Grading Severity and Activity in Thyroid Eye Disease
Peter J. Dolman, M.D., F.R.C.S.C.
Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver,
British Columbia, Canada
Purpose: Thyroid eye disease (TED) is an autoimmune
disorder causing inflammation, expansion, and fibrosis of
orbital fat, muscle, and lacrimal gland. This article reviews the
different methods of grading severity and activity of TED and
focuses on the VISA Classification for disease evaluation and
planning management.
Methods: Accurate evaluation of the clinical features of
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TED is essential for early diagnosis, identification of high-
risk disease, planning medical and surgical intervention, and
assessing response to therapy. Evaluation of the activity and
severity of TED is based on a number of clinical features:
appearance and exposure, periorbital tissue inflammation and
congestion, restricted ocular motility and strabismus, and
dysthyroid optic neuropathy. The authors review these clinical
features in relation to disease activity and severity.
Results: Several classification systems have been devised to
grade severity of these clinical manifestations. These include
the NO SPECS Classification, the European Group on Graves
Orbitopathy severity scale, the Clinical Activity Score of
Mourits, and the VISA Classification as outlined here. The
authors compare and contrast these evaluation schemes.
Conclusions: An accurate clinical assessment of TED,
including grading of disease severity and activity, is necessary
for early diagnosis, recognition of those cases likely to develop
more serious complications, and appropriate management
planning. The VISA Classification grades both disease severity
and activity using subjective and objective inputs. It organizes
the clinical features of TED into 4 discrete groupings: V (vision,
dysthyroid optic neuropathy); I (inflammation, congestion); S
(strabismus, motility restriction); A (appearance, exposure).
The layout follows the usual sequence of the eye examination
and facilitates comparison of measurements between visits and
data collation for research.
(Ophthal Plast Reconstr Surg 2018;34:S34–S40)
T hyroid eye disease (TED) is an orbital inflammatory dis-
order, strongly associated with autoimmune thyroid condi-
tions, that causes expansion and scarring of orbital fat, striated
muscle, and lacrimal gland.1,2 Thyroid eye disease is the most
common orbital disease globally, affects all races and has a
prevalence estimated between 0.5% and 2%, with females
Accepted for publication March 19, 2018.
Sections of this chapter were previously published in Dolman PJ.
Evaluating graves orbitopathy. Best Pract Res Clin Endocrinol Metab
2012;26:229–48.
The author has no financial or conflicts of interest to disclose.
Address correspondence and reprint requests to Peter J. Dolman, M.D.,
F.R.C.S.C., Eye Care Centre, Section I, 2550 Willow Street, Vancouver, BC
V5Z 3N9, Canada. E-mail: peterdolman@hotmail.com
DOI: 10.1097/IOP.0000000000001150
S34 Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018
Copyright © 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
out-numbering males by 4:1.3,4 Although it is self-limited, TED
may significantly disrupt cosmesis, vision, and quality-of-life.5
CLINICAL PHENOTYPES AND DEFINITION OF
SEVERITY AND ACTIVITY
Accurate evaluation of the clinical features of TED is essential
for early diagnosis, identification of high-risk disease, planning
medical and surgical intervention, and assessing response to
therapy.
Thyroid eye disease presents with a spectrum of clinical
findings based on which orbital tissues are affected. The pattern
and extent of involvement are graded as “disease severity.”
Approximately two-thirds of TED patients develop pri-
marily fat expansion, often in association with focal levator
muscle inflammation, resulting in eyelid retraction, proptosis,
and ocular exposure. This pattern typically evolves slowly over
several months in a younger, predominantly female population
(Fig. 1).2
The remaining third have enlargement of 1 or more extra-
ocular muscles and develop more severe features, including
congestion and edema of the conjunctiva and eyelids, restricted
ocular ductions with diplopia, and dysthyroid optic neuropathy
(DON) resulting from apical compression of the optic nerve.
