3 Graves Orbitopathy Flashcards
TED recap
- clinical orbital signs (lid retraction / proptosis / optic neuropathy)
- laboratory tests (positive bloods for autoantibodies)
- typical orbital imaging finding (swollen EOMs)
Symptoms in the mild stage
Foreign body sensation;
dry eye; excessive tearing; conjunctival or eyelid redness
and swelling;
blurred vision;
retro-orbital pain
Clinical features in mild stage
Mild, soft tissue inflammation;
Dilated conjunctival vasculature;
keratoconjunctivitis
Symtoms in moderate stage
Pulling sensation around the eye; Eyelid redness and swelling; Eyelid retraction and bulging eyes, Swelling of extraocular muscles; chemosis; eyelid oedema; proptosis
Clinical features in moderate stage
Horizontal, vertical, and torsional strabismus with double vision;
deteriorating blurred vision; fading colour vision in one or both eyes; decrease in visual acuity, visual field, and colour vision
(signs of optic neuropathy)
Progressive proptosis with eyelid retraction; corneal ulceration; inflammation of extraocular muscles and scarring leading to strabismus and ophthalmoplegia; increased intraocular pressure
Orthoptists role
- Diagnosis
- Assessment of visual function
- Document effects on ocular muscles
- Record disease progress
- Establish when ocular signs stabilise
- Explanation, information and signposting
- Eliminate symptoms / diplopia
- Plan long term management
Orthoptic investigation
- Assess visual function
- Cover test
- Note any AHP (abnormal head posture)
- OM + measure PA
- BSV tests (inc VPFR)
- PCT + torsion (synoptophore or torsionometer)
- Uniocular field of fixation
- Field of BSV
- ?Lees screen / Hess (not useful in bilateral cases)
Colour vision and TED
As the extra ocular muscle swell and the eye becomes proptosed the optic nerve is affected. This can cause colour vision defects.
Colour vision testing
100 hue, D15, Ishihara, City
Visual function testing- pupils
Checks for optic nerve damage which may occur if ON compression
Clinical signs of ON compression
- Reduced VA
- Reduced CS
- Reduced colour vision
- Visual field defect
- RAPD (Relative Afferent Pupillary Defect)
- Optic disc exam – normal / swelling / pallor
- Optic neuropathy – approx 5%
Modified UFOF
Modified technique for UFOF, Reliable, Rapid, minimising discomfort, Change of 8 degrees is significant
Modified UFOF technique
This modified technique for measuring UFOF gives additional quantified information on the vertical extraocular muscles. Its reliability is equivalent to that of other measurement methods in healthy subjects and in those with restricted motility. It can be performed rapidly, minimizing patient discomfort, and may be particularly helpful in the sequential assessment of GO. The data on patients with GO suggest that a difference of 8° is required to detect significant change, particularly where several observers are involved, and this has important implications for the monitoring of GO, especially when using the CAS.
Differential diagnosis
Acute presentation- Carotid-cavernous Fistula, Myositic pseudotumour Ocular myositis,
Leukemia
Chronic presentation- Metastatic tumours, Lymphoma, Chronic progressive external ophthalmoplegia
Myasthenia- Fatigue, Variable ptosis, Limitation not persisting on FDT
Classification systems
Werner’s classification
Mouritis CAS
EUGOGO
Werners classification system
Werner’s classification (NO SPECS)
Stage Ocular signs and symptoms
1 None
2 Only signs, no symptoms
3 Soft tissue involvement
4 Proptosis
5 Extraocular muscle involvement
6 Corneal involvement
7 Sight loss
0 = absent a = minimal b = moderate c = marked
Mouritis clinical activity store (CAS)
Mourits (1997) Clinical Endocrinology 47:9-14
Clinical activity score (CAS) is based on the clinical signs of inflammation:
* Pain
* Redness
* Swelling
* Impaired Function
◦ Ten items considered
◦ For each item present a point is given
First visit score only 1-7, review visits score 1-10
1. Painful oppressive feeling on or behind the globe in last 4 weeks
2. Pain on eye movement in last 4 weeks
3. Redness of eye lid
4. Diffuse redness on conjunctiva covering at least 1 quad
5. Swelling of lid (erythema)
6. Chemosis
7. Swollen caruncle
8. Increase in proptosis >2mm in 1-3 months
9. Reduced motility 5 degrees
10. Reduction in VA of 1 line with PH over 3 months
Mild: Mourits Activity score 4 Moderate: Mourits Activity 4-6 Severe: Mourits
Activity 7 – 10 or presence of vision threatening signs (Exposure, optic nerve oedema, or RAPD
EUGOGO – European Group on Grave’s Orbitopathy
Combines CAS with measures of severity
1. Spontaneous retrobulbar pain
2. Pain on up or downgaze
3. Redness of eye lid
4. Redness on conjunctiva
5. Swelling of lid (erythema)
6. Inflammation of the caruncle
7. Conjunctival oedema
3 or more = active GO
Management options
Surgical: Sight saving, Strabismus, Lid, Encourage use of AHP, Prisms - temporary / incorporated, Occlusion – blenderm / patch / frosted lens , BT and Surgery
Aims of management
Preserve visual function
Keep patient comfortable and symptom free
Allow comfortable BSV in pp and reading position where possible
Reason of surgical intervention
- ON compression – orbital surgery
- Strabismus / ocular issues - realignment surgery
- Lid problems - Lid surgery
MDT- maxfax surgeon, orbital surgeon and strabismologist involvement
Orbital changes
Retrobulbar adipose tissue is increased in volume and may have lymphocytic infiltrate.
