1. Dr. Ali Raza
Associate Professor
Rawalpindi Medical College
Holy Family Hospital
Rawalpindi
2. F: Frontal bone with
Bony Orbit
associated suture and
notch.
<- Trochlea
<- Optical Canal
S: Superior orbital
fissure
I: Inferior orbital
fissure
L: Lacrimal bone
E: Ethmoid bone.
3. Other Locations.
The lacrimal gland and
lacrimal sac as well as the
potential for multiple
compartment involvement.
4. Spaces of the Retrobulbar Orbit
Intraconal space:
Contains CN II, ophthalmic artery, superior
division of CN III, nasociliary nerve (V1),
inferior division of CN III, and CN VI.
Extraconal space:
Contains ophthalmic vein, lacrimal nerve
(V1), CN IV and frontal nerve (V1).
5. Spaces of the Retrobulbar Orbit
Cone:
Composed of the four rectus muscles and
the thin intramuscular membrane which
joins them and extends posteriorly to the
insertion of the muscle tendons at the
orbital apex.
29. THYROID EYE
DISEASE
Autoimmune disorder
characterised by infiltrative
orbitopathy
30. THYROID EYE DISEASE
Associated with normal to abnormal
thyroid function which may coexist,
precede or follow the orbitopathy.
Related to but not the same as Graves
Ophthalmopathy (GO) The natural history
was described by Rundle and Wilson in
1945
31. Thyroid status-
Of those patients with thyroid orbitopathy,
approximately 80% are clinically hyperthyroid
and 20% are clinically euthyroid.4 Most patients
with euthyroid Graves' orbitopathy, however,
have some detectable laboratory evidence of
subclinical hyperthyroidism.
Both hyperthyroid and euthyroid patients can
develop clinical signs and symptoms of thyroid
orbitopathy. In general, patients with euthyroid
Graves' disease tend to have less severe
orbitopathy
32. THYROID EYE DISEASE
The goal is to identify and treat patients who are at
particular risk of sight threatening complications. The
disease has a finite period of activity until it becomes
burnt out.The yellow region shows the early phase
where there is the best response to treatment.
Type 1 younger age group, whiter eyes with proptosis.
Inflammation is mostly in orbital fat not muscles.
Type 11 older patient with red eyes, severe sight
threatening disease, tobacco addiction is frequent.
33. THYROID EYE DISEASE
LID RETRACTION1.
sympathetic overactivity
infiltration of levator / SR
complex. hypotropia
(retraction disappears on
downgaze)
SIGNS:- Dalrymples (lid
retraction), von Graefe
(lid lag), Kocher´s (staring
appearance)
36. Optic neuropathy with visual
loss
The prevalence of optic neuropathy with visual loss in
patients with thyroid orbitopathy is less than 5%.
Optic neuropathy is, however, the most common cause
of blindness secondary to thyroid orbitopathy. Its onset is
often insidious and may be masked by other symptoms.
These patients are usually older (age 50 to 70) are more
frequently male, have a later onset of thyroid disease,
and more often have diabetes.
Optic neuropathy is usually bilateral, but up to one third
of cases may be unilateral.
37. Optic neuropathy
Although a history of decreased vision should be carefully sought, it
is important to realize that optic neuropathy can occur in a
significant number (18%) of patients with visual acuities in the range
of 20/20 to 20/25 (6/6 to 6/7.5).*An afferent pupillary defect is
present in 35%. An abnormal disc (either swollen or pale) is seen in
only 52%. Visual field defects are present in 66%.Other tests that
can be useful include color vision testing and visual evoked
potentials (VEPs). The Farnsworth-Munsell 100-hue test is a
sensitive indicator of optic nerve dysfunction, but
pseudoisochromatic screening procedures (e.g.,Ishihara plates)
rarely identify an acquired color defect unless optic neuropathy is
severe.The pattern reversal VEP is very sensitive at detecting early
optic neuropathy and may be a useful means of following patients
after treatment.
38. Intraocular pressure
The increased intraocular pressure measured during
upgaze in patients with thyroid orbitopathy has been a
controversial finding. When restriction of the inferior
rectus muscle occurs, the intraocular pressure may
increase by 6 mm Hg or more in upgaze as compared
with primary gaze. The increased intraocular pressure in
upgaze is a normal phenomenon exaggerated by thyroid
orbitopathy
In patients with severe infiltrative disease there is an
increased pressure on upgaze as compared with normal
controls and patients with mild disease. It is often not an
indicator of early disease because it occurs infrequently
in patients with minimal eye findings
39. INVESTIGATION
SEROLOGICALT3 (hyperthyroid)T4 TSH
(hypothyroid)TSI (thyroid stimulating immunoglobulin).