This phenotype typically develops rapidly in an older popula-
tion with a more balanced gender distribution and is frequently
associated with smoking and a positive family history of TED
(Fig. 2).2,7,8
This latter presentation often follows a biphasic course,
with a progressive (“active”) phase lasting 6 to 18 months,
followed by a stable (“inactive”) phase. These disease phases
are graded as “clinical activity” and were first represented by
Rundle and Wilson9 as a graph of orbital disease severity plot-
ted against time, with a steeper slope in the progressive phase
reflecting more aggressive disease (Fig. 3).
Immunomodulators and radiotherapy administered
during the early active phase may limit the destructive conse-
quences of the immune cascade.10 Surgery is usually performed
in the postinflammatory phase for orbital cosmesis, comfort,
and function but may be necessary during the progressive phase
to prevent vision loss from DON or corneal breakdown.6,11
FIG. 1.  Fat-centric thyroid eye disease. A, Twenty-eight-year-
old female with slow onset of progressive bilateral proptosis and
upper eyelid retraction. B, Axial CT scan demonstrates proptotic
globes with fat expansion and prolapse of enlarged lacrimal
glands but no significant extraocular muscle involvement. With
permission from Springer.6
 Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018 Grading Severity and Activity in Thyroid Eye Disease
FIG. 2.  Muscle-centric thyroid eye disease. A, Seventy-three-
year-old male with progressive, rapid onset of severe periocular
congestion with restricted ocular motility. B, Axial CT scan
demonstrates bilateral enlargement of horizontal recti muscles
associated with areas of redness and swelling on the bulbar
conjunctiva. There is apical crowding of the optic nerve. With
permission from Springer.6
FIG. 3.  Rundle’s curve shows the biphasic course of myo-
pathic thyroid eye disease with a progressive (active) phase
followed by a quiescent (inactive) phase. Medical therapy and
radiotherapy are offered during the early progressive phase to
limit complications such as ocular motility restriction and optic
neuropathy. Rehabilitative surgery is delayed until the quiescent
phase. Surgery may be necessary emergently during the active
phase for vision-threatening conditions such as compressive
optic neuropathy or corneal ulceration.
Diagnosis of TED is based on 3 aspects of the disease.12
Characteristic clinical findings such as eyelid retraction com-
bined with proptosis and limited motility are suggestive of
TED, especially when bilateral. Abnormal thyroid function
tests (thyroxine and thyroid stimulating hormone levels) or
a history of thyroid dysfunction help confirm the diagnosis,
although 10% of TED patients may be euthyroid at onset.
The presence of thyroid stimulating hormone receptor anti-
bodies has high sensitivity for active Graves disease. CT,
MRI, or ultrasound is useful in atypical or uncertain clinical
situations.13,14
Risk factors for developing TED include a positive fam-
ily history, smoking, life stressors, and poorly controlled hypo-
thyroidism following radioactive iodine.7 Predictors for more
serious involvement by TED include male gender, increasing
age, smoking, and a rapid onset of orbitopathy.7 Cigarette smok-
ing is strongly correlated with both the development of TED and
with its severity.15,16
Recurrence of TED occurs in less than 10% of cases.17
This chapter reviews methods for evaluating and grading
both the severity and activity of TED.
© 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. S35
Copyright © 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
FIG. 4.  CT Scan correlation to clinical upper lid retraction. A,
Isolated right upper eyelid retraction with typical lateral flare.
This affects appearance and may cause dryness and photosen-
sitivity. B, Coronal CT scan demonstrates the thickened levator
muscle aponeurosis near its insertion in the eyelid. The other
extraocular muscles are not involved, and this individual is
unlikely to develop more serious complications of thyroid eye
disease. With permission from Springer.6
CLINICAL FEATURES AND GRADING SEVERITY
Appearance and Exposure. Over 80% of patients with
TED develop upper eyelid retraction, often with an insidious
onset that is first recognized by others.18 The “thyroid stare”
has a lateral flare (Fig. 4A) that is accentuated with emotion,
conveying anger. It is associated with eyelid lag on down-gaze
and lagophthalmos. A recent CT scan review identified a strong
correlation between enlarged levator muscle and upper eyelid
retraction, suggesting that this muscle is the most common
orbital muscle targeted by autoantibodies (Fig. 4B).19 Less than
15% of these patients had enlargement of the inferior rectus
muscle, implying that eyelid retraction occurs less commonly
from an overaction of the superior rectus to compensate for a
tight inferior rectus muscle.
