2. Functional anatomy
• Levator palpebrae superioris (LPS):
– is the primary muscle responsible for lid elevation.
– It arises from the back of the orbit and extends forwards
over the cone of eye muscles.
– It inserts into the eyelid and the tarsal plate, a fibrous
semicircular structure which gives the upper eyelid its
shape.
– The LPS is supplied by the superior division of the
oculomotor nerve.
• Muller’s muscles:
– The way that the LPS attaches to the tarsal plate is modified
by the underlying Müller's muscle.
– This involuntary muscle, comprising sympathetically
innervated smooth muscle,
– has the capacity to 'tighten' the attachment and so raise the
lid a few millimetres.
3.
4. • Frontalis & orbicularis oculi:
– frontalis muscle and the orbicularis oculi, both supplied
by the facial nerve.
– Frontalis contraction helps to elevate the lid by acting
indirectly on the surrounding soft tissues,
– while orbicularis oculi contraction depresses the eyelid.
5. DEFINITION
• Ptosis (from Greek Ptosis -to "fall") is a
drooping or falling of the upper or lower
eyelid.
6. CLASSIFICATION OF PTOSIS
A. Congenital
B. Acquired
1.
2.
3.
4.
5.
Neurogenic
Myogenic
Aponeurotic
Mechanical
Neurotoxic
C. Pseudotosis
7. Congenital ptosis
• It is associated with congenital weakness (maldevelopment)
of the levator palpebrae superioris (LPS).
1. Simple congenital ptosis
– not associated with any other anomaly.
2. Congenital ptosis with associated weakness of superior
rectus muscle.
3. Blepharophimosis syndrome,
– which comprises congenital ptosis, blepharophimosis,
telecanthus and epicanthus inversus .
4. Congenital synkinetic ptosis
– (Marcus Gunn jaw winking ptosis).
– In this condition there occurs retraction of the ptotic lid with jaw
movements i.e., with stimulation of ipsilateral pterygoid muscle.
9. Blepharophimosis syndrome
•
Rare congenital disorder
•
Dominant inheritance
•
•
•
•
•
•
Moderate to severe symmetrical ptos
Short horizontal palpebral aperture
Telecanthus (lateral displacement
of medial canthus)
Epicanthus inversus (lower lid
fold larger than upper)
Lateral inferior ectropion
Poorly developed nasal bridge
and hypoplasia of superior orbital
rims
10. Congenital synkinetic ptosis
• This condition is characterized as a synkinesis:
when two or more muscles that are
independently innervated have either
simultaneous or coordinated movements.
• In MARCUS GUNN PHENOMENON
– The stimulation of the trigeminal nerve by
contraction of the pterygoid muscles of jaw results in
the excitation of the branch of the oculomotor nerve
that innervates the LPS ipsilaterally, so the patient
will have rhythmic upward jerking of their upper
eyelid.
11. • There are two major groups of trigeminooculomotor synkineses:
1) External pterygoid-levator synkinesis:
– is when the eyelid raises upon Jaw thrust to opposite
side (homolateral external pterygoid) Jaw is projected
forward (bilateral external pterygoid) Mouth is opened
widely
2) Internal pterygoid-levator synkinesis
– is when the eyelid raises upon teeth clenching
• External pterygoid-levator synkinesis is the more
common group.
12. Marcus Gunn jaw-winking syndrome
• Accounts for about 5% of all cases of congenital ptosis
• Retraction or ‘wink’ of ptotic lid in conjunction with
stimulation of ipsilateral pterygoid muscles
Opening of mouth
Contralateral movement of jaw
13. • Inverse Marcus Gunn phenomenon:
– Synkinesia between CN V and the levator more rarely causes ptosis on
mouth opening .
• Marin Amat syndrome:
– is a facial nerve aberrant innervation syndrome with levator inhibition
with mouth opening.
• Ptosis in Lambert-Eaton syndrome :
– may temporarily improve after a brief period of upgaze.
• Eyelid myotonia :
– may cause transient difficulty opening the eyes after a forceful
contraction or transient lid retraction after looking up.
• Blepharospasm :
– is a focal dystonia causing involuntary eye closure;
– levator function is normal.
• In apraxia of lid opening,
– the patient has difficulty in voluntarily initiating lid elevation although
there is no levator impairment or blepharospasm.
• Rosenbach's sign:
– A fine tremor of the lid may occur in hyperthyroidism
15. A. Neurogenic ptosis
• It is caused by innervational defects such as
third nerve palsy,
• 3rd nerve misdirection
• Horner’s syndrome,
• Ophthalmoplegic migraine
• Cerebral ptosis
• Multiple sclerosis.
