Surgical Management of Essential Blepharospasm

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Clinical features

The mean age of onset of benign essential blepharospasm is 56 years. Blepharospasm is seen in 1 in 10,000 patients seeking medical attention; women outnumber men by 3:1. The onset is heralded by variable episodes of increased blinking lasting seconds to minutes. The increased blinking may initially be unilateral or predominantly unilateral and progress to bilateral involvement over time. If the spasm remains unilateral, the patient has hemifacial spasm, although unilateral blepharospasm

Differential diagnosis

A thorough ophthalmic and neurologic examination is essential to establish a diagnosis of benign essential blepharospasm. Reflex irritation of the eyes is the commonest cause of blepharospasm; it may be caused by corneal irritation from keratitis sicca, spastic entropion, eyelash abnormalities, or blepharitis. Patients with ocular pain and photophobia secondary to anterior uveitis or posterior subcapsular cataracts may also demonstrate reflex blepharospasm. Symptoms of dryness of eyes,

Therapy

Many blepharospasm sufferers have tried numerous modes of therapy, including biofeedback, acupuncture, hypnosis, faith healing, herbal therapies, and relaxation therapy, with limited relief.

Pharmacotherapy

Several drugs have been reported to ameliorate specific types of blepharospasm, although their efficacy seems to be limited and temporary in most cases. The list includes antipsychotics, affective disorder agents, antianxiety agents, stimulants, sedatives, parasympathomimetics, antimuscarinics, catecholamine synthesis inhibitors, alphamethyl P-tyrosine, antihistamines, and anticonvulsants.

Anti-Parkinsonian drugs have been tried because of an association with Parkinson's disease. Muscle

Chemical myectomy

Various attempts have been made to achieve a chemical denervation or myectomy of the orbicularis muscle. In animals, local injection of doxorubicin has the ability to cause atrophy of the orbicularis oculi muscle fibers with sparing of the surrounding tissues. It has been tried with mixed success as a form of chemical myectomy in blepharospasm patients. Research into chemical myectomy continues, but currently few surgeons use this modality for the treatment of blepharospasm.

Botulinum-A toxin

The pioneering work by Scott and co-workers on the use of botulinum toxin for selective weakening of extraocular muscles has led to its establishment as a form of treatment for a diverse range of conditions. Botulinum-A toxin is the best temporary treatment available for the orbicularis spasms seen in blepharospasm and hemifacial spasm. Botulinum-A toxin interferes with acetylcholine release from nerve terminals, resulting in temporary paralysis of the injected muscles. As many as 86% of all

Surgical treatment

Functionally impaired patients with blepharospasm who have not tolerated or responded well to medication or botulinum-A toxin are candidates for surgical intervention.

The aim of surgery is

  • To correct or improve the functional and, by inference, the cosmetic changes associated with blepharospasm (brow ptosis, dermatochalasis, ptosis, lash ptosis, canthal dystopia, ectropion, and others)

  • To reduce the severity of spasms

  • To decrease the dosage of botulinum toxin and increase the interval between

Surgical options

Although it is recognized that the source of blepharospasm probably resides in the midbrain, little progress has been made in identifying factors responsible for this disabling condition. Surgical therapeutic efforts have therefore tried to address the nerve carrying the impulses resulting in the overaction of the muscles (facial nerve) or manipulation of the muscles involved (various forms of myectomy).

Use of botulinum toxin after myectomy

Statistics concerning the number of patients who are completely free of the need for botulinum toxin are difficult to come by. Anecdotal remarks claiming complete cure of most patients, with no further use of botulinum toxin, should be regarded with caution. Indeed, Chapman et al found that all of their patients undergoing myectomy after 1990 (when botulinum toxin became commercially available) needed postoperative botulinum toxin. The author and colleagues have found that most patients need a

Apraxia of eyelid opening

Many cases of blepharospasm have apraxia of eyelid opening as well, and this condition is more difficult to manage. Some patients have a combination of marked blepharospasm as well as apraxia of eyelid opening. In others, the apraxia is the predominant feature. Even when the eyelid spasms have been appropriately treated with botulinum toxin or with myectomy, the apraxia of eyelid opening persists. Patients close their eyelids and use their brows and other facial muscles in an attempt to elevate

Complications

All patients experience ecchymosis of the periorbital region. Skin necrosis has been described in the literature, but the author and colleagues have not encountered that problem. Postoperative hematomas should be drained immediately to avoid necrosis of overlying tissues, secondary infection, and optic nerve damage. Supraorbital nerve damage was much more common when suprabrow incisions were used. After an endoscopic approach, hypoesthesia or anesthesia may be noted postoperatively for a number

Summary

At present there is no cure for blepharospasm and related dystonias. Systemic medications help some patients. Debilitating blepharospasm continues in most cases, however, rendering many of these patients functionally blind. Botulinum-A toxin is the best temporary therapy and is successful in keeping 86% of patients reasonably well controlled. For the remaining patients, myectomy is currently the best long-term therapy. In patients who do not respond appropriately to botulinum toxin or myectomy

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  • Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, to the Department of Ophthalmology, University of Utah.

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