The lower eyelid rests at or slightly above the inferior
corneal limbus. Lower eyelid retraction is present when sclera
is visible inferiorly and is associated with increasing proptosis
(Fig. 1A).
Proptosis is the second most common finding in TED,
resulting from expansion of the orbital fat and/or muscles. It
may be less apparent in those with tight eyelids limiting for-
ward protrusion. The reliability of the exophthalmometer is 1
to 2 mm; proptosis may also be recorded with photography or
orbital imaging.20
Eyelid retraction combined with proptosis and restricted
Bell’s phenomenon may lead to corneal exposure, with symp-
toms ranging from surface irritation, reflex tearing to visual
loss, and corneal changes ranging from punctate epitheliopathy
to frank ulceration.2
Periorbital Soft-Tissue Inflammation and Congestion.
Periorbital soft-tissue inflammation may present with orbital ache
at rest or with movement, conjunctival and caruncular injection
and edema, eyelid redness and edema, and diurnal variation.
Various grading schemes have been described for each of
these features. The simplest binary scale (present/absent) has good
reproducibility but is insensitive at documenting change, while more
sensitive scales may have poorer inter- and intrarater reliability.21
A recent prospective clinical study by the International
Thyroid Eye Disease Society found an acceptable interrater reli-
ability for eyelid and conjunctival edema using a 0 to 2 scale,
for eyelid and conjunctival redness using a 0 to 1 scale, and poor
reliability for assessing caruncle edema.22 Other studies have
suggested improved reliability using precise verbal descriptors
or an atlas of standardized photographs, although these may be
cumbersome to use in a busy office setting.23
Eyelid redness may be challenging to assess in those with
darkly pigmented skin, while eyelid edema may be hard to dis-
tinguish from orbital fat prolapse.
 P. J. Dolman Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018
Periorbital soft-tissue changes are used by some clini-
cians as a surrogate marker of inflammation and disease activity,
but they may also be significant in patients with chronic conges-
tion and absent in others with progressive disease.
Restricted Ocular Motility and Strabismus. While the
levator muscle is commonly involved in TED, extraocular
muscles become significantly targeted in only one-third of TED
patients.2 The onset of muscle involvement may be heralded
by aching with eye movement and with conjunctival redness
and edema overlying the insertion of the involved muscle.
During the active inflammatory phase, progressive restriction
of motility develops, initially intermittent or with gaze. Later
motility restriction may be due to secondary fibrosis.
The symptom of diplopia may be graded using the Bahn–
Gorman scale: 0 = no diplopia, I = intermittent diplopia (present
with fatigue), II = inconstant diplopia (with vertical or horizon-
tal gaze), III = constant diplopia in straight gaze, correctable
with prisms, and IV = constant diplopia, not correctable with
prisms.2
Ocular ductions can be graded from 0° to 45° in 4 direc-
tions using the Hirschberg principle. The patient is asked to
gaze in 4 directions, while the observer studies the position of
the ocular surface light reflex. If the light reflex is at the pupil-
lary edge, the eye has rotated 15°, between the pupil edge and
the limbus, 30° and at the limbus, 45°. This technique is as reli-
able as the “gold standard” perimetry technique with a coef-
ficient of reliability of 12°.24
Strabismus can be measured objectively by prism cover
testing in different gaze directions and is used for planning sur-
gical alignment.