16. Right third nerve misdirection
• Rare, unilateral
• Aberrant regeneration following acquired third nerve palsy
• Pupil is occasionally involved
• Bizarre movements of upper lid accompany eye movements
Right ptosis in primary
position
Worse on right gaze
Normal on left gaze
17. Horner syndrome
Central
(first order neurone)
Posterior hypothalamus
•
•
Brainstem disease
(vascular, demyelination)
Spinal cord disease
(syringomyelia, tumours)
Pre-ganglionic
(second order neurone)
Superior cervical
ganglion
Intrathoracic lesions
(Pancoast tumour, aneurysm)
•
•
Neck lesions
(glands, trauma)
Post-ganglionic
(third order neurone)
Ciliospinal centre of
Budge( C8 - T2 )
Internal carotid artery disease
• Cavernous sinus mass
•
18. Phenyl ephrine test
• Patients with minimal ptosis (2 mm or less) should
have a phenylephrine test performed in the involved
eye or eyes
• Either 2.5 or 10% phenylephrine is instilled in the
affected eye or eyes. Usually two drops are placed
and the patient is reexamined 5 minutes later.
• The MRD1 is rechecked in the affected and
unaffected eyes .
• A rise in the MRDl of 1.5 mm or greater is
considered a positive test. This indicates that
Müller's muscle is viable
19. Ophthalmoplegic migraine
• Ophthalmoplegic Migraine is a rare eye disorder, previously
called a “complicated migraine”, which is also recognized as
cranial neuralgia by the International Classification of
Headache Disorders (HIS II) .
• This disorder most commonly presents itself in early childhood
or infancy.
• To date, there is no conclusive hypothesis as to the etiology of
this disorder (3, 4).
• Ophthalmoplegic migraines are characterized by
– Severe headaches
– weakening of muscles around the eye.
– these headaches commonly precede episodes of partial paralysis of
one or more ocular nerve (most commonly the third cranial nerve),
– drooping of the eyelid,
– double vision,
– dilation of pupils
20. IHS diagnostic criteria:
• At least 2 attacks fulfilling criterion B
• Migraine-like headache accompanied or
followed within 4 days of its onset by paresis
of one or more of the third, fourth and/or
sixth cranial nerves
• Parasellar, orbital fissure and posterior fossa
lesions ruled out by appropriate investigations
21. Cerebral ptosis
• is due to supranuclear lesions.
• Unilateral cerebral ptosis occurs with lesions,
usually ischemic, of the opposite hemisphere, and
is more common with right hemisphere lesions.
• Bilateral supranuclear ptosis may occur with
unilateral or bilateral hemispheric lesions.
• Ptosis has been reported in as many as 37.5% of
patients with hemispheric strokes.
22. B. MYOGENIC PTOSIS
• It is due to acquired disorders of the LPS
muscle or of the myoneural junction.
1. Myasthenia gravis
2. Myotonic dystrophy
3. Ocular myopathies
4. oculo-pharyngeal muscular dystrophy
5. following trauma to the LPS muscle.
23. Myasthenia Gravis
• The ptosis in MG is frequently asymmetric and may
be unilateral, though it will tend to shift from side
to side
• It characteristically fluctuates from moment to
moment and is worsened by prolonged upgaze
(fatiguable ptosis).
• Cogan's lid twitch sign,
– characteristic of myasthenia, consists of a brief
overshoot twitch of lid retraction following sudden
return of the eyes to primary position after a period of
downgaze.
24. • Curtain sign, seesaw ptosis:
– When the ptosis is asymmetric, the driving discharges
attempting to keep the more ptotic eyelid open are also
transmitted, per Hering's law, to the less ptotic eyelid.
– Manually raising the more ptotic lid causes relaxation
and the eye with less ptosis, sometimes even no ptosis,
may suddenly crash.
25. Myasthenia Gravis
2. Investigations
•
Edrophonium (Camiston) test
•
Electromyography to confirm fatigue
•
Antibodies to acetylcholine receptors
•
CT or MRI for presence of thymoma
3. Treatment options
•
Medical - anticholinesterases, steroids and azathioprine
•
Thymectomy
27. Edrophonium (Tensilon) Test:
• Edrophonium chloride inhibits acetylcholinesterase,
• thereby prolonging the presence of acetylcholine at the neuromuscular
junction.
• This results in enhanced muscle strength.
