Vogt-Koyanagi-Haraa (VKH) syndrome

• bilateral granulomatous panuveitis associated with serous retinal detachments, optic disc edema, neurologic abnormalities and skin pigment changes
• systemic manifestations: tinnitus, vitiligo, alopecia, headache and meningismus
• T cell-mediated autoimmune process directed against melanocyte antigens
• more prevalent in Asians, Latinos and American Indians
• women slightly > men
• any age but usually 4th-6h decades of life

4 phases

• prodromal
• acute uveitic (poor VA, severe AC inflammation w/ or w/o posterior synchiae)
• convalescent
• chronic recurrent (RPE alterations, widespread loss of choroidal melanocytes producing a sunset-glow fundus and choroidal Dalen-Fuchs-like nodules, cutaneous vitiligo, poliosis and alopecia)

Complications

• catarct, glaucoma, CNV, subretinal fibrosis, ERM, macular atrophy

Treatment

• prompt initiation of high-dose systemic corticosteroid therapy (1 to 1.5 mg/kg/day) concurrent with a corticosteroid-sparing immunosuppresive agent) , tapering patients off within 2-3 months
• rapid and aggressive treatment is important to minimize disease duration and lessen the risk of progression into a chronic recurrent form of disease and reduce the incidence of systemic and ocular complications

Lagophthalmos evaluation/treatment

May lead to corneal exposure --> keratopathy --> ulceration/infections keratitis

Taking the history
recent trauma or surgery involving the head/face/eye
past infections e.g. herpes zoster
past symptoms suggestive of thyroid disease or obstructive sleep apnea

Testing the lids and globe
ask patient to look down and gently close both eyes
lagophthalmos is present when a space remains b/w the upper and lower eyelid margins in extreme downgaze
measure this space with a ruler
record the blink rate an the completeness of blink
test cranial nerve function (pay attention to ocular motility and the strength of the orbicularis oculi muscle by evaluating the force generated on attempted eyelid closure)
presence and quality of Bell's phenomenon should be noted (cornea is better protected when the eye rolls upward on attempted closure of the eyelids)

Testing the cornea
test corneal sensitivity by applying soft cotton to the unanesthetized cornea and comparing the blink reaction with that of the fellow eye
describe presence of PEE with NaFl
record TBUT

Etiology

Facial nerve (VII)

• innervates frontalis muscle (raises the eyebrow) and the orbicularis oculi muscle (closes the eyelid)
• loss of function of the VII nihibits eyelid closure, blink reflex, and lacrimal pumping mechanism
• also innervates the muscles of facial expression including the zygomaticus (elevate the cheeks) and corrugator supercilii and procerus (depress the eyebrow) which help facial symmetry

Trauma

• VII is susceptible to blunt trauma or laceration along it's bony course
• fractures to the skull base or mandible can damage the nerve or one of its branches
• neurosurgical procedures

Cerebrovascular accidents

• VII receives its blood supply from the anterior inferior cerebellar artery (susceptible to ischemic damage)

Bell's Palsy

• idiopathic VII palsy thought to be associated with an acute viral infection or reactivation of herpes simplex virus

Tumors

• acoustic neuromas in the cerebellopontine angle and metastatic lesions are most commonly associated with lagophthalmos
• need MRI with gadolinium

Infections, immune-mediated causes

• less common causes: Lyme disease, chickenpox, mumps, polio, Guillain-Barre syndrome, leprosy, diphtheria and botulism

Mobius' syndrome

• rare, congenital condition with CN palsies (esp. VI and VII), motility disturbances, limb anomalies and orofacial defects

Eyelids

damage or degeneration of any of the eyelid tissue structures (skin/subcutaneous tissue, orbicularis oculi muscle, orbital septum, orbital fat, muscles of retraction, tarsus, conjunctiva) may inhibit good eyelid closure

Cicatrices

• chemical or thermal burns
• ocular cicatricial pemphigoid
• Stevens-Johnson syndrome
• mechanical trauma
• above may cause scarring of the soft tissues or retractor muscles

Eyelid surgery

• excessive removal of eyelid skin or muscle (blepharoplsty, tumor excision)
• overcorrection in ptosis repair

Proptosis

• exophthalmos in thyroid ophthalmopathy

Enophthalmos

• aquired causes (orbital blowout fractures, orbital fat atrophy from trauma, infection, inflammation, aging or wasting disease such as linear scleroderma or HIV-AIDS)
• phthisical or prephthisical eye
• scirrhous carcinomas leading to contraction of orbital fat

Floppy eyelid syndrome

• result of severe laxity and flexibility of the superior and inferior tarsal plates
• may be associated with obstructive sleep apnea

Symptoms

• FBS and tearing
• pain in AM from increased corneal exposure and dryness during sleep
• blurry vision from unstable TF

Work-up and treatment

• Medical treatment and supportive care for the cornea (non-preserved artifical tears at least QID, ointments QHS/PRN, moisture gogles, methylcellulose)
• Tarsorrhaphy (suturing lateral 1/3 of eyelids, temporary or permanent)
• Gold weight implantation (gold is inert and doesn't show through thin skin of eyelid)
• Uper eyelid retraction and levator recession (for lagophthalmos due to thyroid ophthalmopathy)
• Lower eyelid tightening and elevation (tightenting procedure will improve apposition fo the lower eyelid to teh globe and decrease tearing)
• Ancillary surgical procedures (facial surgery)