RADIOLOGICAL TESTSOrbital CT (enlarged muscle
belly, tendon normal). Coca-Cola bottle sign = muscle
swelling deforming ethmoidal bones.MRI T2 showing
oedema of muscles; repeating the scan in different
positions of gaze can create a pseudo-video of eye
movements (for assessment of muscle restriction).
RADIOISOTOPE TESTS Octreoscan: quantitative
uptake of radio-labelled octreotide (which is a
somatostatin analogue).
40. VISUAL FIELD
A visual field should be performed in all patients
suspected to have optic neuropathy and is
useful when following patients after initiation of
treatment. Characteristically, a central scotoma
or an inferior altitudinal defect is seen in cases
of compressive optic neuropathy. Other visual
field defects include an enlarged blind spot,
paracentral scotoma, nerve fiber bundle defect,
or generalized constriction.
41. CT findings in thyroid orbitopathy
The most characteristic CT finding in thyroid orbitopathy
is enlargement of the extraocular muscles with normal
tendinous insertions onto the globe. Other findings
include proptosis and anterior prolapse of the orbital
septum due to excessive orbital fat and muscle swelling
(see Fig. 4).Patients at risk for developing optic
neuropathy may also have severe apical crowding, a
dilated superior ophthalmic vein, and anterior
displacement of the lacrimal gland. Of these, apical
crowding is the most sensitive indicator for the presence
of optic neuropathy The CT scan should be done in the
coronal plane to assess the enlargement of the
extraocular muscles at the apex because axial sections
can sometimes be misleading.
43. SYSTEMIC THYROID DISEASE
There are no good recent studies of the natural
history of untreated hyperthyroidism, but based
on older reports, Wilson56 determined that
about one third of patients spontaneously
improve, one third remain chronically
hyperthyroid, and one third progress to thyroid
storm and occasionally death. Because it is not
possible to predict which patients will
spontaneously improve, treatment of thyroid
dysfunction is recommended.
45. TREATMENT
Acute Congestive Orbitopathy
1. SYMPTOMATIC:- elevate bedhead, lubricants, lid taping,
diuretics
2. SYSTEMIC:-
a) Normalise thyroid function with or without thyroxine.
Patients rendered euthyroid do improve their GO score
Tallstedt trial N Eng J Med 1992
antithyroid drugs cause a 10% chance of new or worsening GO
but radio-iodine causes a 30% chance of new or worsening GO.
46. Corticosteroids have been used successfully in the
treatment of acute congestive orbitopathy
Corticosteroids have been used successfully in the treatment of
acute congestive orbitopathy. They are believed to work by altering
cell-mediated immune response and diminishing the production of
mucopolysaccharides by the orbital fibroblasts.Corticosteroids result
in improvement of soft tissue involvement and compressive optic
neuropathy (but do not have as much of an effect on diplopia
Traditionally, a "short burst" of high-dose corticosteroids has been
given, usually in the range of 60 to 120 mg/day of oral prednisone.
Improvement in subjective symptoms such as pain and tearing
usually occurs first, often as early as 24 to 48 hours, followed by
improvement in soft tissue congestion and muscle function over a
period of days to weeks.
47. Steroid Therapy
Prednisone or prednisolone
This is standard treatment but there are frequent side effects. No
response in 35% of patients and anyway the response is only
partial. High dose steroids given early in the disease when muscle
swelling occurs does not necessarily limit the long term course of
the disease. If there is no response to high dose steroids in the first
three weeks they should be rapidly reduced. Prednisolone + orbital
radiotherapy has slightly more effect than either alone.Use high
dose pulsed methylprednisolone if urgent optic nerve
decompression is required, This is more effective than oral
treatment but it is expensive and not justified in most cases of TED.
48. Radiation therapy
During the past few years, radiation therapy has reemerged as a
useful form of treatment of severe orbitopathy. The rationale for the
use of radiation therapy is reduction or elimination of the pathogenic
orbital lymphocytes, which are markedly radiosensitive. It is also
thought that the glycosaminoglycan production by fibroblasts is
reduced, thereby reducing orbital edema, orbital tension, and
conjunctival injection. Although congestive findings improve most
consistently, significant improvement in proptosis and extraocular
muscle function has been reported.Like corticosteroids, radiation
therapy is most effective within the first year, when significant fibrotic
changes have not yet occurred. Mourits and associates,135
however, suggest that periods of active orbital inflammation within
the long natural history of thyroid orbitopathy would benefit from
corticosteroids or radiation therapy.