• In ptosis, a positive test is the elevation of eyelids in 2-5 minutes post
adminstration of Tensilon.
• A negative response is no improvement within 3 minutes.
• the Tensilon test has a relatively low sensitivity, approximately 60% for
MG.
• False positive results occur in patients with
– Lambert-Eaton Myasthenic Syndrome (LEMS),
– Amyothrophic Lateral Sclerosis (ALS), and
– localized intracranial mass lesions.
• Edrophonium chloride can cause overactivation of the parasympathetic
system, and cause unwanted side effects like fainting, dizziness,
involuntary defecation, severe bradycardia, apnea, and even cardiac
arrest. It is important to always have atropine at hand if such side
effects should occur
28. Edrophonium test
Before injection
•
•
Measure amount of ptosis or
diplopia before injection
Inject i.v. atropine 0.3 mg
Positive result
•
•
Inject i.v. test dose of edrophonium
(0.2 ml-2 mg)
Inject remaining (0.8 ml-8 mg) if no
hypersensitivity
29. Myotonic dystrophy
Facial weakness and
ptosis
Release of grip difficult
Hypogonadism
• Frontal baldness in males
• Involvement of tongue and pharyngeal muscles
• Intellectual deterioration
• Ophthalmoplegia - uncommon
• Presenile stellate cataracts
•
Muscle wasting
•
30. Ocular myopathies
Ocular features
Clinical types
•
•
•
Isolated
Ptosis - slowly progressive
and symmetrical
Oculopharyngeal dystrophy
• Ophthalmoplegia - slowly
Kearns-Sayre syndrome
progressive and symmetrical
(pigmentary retinopathy)
(no diplopia)
•
31. C. APONEUROTIC PTOSIS
• It develops due to defects of the levator
aponeurosis in the presence of a normal
functioning muscle.
• It includes
– involutional (senile) ptosis,
– postoperative ptosis
• Ptosis due to aponeurotic weakness associated with
blepharochalasis (Blepharochalasis is an inflammation of the eyelid that is
characterized by exacerbations and remissions of eyelid edema, which results in a
stretching and subsequent atrophy of the eyelid tissue resulting in redundant folds
over the lid margins)
– Posttraumatic dehiscence or disinsertion of the
aponeurosis.
32. Aponeurotic ptosis
Weakness of levator aponeurosis
• Causes - involutional, postoperative and blepharochalasis
•
Mild
High upper lid crease
Good levator function
Severe
Absent upper lid crease
Deep sulcus
33. Senile ptosis
• Senile or involutional ptosis is very common.
Asymmetric lids and redundant lid tissue in the
elderly.
• The levator aponeurosis attaches the levator muscle
to the tarsal plate, which forms the eyelid.
• Aging may cause levator dehiscencedisinsertion
(LDD)—with stretching, thinning, or detachment of
the aponeurosis.
• Normally, with the eyelids gently closed, the upper
lid margin lies 5 mm to 7 mm below the upper lid
fold (the skin fold at the upper part of the lid).
• An increase in this distance suggests LDD.
34.
35. D. Mechanical ptosis
• Due to excessive weight on the upper lid
– lid tumours,
– multiple chalazia
– lid oedema.
• Cicatricial Ptosis
– ocular pemphigoid
– trachoma.
37. E. NEUROTOXIC PTOSIS
• Envenomation by elapids such as cobras, or
kraits.
– Bilateral ptosis is usually accompanied by diplopia,
dysphagia and/or progressive muscular paralysis.
– Regardless, neurotoxic ptosis is a precursor to
respiratory failure and eventual suffocation
caused by complete paralysis of the thoracic
diaphragm.
– It is therefore a medical emergency and
immediate treatment is required.
38. PSEUDOPTOSIS
• Pseudoptosis is the appearance of ptosis in the
absence of levator abnormality.
• Exclude pseudoptosis (simulated ptosis) on
inspection.
• Its common causes are:
– microphthalmos,
– anophthalmos,
– enophthalmos
– Phthisis bulbi.
– Double elevator palsy
– Blepharospasm
– Contralateral proptosis
39. • Blepharochalasis (dermatochalasis)
– refers to age-related lax, baggy skin around the
eyelids;
– it can also simulate ptosis but levator function is
normal
• Duane's syndrome
– the palpebral fissure narrows on ocular adduction
because of globe retraction causing dynamic
enophthalmos.
