Blepharoptosis

Question 1

What is the diagnosis of Blepharoptosis?

Answer 1

• Upper lid covers > 2mm (1/6th) of cornea, lower lid should sit at cornea
• Narrowing of vertical palpebral fissure
• Raising of brows due to frontalis over-action
• Chin up head posture in bilateral ptosis – particularly children

Question 2

What does the Superior Tarsal Plate connect to?
And what is this connection made via (& where does this structure insert)?

Answer 2

Connects to levator palpebrae superioris muscle (crucial muscle in raising lid) via the aponeurosis
Aponeurosis inserts not only into tarsal plate but also into skin of upper lid

Question 3

Where does Muller’s muscle run and how is it innervated?

Answer 3

Running just behind levator palpebrae superioris muscle is Muller’s muscle which is sympathetically innervated (rather than 3rd nerve innervation)

Question 4

Which muscles are supplied by CNIII? And which of these two are intimately linked?

Answer 4

SR and levator palpebrae superioris are intimately linked and both supplied by 3rd nerve. 3rd nerve also supplies IR, MR and IO

Question 5

Which two muscles raise the lid?

Answer 5

85% of raising done by levator palpebrae superioris muscle – runs above SR
Muller’s muscle contributes to ~15% lid raising

Question 6

Where does Horner’s muscle run and which nerve innervates it? What is another name for the muscle?

Answer 6

At front of lid, have superior tarsus muscle – aka Horner’s muscle – runs between tarsal plate and posterior part of the aponeurosis and it is supplied by the sympathetic nerve

Question 7

What is pseudoptosis?

Answer 7

If globe is much smaller as you have in phthisis or microphthalmos, lid sits much lower on globe and look like ptosis = pseudoptosis

Question 8

Examples of Pseudoptosis?

Answer 8

• Enophthalmos – if got orbital floor fracture, globe will sit much lower down in orbit and look like got a ptosis
• Dermatochalasis – vast excess of upper lid skin, can hang over lid and looks like ptosis – lid height when take away upper lid skin is normal
• Micro-opthalmos
• Phthisis bulbi
• Hypotropia
• Contralateral eye -> eyelid retraction
  o e.g. In thyroid eye disease, have unilateral eyelid retraction. Due to Hering’s Law of equal innervation – have less stimulation to levator superioris in contralateral eye because of eyelid retraction. So, in the ipsilateral eye there is an apparent ptosis as the nerves are not letting it be raised as much

Question 9

Describe Simple Congenital Ptosis? Is it myogenic or neurogenic?

Answer 9

• Majority causes by deficiencies in levator muscle
• Hallmark features:
  o Absence or weak lid crease – depends on amount of function left in levator muscle – so v wide variation of function of the muscle – if levator muscle has no function, then will not get a skin crease at all
  o Lid lag on downgaze (levator stiffness & does not relax properly) – child may not close eye fully when they sleep

Myogenic

Question 10

Describe Ptosis Associated with Superior Retuc Dysfunction? Is is myogenic or neurogenic? Is it congenital or acquired?

Answer 10

• Causes poor/ absent Bell’s phenomenon
• ↑ risk of exposure keratopathy with ptosis surgery
• If have combined levator and SR dysfunction – i.e. a double elevator palsy – as well as lid not being able to raise, the eye cannot raise itself and so if you operate on levator muscle & cause lid to be higher up – have an ↑ chance of palpebral aperture not closing properly (and lagophthalmos occurring)
  o Normally in children this isn’t an issue as have good Bell’s phenomenon – eye automatically rotates upwards when have lids coming down – protective measure for cornea
  o With absent Bell’s phenomenon, can raise the lid, possibly give child lagophthalmos, but no protective mechanism to make sure the cornea is underneath lid and covered and continually lubricated and therefore ↑ risk of exposure keratopathy when do ptosis surgery
• Vitally important for these children that they have a full orthoptic assessment to establish if there is SR dysfunction before going ahead with surgery – and may not want to go ahead with surgery because of this

Myogenic
Congenital

Question 11

Describe ptosis due to BPES? What does BPES stand for? Is it myogenic or neurogenic? Is it congenital or acquired?

