Jacqueline Carrasco

BASICS

DESCRIPTION

• Lagophthalmos = incomplete lid closure

• Lid retraction = presence of scleral shown above the superior or below the inferior limbus, with eyes in primary gaze, brows relaxed, and head in normal posture

EPIDEMIOLOGY

Incidence

• Depends on etiology

• Lid retraction is the most common sign of thyroid eye disease (TED).

RISK FACTORS

Depend on etiology

Genetics

N/A, depends on etiology

GENERAL PREVENTION

Prevention of postoperative lid retraction and lagophthalmos involves judicious removal of skin in blepharoplasty, reformation of the lateral canthal tendon if a canthotomy and cantholysis has been performed, possible placement of traction sutures to prevent contracture, severing of fascial attachments of inferior rectus (IR) during recession surgery.

PATHOPHYSIOLOGY

• Lagophthalmos:

– Reduced ability of lids to cover the entire globe may be due to several possibilities:

Globe is proptotic, e.g., TED, retrobulbar mass or hemorrhage; normal variant in patients with shallow orbits

Scarring of anterior lamella (skin and orbicularis), e.g., post op, post herpes zoster, post trauma

Loss of anterior lamella, e.g., post tumor excision, post aggressive blepharoplasty

Posterior lamella (conjunctiva and tarsus) scarring limiting lid excursion, e.g., ocular cicatricial pemphigoid (OCP), chemical burn, trachoma

Neurogenic: Facial nerve (CNVII) palsy or weakness. Orbicularis oculi muscle is responsible for eyelid closure and is innervated by CNVII.

• Lid retraction:

– Sclera is visible above or below the limbus due to eyelid malposition.

– Differential as above; however, the abnormality (lid retraction) is present at rest, in primary gaze.

• Lower eyelid retraction may co-occur with severe ectropion.

ETIOLOGY

Depends on the following conditions:

• Autoimmune (e.g., TED, OCP)

• Inflammatory (e.g., post op, trachoma, leprosy)

• Neurogenic (e.g., CNVII palsy)

• Mechanical (e.g., shallow orbits, lower lid tumor, anterior lamella scarring)

• Involutional/senile (e.g., ectropion)

COMMONLY ASSOCIATED CONDITIONS

• TED

• Facial palsy

• Ectropion

• Postsurgical state

DIAGNOSIS

• Lid retraction – sclera is visible above and/or below the limbus in primary (straight ahead) gaze with brows relaxed.

• Lagophthalmos – incomplete lid closure; patient is asked to close eyes normally (not squeezing maximally) and checked for the presence of a gap between eyelids.

HISTORY

Inquire about

• timing of onset

• tearing, irritation, foreign body sensation, pain

• periorbital ache

• pain with eye movement

• diplopia

• change in vision

• history of prior eyelid or facial surgery or trauma

• history of herpes zoster or simplex infection on face/neck

• history of chronic ophthalmic medication use (possible cause of pseudo-OCP)

• history of chemical burn to eyes/lids/facial skin

PHYSICAL EXAM

• Lid position

• Lid closure

• Examine tarsal conjunctiva for evidence of scarring

• Check for the presence of symblepharon formation

• Visual acuity

• Pupillary reaction (check for rAPD)

• Ocular motility

• Ocular alignment

• Presence of other external signs (e.g., facial scarring, facial nerve palsy/weakness)

• Hertel Exophthalmometry (measures proptosis)

• Anterior segment exam, with careful attention to corneal surface, presence of superficial punctate keratopathy (SPK), corneal erosions or scarring, areas of iris atrophy, presence of active uveitis

• Funduscopy (presence of choroidal folds, optic nerve swelling may indicate the presence of a retrobulbar mass)

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• TSH, T3, free T4, thyroid stimulating immunoglobulin

• Conjunctival biopsy in cases of suspected OCP

Imaging

• CT/MRI of orbits as indicated, e.g., to evaluate for the presence of extraocular muscle enlargement (TED) or mass

• MRI/MRA of brain/brainstem as indicated during work-up of CNVII palsy

Diagnostic Procedures/Other

• Visual field testing (may have various defects in cases of optic neuropathy due to orbital mass or TED)

• External photography (document current lid position, may compare to old family photos to assess for change vs. normal variant as in shallow orbits)

• Color plate deficiencies (decreased color perception may be an indicator of optic neuropathy)

Pathological Findings

Depend on underlying etiology

• TED: Focal and diffuse mononuclear cell infiltrates within EOMs and orbital fat in active disease, fibrotic changes in inactive disease

• OCP: Deposition of IgG, IgA, C3, C4 in conjunctival membrane

DIFFERENTIAL DIAGNOSIS

• Depends on etiology (see the Etiology section):

– DDx of proptosis (TED, tumor, shallow orbits, etc.)

