Introduction

Blepharoptosis, usually referred to as ptosis, can be described as the condition in which the upper lid is at a lower position over the cornea than normal. The question arises, What is the normal position? The usual adult position, with the eyes straight ahead, is for the lid to be about 1.5 mm below the upper limbus of the cornea. It is usually not this low in infants, and it is frequently lower in older persons. It may be lower in some people as a familial trait. If the ptosis is unilateral or asymmetric, the diagnosis is usually made more easily. In some diseases, however, both bilateral and symmetric ptosis occur, making the diagnosis of ptosis more difficult. (A normal form of bilateral and symmetric ptosis is the condition referred to colloquially as bedroom eyes.) Two groups of muscles and nerves elevate the upper lid. The major contributor is the levator muscle, which is innervated by the thircl cranial nerve. This muscle extends as a broad band and inserts into the anterior surface of the cartilaginous tarsal plate. A secondary system inserts into the upper border of the tarsus; it is called Mller's muscle, and It has an associated pupillary sign. Miller's muscle is innervated by the sympathetic nervous system. A peculiar feature of anatomy, which requires understanding, is the intimate relationship between the superior rectus muscle, which elevates the eye, and the levator muscle. Both muscles come from the same anlage, at Zinn's ligament. Not infrequently, both are affected, particularly when trauma is involved.

Examination Techniques
Measure the distance between the two lid margins by measuring the lid fissures with a millimeter ruler, with the patient's eyes looking straight ahead. This method is more accurate than measuring how much of the lid is below the theoretically normal lid position, which, as mentioned, can vary. In planning any surgical correction, it is also important to know where the other normal lid sits in relation to the cornea. Another feature to consider is the function of the levator muscle. Using the millimeter ruler again, observe the excursion of the lid from eyes in the full down-gaze position to the full up-gaze position. Normal excursion is about 15 mm. A major error in evaluating the amount of ptosis can occur when a patient overcomes the ptosis by employing the frontalis muscle. If you observe a wrinkling over one eye and not over the other, suspect a slight ptosis that is being overcome by the use of the frontalis muscle, giving some lift to the ipsilateral lid. Have the patient close the lid, and press your thumb over the center of the patient's eyebrow. Do not push upand thus do the same thing the patient's frontalis muscle doesor push down, thereby depressing the lid and causing a ptosis. Press in toward the bone to prevent the frontalis muscle action on the skin overlying the lid. This maneuver should also be done when measuring excursions of the lid. True ptosis is due to a neuropathic process either in the third cranial nerve innervation to the levator muscle or in the sympathetic innervation to Mller's muscle or to one of several myopathic processes.

Pseudoptosis-Types and Causes

Protective Ptosis
Pseudoptosis has many manifestations, the most common one being protective ptosis associated with an irritated eye. The irritation may be an internal inflammation, such as kids. Photophobia is an early sign of iritis, and frequently it is present before any external inflammatory sign, such as redness. Photophobia may also be seen with inflammation in the posterior segment of the eye but not as consistently as in iritis. The possibility of a corneal foreign body, abrasion, or retained foreign body under the upper lid should be considered. Careful inspection of the upper lid when everted, particularly when doubly everted over a Desmarres retractor, is a necessity.

Hysteric Ptosis
Hysteric ptosis is usually unilateral, and the wrinkling of the orbicularis muscle can be seen as the cause. Cases of flaccid hysteric ptosis do occur, but they are rare.

Enophthalmos

The lid may be ptotic because of lack of support from the globe, a condition that occurs with enophthalmos secondary to a loss of orbital fat. The loss of fat occurs most often after blunt trauma with orbital hemorrhage or after trauma causing a blowout of the orbital floor with damage to the orbital contents.

Microphthalmia and Phthisis Bulbi

In congenital microphthalmia, the lid is not supported properly. A more common variety of microphthalmia is phthisis bulbi, a shrinking of a normal eye after total blindness occurs and internal disruption of all functions such as in end-stage glaucoma or severe disorganization after trauma.