Answer 11

Blepharophimosis Ptosis Epicanthus Inversus Syndrome (BPES):
* Most common syndrome associated with myogenic congenital ptosis
* Autosomal dominant inheritance – runs in families
* Blepharophimosis – decrease in palpebral aperture (distance between the 2 lids)
* Ptosis
* Epicanthus Inversus – fold of skin running from upper lid to lower lid
* Telecanthus – increase space between 2 medial canthi

Myogenic
Congenital

Question 12

Describe Marcus Gunn jaw winking ptosis? Is it myogenic or neurogenic? Is it congenital or acquired?

Answer 12

• Congenital dyskinesis/ abnormal connection between nerve endings
• Congenital neurogenic synkinetic ptosis
• Aberrant connections between CN III and V (motor branches to pterygoids)
• Levator innervated by 5th CN rather than 3rd CN, nerve muscles usually involved in movement of mouth go up and innervate the lid
  o When mouth moves, lid moves
• Vast variation in prominence – some have little wink and some have really obvious wink
• Jaw movement (contraction of pterygoid muscle) elevate the ptotic lid

Neurogenic
Congenital

Question 13

Describe ptosis due to Horner’s Syndrome? Is it myogenic or neurogenic? Is it congenital or acquired?

Answer 13

• Disruption in sympathetic innervation – sympathetic nerve supplies Horner’s muscle which is involved in 15% of the elevation of the lid
• Mild ptosis
• Miosis – as sympathetic nerve also supplies this
• Heterochromia due to hypopigmentation of affected iris
• Congenital Horner’s different from acquired Horner’s (in adults) as acquired Horner’s NEVER get heterochromia

Neurogenic
Congenital

Question 14

Describe the visual function in congenital ptosis?

Answer 14

• Amblyopia is present in 20% with congenital ptosis
• Not only can have the ptotic lid obscuring visual axis but also higher prevalence of:
  o Anisometropia
  o High astigmatism
  o Strabismus
• Vitally important that as well as assessment of ptosis in the child, they also need a full orthoptics assessment & full refractive assessment too

Question 15

Describe aponeurotic acquired ptosis?

Answer 15

• Looking at connection between levator palpebrae superioris muscle and its connection to the tarsal plate
  o via levator aponeurosis (grey part on diagram)
• Most common acquire ptosis
• Usually, age related
• Thinning or disinsertion of levator aponeurosis into tarsal plate and into skin
• Disinsertion from tarsal plate causes retraction of aponeurosis
• Signs:
  o Thinned and deep upper lid sulcus
  o Higher than normal upper lid skin crease (>8-10mm)
  o Near normal levator function – measure of lid excursion from downgaze to up gaze)
  o Absent lid lag on down gaze – eyelid drop on down gaze

Question 16

Describe Myogenic Acquired Ptosis?

Answer 16

• Mainly seen in older people but can occur in late teens, early 20s
• Ptosis caused by muscular issue
• At level of muscle
  o Myotonic Dystrophy
  o Chronic Progressive External Ophthalmoplegia (CPEO)
• At level of myoneural junction (where muscle meets nerves)
  o Myasthenia Gravis
  o Ocular Myasthenia
• Defining feature: ptosis with reduced levator function (levator function around 15mm)
• When do standard ptosis assessment: palpebral aperture, lid show, skin crease, marginal reflex distance, distance between pupil and lid margin and levator function
• Additional systemic abnormalities which need to be investigated – full ocular assessment then referral to cardiology

Question 17

Describe myotonic dystrophy?

Answer 17

• Bilateral symmetrical progressive ptosis
• Autosomal dominantly inherited
• Christmas tree cataract
• Myopathic facies – characteristic appearance to face – hang dog face
• Cardiac conduction abnormalities – pxs sent for cardiology opinion as well as having full eye assessment

Question 18

Describe CPEO and what does it stand for?

Answer 18

Chronic Progressive External Ophthalmoplegia (CPEO)

• Bilateral symmetrical progressive ptosis
• Involvement of other EOMs as well as levator – px can’t look up, down, left or right – so slow and progressive that px often does not realise – no diplopia involved as v symmetrical
• Pigmentary retinopathy
• Cardiac conduction abnormalities – pxs sent for cardiology opinion as well as having full eye assessment

Question 19

Describe Myasthenia Gravis?

