muscular indices of less than Hashimoto thyroiditis and is usually
University Medical Center (St. Louis). A
50% appeared to exclude optic nerve self-limited; most patients have spon-
total of 78 scans from 31 patients were me-
compression. A single case of optic taneous remission of disease within 3 viewed. Six patients had at least two
nerve dysfunction without muscu- months to 3 years (1). Less than 10% high-resolution CT scans that were ob-
lar compression is also discussed. of patients with thyroid eye disease tamed at different times during the
have severe long-term on permanent course of the disease.
Index terms: Exophthalmos, 224.522 Muscles,
#{149}
loss of vision; many have residual CT was performed with a Somatom II
hypertrophy, 2246.522 Nerves,
#{149} optic, 144.52 pmoptosis or ocular motility distur- scanner (Siemens, Iselin, N.J.) at 125 kVp,
#{149}
Orbit, CT, 22.1211 Orbit,
#{149} diseases, 22.522 bances. 40 mA, and 2-mm collimation with no
overlapping between contiguous sec-
Optic nerve dysfunction, known as
Radiology 1988; 167:503-507 tions. Data were acquired in the axial
dysthymoid optic neuropathy, devel-
plane oriented along the canthomeatal
ops in less than 5% of patients with
lines. Scans were processed on a 256 X
thyroid eye disease (1). This is a semi- 256 matrix corresponding to a field of
ous complication that requires view of 125 X 125 mm, resulting in a spa-
prompt decompressive therapy tial resolution of 0.5 mm/pixel. Axial sec-
i From the Mallinckrodt Institute of Radiolo- (high-dose systemic conticosteroids, tions were postpmocessed, generating a
gy (LB., MG.), Washington University School usually followed by surgical decom- longitudinal reformatted scan for each or-
of Medicine, 510 S. Kingshighway, St. Louis, pmession on radiation therapy) to bit along the axis of the optic nerve. For
MO 63110; and the Departments of Ophthal- avoid permanent loss of vision. Cane most cases, four reformatted images were
mology (H.J.G., R.M.B.) and Neurology and generated in the coronal plane. For the
Neurological Surgery (R.M.B., MG.), Washing- must be taken to distinguish loss of
purpose of this retrospective analysis, we
ton University School of Medicine, St. Louis. vision due to dysthyroid optic neu-
reviewed the axial scans, both longitudi-
From the 1986 RSNA annual meeting. Received nopathy from other causes, such as nal reformatted scans, and one comonal me-
December 15, 1986; revision requested Febmu- cataract, macular degeneration, glau-
amy 17, 1987; final revision received November formatted scan in the plane transecting
30; accepted December 22. Address reprint re-
coma, or exposure keratitis. the optic nerves halfway between the
quests to MG. Many authors have proposed that posterior aspect of the globe and the apex
C RSNA, 1988 optic neuropathy is secondary to of the orbit.
503
Clinical Classification
Clinical records were reviewed by one
of the authors (H.J.G.). In all cases, the di-
agnosis of thyroid eye disease had been
made by an experienced neuroophthal-
mologist (R.M.B.) based on characteristic
clinical signs and symptoms and laborato-
my data. Cases were further subdivided
based on the presence or absence of
dysthyroid optic neuropathy. The diagno-
sis of dysthyroid optic neuropathy was
made based on characteristic signs that
included decreased visual acuity, afferent
pupillarv defect, visual field defects, and
diminished color or brightness percep-
tion. Patients with visual loss from causes
other than optic nerve dysfunction were a. b.
excluded. Each case was placed into one
of the following groups: Negative: no ev-
idence of dysthyroid optic neuropathy
prior to or at the time of CT; positive:
dysthyroid optic neumopathy at the time
of CT; resolved: resolved dysthyroid optic
neuropathy at the time of CT. The nega-
tive group was further subdivided into:
never (N): dysthyroid optic neumopathy
tzez’er developed (up to date of last clinical
follow-up); later (L): dysthyroid optic
neuropathy developed later, subsequent
to the time of CT.
