Eyelid Dystrophies PDF

Eyelid Dystrophies Ptosis ○ ○ Abnormal low position of the upper lid in primary gaze ○ Can be congenital or acquired, monocular or binocular ○ Classifications: neurogenic (rule out neurological diseases), myogenic (lack of innervation in mu...

Eyelid Dystrophies Ptosis ○ ○ Abnormal low position of the upper lid in primary gaze ○ Can be congenital or acquired, monocular or binocular ○ Classifications: neurogenic (rule out neurological diseases), myogenic (lack of innervation in muscles; ex: Myasthenia Gravis), aponeurotic (common type of acquired ptosis because it occurs most often in the elderly), mechanical ○ Symptoms: drooping upper eyelid, visual loss especially with reading ○ Signs: drooping upper eyelid, associated to: Anisocoria Horner’s syndrome (miosis and ptosis) CN 3 Palsy (mydriasis present) Any neurological diseases Diplopia Deviation of the muscles Down and out Ocular motility changes Headache Rule out tumors (CN 7 or CN 3) Neck pain More complex and severe Rule out meningitis and encephalitis (usually presenting with fever and inability to move neck ○ Most of them are benign etiologies ○ But rule out: Horner syndrome Tumors obstructing sympathetic pathway (common reason)  Sympathetic Muller muscle is affected (mild ptosis with miosis, with anhidrosis- flushing of the skin [red in the affected area]) CN3 Palsy Myasthenia Gravis More common in females (can appear in young age) Should ask if ptosis gets worse throughout the day (by towards end of the day, either complete closure or severe ptosis) Superior eyelid or orbital mass Big chalazion ○ Always evaluate ocular movements ○ Neurogenic ptosis Caused by an innervational defect such as third nerve palsy and Horner Syndrome ○ Myogenic ptosis - tiredness of the muscle Caused by a myopathy of the levator muscle Or by impairment of transmission of impulses at the neuromuscular junction (neuromyopathic) Acquired myogenic ptosis occurs in Myasthenia gravis (disease that presents in all muscles of body, not only in the eye, affects young females) Myotonic dystrophy ○ Chronic progressive external ophthalmoplegia - CPEO (rare but lack of Ach stimulation) ○ Aponeurotic or involutional ptosis Caused by a defect in the levator aponeurosis, attached more superiorly, sagging ○ Mechanical ptosis Caused by the gravitational effect of a mass or by scarring Evaluate eyelid margin distance Pull up skin and measure distance of center of pupil to eyelid margin (should be 4-5 mm and symmetrical) Neurofibroma Pseudoptosis due to dermatochalasis Chalazion underneath eyelid, rule out tumor ○ Ptosis clinical evaluation Age at onset of ptosis and the duration will usually distinguish congenital from acquired case Symptoms of possible underlying systemic disease, such as associated diplopia, variability of ptosis during the day and excessive fatigue Ask for trauma? Progression? Sudden? Use botox? Steroids? Measurements MRD 4-5 mm Palpebral fissure height: males (7-10 mm), females (8-12 mm) ○ Ptosis mild up to 2 mm, moderate 3 mm, severe 4 mm or more Levator function ○ 15 mm or more normal, fair 5-11, poor 4 or less Upper lid crease (8-10 mm) ○ Increases in aponeurotic ○ Congenital ptosis do NOT have crease  Associated signs Pupils ○ Horner syndrome (miosis) ○ Third nerve palsy (fixed and dilated, loss of accommodation) Increased innervation (Hering Law) ○ May go to the levator muscle of a unilateral ptosis, particularly in upgaze ○ Associated increased innervation to the contralateral normal levator will result in lid retraction ○ The examiner should therefore manually elevate the ptotic lid and look for drooping of the opposite lid ○ Fatigability Asking the patient to look up without blinking for 30-60 seconds while holding eyelids up and then we will see.. Progressive drooping of one or both lids, or an inability to maintain upgaze, is suggestive of myasthenia gravis Ocular motility Jaw-winking can be identified by asking the patient to chew and move the jaws from side to side or up and down Congenital disease (children) - ask to open mouth and eye opens Bell phenomenon Tested by manually holding the lids open, asking the patient to try to shut the eyes and observing upward and outward rotation of the globe Up and out when eyes are closed- normal The tear film ○ Ice pack test to rule myasthenia gravis 2-5 minutes Positive - no ptosis Acetylcholinesterase activity is inhibited Cold improves neuromuscular transmission ○ Pseudoptosis (some have smaller eye than the other, not always ptosis) Lack of support Orbital volume deficit Artificial eye microphthalmos ○ ○ Phthisis bulbi ○ a severe ocular