Answer 19

• Clinical Features:
  o Fluctuating ptosis is characteristic presentation
  o Can be EOM problem as well as ptosis
  o Autoimmune disorder
  o Antibodies to acetylcholine receptors
  o Easy fatiguability – ptosis worsens on prolonged up-gaze
  o Life threatening symptoms – dysphagia & dyspnoea
  o Ptosis and diplopia
  o Pupil always normal
  o Consider myasthenia in every case of ptosis or diplopia
• Diagnosis & Treatment:
  o Tensilon (Edrophonium chloride) test is the classical diagnostic test to confirm diagnosis
   IV anti-acetylcholinesterase drug, ptosis improves on injection
  o Other tests:
   Ice pack test – should improve ptosis
   Prolonged up-gaze – should worsen ptosis
   Rest/sleep test – should improve ptosis
  o Anticholinesterase drugs
  o Corticosteroids – particular if px also has systemic symptoms
  o Immunosuppressants
  o Thymectomy

Question 20

Describe Neurogenic Acquire ptosis due to oculomotor (CNIII) nerve paralysis?

Answer 20

o Ptosis
o EOM disorders – eyeball being down and out and only abduction (LR still being supplied) and intorsion (SO still being supplied) movements present
o Pupil may or not be involved – particularly worried if pupil is involved
o Accommodation is absent
o Vasculopathic Causes – something interrupting blood supply:
 Diabetes
 Hypertension
 Atherosclerosis
* Sudden onset
* Pupil sparing
* Recovery within 3-6 months – often spontaneous
o Compressive Causes:
 Aneurysm
 Neoplasm
* Total or partial progressive symptoms
* Pupil involved
* Emergency workup
o Any 3rd nerve gets imaged now as can occasionally get pupil sparing in compressive causes

Question 21

Describe Neurogenic Acquire ptosis due to Horner’s syndrome? What is another name for it?

Answer 21

oculosympathetic palsy

o Mild ptosis as a rule – ptosis is caused by abnormality of Horner’s muscle – smooth muscle supplied by the sympathetic output – only supplies 15% of the raising of the lid so ptosis is mild as a rule
 Miosis
 Normal pupillary reactions
 Enophthalmos – as well as ptosis, have inverse ptosis of lower lid – which makes the palpebral aperture smaller & the enophthalmos is an apparent enophthalmos
 Anhidrosis – lack of sweating on that side – sweating supplied by the sympathetic nerve
o Causes:
 Intracranial aneurysm/tumour/inflammation
 Pancoast’s tumour/carotid aneurysms/malignant cervical lymph nodes – tumour along the sympathetic pathway
o Must image the lung as can get tumours here too

Question 22

Describe traumatic acquire ptosis?

Answer 22

• Often follow an orbital injury

Question 23

Describe mechanical acquired ptosis?

Answer 23

• Something weighing down the lid so difficult for lid to keep raised
• e.g. inflammatory mechanical ptosis cause by a chalazion
• plexiform neurofibroma – ‘S’ shaped deformity of upper lid where tumour is pulling lid down – commonly seen in condition called neurofibromatosis

Question 24

Describe the management for ptosis?

Answer 24

• Assessment:
  o Measurements in ptosis:
   Palpebral fissure height – palpebral aperture – vertical distance between the 2 lids
   Marginal reflex distance (MRD) 1 – distance between pupillary light reflex when shine a light into the eye & upper lid margin – often only take MRD 1
• MRD 2 – distance between pupillary light reflex and lower lid margin
   Upper lid crease and fold
   Levator excursion:
• Ask px to look down then fixate (place finger on) brow so the frontalis cannot cloud any readings taken
• Position ruler so bottom end is at level of lid as px is looking down
• Then get px to maximally look up and then record how far the lid has moved up on the ruler
• V difficult in children, more of an estimation
   Lid show
   EOM, pupils, synkinesis – e.g. Marcus Gunn, dryness or anhidrosis – can inform of other conditions which are coexisting

Question 25

What is the classification of levator function?

Answer 25

Levator muscle excursion of 15mm or above = Normal
Levator muscle excursion of greater than or equal to 8mm = Good
Levator muscle excursion of 5-7mm = Fair
Levator muscle excursion of less than or equal to 4mm = Poor