Fall Meeting Notes, 2004
Guest Speaker - Dr. Yasser A. Khan
From a historical perspective the diagnosis of or the phenomenon of blepharospasm goes back even to the sixteenth century and was depicted in a painting, by DeGaper, and showed the subject with eyelid and facial spasms.
The first report of blepharospasm in medical literature was in 1870, followed by the classic Meige Syndrome that was described in 1907. It wasn't until the early to mid nineteen hundreds that treatment for blepharospasm was started. They used more radical options like alcohol injections on the facial nerve: basically, just killing the entire facial nerve. They also used other surgical procedures to de-bulk the facial nerve or even cut the facial nerve. All these procedures, as you can well imagine, were associated with several side effects including complete paralysis of one side of the face or both; depending on which side was done. In the early 1970's people started playing around with the idea of a total myectomy or total removal of all eyelid muscle from the upper and lower eyelids.
It wasn't until the early 1980's and, in particular 1981, when three key things happened. There was of course the founding of the Benign Essential Blepharospasm Research Foundation in the United States by Mattie Lou Koster which was a key event in educating people and physicians alike as well as progressing the management of this condition. Secondly, Scott, a neurologist, first started using botulism toxins for strabismus, or eye muscle procedures. He then discovered that it was actually affecting eyelid blepharospasms and started using it for the eyelids. Thirdly, it was Richard Anderson who really pioneered the surgical aspects of this condition in the early 80's when he started performing a full myectomy procedure. Currently the trend has gone from full myectomy surgery to a limited or partial myectomy procedure. A limited myectomy procedure is a much less aggressive procedure where a smaller amount of eyelid muscle is removed usually from the upper eyelids. This limited or partial myectomy is often combined with other eyelid surgical procedures such as “ptosis repair” (eyelid droop repair) or entropion repair (spastic lower eyelid repair). These other procedures help BEB patients by correcting other eyelid malformations that can worsen blepharospasms.
Classification of Blepharospasm-Related Disorders
Dr. Khan spoke about Dr. Richard Anderson’s classification of blepharospasm-related disorders as follows:
- There is the classic Meige Syndrome, which essentially is spasms in both the eyelids and the mid face.
- There is Brueghel Syndrome, which are spasms in the eyelids and the lower face and neck.
- There is Segmental Cranial Dystonia which is eyelid and facial spasms where other facial nerves are involved, and,
- There is Generalized Dystonia, which is basically eyelid and facial spasms with spasms in other parts of the body.
These are all part and parcel of one condition or disease complex.
As far as treating Benign Essential Blepharospasm (BEB) there are four main areas:
- Education and support is fundamental and all of us who treat patients with BEB will agree on this and definitely the Foundation has been a huge part in helping us with that.
- Systemic drugs to sort of use a shotgun approach to treating this condition has been tried in the past and still is relevant in the select blepharospasm patients.
- Of course, BOTOX®, which is the most common way of treating patients with blepharospasm, and,
- Surgery.
Dr. Khan views the causes of blepharospasm as very complex and multiple. Blepharospasm occurs due to some imbalance in the area of the brain responsible for blinking. A stimulus such as dry eyes, stress, light or emotion causes a blink response. Defective circuits in an unknown “blinking center” in the brain fail to control this blinking. This results in uncontrollable blinking and the blepharospasm cycle. All treatments therefore aim to break this cycle.
Non-medical ways to modulate this cycle include the use of sunglasses, eye drops, emotional counseling, and changing the stressful factors in our lives. Medical methods include oral medications, botulinum toxin injections and surgery.
Surgical Options
Surgical options for treating BEB traditionally include the classic full myectomy first pioneered by Dr. Richard Anderson in the early 1980's. A more modified and current surgical procedure is the limited or partial myectomy. Whereas the full myectomy involved excision of upper and lower eyelid muscle, the partial myectomy involves excision of only a select amount of upper eyelid muscle. Usually it also involves the tightening of the eyelid muscle responsible for opening the eyelid or “ptosis repair”. The limited myectomy is a much less extensive procedure and is most commonly indicated for treating apraxia of eyelid opening. Apraxia of eyelid opening is a less common type of BEB where not only are blepharospasms present but also a debilitating inability to open the eyelids once closed. Surgery is often the only way to deal with this condition. Some patients with BEB often have other underlying eyelid malformations that are either pre-existing or a result of their continual squeezing of their eyelids: these are also surgical candidates. Surgery does not remove the need for botulinum toxin injections but rather enhances the effects of the toxin. Surgery allows a lesser amount of toxin to be used to achieve the desired result.
In more detail, a full myectomy is the excision of nearly all the eyelid muscle (orbicularis oculi) starting from just above the eyebrow and involving the upper lower eyelids. A full myectomy is an option for patients who either do not respond to botulinum toxin injections or have stopped responding (become “immune”).
Dr. Richard Anderson, has performed the full myectomy for over the past 15 years. He investigated his results over this time period. Out of 320 patients who underwent full myectomy, either of the upper and lower lid or just the upper eyelid: overall 88% of patients noted some type of improvement after a full myectomy. And of this group, 38% required BOTOX® after myectomy as sort of an adjunctive or secondary treatment.
A full myectomy is major surgery and as such has some negative side effects. It may take six months to one year because of swelling and wound healing. There is a phenomenon call lymphedema, which is basically a swelling of the eyelid, and that can also persist upwards of six months to a year. There is a nerve in the area where we do the full myectomy that supplies sensation to the facial area and, if that nerve inevitably gets damaged in the procedure it can cause anaesthesia or numbness in that area: another negative side affect. However, most patients after six months to a year get the sensation back. Finally, because all of the muscle is removed, there is a risk of having decreased eyelid function and closing, which can cause dry eyes and exposure of the cornea.