49. Radiotherapy
RETROBULBAR RADIOTHERAPY:- Trial of
prednisone versus radiotherapy showed no
difference in clinical improvement (about
50%).The patients all tolerated retrobulbar
radiotherapy better than steroids Consider if
steroid maintenance > 25mg/ day. Best effect in
acute disease.Do not irradiate patients with
diabetes mellitus as they are more susceptible
to radiation retinopathy.2000rads/ 10days, effect
starts at 4 weeks, maximal 4 months.
50. Compressive Optic Neuropathy
Compressive optic neuropathy can cause
permanent visual loss. The treatment
possibilities include high doses of
corticosteroids, irradiation, and orbital
decompression. Some patients require only one
of these modalities, while other patients need
combined therapies.
51. Compressive Optic Neuropathy
As in the treatment of acute congestive thyroid
orbitopathy, radiation therapy is becoming increasingly
popular. A retrospective series of 84 patients with
compressive optic neuropathy treated with either
corticosteroids or radiation therapy supports mounting
evidence that radiation therapy may be safer and more
effective than corticosteroids.
Radiation therapy, however, must be administered in
fractionated doses, which delays its beneficial effect. For
this reason, if visual dysfunction progresses while the
patient is on corticosteroids, surgical decompression is
usually recommended if the patient is a surgical
candidate.
52. Orbital decompression
Orbital decompression is indicated for compressive optic
neuropathy when there has been failure of or
contraindication for corticosteroids or radiation therapy or
if corticosteroid dependence has developed with
intolerable side effects. Other indications include
excessive proptosis with exposure keratitis and corneal
ulceration, pain relief, and cosmesis for disfiguring
exophthalmos. Orbital decompression may also be
indicated as a preliminary procedure to extraocular
muscle surgery on a patient with sufficient proptosis to
suggest that decompression might ultimately be
required.
53. Orbital decompression
A variety of approaches may be used, each with
its own advantages and associated
complications.
The transorbital (via fornix or eyelid) approach to
inferior and medial wall decompression is the
most common approach used by
ophthalmologists. The addition of a lateral wall
advancement has the advantage of both further
increasing the orbital volume and simultaneously
improving upper eyelid retraction; this is the
technique we prefer.
54. ORBITAL DECOMPRESSION
Subciliary approach.Inferior & medial wall (6mm
proptosis).Remove bone to posterior wall
maxillary sinus (5mm more posterior on medial
wall), Avoid IO neurovascular bundle, and the
anterior and posterior ethmoidal arteries.Incise
periosteum in A-P direction posteriorly and
circumferentially anteriorly.
Complications:
visual loss,
A pattern ET
55. Motility Disorders
A major source of morbidity in thyroid orbitopathy, and the most
frequent problem associated with orbital decompression surgery,
has been strabismus. In patients with relatively minimal degrees of
ocular misalignment, diplopia can be avoided with a compensatory
head posture, Fresnel plastic press-on prisms, or temporary
occlusion. Unfortunately there is significant image degradation as
larger prisms are used, limiting their efficacy. If there is marked
asymmetry in ocular deviation in different fields of gaze, prisms are
also less effective. In some cases during the inflammatory period,
use of intramuscular botulinum toxin has shown some efficacy.
Extraocular muscle surgery should be postponed until the muscles
are no longer inflamed and the deviation has remained stable for at
least 6 months.
56. Eyelid Abnormalities
As with other thyroid eye problems, eyelid retraction will often
improve with time, and only an estimated 50% of patients with eyelid
retraction have a significant eyelid abnormality 5 years later.
Eyelid retraction can result from excessive autonomic discharge,
levator fibrosis, or contraction of the inferior rectus muscle.
Surgical correction of eyelid abnormalities should be performed only
after orbital or extraocular muscle surgery because these operations
may change eyelid position. For example, inferior rectus muscle
restriction may cause upper eyelid retraction because of the
superior rectus/levator palpebrae superioris overaction against the
restriction. Specific techniques for repair of eyelid retraction are
discussed in other chapters.
57. Steroid Therapy
Prednisone or prednisolone
This is standard treatment but there are frequent side effects. No
response in 35% of patients and anyway the response is only
partial. High dose steroids given early in the disease when muscle
swelling occurs does not necessarily limit the long term course of
the disease. If there is no response to high dose steroids in the first
three weeks they should be rapidly reduced. Prednisolone + orbital
radiotherapy has slightly more effect than either alone.Use high
dose pulsed methylprednisolone if urgent optic nerve
decompression is required, This is more effective than oral
treatment but it is expensive and not justified in most cases of TED.