Blepharochalasis
A common finding, and one that may mask an associated subtle but true ptosis, such as exists in Horner syndrome, is the blepharochalasis of the elderly. This condition results from loss of fascial attachments of the overlying skin to the levator muscle, causing the skin to fall down and cover the lid margin. Usually the skin can be gently lifted by pushing up on the brow; and the lid margin may then be found to be in a normal position.

Ptosis after Operation

Minimal ptosis may occur after cataract or retinal detachment operation, with traction sutures under the superior rectus muscle, or after temporary removal of that muscle.

Vertical Muscle Imbalance

The most subtle type of ptosis, and one that is frequently overlooked, is that caused by vertical muscle imbalance. If the patient fixes with the lower eye, the action causes the other eye to move up and places the lid lower on the cornea, thereby giving the appearance of ptosis. To detect this condition, alternately cover one eye and then the other. Two observations can be made. The apparently ptotic side will have that eye come down to fix when the cover is moved to the other eye. When each eye fixes, each lid will be in the same position in reference to the pupil and the cornea. The measurement of the fissures will also be found equal (Fig. 5.1, A and B).

Figure 5.1 Patient with a right hypertropia. A. She is fixing with the hypotropic eye, and no difference in the lids is noticed. B. She is fixing with the hypertropic eye, causing the left eye to look down and the left lid also to be down, giving the impression of a ptosis. The relative position of the pupil as a result of a tropia will influence which eye appears to have a pseudoptosis.

Apraxia of Lid Opening

Apraxia of lid opening is not true ptosis. A patient with this disorder has difficulty in opening the lids after forced closure. The condition is not related to blepharospasm, and the patient shows no contraction of the orbicularis muscle. Characteristically, to initiate movement, the patient may thrust the head back, as if to break some neuronal connection inhibiting lid opening. No ptosis occurs between attacks, which are associated with a variety of supranuclear lesions, including Huntington's chorea.

True Ptosis (FIGS. 5.2, 5.3)

Figure 5.2. Ptosis evaluation, part 1

Figure 5.3. Ptosis evaluation, part 2

Congenital Ptosis
Most kinds of congenital ptosis are not hereditary, with one notable exception. The ptosis associated with epicanthus inversus and blepharophimosis, which is usually bilateral, is frequently familial and may be a dominant characteristic. The other kinds of congenital ptosis have no familial association (and the ptosis is unilateral in 70% of the cases).

SUPERIOR RECTUS MUSCLE WEAKNESS

A feature often overlooked in congenital ptosis--and sometimes in acquired ptosis--is an associated weakness of the superior rectus muscle (found in about 10% of the cases). This feature must be considered in evaluating a patient for correction of ptosis. It is also important to evaluate the patient's Bell's phenomenon. Failure to recognize a superior rectus muscle weakness or an inadequate Bell's phenomenon may result in a corneal exposure problem that may be more serious than the original ptosis. Once a corrective surgical procedure has been performed on the lid, the superior rectus muscle weakness may be a greater cosmetic problem than the ptosis ever was. The patient may complain that the now obvious vertical displacement of the eye was caused by the operation. Improper selection of operative procedure may make the vertical problem worse.

LEVATOR APONEUROSIS DEHISCENE

In congenital ptosis, the distance from lid sulcus to lid margin is normal. The congenital ptotic lid is the higher not the lower lid on down-gaze because of structural changes that keep the lid from relaxing on down-gaze (Fig. 5.4). In cases of levator dehiscence, this impediment is absent, and the ptotic lid is the lower lid on both up-gaze and down-gaze (Fig. 5.5).

Figure 5.4 Patient with congenital ptosis. Note that the ptotic left lid is higher on down-gaze than normal lid.

Figure 5.5. Patient with dehiscence of the levator tendon. Note that the ptotic lid is lower than the normal eye in all positions of gaze, including down-gaze.