In addition there are the basic complications of any kind of surgical procedure of this magnitude. They include infection, bleeding, loss of eyebrow hair, scar tissue and ptosis, which is basically an eyelid droop. You can get upper eyelid retraction where the eyelid retracts upward or no eyelid re-traction where the eyelid can get retracted downwards. It may also include a basic eyelid deformity or an entropion, which is an inward turning of the lower eyelid.
The limited myectomy involves the removal of upper eyelid muscles and selected muscles around the eyelids and eyebrows. This is a much less extensive procedure. A surgeon may also select the muscles with high amounts of blepharospasm and selectively excise them – a tailored solution for a particular patient. Typically with a limited myectomy the lower eyelid is usually spared with the limited myectomy.
Why Surgery?
The advantages of a limited myectomy over a full myectomy include the incision which is identical to that done for a cosmetic blepharoplasty (“eyelid lift”) – so an improved cosmetic outcome. The incision is made in the eyelid crease, which means that the incision or the scar is hidden and not seen, whereas in a full myectomy the incision is made over the eyebrow. Therefore, there is a decreased amount of swelling and, healing is much faster; anywhere from six weeks to three months as compared to up to one year for a full myectomy. There is a decreased risk of loss of eyelid function. In addition, the lower eyelid is typically not approached.
Why perfom a limited myectomy? The procedure is primarily for patients who respond to botulinum toxin injections but do so inadequately, especially individuals with associated apraxia of eyelid opening. Studies have shown that 80% of patients undergoing limited myectomy note a subjective relief of symptoms. However, the majority of patients with a limited myectomy will continue to require botulinum toxin injections. Presumably, due to the presence of decreased amounts of eyelid muscle, the same amount of toxin in the injections or a lesser dosage will be more potent and thus the toxin will “work better” - a more efficient use of BOTOX®.
Apraxia of eyelid opening can be a very frustrating condition to manage. It is the inability of the normal eyelid to open even in the absence of squeezing. There are two different muscles that control eyelid function. The muscles (orbicularis oculi) that are affected most commonly in BEB are ones that close the eyelid. The muscle that opens the eyelid is called the levator palpebris. These muscles work at opposite times; so when the eyelid is opening the closing muscles are inhibited. Seven percent (7%) of BEB patients have apraxia of eyelid opening.
It has been reported that about 50% or half of patients who have not responded to BOTOX® have apraxia of eyelid opening and, is the most common cause of failure with botulinum therapy. Increasing the dose of BOTOX® may actually worsen apraxia of eyelid opening.
Other causes which may aggravate an inability to open the eyelids despite absence of spasms include ptosis and dermatochalasis. Ptosis is an eyelid droop where the muscle that opens up the eyelid (levator palpebris) has become detached or damaged (i.e. by excessive blinking).
Dermatochalasis is excessive upper eyelid skin that may exacerbate BEB. Surgical correction doesn't necessarily take the blepharospasms away but may help to control them better and slow down the cycle BEB patients get into.
A limited myectomy with ptosis repair (tightening of the muscle that opens the eye) has helped the patient’s apraxia. Patients who do not respond to this may also benefit from a second procedure known as a frontalis sling procedure. In this procedure, the upper eyelid is internally attached to the eyebrow muscle using various materials. Patients use their eyebrow muscles to help lift their eyelid.
Other Conditions Affecting BEB
Other eyelid conditions that can exist alongside BEB or, be a result of BEB, significantly exacerbate BEB. These include eyebrow ptosis, entropion, ectropion, lagophthalmos and lower eyelid retraction. An eyebrow ptosis or eyebrow droop causes eyelid tissue to weigh down on the upper eyelid and cause aggravation with blepharospasms. Therefore, by lifting the eyebrow surgically it will improve the spasms.
Entropion is basically the inward turning of the eyelid; the eyelid muscle starts to get lax and as a result the eyelashes turn in and can rub against the cornea, a very sensitive area, causing a great amount of discomfort. This in turn causes an increase in the amount of blinking, and, hence, a worsening of the BEB. This may be corrected surgically giving some respite to the patient. Ectropion is an outward turning of the eyelid that can cause a lot of eye irritation and discomfort. After surgery the eyelid is returned to its normal position resulting in less discomfort and a greater response to Botox injections.
Lagophthalmos is the incomplete closure of the eyelids causing irritation and dryness of the eye surface. The eye may not close fully at night while sleeping and one may wake up in the morning with dry, irritated and painful eyes. In this case it is recommended that the patient use an eye-lubricant just before they retire at night.
Eyelid retraction is where the lower eyelid is retracted and the white-of-the-eye is showing. Normally there should be no white showing in the bottom - surgery is used to correct this fault.
In summary, blepharospasm may be viewed as a cycle and all parts of the cycle should be addressed in its management. The various types of surgical treatment options are summarized:
- A full myectomy is an effective procedure for those who don't respond to botulinum toxic (Botox) injections.
- A limited myectomy is very effective and current. It’s a procedure with which Dr. Khan has had good patient outcomes – especially for apraxia for eyelid opening.
- Apraxia of eyelid opening; is often present in patients unresponsive to Botox.
- It is important to correct other eyelid malformations that are often associated with patients who have BEB.
Dr. Khan concluded his talk by stating that he wanted “to emphasize that surgery is not all that frightening and it is a valid option for appropriate patients”.
Return to the Top of the Page
- BOTOX® registered trademark of Allergan Corporation
- DeGaper Painting (kein Copyright,siehe http;//nl.wikipedia.org/wiki/Abfeelding:DeGaper.gif) Prof. Dr. Ch. Kubisch/Dr. Ch. Netzer, Uniklinik Koln