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

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

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

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)

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)

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

Upper lid crease Pretarsal show Distance between lid margin and lid fold crease Distance between lid margin and lid crease in down-gaze Distance between lash line and skin fold in primary position of gaze Normals - females 10 mm; males 8 mm Absence in congenital ptosis indicates poor levator function High crease suggests an aponeurotic defect

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

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

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

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

Posterior hypothalamus (second order neurone) 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

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

Myasthenia Gravis 1. Clinical features 2. Investigations 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

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

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

Myotonic dystrophy Facial weakness and Release of grip difficult 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

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)

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

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

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

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

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

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

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