Lids 3 - Blepharoptosis

Question 1

Ptosis physiological features that aid diagnosis?

Answer 1

• Drooping of the upper lid Upper lid covers more than 2mm (one sixth) of the cornea
• Narrowing of palpebral fissure
• Raising of brows due to frontalis over-action
• Chin up head posture in bilateral ptosis

Question 2

What are the Classifications if Pseudootosis?

Answer 2

•Enophthalmos
•Dermatochalasis
• Micro-ophthalmos
•Phthisis bulbi
•Hypotropia
• Contralateral eye; herrings law
- Eyelid retraction

Question 3

What are the Classifications of Ptosis:

Answer 3

•Congenital
- Myogenic
- Neurogenic

•Acquired
- Aponeurotic
- Myogenic
- Neurogenic
- Traumatic
- Mechanical

Question 4

Congenital Ptosis Classification and types in each classification:-

Answer 4

• Neurogenic
- Marcus Gunn Jaw winking ptosis
- Horners syndrome

•Myogenic
- Simple congenital Ptosis
Associated with superior rectus dysfunction
- Associated with a Syndrome (commonly BEPS)

Question 5

Features of Myogenic Congenital Ptosis?

Answer 5

• Levator muscle
•Absent weak lid crease
• Lid lag on down gaze (levator is stiff muscle)

Question 6

What does Superior rectus dysfunction cause?

Answer 6

• Causes poor/ absent Bell’s phenomenon
• Increases risk of exposure keratopathy with ptosis surgery

Question 7

What is, Blepharophimosis Ptosis Epicanthus Inversus Syndrome, Or BPES?

Answer 7

• Associated with myogenic congenital ptosis
• Autosomal Dominant inheritance
• Blepharophimosis: decreased vertical Palpebral aperture
• Ptosis
• Epicanthus Inversus
• Telecanthus - Increased distance between medical canthi

Question 8

Neurogenic Congenital Ptosis types?

Answer 8

•Marcus Gun Jaw winking Ptosis
• Congenital Horners syndrome

Question 9

What is Marcus jaw winking ptosis?

Answer 9

•Causes a: Congenital neurogenic Synkinetic ptosis
• Aberrant connections between cranial nerves IlI and V (motor branches to pterygoids)
• Jaw movement (contraction of the pterygoid muscle) elevates the ptotic lid

Question 10

What is Congenital Horners syndrome and its signs?

Answer 10

•Neurological syndrome that disrupts sympathetic nerve branch to eye.

Signs:-
• Mild Ptosis
• Miosis
• Heterochromia due to hypopigmentation of affected Iris

Question 11

What defects are associated with congenital ptosis?

Answer 11

• Amblyopia is present in 20% with congenital ptosis
• Ptotic lid obscures visual axis causing:
- Anisometropia
- High astigmatism
- Strabismus

Question 12

What is Aponeurotic acquired ptosis? And how does it present?

Answer 12

• Connection between levator palpebral connection to tarsal plate, via aponeurosis
•Most common
• Usually age related
• Thinning or disinsertion of levator aponeurosis
• Disinsertion from tarsal plate, causes retraction of aponeurosis

• Thinned upper lid, deep sulcus
• Higher upper lid crease (>8-10mm)
• Near normal levator function
• Absent lid lag on down gaze

Question 13

What are the differences between Congenital Myogenic vs Acquired Aponeurotic ptosis regarding (severity, upper crease, levator function and eyelid on downgaze)?

Answer 13

•Congenital Myogenic
- Mild to severe ptosis
- Weak or absent upper crease
- Reduced levator function
- Eyelid lag on downgaze
• Acquired Aponeurotic
- Mild to severe ptosis
- Higher than normal upper crease
- Near normal levator function
- Eyelid drop on downgaze

Question 14

Which levels can Myogenic acquired ptosis affect?