Orbital CT scan identifies which muscles are enlarged
and with contrast may show enhancement and fat stranding
around actively inflamed muscles. In later stages, lucent zones
of hyaluronate develop within the enlarged muscles (Fig. 5A,
B). Short T1 Inversion Recovery and T2-weighted MRI may
show enhancement in edematous muscles.13
Dysthyroid Optic Neuropathy. Dysthyroid optic neuropathy
is a potentially reversible optic nerve dysfunction seen in 5%
to 7% of all cases of TED. Most cases are caused by direct
compression of the nerve by swollen muscles in the narrow
confines of the bony orbital apex, possibly impairing axoplasmic
FIG. 5.  CT scan extraocular muscle changes between active
and quiescent disease. A, Coronal CT scan of a 65-year-old
man immediately following right orbital medial wall and floor
orbital decompression for a compressive optic neuropathy with
impaired central and color vision. Note the enlarged medial and
inferior recti muscles with contrast enhancement. B, Five years
later, he developed left periorbital soft-tissue congestion with
reduced left color and central vision. CT scan shows interval
enlargement of the left extraocular muscles with optic nerve
crowding and contrast enhancement indicating active inflam-
mation. The right muscles remain enlarged, but the clear zones
represent hyaluronic acid deposition and are typical in long-
standing quiescent disease. He was treated successfully with left
orbital decompression and adjuvant corticosteroids and radio-
therapy. With permission from Springer.6
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Copyright © 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
flow (Fig. 5A, B).25 In rare cases with severe fat expansion,
vision loss has been reported from optic nerve stretch.26
Symptoms typically consist of desaturation of colors and
blurring of central vision. This is usually confirmed on clinical
examination, although central vision loss may be subtle in early
stages.27 An afferent pupil defect is a specific sign of DON but is
not detected in more than 60% of patients because of symmetric
visual impairment. Disc edema is also a specific sign when pres-
ent but is absent in over 40% of patients with DON.27
Dysthyroid optic neuropathy usually presents during the
active phase of TED because the onset of visual loss provokes
the patient to seek help. In early cases, the diagnosis may be
uncertain.
Most cases are associated with muscle enlargement, with
diplopia and restricted ocular ductions. Congestive and inflam-
matory features are typically present because of the enlarged
muscles but may be subtle. Proptosis is not strongly correlated
with DON.
Coronal CT scans demonstrate enlarged extraocular mus-
cles compressing the apical optic nerve with effacement of the
surrounding fat.25
Ancillary tests include visual field testing, which dem-
onstrates paracentral scotomas or generalized loss in 70% of
DON cases.
Grading Severity. Several classification systems have been
devised to grade severity of these clinical manifestations.
NO SPECS Classification by Dr. Werner28 grades TED-
related symptoms and signs and assigns a Global Severity Score
(Table 1). The acronym highlights disease features in order of
frequency of presentation, but the descriptors for each grade
are loosely defined and often based on only 1 variable such as
Snellen visual acuity for sight loss. Summary scores also tend
to hide details about how the patient is specifically affected and
make it difficult to assess disease progression or response to
therapy. It does not assess clinical activity nor provide a guide
for management.2
The European Group on Graves Orbitopathy severity
scale is based on 3 management categories.11 “Mild disease”
includes patients with eyelid retraction, mild proptosis, and min-
imal muscle involvement and is usually treated conservatively.
“Moderate to severe disease” incorporates individuals with
greater proptosis (>25 mm), inflammation, or significant motil-
ity restriction that impairs daily function and is often treated
medically. “Very serious disease” refers to sight-threatening
conditions such as DON or corneal ulceration and is often man-
aged surgically. In this system, the distinction between mild and
moderate disease is imprecise and the moderate class is a broad,
heterogeneous category, including individuals with soft-tissue
TABLE 1.  NO SPECS classification
Class Grade
0 No physical signs or symptoms
I Only signs (eyelid retraction)
II Soft-tissue involvement (0: absent, a: minimal, b: moderate,
c: marked)
III Proptosis (0: absent, a: minimal, b: moderate, c: marked)
IV Extraocular muscle signs (0: absent, a: limitation in extremes
of gaze, b: evident restriction, c: fixation of globe[s])
V Corneal involvement (0: absent, a: stippling, b: ulceration,
c: clouding, necrosis, perforation)
VI Sight loss (optic nerve compression) (0: absent, a:
visual acuity 0.63–0.5, b: visual acuity 0.4–0.1, c: visual
acuity <0.1 to no light perception)
 Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018 Grading Severity and Activity in Thyroid Eye Disease
congestion, motility disturbances, and severe proptosis.2 Also,
there is an implied rank order for severity that may not match
the patient’s perception of their disease. For example, an indi-
vidual with early DON may be oblivious to their color vision
loss but would be classified as “very severe,” while another indi-
vidual with disabling torsional diplopia would be graded with
“moderate” disease.
