Decompression of the facial nerve in symptomatic cases is effective for hemifacial spasm. Surgery for cryptogenic cases and decompression of the facial nerve from aberrant blood vessels have been recommended.

The surgical approach for hemifacial spasm requires a retromastoid craniectomy and microneurosurgical techniques and should not be undertaken unless symptoms warrant. Although some patients are extremely grateful to have had this surgery, the potential risks cannot be overlooked.

Supportive

Most patients are helped by support groups, although a few patients have been adversely affected by participation when they have been exposed to patients with disease that was far more severe than their own.

Because there is substantial variability in the severity of blepharospasm, the referral of patients should be individualized so as not to increase patient anxiety.

COMMENTS

The treatment of benign essential blepharospasm has three phases: a medical phase, the trial of botulinum toxin type A, and the surgical phase. Hemifacial spasm responds best to botulinum toxin type A and, if this fails, to surgical decompression. There is no role for medication. Individuals show a substantial variation in their symptoms and their responses to medication. Both doses of medication and toxin injections require careful titration. Often several trials of toxin are required to find the dose and pattern that suits the patient best.

SUPPORT GROUP

Benign Essential Blepharospasm Research Foundation

755 Howell Street

Beaumont, TX 77706

REFERENCES

Frueh BR, Felt DP, Wojno TH, et al: Treatment of blepharospasm with botulinum toxin: a preliminary report. Arch Ophthalmol 102:1464– 1468, 1984.

Gillum WN, Anderson RL: Blepharospasm surgery: An anatomical approach. Arch Ophthalmol 99:1056–1062, 1981.

Jankovic J: Clinical features, differential diagnosis and pathogenesis of blepharospasm and cranial cervical dystonia. In: Bosniak SL, Smith BC, eds: Advances in ophthalmic plastic and reconstructive surgeryblepharospasm. New York, Pergamon, 1985:4.

Jones FTW, Samples JR, Waller RR: The treatment of essential blepharospasm. Mayo Clin Proc 60:663–666, 1985.

Lingua RW: Sequelae of botulinum toxin injection. Am J Ophthalmol 100:305–307, 1985.

242 FLOPPY EYELID SYNDROME

374.9

Lee K. Schwartz, MD

San Francisco, California

Gary L. Aguilar, MD

San Francisco, California

ETIOLOGY/INCIDENCE

The floppy eyelid syndrome (FES) is an uncommon and frequently unrecognized cause of noninfectious, chronic unilateral or bilateral papillary conjunctivitis. The condition is characterized by loose, ‘floppy’ eyelids associated with punctate epithelial keratopathy (PEK), ptosis of lateral eyelashes and the typical conjunctival changes.

The diagnosis has been made in patients of both sexes ranging in age from 2 to 80, but the disorder most commonly affects the middle-aged, and in particular middle-aged, obese males. The floppiness of the eyelids is due to laxity of the tarsus, which is a consequence of a decrease in tarsal elastin.

There may be several pathways that lead to FES. The primary cause of the tarsal laxity has been explained by the frequent observation that the affected side in unilateral cases corresponds to the side the patient preferentially sleeps on. Pressure on the dependent eyelid during sleep may result in local ischemia and lid eversion, and may provoke the patient to rub the eyelid, exacerbating the ischemia while stretching the tarsal plate. Patients with bilateral disease commonly alternate sides while asleep or sleep face down.

Studies have shown a significant decrease in the amount of elastin within the tarsal plate and eyelid skin as compared to normal controls. It is thought that the up regulation of elastolytic enzymes, most probably induced by repeated mechanical stress, associated with eye rubbing or by sleeping habits, participates in elastic fiber degradation and subsequent tarsal laxity and eyelash ptosis in FES.

Also poor contact of the lax eyelid with the globe in conjunction with meibomian gland and tear film abnormalities may contribute further to the syndrome. Patients with more severe symptoms tend to have floppier eyelids.

A contributing factor to the syndrome appears to be an abnormality in tear film dynamics. Tear film abnormalities have been shown to be prevalent in patients with FES and are characterized by a lipid deficiency, with a consequent rapid rate of tear evaporation. The eyelid skin of FES patients is remarkable for its high temperature, high water evaporation rate, and a tendency toward hyperpigmentation.

In addition obstructive sleep apnea (OSA) may contribute to the development of FES. The physician should inquire about the presence of symptoms of nocturnal breathing disorder. OSA may contribute to local eyelid ischemia that may play an important role in the development of FES. This ischemia may be exacerbated by the hypoventilation of OSA. In addition the pressure that is placed on the dependent eyelid during sleep and/or the act of rubbing the eyelid may also contribute to the syndrome.

