Ptosis

BASICS


DESCRIPTION


• Drooping of the upper eyelid


– May be congenital or acquired


– May cause blurred vision or loss of visual field


EPIDEMIOLOGY


Unknown


RISK FACTORS


See associated conditions


Genetics


• Most commonly idiopathic


• May be inherited as autosomal dominant trait


• Familial occurrences suggest that genetic or chromosomal defects are likely, although yet unknown.


PATHOPHYSIOLOGY


• Aponeurotic: levator stretching or dehiscence (1)


• Myogenic: primary or secondary levator and/or Muller’s muscle abnormality


• Neurogenic: damage to nerves innervating the levator and/or Muller’s muscle


• Mechanical


• Traumatic: damage to eyelid retractors


ETIOLOGY


• Congenital:


– Myogenic: levator dysgenesis


– Blepharophimosis


– Neurogenic: synkinetic, Horner’s syndrome, cranial nerve (CN) III palsy


• Acquired:


– Aponeurotic: normal aging changes, repetitive trauma in patients who rub eyes, contact lens wearers, patients with previous intraocular surgery


– Myogenic: myasthenia gravis, chronic progressive external ophthalmoplegia (CPEO), myotonic dystrophy, oculopharyngeal dystrophy


– Neurogenic: CN III palsy, Horner’s syndrome


– Mechanical


– Traumatic: history of eyelid laceration with levator transection, contusion injury to the levator


– Reactive: due to ocular or orbital inflammation


COMMONLY ASSOCIATED CONDITIONS


• CN III palsy


– Congenital, compressive, vasculopathic, traumatic


• Horner’s syndrome


• Myasthenia gravis


• Other: Marcus Gunn jaw winking syndrome, ophthalmoplegic migraine, multiple sclerosis


DIAGNOSIS


HISTORY


• Determine the onset and duration of ptosis.


• History of surgery in either eye?


• Symptoms of ocular irritation?


• Orbital or eyelid trauma?


• Variability with fatigue?


• Associated with headache or neck pain?


• Associated diplopia?


• History of autoimmune disease (lupus, Sjögren’s syndrome)?


PHYSICAL EXAM


• Visual acuity


• External


– Position of upper and lower eyelid margins (primary, up and down gaze)


– Levator function


– Presence and position of eyelid crease


– Amount of dermatochalasis, position of eyebrows


– Preexisting eyelid scars


– Evert upper eyelid


– Orbital exam: palpable masses or proptosis?


• Pupils


– Irregular, miosis or mydriasis


• Motility


– Ductions and versions


– Bell’s phenomenon


– Strabismus or diplopia


• Anterior segment


– Conjunctival inflammation, filtering blebs


– Tear film


– Keratopathy


– Anterior segment inflammation


• Retina


• Pigmentary changes


DIAGNOSTIC TESTS & INTERPRETATION


Lab


Acetylcholine receptor antibody testing and/or edrophonium chloride (Tensilon) testing under monitored conditions are recommended in cases of suspected myasthenia gravis.


Imaging


• Suspected orbital mass: orbital CT or MRI


• Painful Horner’s syndrome: rule out carotid artery dissection with emergent CT/CT angiography (CTA) or MRI/magnetic resonance angiography (MRA) of the head/neck.


• CN III palsy (partial or pupil involving): rule out intracranial aneurysm with emergent CT/CTA, MRI/MRA, or angiography.


Diagnostic Procedures/Other


• Visual fields to determine functional impact


• Clinical photographs


• Ice test


– Improvement of ptosis with ice suggests myasthenia gravis.


• Cocaine and/or hydroxyamphetamine tests


– In cases of suspected Horner’s syndrome


• Single fiber electromyography (EMG)


– In cases of suspected myasthenia gravis


Pathological Findings


• Congenital ptosis


– Underdeveloped levator muscle, may be infiltrated by fatty tissue


• Aponeurotic ptosis


– Thinning, lengthening, and/or disinsertion of the levator aponeurosis from the tarsal plate


• Myogenic ptosis


– Ragged red fibers in CPEO


DIFFERENTIAL DIAGNOSIS


• Contralateral upper lid retraction


• Ipsilateral exophthalmos


• Small globe: microphthalmos, phthisis bulbi


• Dermatochalasis


• Brow ptosis


• Eyelid edema


TREATMENT


MEDICATION


• Myasthenia gravis: treatment of primary disorder will improve ocular symptoms


– Pyridostigmine, prednisone, intravenous immunoglobulin (IVIG), steroid-sparing agents


ADDITIONAL TREATMENT


General Measures


• Observation


• Management of chalazion and eyelid and/or orbital neoplasms with excision


• Treatment of ocular irritation in reactive ptosis


• Traumatic ptosis should be observed for spontaneous improvement for at least 6 months.


Issues for Referral


• Congenital ptosis


– Referral to pediatric ophthalmologist for monitoring/management of possible amblyopia and/or strabismus


• Neurogenic ptosis


– Referral to neuro-ophthalmologist, neurologist, and/or neurosurgeon


COMPLEMENTARY & ALTERNATIVE THERAPIES


Taping and eyelid crutches attached to glasses


SURGERY/OTHER PROCEDURES


• External levator resection/advancement


• Internal levator resection


• Frontalis sling procedure: for patients with poor levator function


ONGOING CARE


FOLLOW-UP RECOMMENDATIONS


See issues for referral.


Patient Monitoring


Congenital ptosis: see issues for referral.


PROGNOSIS


Dependent of etiology


COMPLICATIONS


• After surgical repair:


– Orbital hemorrhage


– Under-/overcorrections


– Eyelid asymmetry


– Infection


– Dry eye syndrome


– Corneal exposure/lagophthalmos



REFERENCE


1. Frueh BR. The mechanistic classification of ptosis. Ophthalmology 1980;87:1019–1021.

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Nov 9, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Ptosis
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