DISEASE COURSE AND GRADING ACTIVITY
Rundle’s Curve. While “severity” documents the status of TED
disease on a single visit, “activity” reflects the course of the
disease (onset and progression) with the aid of Rundle’s curve,
which plots change in severity over time.
Mild TED cases tend to have an indolent disease onset
and progression with a flatter curve, making it hard to distin-
guish active from quiescent phases. They often present in a
stable phase when surgery may be offered on a nonurgent basis.
More severe cases with inflammation and distension of
the extraocular muscle tend to have an acute onset and follow
the typical Rundle’s curve (Fig. 3) with obvious progression
from active to inactive phases.9 A steeper activity curve sug-
gests a greater risk of severe consequences, necessitating early
preventative intervention.2
Disease Duration and Progression. Individuals with TED pay
keen attention to their disease and often document the date and
rate of onset and the recent progression (worsening, stabilizing,
or improving). Immunosuppressive therapy and radiotherapy
are most effective in early TED, so identification of active and
high-risk cases can be determined even on the first clinic visit
based on patient reports, rather than waiting for a follow up to
document clinical changes.
Disease progression may also be documented by deter-
mining changes in ophthalmic signs on subsequent office visits.
An observed change is considered significant if it is greater than
the known coefficient of reliability for the measurement.24
Clinical Activity Score. Enlarged muscles may limit orbital
venous drainage resulting in periocular soft-tissue changes. The
Clinical Activity Score (CAS) is a summed score of periocular
inflammatory features proposed by Mourits et al.21 as a means of
identifying TED patients with active disease who could benefit from
immunosuppressive therapy (Table 2). This uses a binary scale with
a single point for 7 periocular soft-tissue inflammatory symptoms
and signs as surrogate markers of disease activity. On follow-up
visits, additional points are given for increased proptosis (2 mm or
more), decreased ocular motility (8° or more), or decreased visual
acuity over the previous 3 months. The scale is relatively easy to
score and a CAS score of 4 or higher has been shown to have an
80% positive predictive value and a 64% negative predictive value
in predicting response to corticosteroid therapy.
TABLE 2.  Clinical activity score
•  Painful feeling behind globe
•  Pain on attempted gaze
•  Redness of eyelids
•  Redness of conjunctiva
• Chemosis
•  Inflammatory eyelid swelling
•  Inflammation of caruncle or plica
•  Increase of 2 mm or more in proptosis in last 1–3 months
•  Decrease in visual acuity in last 1–3 months
•  Decrease in eye movements of 8° or more in last 1–3 months
© 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. S37
Copyright © 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
The CAS was intended to identify active disease but
has not been shown to correlate with risk of developing sig-
nificant complications such as diplopia or DON. Limitations
of this binary scale are that each clinical feature carries equal
weight (development of optic neuropathy is scored the same
as the onset of conjunctival redness), and that positive or
negative changes are documented only when they appear or
resolve.
While these inflammatory periocular soft-tissue symp-
toms and signs may reflect underlying TED activity, severe dis-
ease complications such as DON, eyelid retraction, or proptosis
often develop with low CAS scores, while patients with high
CAS scores may have long-standing congestive changes that
are unresponsive to any immunotherapy but that respond best to
mechanical surgical decompression.
Laboratory and Imaging. Several potential serum markers for
TED activity have been investigated including urine and serum
glycosaminoglycans29 and thyrotropin (thyroid stimulating
hormone) receptor antibodies. Clinical Activity Scores have
been shown to correlate with changing thyroid-stimulating
immunoglobulin assays.
Imaging techniques looking for tissue inflammation
include contrast enhancement within and around extraocu-
lar muscles using CT scans, muscle edema on T2-weighted
or Short T1 Inversion Recovery-sequenced MRI scans, and
orbital inflammation using gallium or octreotide scintigraphy.30
Facial thermography, positron emission tomography scans,
and Doppler ultrasonography have also been studied, but none
appear better than the clinical assessment tools.
