Ptosis

PTOSIS

1. Ptosis

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. 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.

4. DEFINITION Ptosis ( from Greek Ptosis -to "fall") is a drooping or falling of the upper or lower eyelid .

5. CLASSIFICATION OF PTOSIS Congenital Acquired Neurogenic Myogenic Aponeurotic Mechanical Neurotoxic Pseudotosis

6. Congenital ptosis It is associated with congenital weakness ( maldevelopment ) of the levator palpebrae superioris (LPS ). Simple congenital ptosis not associated with any other anomaly. 2. Congenital ptosis with associated weakness of superior rectus muscle. Blepharophimosis syndrome , which comprises congenital ptosis, blepharophimosis , telecanthus and epicanthus inversus . 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.

7. Telecanthus Epicanthus inversus

8. Blepharophimosis syndrome Rare congenital disorder Dominant inheritance Moderate to severe symmetrical ptosis 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

9. 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 .

10. There are two major groups of trigemino-oculomotor 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.

11. 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

12. I nverse 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

13. ACQUIRED PTOSIS 1. Neurogenic Third nerve palsy Third nerve misdirection Horner syndrome 2. Myogenic 3 . Aponeurotic Mechanical Neurotoxic

14. A. Neurogenic ptosis It is caused by innervational defects such as third nerve palsy, 3 rd nerve misdirection Horner’s syndrome , Ophthalmoplegic migraine Cerebral ptosis Multiple sclerosis .

15. 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

16. 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 Posterior hypothalamus Ciliospinal centre of Budge( C8 - T2 ) Superior cervical ganglion

17. 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

18. 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

19. 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

20. 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.

21. B. MYOGENIC PTOSIS It is due to acquired disorders of the LPS muscle or of the myoneural junction . Myasthenia gravis Myotonic dystrophy Ocular myopathies oculo -pharyngeal muscular dystrophy following trauma to the LPS muscle.

22. 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 .

23. 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.

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

25. Ocular myasthenia Insidious, bilateral but asymmetrical Worse with fatigue and in upgaze Ptosis Ptotic lid may show ‘twitch’ and ‘hop’ signs Intermittent and usually vertical Diplopia

26. 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

27. Edrophonium test Measure amount of ptosis or diplopia before injection Inject i.v. atropine 0.3 mg Inject i.v. test dose of edrophonium (0.2 ml-2 mg) Inject remaining (0.8 ml-8 mg) if no hypersensitivity Before injection Positive result

28. Myotonic dystrophy Facial weakness and ptosis Involvement of tongue and pharyngeal muscles Ophthalmoplegia - uncommon Muscle wasting Hypogonadism Frontal baldness in males Intellectual deterioration Presenile stellate cataracts Release of grip difficult

29. Ocular myopathies Isolated Oculopharyngeal dystrophy Kearns-Sayre syndrome ( pigmentary retinopathy) Ptosis - slowly progressive and symmetrical Ophthalmoplegia - slowly progressive and symmetrical (no diplopia) Clinical types Ocular features

30. 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 .

31. Aponeurotic ptosis Weakness of levator aponeurosis Causes - involutional, postoperative and blepharochalasis High upper lid crease Good levator function Absent upper lid crease Deep sulcus Mild Severe

32. 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.

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

34. Mechanical ptosis Causes Dermatochalasis Large tumours Severe lid oedema Anterior orbital lesions

35. 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.

36. 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

37. 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 .

38. Causes of pseudoptosis Ipsilateral hypotropia Brow ptosis - excessive eyebrow skin Dermatochalasis - excessive eyelid skin Lack of lid support Contralateral lid retraction

39. EVALUATION OF PTOSIS

40. HISTORY Ptosis Age of onset Duration One/both eye Diurnal variability Associated history : Diplopia Dysphagia Muscle weakness Vision

41. 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?

42. 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.

43. 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 .

44. 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 3 rd 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 diagnosi s Horner’s syndrome Involvement in a case of third nerve palsy

45. Measurements Margin reflex distance Vertical fissure height LPS action Lid crease level Lid level on down gaze

46. 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.  

47. 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)

48. 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

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

50. 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

51. Reflects levator function Normal (15 mm or more) Good ( 8 mm or more) Fair ( 5-7 mm) Upper lid excursion- burke’s ,method Poor (4 mm or less)

52. Upper lid 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 Pretarsal show crease fold

53. Ice test

54. Investigation Serum acetylcholine receptor assay Tensilon test EMG ECG ERG T3, T4, TSH

55. Surgical management

56. Surgical management