HORNER SYNDROME
Horner syndrome has ptosis as one of its prime signs. Just as the ptosis may be overlooked because of associated frontalis muscle assistance, incompleteness of Horner syndrome or the degree of alertness of the patient may cause the miosis to be overlooked if all the pupilinfluencing factors are not considered. A detailed description of these factors is given in Chapter 3. The presence of heterochromia on the side of the ptosis should alert one to look even harder for a smaller pupil on that side, since it goes along with a congenital Horner syndrome (Fig. 5.6). Heterochromia is seen only in a ptosis caused by sympathetic paralysis with a congenital or neonatal onset.

Figure 5.6. Patient with typical Horner syndrome with right ptosis and miosis of the right pupil. Even in a black and white photograph, the lighter color of the right iris can be detected. Heterochromia is a sign of congenital Horner syndrome.

CYCLIC THIRD CRANIAL NERVE PARALYSIS

Cyclic paralysis of the third cranial nerve may be complete or incomplete. This rare condition usually begins in childhood, remains throughout life, and is benign. It is gradual in onset until a certain point, and then it gradually recovers without any deficit. An entire episode lasts only a few minutes. After pupillary involvement, ptosis is one of the most constant features of this syndrome. The inclusion of other parts of the third cranial nerve varies from patient to patient. Lid twitching prior 1:0 the onset of each new phase is a characteristic sign. The extraocular muscles are infrequently involved and then only the medial rectus muscle. The onset in childhood, repetitiveness of course, and lack of residual deficit essentially

differentiate this disorder from third cranial nerve paralysis associated with diabetes or aneurysm, which comes on later in life. The cause is unknown, as is the treatment.

Acquired Ptosis
Hereditary ptosis coming on later in life is not common. The main example that I see is that associated with chronic progressive external ophthalmoplegia. The leading initial sign of the condition is ptosis, and it may be the only sign for many years. Observing other members of the patient's family, either in person or from photographs, is important. Many patients either suppress a family history or are truly unaware of it; since all members look alike, nobody thinks to comment about it. The Tensilon test is negative and excludes myasthenia gravis. The normal pupil and normal accommodation absolve the third cranial nerve.

MYASTHENIA GRAVIS
Ptosis is usually the first sign of myasthenia gravis in young people, just as it is in chronic progressive external ophthalmoplegia. The ptosis caused by myasthenia gravis, however, responds to a Tensilon injection. In addition, no pupillary or accommodative abnormalities exist, and the ophthalmoplegia that usually comes on later is similar to other myopathies, with the elevation of the eye being primarily affected. Keeping the eyes in sustained up-gaze for 1 to 2 minutes causes the ptosis to get worse. This factor is almost pathognomonic of myasthenia gravis and is not seen in Horner syndrome or third cranial nerve disease. When the cause of the ptosis is still in doubt, the lid peek sign may be useful. Just as the levator is weakened to hold the upper lid in its proper position, the orbicularis is also weak in keeping the fissure closed for a prolonged period. When a patient is asked to close the eyes and keep them closed, the fissure opens up in a few seconds because of the orbicularis weakness. This phenomenon needs to be differentiated from motor inpersistence, which is part of the syndrome of the nondominant hemisphere or which occasionally can be seen in bilateral hemisphere lesions. The associated hemisphere signs and symptoms should easily differentiate it from the lid peek sign of myasthenia gravis. In contrast to ptosis, lid retraction in myasthenia gravis is uncommon. When it occurs, three different types are categorized by the duration of the lid retraction. The most common form is the lid retraction associated with weakness of the contralateral levator muscle, which may be prolonged. This type of lid retraction is an example of Hering's law of equal innervation to yoke muscles at work. Hering's law can be temporarily altered toward the more normal side during the Tensilon test. During this test, less effort is required of the myasthenic lid, and no lid retraction develops in the other lid. The second type of lid retraction is Cogan's lid twitch sign. The upper lid overshoots when the eyes are moved rapidly from down-gaze to a position straight ahead. There is no sustained lid retraction in these instances. The third type of lid retraction was reported by Puklin, Sacks, and Boshes in a small series of myasthenic patients. This type of lid retraction is over in a few seconds and is caused by posttetanic facilitation of the levator, seen particularly after prolonged up-gaze. A similar mechanism was postulated for a comparable phenomenon in the small muscles of the hand of myasthenic patients. In patients with only ptosis or ocular signs of myasthenia gravis, the acetylcholine receptor antibody is positive in 65% of cases but in over 90% of patients with generalized myasthenia gravis. Medications can induce a myasthenic syndrome. These include curare, penicillamine, and the aminoglycosides such as gentamycin, neomycin, and tobramycin. These drugs appear to work at the myoneural junction.