– DDx of anterior and posterior lamella scarring

– DDx of CNVII palsy

• Lower lids may appear falsely retracted in patients with chronic neck flexion forcing chronic chin down head posture and resulting in chronic upgaze.

TREATMENT

ADDITIONAL TREATMENT

General Measures

• If asymptomatic and the cornea is well lubricated, the patient may be observed.

• Both lid retraction and lagophthalmos may result in corneal exposure with symptoms and signs of ocular irritation (pain, foreign body sensation, gritty/sandy sensation, redness, excessive tearing, photophobia, etc.).

• Treatment is directed at alleviating the above symptoms.

• Initial measures include frequent lubrication with artificial tears, use of artificial tear gels or ointments at bedtime to protect the corneal surface.

• Eyelids may be taped closed at night.

• Avoiding excessively dry environments is recommended (e.g., if fans are used in the bedroom, they should be pointed away from the patient; use of humidifiers is considered, etc.).

• Concurrent conditions such as blepharitis or ocular allergies are treated appropriately.

• Underlying conditions (e.g., TED, OCP) treated as appropriate.

SURGERY/OTHER PROCEDURES

• If no relief with supportive treatments, surgery may be indicated.

• In cases of CNVII involvement need for surgery is determined by prognosis for CNVII recovery. If no recovery is expected, the upper eyelid retraction, lagophthalmos, and paralytic ectropion should be surgically addressed.

• Eyelid surgery:

– Approach depends on the underlying condition (e.g., in postoperative lower lid retraction due to aggressive blepharoplasty a skin graft may be required whereas in the case of paralytic ectropion secondary to CNVII palsy the lower lid may need to be horizontally shortened and tightened).

– Goal is to restore eyelid closure, coverage during blink, reduce symptoms of exposure.

– Secondary goal is to improve cosmesis, restoring a more normal appearance.

– Temporary or permanent tarsorrhaphies (parts of the upper and lower lid are sutured together) may be placed.

– In cases of anterior lamella deficit, a skin graft may be required.

– Upper eyelids:

– Transcutaneous or transconjunctival approaches may be utilized. Upper eyelid retractors (Muller’s muscle and/or levator palpebrae) are cut (recessed).

– Gold or platinum weight placement into the upper eyelid is used to improve lid closure.

– Eye springs may be used.

– Lower eyelids:

Lower lid retractors may be recessed.

Spacer graft (AlloDerm, buccal mucosa, banked sclera, etc.) may be used to replace posterior lamella and to elevate the lower lid.

Lower lids may be tightened (lateral canthoplasty).

Mini-tendon grafting may be performed.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Primary care provider and/or endocrinologist involved if thyroid disease is suspected.

• Ophthalmologist with subspecialty involvement of an oculoplastic surgeon can manage eye complications and surgery.

• Others may include neurology, ENT, or neurosurgery in cases of CNVII palsy.

Patient Monitoring

Frequency depends on severity of corneal disease: Daily or possible inpatient admission in cases of corneal ulcers to yearly if mild or stable.

PATIENT EDUCATION

Patients are informed about the status of their cornea and/or underlying condition and advised as to the recommended treatment and prognosis.

PROGNOSIS

Depends on underlying etiology and extent of corneal exposure. Ranges from severe visual loss and possible loss of the eye in cases of severe nonhealing or perforated corneal ulcers to mild or asymptomatic cases which require supportive care and monitoring only.

COMPLICATIONS

• Visual loss

• Disfigurement

ADDITIONAL READING

• Abenavoli FM, De Gregorio A, Corelli R. Upper eye lid loading with autologous cartilage in paralytic lagophthalmos. Plast Reconstr Surg 2006;117(7):2511–2512.

• Garg RK. Unusually thickened ulnar nerve and lagophthalmos in leprosy. Am J Trop Med Hyg 2010;82(5):758.

• Golio D, De Martelaere S, Anderson J, Esmaeli B. Outcomes of periocular reconstruction for facial nerve paralysis in cancer patients. Plast Reconstr Surg 2007;119(4):1233–1237.

• Hassan AS, Frueh BR, Elner VM. Müllerectomy for upper eyelid retraction and lagophthalmos due to facial nerve palsy. Arch Ophthalmol 2005;123(9):1221–1225.

• Liao SL, Shih MJ, Lin LL. A procedure to minimize lower lid retraction during large inferior rectus recession in graves ophthalmopathy. Am J Ophthalmol 2006;141(2):340–345.

CODES

ICD9

• 374.20 Lagophthalmos, unspecified

• 374.22 Mechanical lagophthalmos

• 374.41 Lid retraction or lag

CLINICAL PEARLS

• Lid retraction is most common sign of TED.

• Severe corneal exposure can lead to visual loss.

• Simple measures such as use of over-the-counter artificial tears and gels may be the extent of required treatment.

• Head posture should be assessed as it can affect gaze position and apparent lid position.

Stay updated, free articles. Join our Telegram channel

Join