Increased volume of orbital contents typically leads to exophthalmos/proptosis
May lead to ON compression / neuropathy
Optic neuropathy management
Steroids, Radiation and Orbital Decompression
Steroids for optic neuropathy mechanism
anti-inflammatory
immune response - immunosuppressive
decrease
mucopolysaccharide production by orbital fibroblasts
Steroids for optic neuropathy indications
acute inflammatory disease
optic neuropathy - mild VA loss
recent onset <6m, predominantly severe soft tissue signs
following other treatments
pre / post decompression
Steroids and azathioprine- they are another immunosuppressant. It allows the reduction of steroids if prolonged large doses – reduces side effects of steroids
Effects are modest
Radiation therapy in affected areas
It is well tolerated and has no short term side affects usually no long term side affects* either. Reduces symptoms but not the course of the disease. Referral to oncologist, face mask to immobilise patient and allow accurate delivery of radiation, planning with CT scans, 2 weeks of treatment delivered daily and it can feel intensive for patient
Radiotherapy reduce the cells which cause the inflammation and so reduce the swelling behind the eye.It may take up to a year to notice the full effect. Ten radiotherapy treatments given over two weeks. Face mask to immobilise patient and allow accurate delivery of radiation.
Radiation therapy risks
There is a risk of cataract/risk of dry eyes
Radiation therapy process
Radiation therapy is given in daily doses of radiation beams directed at the orbital area- Low dose of 2Gy x ten days. It destroys lymphocytes and fibroblasts reducing auto-immune response. First weeks often in conjunction with steroids. There is increased chemosis in first week of treatment. There is Improvement after 2 weeks of treatment. If no improvement in 1 month of treatment unlikely to improve. Radiotherapy is indicated in patients with
severe acute soft tissue signs - (steroids initially as radiotherapy not immediate)
recent onset progressive proptosis, acute ophthalmoplegia, acute vision loss. Where steroid treatment has failed
Radiation is not as effective in…
- chronic TED
- minimal or no inflammation
- proptosis without inflammatory changes
- longstanding restrictive myopathy or rapid progression of disease
- male patients / smokers
- age >50 years
Radiation therapy affects on Mourits CAS score
Patients with CAS > 4 = 80% chance of improvement
Patients with CAS < 4 = 36% chance of improvement
ITS BETTER FOR SEVERE CASES
Orbital decompression
Orbital decompression is an operation to remove bone from the walls of the orbit (eye socket) in order to reduce the amount of petrusion of the eye. Indications; sight threatening situations and improves cosmesis (40%, Lyons & Rootman 1994)
Moderate proptosis
lateral orbital (single)
Severe proptosis
a two-and-a-half wall decompression (the medial wall, medial half of the floor, and lateral wall are removed
Patients with sight threatening orbitopathy
A medial one-and-a-half wall approach (where the medial wall and medial half of the floor is removed) is used after a trial of three daily/alternate day injections of high-dose intravenous methylprednisolone.
Mild proptosis
Orbital fat decompression alone
Complications of orbital decompression- many
Temporary lip numbness
Asymmetric correction of proptosis
Sinusitis*
Apparent upper lid retraction
Orbital cellulitis*
Late endophthalmos
Meningitis*
Epiphora
Lower lid entropian
Diplopia
Blindness 1/10000
Strabismus and TED
Due to EOMs swelling and it occurs in 15-51% of patients with TED
Diplopia which impacts on work / driving / ability to function independently
Usually vertical
Indications for strabismus surgery
Six months stability seems a general rule that’s accepted
- Medical condition stable
- Eye condition stable
- Problematic diplopia
- Uncomfortable head posture
- Centralise and / or enlarge field of BSV
Affect on lids
retraction of upper and lower lid
Levator palpebral superioris - muscle fibre enlargement and oedema
Overaction of Muller’s muscle (sympathetic overaction)
Innervation to SR and levator palpebral superioris
Lid retraction drugs
Guanethidine- Topical (drops) alpha-adrenergic blocking agent
Triamcinolone – oral anti-inflammatory / anti- fibrotic effect
Levator muscle procedures
Müller’s muscle procedures
Combination of above
Aim to leave ptotic - gradually elevates
Lid retraction surgeries- lateral tarsorrhaphy is rarely used now and not very effective. It may prevent exophthalmos. Can also perform the Henderson’s operation which is a weakening of the muller muscle. You can perform a recession, tenotomy or use BT on the levator muscles.
Periorbital oedema
Blepharoplasty (surgical correction of a lid deformity)
◦ mark out excess skin
◦ excise skin and orbicularis
◦ remove fat
Complications can include infection, bleeding, dry / irritated eyes and difficulty closing eyelids
Corneal issues
- Tape lids, glasses with side protection, Hypromellose, steroids, orbital decompression and tarsorraphy
Newer treatment for GO -2
Rituximab
Teprotumumab (Tepezza)
Rituximab
(MONOCLONAL ANTIBODY) is a drug which depletes B cells, thus promoting antibody-dependent cellular toxicity. Used in non-Hodgkin B-cell lymphoma and rheumatoid arthritis with some very promising results. Currently under review in some studies with GO but nothing concrete yet
Tepezza
A new medicine used to treat GO. Human monoclonal antibody. Approved for use in USA in Jan 2020. It is expensive - $343000 per patient for 6 months of treatment (approx. £250k)
Tepezza- positive findings
Its the only medication that reduces the fat and muscle expansion within the orbit
Only medicine to possibly reduce ON compression
Some patients remain non-responders to treatment
Possible side effects: hearing loss, hyperglycemia, and muscle spasm
Summarising GO
Graves orbitopathy is a debilitating condition and is sigh threatening and requires complex management and the patients need support and signposting.