Analysis of CT Scans
High-resolution CT scans were ana-
c. d.
lyzed by one of the authors (LB.). This Figure 1. Images of a normal orbit. (a) Axial scan. White line indicates the plane of the co-
observer knew that thyroid eye disease ronal reformatted scan. This plane is halfway between the orbital apex and the insertion of
was diagnosed in all patients but had no the optic nerve on the globe. (b) Reformatted scan. (c) Schematic of b to illustrate the homi-
zontal index, which is an objective assessment of impingement of the horizontal mectus mus-
knowledge of the optic nerve function of
each patient. For each of the 78 scans, the des on the optic nerve. The horizontal index is the percentage of orbital width (C) occupied
by the medial mectus (A) and lateral rectus muscles (B) along the line through the optic
number of enlarged muscles and the pat-
nerve. (d) Schematic of b to illustrate the vertical index, which is an objective assessment of
tern of enlargement were subjectively as-
impingement by the vertical mectus muscles on the optic nerve. The vertical index is the per-
sessed by the observer. Axial scans were
centage of orbital height (F) occupied by the superior mectus (D) and inferior rectus muscles
used to assess medial and lateral rectus
(E) along the line through the optic nerve. The larger of the two indices represents the most
muscle enlargement. Longitudinal refor-
significant impingement on the optic nerve space and is called the muscular index; thus, this
matted and axial scans were used to assess patient has a muscular index of 43%.
the superior and inferior rectus muscles.
When coronal reformatted scans were
available, a single scan transecting the ferior mectus muscle (E), and orbital used to compare the muscular indices and
optic nerves halfway between the posteri- height (F) were measured in the same number of enlarged extmaoculam muscles
or aspect of the globe and the orbital apex manner along a vertical line through the between the groups.
had been routinely obtained. This single optic nerve to determine the vertical mus-
scan was used to determine the extent of cle index ([D + E/F] X 100). We postulat-
encroachment of the enlarged extraocular ed that the larger of these two percent- RESULTS
muscles on the optic nerve space. ages reflected the maximum extraocular
The mean muscular index of the
muscle impingement on the optic nerve
positive group was significantly
space. The larger value, the muscular in-
Muscular Index dex, was used in subsequent data analy- greater than that of the negative
sis. In the orbit in Figure 2, theme is a very group (P < .001, Table 1). Two-thirds
A simple method was devised to quan-
high vertical index and a very low hori- of the positive cases had muscular in-
tify extraoculam muscle impingement on
the optic nerve space. The coronal refom- zontal index. It would seem that the com- dices greater than 70%; none were
matted scan halfway between the posteri- pression in this case is predominantly, or less than 50%. None of the negative
or globe and the orbital apex optimally even exclusively, in the vertical axis. We cases had muscular indices above
demonstrated muscle impingement on thought that the amount of optic nerve 67%. Muscular indices between 50%
the optic nerve space. The transverse di- compression in these cases would be best
and 67% were found in both positive
mensions of the medial rectus muscle (A), represented by the larger index (in this
and negative groups. The case with
lateral rectus muscle (B), and orbital case the vertical index), rather than by an
average of the horizontal and vertical in-
the lowest muscular index (50%) in
width (C) were measured with a ruler
dices. the positive group was the only one
along a horizontal line through the optic
in the study with enlargement of the
nerve. The horizontal muscular index was
expressed as the percentage of orbital optic nerve and sheath complex dem-
Statistical Analysis onstrated by CT (Fig. 3).
width occupied by the lateral and medial
rectus muscles ([A + B/C] X 100) (Figs. 1, For each group, the mean and standard The average number of enlarged
2). The vertical dimensions of the supemi- deviation (SD) of the muscular indices muscles that was subjectively as-
or rectus/levator muscle complex (D), in- were calculated. A two-tailed t test was sessed was 3.2 in the positive group
504 Radiology
#{149} May 1988
Table 1
Muscular Indices for Patient Groups
Mean
Muscular
No. of Index
Group Orbits (%) [SD]
Negative 49 42 [11]
Never developed 43 41 [11]
Developed later 6 46 [7.6]
Three or four
extraocular
muscles involved 16 51 [8.4]
Positive 6 67 [9.2]
Three or four
extmaocular
muscles involved 5 70 [5.8]
Resolved 7 60[11]
a. b.
