17 - orbitopathy and NMJ disease

What are causes of orbitopathy?

1. carotid-cavernous fistula

2. TED

3. orbital trauma

4. orbital inflammation → orbital cellulitis, sarcoid, idiopathic

5. orbital tumors

What are the general signs of orbitopathy?

1. proptosis

2. red eye

3. chemosis

4. EOM restriction + strabismus

What is a carotid-cavernous fistula?

abnormal communication between the carotid arterial system and cavernous sinus causing orbitopathy

What are the symptoms of a carotid-cavernous fistula?

1. VAs normal to poor

2. diplopia

3. red eye

4. headache

What are the signs of carotid-cavernous fistula?

1. proptosis

2. ophthalmoplegia

3. RAPD

4. engorged conj vessels

5. iris neovasc

6. increased IOP

7. may have retinal involvement

What is the treatment for a carotid-cavernous fistula?

low flow fistula with no increased IOP → observe

high flow fistula → surgery

good prognosis

What is thyroid eye disease (TED)?

autoimmune disease causing overactivity of thyroid gland → hyperthyroidism

Who gets TED?

1. female (but male more likely to have severe disease)

2. middle aged

3. smokers

those who get worsening of thyroid state after achieving good control

What are the symptoms of TED?

1. irritation

2. red eye

3. blurred vision

4. diplopia

What are the signs of TED?

1. lid retraction

2. proptosis

3. periorbital swelling

4. EOM restriction

5. corneal involvement

6. sight loss (optic neuropathy → pale or swollen disc)

What is NOSPECS for TED?

*N*o signs/symptoms

*O*cular symptoms (red, FB sensation, lid retraction)

*S*oft tissue involvement (periorbotal edema, conj chemosis)

*P*roptosis

*E*OM involvement

*C*orneal involvement

*S*ight (optic neuropathy)

What is the time course of TED?

self limiting → active inflammatory phase with worsening signs and symptoms followed by acute phase followed by plateau and inactive phase

What is the management for TED (how are you following this patient)?

monitor for progression to optic neuropathy with frequent F/U + self monitoring for red desaturation

What is the treatment for TED?

1. control thyroid state

2. systemic steroids

3. surface disease tx

4. EOM surgery

5. Tepezza

6. orbital radiotherapy

7. orbital decompression surgery

what is Tepezza?

monoclonal antibody

What were the results of the two studies that look at the effect of Tepezza?

Study 1 found that 71% of participants had a >2mm decrease in proptosis

Study 2 found the same but for 83% of participants

What is myasthenia gravis?

antibodies to the Ach receptor at neuromuscular junction interrupt normal function

What is often the first symptom in myasthenia gravis?

ptosis → 50%

What are the symptoms of myasthenia gravis?

1. ptosis + diplopia that worsen as day progresses

2. muscle group weakness → including pharyngeal muscles causing voice changes

3. difficult swallowing or breathing

What are the signs of myasthenia gravis?

1. various ocular motility defects

2. ptosis that worsens with extended upgaze

3. Cogan sign

4. if ptotic lid is manually elevated, other lid droops more

What is cogan sign?

looking in downgaze to primary → lids overshoot/twitch → then comes back to ptosis position

What are the tests used to diagnose myasthenia gravis?

1. ice test → ice over closed lid improves ptosis and EOM fxn

2. rest test → ptosis improves

3. tensilon test

4. blood test for antibodies

What is the treatment for myasthenia gravis?

1. anti-AchE

2. corticosteroids

3. immunosuppressants

4. thymectomy