(37% of patients are females) who presents with a chronic red eye with a mucoid discharge. By the time the diagnosis is finally made, the patient has usually received multiple ocular medications. The duration of symptoms before diagnosis has ranged from 8 months to 14 years.

DIAGNOSIS

Clinical signs and symptoms

The syndrome is characterized by a triad of diffuse papillary conjunctivitis, a loose upper eyelid that readily everts by pulling it upward (positive lid eversion sign), and a soft rubbery tarsus that can be easily folded on itself. The lower eyelids may also be involved. Lash ptosis is common, usually involving the lateral lashes of the upper eyelid.

The consequences of untreated FES can be serious. Staphylococcus aureus corneal ulceration has been described, as has bilateral corneal neovascularization. Corneal scarring may occur necessitating a penetrating keratoplasty in rare cases. A case has been reported of a patient with FES and rheumatoid arthritis who had dry eyes and who developed a corneal melt with perforation; ultimately requiring evisceration after endopthalmitis supervened. This highlights the fact that comorbities may complicate FES with severe clinical consequences.

The diagnosis is often missed because patients usually present complaining of nonspecific ocular irritation and so can be confused with patients suffering from dry eyes. Typically, patients complain of a red eye(s), a foreign body sensation, eyelid swelling, dryness and a mucoid discharge. Symptoms are usually worse on waking. Patients often come in with multiple ocular medications that have been prescribed by previous physicians without success. The syndrome may be masked or complicated by a toxic medicamentosa from the numerous eye medications.

The lids may appear swollen with mucus discharge. A diffuse, velvety, papillary conjunctivitis is present in the tarsus of one or both eyelids. The upper lid may be thickened and is easily everted, (positive lid eversion sign). The eyelashes may be nonparallel and ptotic, especially laterally, tending to curl toward the globe. The cornea may show punctate epithelial keratopathy, corneal neovascularization, even ulceration.

Associated ocular findings not present in all patients

●PEK (41% to 45%).

●Keratoconus (10% to 16%).

●Blepharitis.

●Blepharochalasis.

●Blepharoptosis.

●Corneal ulcers and neovascularization.

●Dermatochalasis.

●Eyelash ptosis (loss of parallelism of cilia).

Associated systemic disorders not present in all patients

●Arteriosclerotic heart disease.

●Corneal perforation associated with rheumatoid arthritis.

●Chronic bronchitis.

●Diabetes mellitus.

●Epibulbar fasciitis.

●Gout.

●Hay fever.

●Hyperextensibility and hypermobility of joints.

●Hyperglycinemia.

●Hyperlipidemia.

●Hypertension.

●Interstitial pneumonitis.

●Mental retardation.

●Nephrolithiasis.

●Obesity.

●Obstructive sleep apnea.

●Psoriasis.

Nocturnal ectropion of the upper eyelid is probably responsible for the clinical manifestations in most patients.

It is not clear that all cases of FES represent the same syndrome. It is possible that a clinical picture similar to FES occurs in patients with lax upper eyelids of any cause.

Laboratory findings

Conjunctival scrapings reveal keratinized epithelial cells. Bacterial cultures of the conjunctiva should be obtained, and treatment should be instituted if appropriate.

Histopathologic investigations have revealed chronic conjunctival inflammation and fibrosis and scarring. Meibomian gland cystic degeneration and squamous metaplasia have been noted, along with abnormal keratinization and granuloma formation. There is no definite association with systemic diseases of collagen or elastic tissue. Various associations have been reported with keratoconus, hyperglycinemia, and other disorders.

Consider FES patients for sleep studies if OSA is suspected. Symptoms may include snoring, daytime somnolence, waking up feeling un-refreshed, apnea during sleep observed by others, waking gasping for air during the night and morning headache.

Differential diagnosis

A study of 58 patients with chronic conjunctivitis of more than 2 weeks’ duration revealed that FES was the cause of chronic conjunctivitis in 2% of patients. In 31% of patients with chronic conjunctivitis, no specific cause was detected.

Because these patients often present with multiple eye

The diagnosis is made clinically by a localized area of tenderness in the region of obvious inflammation, but may require confirmation based on culture or biopsy results if complications occur.