58. Radiation therapy
During the past few years, radiation therapy has reemerged as a
useful form of treatment of severe orbitopathy. The rationale for the
use of radiation therapy is reduction or elimination of the pathogenic
orbital lymphocytes, which are markedly radiosensitive. It is also
thought that the glycosaminoglycan production by fibroblasts is
reduced, thereby reducing orbital edema, orbital tension, and
conjunctival injection. Although congestive findings improve most
consistently, significant improvement in proptosis and extraocular
muscle function has been reported.Like corticosteroids, radiation
therapy is most effective within the first year, when significant fibrotic
changes have not yet occurred. Mourits and associates,135
however, suggest that periods of active orbital inflammation within
the long natural history of thyroid orbitopathy would benefit from
corticosteroids or radiation therapy.
59. Radiotherapy
RETROBULBAR RADIOTHERAPY:- Trial of
prednisone versus radiotherapy showed no
difference in clinical improvement (about
50%).The patients all tolerated retrobulbar
radiotherapy better than steroids Consider if
steroid maintenance > 25mg/ day. Best effect in
acute disease.Do not irradiate patients with
diabetes mellitus as they are more susceptible
to radiation retinopathy.2000rads/ 10days, effect
starts at 4 weeks, maximal 4 months.
60. Compressive Optic Neuropathy
Compressive optic neuropathy can cause
permanent visual loss. The treatment
possibilities include high doses of
corticosteroids, irradiation, and orbital
decompression. Some patients require only one
of these modalities, while other patients need
combined therapies.
61. Compressive Optic Neuropathy
As in the treatment of acute congestive thyroid
orbitopathy, radiation therapy is becoming increasingly
popular. A retrospective series of 84 patients with
compressive optic neuropathy treated with either
corticosteroids or radiation therapy supports mounting
evidence that radiation therapy may be safer and more
effective than corticosteroids.
Radiation therapy, however, must be administered in
fractionated doses, which delays its beneficial effect. For
this reason, if visual dysfunction progresses while the
patient is on corticosteroids, surgical decompression is
usually recommended if the patient is a surgical
candidate.
62. Orbital decompression
Orbital decompression is indicated for compressive optic
neuropathy when there has been failure of or
contraindication for corticosteroids or radiation therapy or
if corticosteroid dependence has developed with
intolerable side effects. Other indications include
excessive proptosis with exposure keratitis and corneal
ulceration, pain relief, and cosmesis for disfiguring
exophthalmos. Orbital decompression may also be
indicated as a preliminary procedure to extraocular
muscle surgery on a patient with sufficient proptosis to
suggest that decompression might ultimately be
required.
63. Orbital decompression
A variety of approaches may be used, each with
its own advantages and associated
complications.
The transorbital (via fornix or eyelid) approach to
inferior and medial wall decompression is the
most common approach used by
ophthalmologists. The addition of a lateral wall
advancement has the advantage of both further
increasing the orbital volume and simultaneously
improving upper eyelid retraction; this is the
technique we prefer.
64. ORBITAL DECOMPRESSION
Subciliary approach.Inferior & medial wall (6mm
proptosis).Remove bone to posterior wall
maxillary sinus (5mm more posterior on medial
wall), Avoid IO neurovascular bundle, and the
anterior and posterior ethmoidal arteries.Incise
periosteum in A-P direction posteriorly and
circumferentially anteriorly.
Complications:
visual loss,
A pattern ET
65. Motility Disorders
A major source of morbidity in thyroid orbitopathy, and the most
frequent problem associated with orbital decompression surgery,
has been strabismus. In patients with relatively minimal degrees of
ocular misalignment, diplopia can be avoided with a compensatory
head posture, Fresnel plastic press-on prisms, or temporary
occlusion. Unfortunately there is significant image degradation as
larger prisms are used, limiting their efficacy. If there is marked
asymmetry in ocular deviation in different fields of gaze, prisms are
also less effective. In some cases during the inflammatory period,
use of intramuscular botulinum toxin has shown some efficacy.
Extraocular muscle surgery should be postponed until the muscles
are no longer inflamed and the deviation has remained stable for at
least 6 months.
66. Surgery
STRABISMUS SURGERY:-Aim for maximal area of fusion without abnormal
head posture.IR recession on adjustable +/- contra SR recession
iii) EYELID SURGERY:-
Upper Lid retraction - Muller´s tenotomy (<2mm), levator Z myotomy or
recession on hangback sutures, levator tenotomy +/- horns.
Lower Lid retraction - Usually needs a spacer from donor sclera (lid
retraction X 2 = amount of sclera required)
iv) BLEPHAROPLASTY for excess skin and fat
Ideally treatment combines a multidisciplinary coherent approach such as
Combined radiotherapy and immunosuppression trial