condition characterized by the shrinkage and disorganization of the eye, often resulting in complete functional loss ○ Can arise from various causes, including trauma, infections, inflammation, malignancies, and complications from eye surgeries, use of prosthetics ○ Affected eye typically presents as shrunken, soft, and non-functional, often described with the mnemonic "7S": Soft, Shrunken, Shapeless, Sightless, Structureless, Squared, and Sore  ○ Enophthalmos (eyes sinking deeper in eye socket) Contralateral lid retraction Comparing the levels of the upper lids Ipsilateral hypotropia Causes pseudoptosis because the upper lid follows the globe downwards Brow ptosis Due to excessive skin on the brow, or seventh nerve palsy Dermatochalasis Overhanging skin on the upper lids may be mistaken for ptosis But may also cause mechanical ptosis ○ Simple congenital ptosis Failure of neuronal migration or failure in muscular development Some patients have a family history AD Signs Unilateral or bilateral ptosis of variable severity Absent lid crease Poor levator function (muscle fibers have not fully formed) ○ Congenital ptosis In downgaze, the ptotic lid higher than the normal because of poor relaxation of the levator muscle In contrast to acquired ptosis, in which the affected lid is either level with or lower than the normal lid on downgaze Associations Superior rectus weakness (embryological association with the levator) Compensatory chin elevation Refractive errors are common Treatment Surgical treatment preschool years ○ Levator resection ○ Marcus Gunn Jaw Winking Syndrome 5% of all cases of congenital ptosis are associated with the Marcus Gunn jaw-winking phenomenon Most are unilateral Exact etiology is unclear Branch of the mandibular division of the CN V is misdirected to the levator muscle Signs: Retraction of the ptotic lid in conjunction with stimulation of the ipsilateral pterygoid muscles by chewing, sucking, opening the mouth or contralateral jaw movement Less common stimuli ○ Jaw protrusion, smiling, swallowing and clenching of teeth Does not improve with age Treatment: Surgery ○ Levator muscle disinsertion and advancement w/ wo brow suspension ○ Horner’s Syndrome Ptosis, miosis, anhidrosis A moderate ptosis Cocaine 2-4% No NE is released, and cocaine will NOT dilate the pupil ○ Third Nerve Misdirection Syndromes May be congenital More frequently follow acquired third nerve palsy Bizarre movement so the upper lid accompany various eye movements Treatment: levator disinsertion and brow suspension  ○ Third Nerve Palsy Down and out position (SO4LR6)  ○ Involutional ptosis Involutional (aponeurotic) ptosis Age-related condition caused by dehiscence, disinsertion or stretching of the levator aponeurosis Due to fatigue of the Muller muscle, frequently worsens towards the end of the day (can sometimes be confused with ptosis secondary to myasthenia) Headaches can be a complaint also  Treatment: Include levator resection, advancement with reinsertion or anterior levator repair Should have surgery immediately ○ Eyelid crutches If patient cannot have surgery due to money or insurance Prevents headaches and chin elevation ○ Mechanical ptosis Result of impaired mobility of the upper lid May be caused Dermatochalasis Large tumors Heavy scar tissue Severe edema Anterior orbital lesions  ○ Ptosis Surgical Treatment Conjunctiva-Muller resection Excision of Muller and overlying conjunctiva with reattachment (2-3 mm) Mild ptosis (at least 10 mm levator function) Levator advancement Levator is shortened to the anterior skin or posterior conjunctiva Any cause of ptosis Levator function at least 5 mm (depends in the severity) Brow (frontalis) suspension Severe ptosis (poor levator function scarring) FBS because of the dryness and exposure Epiphora Redness Asymptomatic ○ Signs: Outward turning of eyelid margin Slow appositional return (mild) - pulling eyelid down and it should return to normal immediately (if it delays, then you grade the severity) Positive snap back test Mild return slow Advanced not in apposition w/ globe, lacrimal punctum not in apposition Conjunctival hyperemia Corneal staining (SPK)  Dry eye Corneal ulcer Chronic Thickening and keratinization ○ Mucosa gets thick without keratin in conjunctiva Scarring of skin in cicatricial cases ○ Involutional (senile) Age-related ectropion, the most common to see Etiology: Eyelid horizontal laxity and gravity act to evert lid or disinsertion of lower lid retractors Signs: Lower lid in elderly Causes epiphora May exacerbate dry eye  Hypermic conjunctiva Tarsal conjunctiva chronically inflamed, thickened and keratinized