Trial of Therapy. In some cases, determination of activity is
uncertain based on an equivocal history of progression and
borderline inflammatory changes. A trial of therapy using a
3-day course of oral prednisolone 50 mg can determine whether
clinical features show improvement and if the disease is
responsive, indicate more definitive therapy using intravenous
(IV) corticosteroids or radiotherapy.
THE VISA CLASSIFICATION AND ITS
APPLICATIONS
Evaluation of Severity and Grade. This recording form grades
both disease severity and activity using subjective and objective
inputs.31 It organizes the clinical features of TED into 4 discrete
groupings: V (vision, DON); I (inflammation, congestion); S
(strabismus, motility restriction); A (appearance, exposure).
The layout follows the usual sequence of the eye examination
and facilitates comparison of measurements between visits and
data collation for research.
The standard visit form (Fig. 6) is divided into 4 sections
recording specific symptoms on the left and validated signs for
each eye on the right. After each section is a progress row (bet-
ter, same, worse) documenting the impression of both patient
and clinician of the change in that parameter since the previous
visit. Progress is judged based on defined interval changes (i.e.,
2 mm change in proptosis, 12° change in ocular ductions, and a
change of 2 or more on the inflammatory score) rather than on
global scores.
Summary grades for the severity and progress for each
of the 4 disease parameters are documented at the bottom of the
form. Unlike the European Group on Graves Orbitopathy scale
that rates severity based on a rank order of clinical features, the
VISA Classification grades the severity and activity of each
parameter independently.
 P. J. Dolman Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018

FIG. 6.  International Thyroid Eye Disease Society: VISA Classification follow-up form.

S38 © 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Copyright © 2018 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
 Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018 Grading Severity and Activity in Thyroid Eye Disease

Activity is determined by a worsening in symptoms and

signs within any one of the 4 VISA parameters. Unlike CAS,
which uses an absolute score >4 to define disease activity, the
VISA system requires a worsening of the inflammatory score
of 2 or more as evidence of disease progress and activity. The
VISA I-score also differs from CAS in using a more sensitive 0
to 2 score for chemosis and eyelid edema, confirmed as main-
taining reliability in a recent prospective study.22
On the first visit, the date and rate of onset as well as
historic progress of both the systemic and orbital symptoms are
recorded, allowing immediate decisions to be made based on
disease course. Additional questions identify risk factors for
more serious TED outcomes including smoking, family history
of TED, and comorbidities such as diabetes.
A first visit form (2 pages) and follow-up form (1 page)
may be downloaded from the International Thyroid Eye Disease
Society website: www.thyroideyedisease.org.
An associated quality-of-life form (TED-quality of life),
downloadable from the same website, allows patient feedback
concerning the effect of the disease on their overall quality-of-
life, satisfaction with appearance, and ability to function.32,33
FIG. 7.  Periocular soft tissue inflammatory changes and
response to corticosteroids. A, Forty-seven-year-old woman
with rapid onset of inflammatory changes (Clinical Activity
Score, 7/10; VISA inflammatory score, 9/10). The recent onset
and history of progression indicate “active disease,” while the
congestive changes indicate extraocular muscle enlargement
(and the risk for serious sequelae) based on the VISA Classifica-
tion criteria. B, She was treated with combination corticosteroid
and radiotherapy for control of the inflammatory changes and
to prevent onset of motility disruption and optic neuropathy.
Although the inflammatory soft-tissue changes have resolved,
the upper eyelids remain retracted, suggesting that levator scar-
ring has already occurred. With permission from Springer.6

Management Planning. The layout of the VISA form facilitates

management planning.
V: Vision. Dysthyroid optic neuropathy is recognized by
central and color vision loss combined with a possible afferent
pupil defect and/or optic nerve head changes. Ancillary tests
to confirm the diagnosis include visual fields, visual evoked
potential, and coronal CT scans. Most cases are identified
during the progressive phase with the patient aware of the date
of onset and the rate of recent deterioration. In other cases, the
clinician may identify early subtle signs.