DISCUSSION
dysthyroid optic neuropathy and dices supports this hypothesis. by extraoculan muscle impingement
found that total muscle volume was Coronal reformatted scans used for on the optic nerve.
increased significantly in patients measurement are electronically gen- A single patient with optic nerve
with dysthyroid optic neuropathy erated by stacking axial scan data in dysfunction had a relatively low
compared with patients in whom op- the computer memory. The reformat- muscular index (50%); this was the
tic neumopathy never developed. ted scan has a potential source of em- only patient in the study who had an
However, no CT cmitiemia were de- mom; namely, slight changes in the pa- enlarged optic nerve and sheath
scnibed to diagnose the presence or tient’s position between scanning. complex. The clinical diagnosis of
absence of compressive optic neunop- Furthermore, the spatial resolution thyroid eye disease in this patient
athy in an individual patient, and in a reformatted scan is degraded be- was made on the basis of bilateral up-
their complex planimetnic method cause of the low spatial resolution in pen eyelid retraction, an increase in
for quantitation is impractical for a the z-axis (2-mm section thickness) intraocular pressure while looking
nadiologic practice. inherent with CT. Direct, coronal CT up, abnormal ocular motility, and a
Our method for obtaining a mus- scanning has both technical and sta- history of hypenthynoidism previous-
cular index is a simple one that me- tistical problems. The angle of the di- ly treated with surgical and chemical
quines no special hardware on soft- mect coronal plane varies widely from thyroidectomy. Signs of optic nerve
ware. Measurements can be per- patient to patient, usually due to the dysfunction developed, including di-
formed on the hardcopy for inability of a patient to adopt a hy- minished visual acuity (to 20/400),
retrospective analysis or can be per- pemextended neck position. In other affenent pupillary defect, abnormal
formed with interactive use of the instances, the scanning plane must Goldmann visual fields, and a swol-
cursor on light pen provided in the be deliberately altered to avoid me- len optic disk. Hem visual acuity
console of most CT units. Although tallic artifacts caused by dental amal- reached a nadir 1 week after the on-
optic nerve compression by enlarged gam. As a result of this variability in set of visual loss, at which time sys-
extmaoculam muscles is thought to oc- the orientation of the section in a di- temic conticosteroids were adminis-
cur near the orbital apex, we chose to rect coronal scan, measurements of tered. After a 1-week course of sys-
make our measurements from the co- muscle thickness could not be com- temic conticostenoids, visual acuity
monal reformatted scan taken halfway pared between patients. Reformatted improved to 20/40. Vision has me-
between the orbital apex and the pos- scans requiring no unusual position mained stable after 3’/2 years of fol-
tenon globe. At the orbital apex, be- or additional ionizing radiation are low-up. Surgical decompression has
cause the orbital area is small, the op- always created in a plane pempendic- not been performed, and long-term
tic nerve is encircled by the extmaocu- ulan to the plane of the axial scan; administration of conticosteroids has
lam muscles, and it is difficult to with our technique, they were cneat- not been necessary.
identify the optic nerve and muscles ed in a plane perpendicular to the Enlargement of the optic nerve
as separate structures. At the mid-op- canthomeatal line. and sheath is a nonspecific finding
tic nerve plane we used, the optic Marked differences in the muscu- that can be due to optic neuritis, in-
nerve and muscles are clearly identi- lam indices were found between the creased intracranial pressure, orbital
fied as separate structures because of positive and negative groups. Two- pseudotumon, or optic nerve tumors
the larger orbital area. The plane thirds of positive cases had muscular (7). Although enlargement of the op-
halfway between the apex and poste- indices greaten than 70%, while none tic nerve and sheath has been de-
nor globe is posterior to the usual of the negative cases had muscular scnibed in patients with Graves dis-
area of anterior muscular tapering indices greater than 67%. Two-thirds ease with severe muscle enlargement
and tendinous sparing seen in dys- of the negative cases had muscular and optic neunopathy, the patient in
thyroid muscle enlargement. Theme- indices less than 50%, whereas none our study with enlargement of the
fore, muscle enlargement at mid-op- of the positive cases had muscular in- optic nerve and sheath had only mild
tic nerve hypothetically should me- dices less than 50%. These results muscle enlargement. Perhaps the en-
flect muscle enlargement near the generally support the theory that lamgement and optic nerve dysfunc-
apex. The significant correlation of dysthyroid optic neumopathy is a tion in this case were due to a mecha-
positive cases with high muscular in- compressive optic neumopathy caused nism other than compression. It is