CLASSIFICATION OF PTOSIS

1. CLASSIFICATION OF PTOSIS 1. Neurogenic • Third nerve palsy • Third nerve misdirection • Horner syndrome • Marcus Gunn jaw-winking syndrome 2. Myogenic • Myasthenia gravis • Myotonic dystrophy • Ocular myopathies • Simple congenital • Blepharophimosis syndrome 3. Aponeurotic 4. Mechanical

2. PTOSIS 1. Evaluation • Pseudoptosis • True ptosis 2. Classification • Neurogenic ptosis • Myogenic ptosis • Aponeurotic ptosis • Mechanical ptosis 3. Treatment options

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

4. Marginal reflex distance • Distance between upper lid • margin and light reflex (MRD) • Mild ptosis (2 mm of droop) • Moderate ptosis (3 mm) • Severe ptosis (4 mm or more)

5. Upper lid excursion • Reflects levator function • Normal (15 mm or more) • Good (12 mm or more) • Fair (5-11 mm) • Poor (4 mm or less)

6. Vertical fissure height • Distance between upper and lower lid margins • Normal upper lid margin rests about 2 mm below upper limbus • Normal lower lid margin rests 1 mm above lower limbus • Amount of unilateral ptosis is determined by comparison

7. Upper lid crease Pretarsal show fold crease • Distance between lash line and skin fold • in primary position of gaze • 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

8. Bell’s phenomenon Upward rotation of globe on lid closure Good Poor - risk of postoperative corneal exposure

9. Left third nerve palsy Severe unilateral ptosis and Normal abduction defective adduction Defective elevation Defective depression

10. 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

11. Horner syndrome • Caused by oculosympathetic • palsy • Usually unilateral mild • ptosis and miosis • Normal pupillary reactions • Slight elevation of lower lid • Iris hypochromia if • congenital or longstanding • Anhydrosis if lesion is below • superior cervical ganglion

12. Important causes of 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) • Internal carotid artery disease Ciliospinal centre of Budge( C8 - T2 ) • Cavernous sinus mass

13. 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 Contralateral movement of jaw Opening of mouth

14. Myasthenia Gravis 1. Clinical features • Uncommon, typically affects young women • Weakness and fatiguability of voluntary musculature • Three types - ocular, bulbar and generalized 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

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

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

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

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

19. Simple congenital ptosis • Developmental dystrophy of levator muscle • Occasionally associated with weakness of superior rectus In downgaze ptotic eyelid is slightly higher Unilateral or bilateral ptosis of varying severity Frequent absence of upper lid crease Usually poor levator function

20. 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

21. 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

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

23. Fasanella-Servat procedure Indicated for mild ptosis with good levator function .. Excision of upper border of tarsus, lower border of Muller muscle and overlying conjunctiva

24. Levator resection Indicated for any ptosis provided levator function is at least 5 mm Shortening of levator complex Amount determined by levator function and severity of ptosis

25. Frontalis brow suspension Main indications • Severe ptosis with poor levator function ( 4 mm or less ) • Marcus Gunn jaw-winking syndrome Attachment of tarsus to frontalis muscle with sling