ptosis, types, clinical features, associated syndromes and management. credits: by my colleague Dr. Akansha Sharma

1. PTOSIS
BY- DR. PRIYANKA RAJ

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,
– It has the capacity to 'tighten' the attachment and so raise the lid a few millimetres.

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
A. Congenital
B. Acquired
1. Neurogenic
2. Myogenic
3. Aponeurotic
4. Mechanical
5. 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

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

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.

14. • 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. ACQUIRED PTOSIS
1. Neurogenic
– Third nerve palsy
– Third nerve misdirection
– Horner syndrome
2. Myogenic
3. Aponeurotic
4. Mechanical
5. Neurotoxic

16. 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.

17. 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

18. Horner syndrome
Central (first order neurone)
• Brainstem disease (vascular, demyelination)
• Spinal cord disease (syringomyelia, tumours)
Pre-ganglionic (second order neurone)
• Intrathoracic lesions (Pancoast tumour, aneurysm)
• Neck lesions (glands, trauma)
Post-ganglionic (third order neurone)
• Internal carotid artery disease
• Cavernous sinus mass

19. 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 re-examined 5 minutes later.
• The MRD1 is rechecked in the affected and unaffected eyes .
• A rise in the MRD l of 1.5 mm or greater is considered a positive test.
-This indicates that Müller's muscle is viable

20. 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.

21. 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.

22. • 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.

23. 2. Investigations
Edrophonium (Camiston) test
Antibodies to acetylcholine receptors
CT or MRI for presence of thymoma
Electromyography to confirm fatigue
3. Treatment options
• Medical
- anticholinesterases, steroids and azathioprine
• Thymectomy

24. Edrophonium test

25. Myotonic dystrophy

26. 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.

28. D. Mechanical ptosis
• Due to excessive weight on the upper lid
– lid tumours,
– multiple chalazia
– lid oedema.
• Cicatricial Ptosis
– ocular pemphigoid
– trachoma.

30. 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.

31. 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.
– Blepharospasm
– Contralateral proptosis

32. • 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.

33. EVALUATION OF PTOSIS
(HISTORY)
• Ptosis
– Age of onset
– Duration
– One/both eye
– Diurnal variability
• Associated history :
– Diplopia
– Dysphagia
– Muscle weakness
• Vision

34. • 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

35. 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

36. • 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 rule out 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

37. measurements
1. MARGIN REFLEX DISTANCE
• Margin-to-reflex distance 1 (MRD1) :
– When light is thrown on the cornea a reflection occurs .
-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
MRD1 would be a –ve value.

40. 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

42. Grading of severity of ptosis
< or = 2mm : mild ptosis
= 3 mm : moderate ptosis
= or > 4 mm : severe ptosis

43. 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

49. Thank you !