• 13 SECTIONDisorders Eyelid

Ptosis

Key Facts

•Droopy upper lid caused by innervational failure (neurogenic ptosis) or levator palpebrae superioris muscle damage (myogenic ptosis)

•Causes of neurogenic ptosis:

•third cranial nerve palsy • Horner syndrome • myasthenia gravis • botulism

•Guillain–Barré syndrome • chronic inflammatory demyelinating polyneuropathy (CIDP)

•Causes of myogenic ptosis:

•congenital ptosis • senescent levator aponeurosis dehiscence • eyelid or orbital trauma or surgery • superior sino-orbital inflammation, mass, or infiltrative lesion • mitochondrial myopathy • congenital myopathy • oculopharyngeal dystrophy • myotonic dystrophy • herpes zoster • chronic contact lens wear

•chronic topical steroid use

Clinical Findings

•Droopy upper lid with or without normal excursion • To diagnose neurogenic ptosis, first rule out eyelid or orbital signs then hunt for signs of ocular motor palsies, Horner syndrome, or myasthenia • To diagnose myogenic ptosis, rule out cranial nerve palsies, pupil abnormalities, and myasthenic weakness and hunt for eyelid masses, orbitopathy, and signs associated with congenital or heredodegenerative myopathies

Ancillary Testing

•Suspected neurogenic ptosis:

•topical pharmacologic testing for Horner syndrome • neck and brain imaging for intracranial lesions • lumbar puncture • nerve conduction velocities for Guillain–Barré syndrome or CIDP • electromyography and serologic testing for myasthenia gravis

•Suspected myogenic ptosis:

•DNA testing • muscle biopsy for mitochondrial or other myopathies • orbital imaging to rule out mass or inflammation

Differential Diagnosis

•Pseudoptosis caused by:

•blepharochalasis (dermatochalasis) • blepharospasm • apraxia of eyelid opening • facial contracture after facial nerve palsy • enophthalmos • eyelid or orbital deformity • supraducted eye • eyelid retraction of fellow eye

•Third cranial nerve palsy: look for ductional deficits with or without mydriatic pupil

•Horner syndrome: look for miosis

•Myasthenia gravis: look for fatigable ptosis, ocular misalignment, orbicularis oculi, bulbar, neck, or proximal extremity muscle weakness

•Guillain–Barré and CIDP: look for absent deep tendon reflexes

•Botulism: look for accommodative paresis, ductional deficits, gastrointestinal symptoms

•Congenital ptosis: present at birth, look for impaired lid relaxation on downgaze

•Congenital myopathy, mitochondrial, oculopharyngeal, myotonic dystrophy: get history of longstanding bilateral ptosis with or without ophthalmoplegia or other neurologic signs

•Levator dehiscence: look for indistinct upper eyelid crease, normal lid excursion

•Orbital masses: look for resistance to retropulsion, imaging abnormalities

•Contact lens wear, steroid use, previous eyelid or orbital trauma or surgery: get history

Treatment

• Depends on underlying condition

Prognosis

• 13 SECTIONDisorders Eyelid

Lid Retraction

Key Facts

•Upper lid is elevated so that sclera shows above superior cornea

•Unilateral or bilateral

•Common causes:

•Graves disease

•hyperalert state

•psychoneurotic state

•parkinsonism

•dorsal midbrain (pretectal) syndrome

•eyelid inflammation, trauma, or surgery with scarring (cicatricial lid retraction)

•If stable and longstanding and causing exposure of the eye, surgical eyelidlowering procedures will be beneficial

Clinical Findings

•Upper lid is shortened so that sclera shows above superior cornea

•Unilateral or bilateral

•Graves disease: lid relaxation is delayed as eyes go into downward gaze (lid lag)

•Orbital soft tissue signs of Graves ophthalmopathy present

•Cicatricial lid retraction: lid fails to lower completely on downgaze (lagophthalmos)

•Hyperalert and/or psychoneurotic state: eyelid retraction is bilateral

•Lid lag and lagophthalmos may be present

•Signs may disappear if patient can be made to relax

•Parkinsonism: eyelid retraction is part of facial immobility, bradykinesia

•Dorsal midbrain syndrome: eyelid retraction (Collier sign) accompanied by upgaze deficit

•Light-near dissociation or completely unreactive pupils

•Sometimes convergence–retraction on attempted upgaze

Ancillary Testing

•Suspected Graves: orbital imaging, thyroid function studies

•Suspected psychoneurotic state: psychiatric evaluation

•Suspected parkinsonism: neurologic evaluation

•Suspected dorsal midbrain syndrome: brain imaging

Differential Diagnosis

•Suspected Graves disease: orbital imaging, thyroid function studies

•Cicatricial lid retraction: history or evidence of previous trauma, surgery, or inflammation

•Hyperalert state: behavioral features, medication or recreational drug use

•Psychoneurotic state: behavioral features

Treatment

•In longstanding and stable Graves or cicatricial lid retraction causing exposure, surgical eyelid-lowering procedures

Prognosis

• Lid-lowering surgery often provides benefit