Differential diagnosis

When the lesion does not resolve with appropriate therapy then preseptal cellulitis, sebaceous cell carcinoma, and pyogenic granuloma should be considered.

Briner AM: Surgical treatment of a chalazion or hordeolum internum. Aust Fam Phys 16:834–835, 1987.

Briner AM: Treatment of common eyelid cyst. Aust Fam Phys 16:828–830, 1987.

Diegel JT: Eyelid problems: Blepharitis, hordeola, and chalazia. Postgrad Med 80:271–272, 1986.

Wilson LA: Bacterial conjunctivitis. In: Duane TD, ed: Clinical ophthalmology. Philadelphia, Harper & Row, 1979:IV:12–16.

PROPHYLAXIS

Prophylaxis is based on lid hygiene, control of associated skin or systemic disease, and improved overall general health.

TREATMENT

Systemic

●If multiple hordeola, recurrent hordeola, or adenopathy occur, consider systemic antibiotics: 250 mg erythromycin q.i.d., 125 to 250 mg dicloxacillin, or 500 mg cloxacillan q.i.d. for as long as 2 weeks

244 LAGOPHTHALMOS 374.2

Eric A. Steele, MD

Portland, Oregon

Roger A. Dailey, MD

Portland, Oregon

ETIOLOGY

Lagophthalmos, which is the inability to fully close the eyelids, can occur for a number of reasons, including excessive projection of the eye in the orbit, inadequate vertical dimensions of

Periocular

●Hot moist compresses for 5 to 15 minutes hourly or q.i.d.

●Removal of eyelashes in area to enhance suppuration.

●Once drainage occurs, topical ocular antibiotic solutions or ointments: bacitracin, erythromycin, gentamicin, tobramycin, ciloxin, and others applied q.i.d. during the acute phase.

COURSE/PROGNOSIS

The presence of lagophthalmos often requires prompt efforts to protect the eye. Neglect of lagophthalmos can result in decreased vision, painful dryness, keratoconjunctivitis, corneal erosion, ulceration, and even endophthalmitis and loss of the eye.

discoloration.

●Lid hygiene.

Surgical

●The gland usually drains spontaneously.

●Stab incision, from either skin or palpebral conjunctiva depending on where the lesion is pointing, may spread infection if the incision is made before the eyelid skin or palpebral conjunctiva appears white or cream colored.

●Multiple pockets may be present; all must be incised.

●Occasionally, a firm central abscess core occurs and must be removed or the lesion will not easily heal.

If a local anesthetic is used, the injection is not administered directly in the area of inflammation but either above the upper border of the upper tarsus or below the lower border of the lower tarsus. A chalazion clamp is applied, and a horizontal incision is made over the pointing area if it is on the skin, or a vertical incision is made if the lesion is conjunctival. The lash margins should be avoided so as not to cause a lid margin defect or injury to the lash roots. The wound edges are not sutured, and postoperative warm compresses and antibiotic ointment are used until closure occurs spontaneously.

The patient often presents with foreign body sensation and tearing, but may be asymptomatic if corneal hypoesthesia is present. On examination, there is incomplete closure of the eyelids. There may be associated corneal epithelial irregularities, or even ulceration with frank perforation.

TREATMENT

Appropriate therapy is dependent on an accurate diagnosis. Proptosis may be caused by congenital deformity, tumor, trauma, or thyroid related immune orbitopathy. Lid retraction can result from traumatic loss of eyelid tissue or cicatrization from many causes. Paralytic lagophthalmos secondary to decreased orbicularis oculi muscle tone commonly occurs with Bell’s palsy.

Ocular

The maintenance of a moist surface on the eye is critical to the management of lagophthalmos. Treatment begins with artificial tears. Lubricating ointments protect the ocular surface longer, but cause bothersome blurring of vision, and may be best tolerated at bedtime. Punctal plugs or thermal occlusion

Fine contractions of the eyelid are visible but are usually more apparent to the patient than to the observer. Rarely, the contractions may be vigorous enough to cause movement of the globe, producing oscillopsia and pseudonystagmus. The focus of irritation is most likely the nerve fibers within the muscle.

TREATMENT

●Reassurance and reduction in precipitating factors, if identifiable, are appropriate for most patients.

DIAGNOSIS

Clinical signs and symptoms

The normal resting upper eyelid position is approximately 4.0 to 4.5 mm above the central corneal light reflex, and the normal lower eyelid position is approximately 5.5 mm below the central corneal light reflex. Normally, eyelid position is symmetric (within 1 mm of the contralateral eyelid). Significant deviation of the eyelid from normal may be considered eyelid retraction.