Initial therapy is a trial of systemic corticosteroids (oral
prednisone 1.5 mg/kg/d or IV methylprednisolone 1 g for 3
doses). The response to this trial of therapy often helps pre-
dict whether benefit will be gained from subsequent external
beam radiotherapy or surgical decompression. Complete lack
of response or the presence of a pale optic nerve head signi-
fies a poorer prognosis. In most cases, apical decompression can
restore even long-standing vision loss. Adjuvant radiotherapy
is often useful to prevent continued postoperative expansion of
muscle and recurrence of visual loss.10
I: Inflammation/Congestion. Symptoms include diurnal
variation, and orbital ache at rest or with movement, while signs
include injection and edema of the ocular surface or eyelid.
These are summed to form a VISA Inflammatory Score based
on the worst score for either eye or eyelid.
Mild soft-tissue inflammatory changes may be treated
with cold compresses and head elevation. Those with recent
onset and worsening scores may be treated medically with
oral or IV corticosteroids (Fig. 7A, B).34 The authors recently
reviewed 144 patients who had received monotherapy IV cor-
ticosteroids and found that 35% still developed strabismus and
15% developed DON in spite of adequate IV corticosteroids
therapy.35
External beam radiotherapy has been used for over 50
years for active thyroid orbitopathy with 60% efficacy in reduc-
ing soft-tissue inflammation and stabilizing strabismus, possibly
by targeting lymphocytes and fibrocytes that play an important
role in disease evolution. A recent retrospective review at the
authors’ institution of 258 patients found that there was 0% inci-
dence of new-onset DON in those treated with external beam
radiotherapy/corticosteroids, compared with 17% for those
treated with IV corticosteroids alone.35
FIG. 8.  Congestive orbitopathy and response to orbital
decompression. A, This lady had a high VISA inflammatory
score (and high Clinical Activity Score) based on her soft-tissue
changes. However, she had been on combination oral corti-
costeroids and cyclosporine for over a year with no history of
progression and was “inactive” following the VISA Classification
guideline (although her Clinical Activity Score would have been
interpreted as active). B, One month following bilateral orbital
decompression and upper eyelid lowering, her congestive fea-
tures had resolved and her medications were tapered off. Both
the VISA inflammatory score and Clinical Activity Scores were
reduced to zero. With permission from Springer.6
FIG. 9.  A, Progressive disease with bilateral upgaze restriction
and constant diplopia. B, Quiescent disease following combined
intravenous corticosteroids and radiotherapy and subsequent
ocular alignment surgery and upper eyelid lowering surgery.
With permission from Springer.6
In chronic cases refractory to medical therapy with no
other signs of progression, the high VISA inflammatory score
may represent chronic orbital venous congestion, rather than
true inflammation. In these cases, medical therapy may be dis-
continued and surgical decompression considered (Fig. 8A, B).
S: Strabismus/Motility Restriction. Three aspects are documented.
Symptoms of diplopia are recorded using a modified Bahn–
Gorman scale and can be graded from 0 to 3. Ocular ductions
are measured to the nearest 5° in 4 directions using the corneal

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 P. J. Dolman Ophthal Plast Reconstr Surg, Vol. 34, No. 4S, 2018
light reflex technique described above. Ocular restriction can
be graded from 0 to 3 based on the range of ductions (0°–15°,
15°–30°, 30°–45°, >45°). Strabismus can be measured
objectively by prism cover testing in different gaze directions to
plan surgical alignment.
Diplopia is treated with prisms or patching during the
active phase, and systemic corticosteroids and external beam
radiotherapy considered to limit ocular restriction. Once a sta-
ble phase is documented, alignment surgery or prisms may be
offered (Fig. 9A, B).
A: Appearance/Exposure. This section records features
relating to appearance and exposure including eyelid retraction,
exophthalmometry, and corneal exposure changes.
Exposure changes are treated with lubricant drops and
patching during the active phase. Rarely a tarsorrhaphy or even
an orbital decompression may be required for corneal break-
down or ulceration to prevent vision loss. Once the disease is
nonprogressive, surgery may be offered to deal with proptosis,
eyelid retraction, and orbital fat prolapse.36
CONCLUSIONS
An accurate clinical assessment of TED (including grading of
disease severity and activity) is necessary for early diagnosis,
recognition of those cases likely to develop more serious com-
plications, and appropriate management planning.
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