Answer 14

• At level of the muscle
- Myotonic dystrophy
- Chronic progressive external ophthalmoplegia (CPEO)

• At level of myoneural junction:-
- Myasthenia Gravis
(Ocular myasthenia)

Question 15

Muscular Dystrophies 2 types and their associated signs?

Answer 15

• Myotonic Dystrophy
- Bilateral symmetrical progressive ptosis
- Christmas Tree cataract
- Myopathic facies
- Cardiac conduction abnormalities

• СРЕО
- Bilateral symmetrical progressive ptosis
- Involvement of other extraocular muscles
- Pigmentary retinopathy
- Cardiac conduction abnormalities

Question 16

What is Myasthenia Gravis and its symptoms and signs?

Answer 16

• Autoimmune disorder
• Antibodies to acetylcholine receptors

Symptoms:
• Easy fatiguability
• Life threatening symptoms - dysphagia and dyspnoea
• Fluctuating ptosis characteristic presentation

Signs:
- Ptosis and diplopia
- Ptosis worsens on prolonged up-gaze
- Pupil always normal
- Consider myasthenia in every case of ptosis or diplopia

Question 17

Myaesthenia Gravis - Diagnosis tests and treatment

Answer 17

• Tensilon (Edrophonium chloride) test is the classical diagnostic test to confirm diagnosis
- IV anti-acetylcholinesterase drug, ptosis improves on injection
• Other tests
- Ice pack test - improves ptosis
- Prolonged up-gaze - worsens
ptosis
- Rest/ sleep test - improves ptosis

•Anti cholinesterase drugs
• Corticosteroids
• Immunosuppressants
• Thymectomy

Question 18

Adult Myogenic Ptosis cause and concern?

Answer 18

• Ptosis with reduced levator function
• Additional systemic abnormalities which need to be investigated

Question 19

Acquired neurogenic cause and categories:

Answer 19

• 3rd nerve paralysis
- levator muscle
- Superior rectus
- Inferior rectus
- Medial rectus
- Inferior oblique

• Horners syndrome

Question 20

Oculomotor nerve palsy:-

Answer 20

• Ptosis
• Eyeball down and out
• Only abduction and intorsion movements present
• Pupil may or may not be involved
• Accommodation absent

Question 21

Acquired Oculomotor nerve palsy divisions and their possible causes:-

Answer 21

• Vasculopathic causes
- Diabetes
- Atherosclerosis
- Hypertension

• Compressive causes
- Aneurysm
- Neoplasm

Question 22

Vasculopathic palsy presentation?

Answer 22

• Sudden onset
• Pupil sparing
• Recovery within 3-6months

Question 23

Compressive nerve palsy presentation

Answer 23

• Total or partial
• Progressive symptoms
• Pupil involved
• Emergency workup

Question 24

What causes Acquired horners syndrome: And what are the signs?

Answer 24

• Causes
- Intracranial aneurysm/tumour/inflammation
- Pancoast’s tumour/ carotid aneurysms/ malignant cervical lymph nodes

Signs:
• Mild ptosis as a rule
• Miosis
• Normal pupillary reactions
• Anhydrosis

Question 25

Acquired - Traumatic causes:-

Answer 25

• Orbital injury

Question 26

Mechanical acquired ptosis causes:

Answer 26

• Chalazion • hordeolum • Tumour • Something “mechanically weighs down lid”

Question 27

What are checked during slit lamp exam of Ptosis:-

Answer 27

• Measurement of ptosis:- - Palpebral fissure height - Marginal reflex distance - upper lid crease and fold - Levator excursion • Always check EOM, pupils, synkinesis, dryness

Question 28

Levator Function classification:- (and how they are surgically managed)

Answer 28

•15mm - Normal • Greater or equal 8mm - Good - Mullers muscle - conjunctival resection MMCR - Levator aponeurosis advancement • 5-7mm - Fair - Levator resection • Less or equal to 4mm - Poor - Frontalis brow suspension