When the upper eyelid is affected, the resting position of the upper eyelid is at a higher-than-normal level, and when the

ing of local injections of 2.5 to 5.0 units each to the affected eyelid region usually provide adequate relief lasting 12 to 16 weeks. Adverse effects include temporary lid laxity, which may produce lagophthalmos and exposure keratopathy.

●Oral quinine, antihistamines, clonazepam, or baclofen may be tried in the unlikely event that botulinum toxin type A is unsuccessful, but the efficacy of these agents has not been proved. In addition, quinine has numerous side effects and is contraindicated in pregnancy.

REFERENCES

Givner I, Jaffe NS: Myokymia of the eyelids: a suggestion as to therapy: preliminary report. Am J Ophthalmol 32:51–55, 1949.

Lowe R: Facial twitching. Trans Ophthalmol Soc Aust 11:129–133, 1951.

Reinecke RD: Translated myokymia of the lower eyelid causing uniocular vertical pseudonystagmus. Am J Ophthalmol 75:150–151, 1973.

Scott AB: Botulinum toxin for blepharospasm. In: Spaeth G, Katz LJ, Parker KW, eds: Current therapy in ophthalmic surgery. Toronto, Decker, 1989:322–324.

Sogg RL, Hoyt WF, Boldrey E: Spastic paretic facial contracture: a rare sign of brain stem tumor. Neurology 13:607–612, 1963.

246 LID RETRACTION 374.41

Dale R. Meyer, MD, FACS

Albany, New York

Lid retraction is a disorder of eyelid position that can affect the upper eyelid, lower eyelid, or both, and may be unilateral or bilateral.

cally, inferior scleral show occurs when the lower eyelid is affected, and superior scleral show occurs with more advanced upper eyelid retraction. Upper eyelid retraction makes the person appear as if he or she is staring and can produce the illusion of exophthalmos (Figure 246.1).

Eyelid position can be affected by both vertical and horizontal gaze. In downgaze, the upper eyelid normally drops slightly or stays at the same level relative to the mid cornea or visual axis. The term lid lag describes the situation in which the upper eyelid assumes a higher position when the eyes are in downgaze. Lid lag is not to be confused with von Graefe’s sign, which is a dynamic sign describing the pause or retarded descent of the eyelid when the eye moves from primary position to downgaze. Stellwag’s sign describes retraction of the upper eyelid or eyelids associated with infrequent or incomplete blinking. Dalrymple’s sign describes an abnormal vertical wideness of the palpebral fissure.

Resting eyelid position is determined by a balance of the eyelid protractors and retractors. The primary protractor of the eyelids is the orbicularis oculi muscle, which is responsible for eyelid closure and blinking. In the resting position, the orbicularis oculi muscle exhibits low-grade tonic activity only. The retractors of the upper eyelid are the levator palpebrae superioris muscle innervated by the third cranial (oculomotor) nerve and Müller’s muscle, which is smooth muscle innervated by sympathetics. The lower eyelid retractors are more rudimentary and include the capsulopalpebral fascia (an extension of the capsulopalpebral head from the inferior rectus muscle) and the inferior tarsal muscle, which is also smooth muscle innervated by sympathetics.

Determination of the underlying cause of eyelid retraction is usually straightforward. History regarding previous surgery or

vally just above the superior tarsal border can temporarily reduce lid retraction. The individual response to treatment is variable. Effects generally last 3–4 months. Diplopia and overcorrection (ptosis) are potential side-effects.

Surgical

●Approaches vary for upper eyelid, lower eyelid, and severity of lid retraction.

●The upper eyelid usually requires recession of Müller’s muscle, levator aponeurosis, or both.

●The lower eyelid usually requires recession of lower eyelid retractors; advanced cases also require spacer graft (hard palate, ear cartilage, eye bank sclera, or other). A cheek suspension procedure may also be considered.

Upper eyelid retraction surgery

●Posterior (internal) approach.

aponeurosis then is disinserted from the tarsus, with less dissection performed medially than laterally to avoid overcorrection. The patient is placed in the sitting position, and the lid position is assessed. If further lowering of the eyelid is desired, Müller’s muscle can be recessed/extirpated. The plane between Müller’s muscle and conjunctiva is infiltrated with 2% xylocaine with 1 : 100,000 epinephrine; then, Müller’s muscle is undermined and excised. With the anterior approach, the conjunctiva is usually preserved. The patient is again placed in the sitting position, and the eyelid position is checked. Further recession of the levator aponeurosis and Müller’s muscle can be performed with blunt dissection. The levator aponeurosis can be secured in its recessed position with an absorbable suture. The skin is closed in the usual fashion, and the lid crease can be sutured to the superior portion of the tarsus with deeper bites to re-establish the eyelid crease.