in chronic cases Include Horizontal lid laxity ○ Pull the skin down even 8 mm from the globe and returns to its normal position Lateral canthal tendon laxity ○ Rounded appearance of the lateral canthus ○ Can be pulled down more than 2 mm Medial canthal tendon laxity ○ Can be pulled down and memdially the punctum Treatment Treat exposure keratitis ○ Lubricants, and ointments to prevent exposure and dryness Advise q 2 hrs ○ Tape eyelids ○ Inflammation - mild steroids Surgery ○ Lateral tarsal strip procedure ○ Horizontal ldi shortening ○ Medial spindle procedure ○ Cicatricial ectropion Caused by scars or burns with fire or acid Caused by scarring or contracture of the skin and underlying issues, which pulls the eyelid away from the globe  Causes Trauma Burns Dermatitis Ichthyosis Excessive skin excision (or laser) in blepharoplasty Glaucoma drops: Dorzolamide- (Trusopt), Brimonidine (Alphagan), due to allergic rxn - d/c Treatment Surgery - Z plasty, transposition flaps or free skin grafts ○ Paralytic (facial nerve palsy) ectropion Mainly caused by ipsilateral facial nerve palsy, cannot lift eyebrow Other etiologies: Congenital moebius syndrome Vascular lesions Bell’s palsy Tumors ○ Acoustic neuroma, parotid gland Trauma at birth to temporal bone fracture All are lack of innervation secondary to CN VII Signs: Flattenign of the entire face with loss of forehead wrinkles, infraorbital fold and nasolabial fold Ocular ○ Eyebrow ptosis ○ Elevation of the upper eyelid ○ Ptosis and ectropion of the lower eyelid ○ Epiphora ○ Lagophthalmos (lack of closure from palpebral fissure) ○ Exposure keratopathy (complication)  Treatment: Because it is paralytic, we have to wait until the movement is recovered ○ Antivirals sometimes, multiple studies that most of the facial nerves are secondary to viral diseases Acyclovir, Dicyclovir with combination of corticosteroids (high doses) ○ Facial therapy for muscles to recover quickly ○ Protect the cornea ○ Lubrication (ointment is usually what is given) ○ Close the eye during sleep Botulinum toxin injection into levator (induce temporary ptosis) [BOTOX] Temporary tarsorrhaphy (lateral aspects of upper and lower lids are sutured together) Permanent paralytic ectropion ○ Medial canthoplasty with lateral canthal sling ○ Mechanical ectropion Caused by tumors on or near the lid margin that mechanically evert the lid Treatment Removal of the cause Correction of significant horizontal lid laxity  ○ Grading in ectropion Mild: normal eyelid appearance with minimal lid laxity Moderate: ectropion with sclera show, without extension of the inferior lacrimal punctum Marked: advanced ectropion with eversion of the punctum Extreme: ectropion with complications (e.g., conjunctival metaplasia) ○ Lid laxity evaluation Look at the grading and timing Entropion ○ Symptoms Irritation FB sensation Pain Spastic closure of lids ○ Signs Lid margin toward the globe Trichiasis (“Secondary trichiasis”) ○ Types Involutional Attenuation or disinsertion of lower lid retractors Epidemiology: older patients; most common type Etiology: age-related changes Treatment: Botox injections, surgery Age-related entropion that affects mainly the lower lid Pseudotrichiasis cause ○ Corneal punctate epithelial erosions in severe cases pannus or ulceration ○ Include: ○ Horizontal lid laxity ○ Vertical lid instability (dehiscence or disinsertion of lower lid retractors) ○ Over-riding of the pretarsal (tipping the lid inwards) ○ Orbital septum laxity (fat prolapse) Treatment ○ Lubricants ○ Taping ○ Soft bandage contact lenses ○ Orbicularis chemodenerbation with botox injection ○ Antibiotic if severe ○ Surgery: over-riding and disinsertion, Wies procedure: return eyelid to straight position without over-riding Spastic  Secondary to ocular inflammation irritation/surgery Epidemiology: usually older patients Etiology: secondary to surgery or inflammation Treatment: may self-limit with time Cicatricial Trauma, chemical injuries, trachoma scarring, cicatrizing conjunctivitis Epidemiology: any age Etiology: chemical burn, ocular cicatricial pemphigoid, Stevens-Johnson syndrome, trachoma, others Treatment: Manage underlying etiology, botox injections, surgery Scarring of the palpebral conjunctiva can rotate the upper or lower lid margin towards the globe Causes: ○ Cicatrizing conjunctivitis ○ Trachoma ○ Trauma ○ Chemical injuries Temporary measure are similar to involutional entropion Surgical treatment: tarsal fracture (transverse tarsotomy) - break tarsal and expand area to prevent movement inside of eyelid Congenital Lower lid retractors dysgenesis (malformation) Epidemiology: children,