CLASSIFICATION OF PTOSIS

• Third nerve palsy1. Neurogenic

• Third nerve misdirection• Horner syndrome• Marcus Gunn jaw-winking syndrome

• Myasthenia gravis• Myotonic dystrophy• Ocular myopathies• Simple congenital

2. Myogenic

3. Aponeurotic4. Mechanical

• Blepharophimosis syndrome

PTOSIS

• Pseudoptosis• True ptosis

1. Evaluation

• Neurogenic ptosis• Myogenic ptosis• Aponeurotic ptosis• Mechanical ptosis

2. Classification

3. Treatment options

Causes of pseudoptosis

Ipsilateral hypotropia Brow ptosis - excessive eyebrow skin

Dermatochalasis - excessiveeyelid skin

Lack of lid support Contralateral lid retraction

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)

• Reflects levator function

• Normal (15 mm or more)

• Good (12 mm or more)

• Fair (5-11 mm)

Upper lid excursion

• Poor (4 mm or less)

• 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

Vertical fissure height

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

Bell’s phenomenonUpward rotation of globe on lid closure

Good Poor - risk of postoperativecorneal exposure

Left third nerve palsy

Severe unilateral ptosis anddefective adduction Normal abduction

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 primaryposition

Worse on right gaze Normal on left gaze

Horner syndrome

• Caused by oculosympathetic palsy• Usually unilateral mild ptosis and miosis

• Slight elevation of lower lid

• Normal pupillary reactions

• Iris hypochromia if congenital or longstanding

• Anhydrosis if lesion is below superior cervical ganglion

Important causes of Horner syndromeCentral

(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 cervicalganglion

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

• Uncommon, typically affects young women1. Clinical features

• Edrophonium (Camiston) test2. Investigations

• Medical - anticholinesterases, steroids and azathioprine3. Treatment options

• Weakness and fatiguability of voluntary musculature• Three types - ocular, bulbar and generalized

• Antibodies to acetylcholine receptors• CT or MRI for presence of thymoma

• Electromyography to confirm fatigue

• Thymectomy

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

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

Myotonic dystrophyFacial 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

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

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• Weakness of levator aponeurosis• Causes - involutional, postoperative and blepharochalasis

High upper lid crease Good levator function

Absent upper lid crease Deep sulcus

Mild

Severe

Mechanical ptosis

Causes

Dermatochalasis Large tumours

Severe lid oedema Anterior orbital lesions

Fasanella-Servat procedure

Excision of upper border of tarsus, lower border of Muller muscle and overlying conjunctiva

Indicated for mild ptosis with good levator function

..

Levator resection

Shortening of levator complex

Indicated for any ptosis provided levator function is at least 5 mm

Amount determined by levator function and severity of ptosis

Frontalis brow suspension

Attachment of tarsus to frontalis muscle with sling

Main indications• Severe ptosis with poor levator function ( 4 mm or less )• Marcus Gunn jaw-winking syndrome