PTOSIS management and caseswiith discussion

Ptosis ppt and workup

1. PTOSIS (ETIOLOGY, CLASSIFICATION AND WORK-UP) MODERATOR- DR. MANEESH KUMAR PRESENTER- DR. DIVYA ROY

2. Ptosis is an abnormally low position(drooping) of the upper lid, which may be congenital or acquired . 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.

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 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.

4. ETIOLOGY

5. CLASSIFICATION Congenital Acquired Neurogenic Myogenic Apone u r otic 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. Congenital ptosis with associated weakness of superior rectus muscle. Blepharophimosis syndrome , 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 m o v e m e n ts i. e. , with s timul a tion o f i p sil at e r al p t e r y g oi d muscl e .

7. SIMPLE CONGENITAL PTOSIS It probably results from a failure of neuronal migration and development with muscular sequelae secondary to this. SIGNS: U/L or B/L ptosis of variable severity Absent lid crease and poor levator function In downgaze the ptotic lid is higher than the normal because of poor relaxation of the levator muscle Superior rectus weakness may be present due to its close embryological association with the levator . Compensatory chin elevation Refractive errors are common and usually responsible for amblyopia .

8. 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.

9. There are two major groups of trigemino- oculomotor synkineses : 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 Internal pterygoid-levator synkinesis is when the eyelid raises upon teeth clenching External pterygoid-levator synkinesis is the more common group.

10. Blepharophimosis Syndrome : A. Blepharophimosis B: Epicanthus Inversus C: Telecanthus D: Ptosis 

11. ACQUIRED PTOSIS Neurogenic Third nerve palsy Third nerve misdirection Horner syndrome Myogenic Aponeurotic Mechanical Neurotoxic

12. 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.

13. Third nerve palsy Ptosis : Due to paralysis of LPS ( levator palpebrae superioris) muscle Ocular deviation : In case of third-nerve palsy, the lateral rectus and superior oblique are spared, and their unopposed action brings the eye in a “down and out” position. Pupil :  In compressive third-nerve palsy, the pupil becomes fixed and dilated due to paralysis of sphincter pupillae. Ciliary muscle paralysis also leads to loss of accommodation. However, in ischemic lesions, the pupil is spared, and there is no loss of accommodation. Diplopia :  This occurs due deviation of the affected eye resulting in the image falling on an extrafoveal point. However, due to ptosis the patient usually doesn’t complain of double vision as ptosis acts as a barrier to diplopia .

14. T hird nerve misdirection syndrome 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

15. HORNER’S SYNDROME Horner syndrome is a rare condition classically presenting with partial ptosis (drooping or falling of upper eyelid), miosis (constricted pupil), and facial anhidrosis (loss of sweating) due to a disruption in the sympathetic nerve supply.

16. 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 . 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

17. Cerebral ptosis It 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.

18. 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.

19. MYASTHENIA GRAVIS Myasthenia gravis (MG) is an autoimmune disease in which antibodies mediate damage and destruction of acetylcholine receptors in striated muscle. The resultant impairment of neuromuscular conduction causes weakness and fatigability of skeletal musculature. Ocular involvement occurs in 90% of cases and is the presenting feature in 60%. Two-thirds of patients have both ptosis and diplopia- Ptosis is insidious, bilateral and frequently asymmetrical. Typically worse at the end of the day. Worse on prolonged (60 second) upgaze due to fatigue. If one eyelid is elevated manually as the patient looks up, the fellow eyelid may show fine oscillatory movements. Diplopia is frequently vertical. Nystagmoid movements may be present on extremes of gaze.

20. Investigations Ice pack test Edrophonium test Electromyography to confirm fatigue Antibodies to acetylcholine receptors CT or MRI for presence of thymoma Treatment options Medical - anticholinesterases, steroids and azathioprine Thymectomy INVESTIGATION/TREATMENT

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

22. Chronic progressive external ophthalmoplegia (CPEO) refers to a group of disorders characterized by ptosis and slowly progressive bilateral ocular immobility. Features- Ptosis, usually the first sign, is bilateral and may be asymmetrical. Pupils are usually not involved. External ophthalmoplegia begins in young adulthood and is typically symmetrical. It is characterized by a progressive course without remission or exacerbation. Investigations- Electromyography shows myotonic and myopathic potentials; serum creatine kinase is elevated. Treatment involves exercise and prevention of contractures. A minority of patients with diplopia may benefit from surgery. CPEO

23. 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.

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

25. MECHANICAL PTOSIS Due to excessive weight on the upper lid lid tumours, multiple chalazia lid oedema. Cicatricial Ptosis ocular pemphigoid trachoma.

26. Causes Dermatochalasis Large tumours Severe lid oedema Anterior orbital lesions

27. 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

28. CAUSES OF PSEUDOPTOSIS e Ipsilateral hypotropia Brow ptosis - excessive eyebrow skin Dermatochalasis - excessiv eyelid skin Lack of lid support Contralateral lid retraction

29. EVALUATION OF PTOSIS

30. HI S T O R Y Ptosis Age of onset Duration One/both eye Diurnal variability Associated history : Diplopia Dysphagia Muscle weakness Vision

31. 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?

32. 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.

33. 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.

34. 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, T o R/O 3 r d ne r v e pal s y 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

35. MEASUREMENTS Margin reflex distance Vertical fissure height LPS action Lid crease level Lid level on down gaze

36. 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.

37. MARGIN REFLEX DISTANCE 1

38. MARGIN REFLEX DISTANCE 2

39. MARGIN REFLEX DISTANCE 3

40. 2 . VERTICA L FISSURE HEI G HT 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

41. GRADING OF SEVERITY OF PTOSIS < or = 2mm : mild ptosis = 3 mm : moderate ptosis = or > 4 mm : severe ptosis

42. 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

43. 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)

44. UPPER LID CREASE crease fold 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

45. OTHER EXAMINATIONS

46. BELL’S PHENOMENON

47. CORNEAL SENSATION/ TBUT TEST/ LAGOPTHALMOS TEST

48. OCULAR MOTILITY ASSESSMENT

49. INVESTIGATION Serum acetylcholine receptor assay Tensilon test EMG ECG ERG T3, T4, TSH

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