40. Causes of pseudoptosis
Lack of lid support
Ipsilateral hypotropia
Contralateral lid retraction
Brow ptosis - excessive
eyebrow skin
Dermatochalasis - excessive
eyelid skin
44. HISTORY
• Ptosis
– Age of onset
– Duration
– One/both eye
– Diurnal variability
• Associated history :
– Diplopia
– Dysphagia
– Muscle weakness
• Vision
45. • Association with
– Jaw movements
– Abnormal ocular movements
– Abnormal head posture
• History of
– Trauma or previous surgery
– Poisoning
– Use of steroid drops
– Any reaction with anesthesia
– Bleeding tendency
• Previous photographs may prove to be of great
help.
• Is there a family history of ptosis or of other muscle
weakness?
46. Ocular Examination:
NORMAL POSITION OF EYELIDS
• The normal upper eyelid in primary position
– crosses the iris between the limbus (junction of the iris
and sclera) and the pupil,
– usually 1 mm to 2 mm below the limbus;
– the lower lid touches or crosses slightly above the
limbus.
– Normally there is no sclera showing above the iris.
• The palpebral fissures:
– are normally 9 mm to 12 mm from upper to lower lid
margin.
47. Ptosis
• U/L or B/L
• Complete /incomplete
• Total unilateral ptosis
– complete third nerve palsy.
• Mild to moderate unilateral ptosis
– Horner's syndrome,
– partial third nerve palsy.
• Mild to moderate bilateral ptosis
– neuromuscular disorders, such as MG,
– muscular dystrophy,
– Ocular myopathy.
48. • Head Posture:
– chin elevation as the ptosis is minimum in downgaze in a
patient with congenital ptosis is also one of the indications
for surgery especially in the pediatric age group.
– In ptosis there will be superior altitudinal defect which is
corrected by elevating the eyelids
• Ocular Motility:
– Importance in myogenic ptosis,
– To R/O 3rd nerve palsy
– presence of strabismus, especially vertical strabismus entails
that it be corrected prior to the correction of the ptosis.
• Visual acuity
– Best-corrected visual acuity should be assessed to record any
amblyopia if present, especially in cases of congenital ptosis.
• Pupillary Examination:
– TO diagnosis Horner’s syndrome
– Involvement in a case of third nerve palsy
50. 1. MARGIN REFLEX DISTANCE
• Margin-to-reflex distance 1 (MRD1) :
– When light is thrown on the cornea a reflection occurrs
.the distance from the central pupillary light reflex to the
upper eyelid margin with the eye in primary gaze.
• NORMAL : 4 - 5 mm.
• If the margin is above the light reflex the MRD 1 is a
+ve value.
• If the lid margin is below the corneal reflex in cases
of very severe ptosis the MRD 1 would be a –ve
value.
51.
52. Marginal reflex distance
• Distance between upper lid
margin and cornal light reflex
(MRD)
• Mild ptosis (2 mm of droop)
• Moderate ptosis (3 mm)
• Severe ptosis (4 mm or more)
53. 2. Vertical fissure height
• The distance between the upper and lower
eyelid in vertical alignment with the center of
the pupil in primary gaze, with the patient’s
brow relaxed.
• Normal – 9-10mm in primary gaze
• Should be seen in up gaze, down gaze and
primary gaze
• Amount of ptosis = difference in palpebral
apertures in unilateral ptosis or Difference
from normal in bilateral ptosis
54.
55. Grading of severity of ptosis
< or = 2mm : mild ptosis
= 3 mm : moderate ptosis
= or > 4 mm : severe ptosis
56. 3. Levator function assesment
• It is determined by the lid excursion caused by LPS
muscle (Burke’s method).
– Patient is asked to look down, and thumb of one hand is
placed firmly against the eyebrow of the patient (to block
the action of frontalis muscle) by the examiner.
– Then the patient is asked to look up and the amount of
upper lid excursion is measured with a ruler held in the
other hand by the examiner.
– Levator function is graded as follows:
•
•
•
•
Normal 15 mm
Good 8 mm or more
Fair 5-7 mm
Poor 4 mm or less
57. Upper lid excursion- burke’s ,method
•
Reflects levator function
•
Normal (15 mm or more)
•
Good (8 mm or more)
•
Fair (5-7 mm)
•
Poor (4 mm or less)
58. Upper lid crease
Pretarsal show
fold
crease
•
•
•
•
Distance between lid margin and lid
crease in down-gaze
Normals - females 10 mm; males 8 mm
Absence in congenital ptosis indicates
poor levator function
High crease suggests an aponeurotic
defect
•
Distance between lash line and skin
fold in primary position of gaze