Lower eyelid retraction surgery

A 4-0 silk suture is placed through the eyelid margin, and the lid is everted over a Desmarres retractor. Local anesthetic (2% xylocaine with 1 : 100,000 epinephrine) is infiltrated in the subconjunctival space above the superior tarsal border. The conjunctiva is grasped 2 to 3 mm above the superior tarsal border, and a small ‘buttonhole’ incision is made with Stevens scissors through the conjunctiva and Müller’s muscle. The surgical plane between Müller’s muscle and the levator aponeurosis is undermined with Stevens scissors, and the conjunctiva and Müller’s muscle are then incised medially and laterally. The extent of the medial dissection is somewhat less than laterally to avoid overcorrection. Müller’s muscle can be excised directly with Stevens scissors. The patient is placed in the sitting position, and the lid position is assessed. Usually, simple recession/extirpation of Müller’s muscle, as described, will produce approximately 2-mm lowering of the eyelid margin. If further lowering of the eyelid is required, the levator aponeurosis itself can be disinserted from the tarsus via the posterior approach and recessed. The patient is placed in the sitting position, and further recession of the levator aponeurosis usually can be performed through additional blunt dissection to achieve the desired eyelid position. Some surgeons suture back together the conjunctiva at the end of the procedure, but this author has not found this to be necessary or desirable. The conjunctiva usually reepithelializes in approximately 1 week. The silk suture at the lid margin can be taped down to the cheek for several days, but for less advanced cases of upper eyelid retraction, this is not necessary. Although spacer grafts have also been used to treat upper eyelid retraction, they usually are not necessary. Postoperative eyelid position can vary somewhat (either upward or downward) but usually approximates the position noted at the end of surgery.

●Anterior (external) approach.

The levator aponeurosis and Müller’s muscle also can be approached from an anterior eyelid crease incision. The advantage of the anterior approach is that blepharoplasty, if desired, can be performed with further debulking of excess orbital fat. The procedure is performed after the eyelid crease is marked with a skin-marking pen, and if blepharoplasty is planned, a skin pinch technique is used to assess the amount of redundant tissue. Caution should be taken not to excise too much skin because the amount of apparent redundancy may be less when

Surgical correction of lower eyelid retraction can be divided into three approaches:

1.Lower eyelid retractor lysis/recession (extirpation).

2.Recession with spacers (e.g. hard palate mucosa, cartilage,

sclera, acellular dermis).

3. Orbicularis/prezygomatic cheek flap advancement and fixation.

Eyelid retraction procedures are frequently coupled with horizontal eyelid tightening (e.g. tarsal strip procedure or ‘lateral canthoplasty’). These techniques generally involve a conjunctival incision, with identification of the lower eyelid retractors. The eyelid retractors can be dissected from the conjunctiva, followed by recession or extirpation; alternatively, the conjunctiva and retractors can be recessed together, which is this author’s favored approach. In virtually all techniques for the correction of lower eyelid retraction, some type of upward traction (usually via a Frost suture) is applied postoperatively to the lower eyelid for several days. Because gravity ‘works against’ lower eyelid surgery, it may be necessary to place a spacer between the recessed conjunctiva/retractors and the tarsus, particularly if the lower retraction is more than 2 mm. Hard palate mucosa grafts and auricular cartilage, as well as commercially prepared acellular dermis are useful for this purpose. Eye bank sclera can also be used, but results may be less predictable because of reabsorption and fibrosis. Finally, if lower eyelid retraction is due to a deficiency of skin such as occurs after lower eyelid blepharoplasty or burns, consideration must be given to advancement of the remaining eyelid and cheek skin, usually via dissection and mobilization of a composite cheek flap (e.g. ‘SOOF lift’). In cases of extreme vertical skin deficiency, a full-thickness skin graft, usually obtained from behind the ear, may be required to adequately reposition the lower eyelid.

COMPLICATIONS

●The main complication of eyelid retraction is corneal exposure.

●Failure of eyelid retraction surgery to adequately correct eyelid retraction requires more aggressive secondary surgery,

including further recession of the eyelid retractors,