THIRD CRANIAL NERVE PARALYSIS

One of the major causes of acquired ptotis is third cranial nerve paralysis owing to an aneurysm. If the ptosis is associated with diplopia or, more significantly, with a pupillary abnormality, aneurysm as the cause must be the first consideration. The condition requires prompt evaluation and treatment because of its catastrophic characteristics. Not all third cranial nerve paralyses come on with marked involvement of all parts of the third cranial nerve. In the overwhelming majority of patients with aneurysms causing third cranial nerve paralysis, however, the pupil is affected to some degree. Subtle changes in pupil size in dim illumination with distance fixation should be scrupulously evaluated. The extraocular muscle weakness may not be apparent or considered, since the patient does not complain of diplopia. When the patient is examined with the eyes in the extremes of gaze, particularly with the

affected eye turned up and out in the field of action of the superior rectus muscle, a small hypotropia may be seen that is not present in the opposite field of gaze. The other prime cause of third cranial nerve paralysis, diabetes, does not always spare the pupil and accommodation. In about 20% of cases of diabetic third cranial nerve paralysis, the pupil is involved; and these cases cannot be differentiated clinically from those caused by aneurysm, even when the urine gives a 4+ reaction for sugar. A third cranial nerve paralysis with the pupil involved must be considered to be caused by an aneurysm until that is adequately ruled out. Some degree of ptosis is almost always present in third cranial nerve paralysis from any cause. Adults with acquired Horner syndrome do not have the heterochromia associated with congenital Horner syndrome (Chapter 3). In any case of ptosis, examination of the pupils for size abnormalities is essential. When in doubt as to the equality of pupils, observe the pupils in dim light with the patient's gaze directed at a distance. The other test is to measure the near point of accommodation or the point at which the patient begins to notice blurring. The side with Horner syndrome will read considerably closer. Measure with the patient's glasses on for full distance correction and an equal amount of bifocal correction if needed. The procedure is most effective with patients over 40 years of age who require reading glasses. The high degree of residual accommodation in the young person makes the measurement difficult in those under the age of 35.

TRAUMA
Any injury often causes either direct damage to the levator muscle or damage to its oculomotor innervation. Since the superior rectus muscle has 'a common origin with the levator muscle, it is also frequently injured, with a resultant muscle imbalance. The damage may not be immediately apparent if the ptosis is severe enough to cover the pupil and prevent diplopia. The recovery from ptosis may take many months, and no corrective surgery should be considered until at least 6 months have elapsed with no improvement. Resorption of edema of the lids should not be interpreted as improvement of ptosis. Be conservative and do not overestimate the final outcome to the patient.

EXTERNAL TUMORS OF THE LID

Any external lid tumors large enough to pull the lid down are readily visible. Those that are in the superior orbit or in the upper fornix between the lids and the globe may not be appreciated on simple inspection. Diagnosis may require digital palpation of the area beneath the superior bony orbital rim, as well as double eversion of the upper lid and observation beneath it. Plexiform neuromas of the lid feel like a bag of worms rather than a single mass, and they frequently extend laterally beneath the skin of the cheek and into the temporal fossa. These neuromas are associated with generalized neurofibromatosis and have other signs, such as caf au lait spots and epilepsy.

STRABISMUS OPERATION
When an operation to correct strabismus shortens the superior rectus muscle, it may also pull the levator muscle down, causing ptosis, Such an operation, if carried too far posteriorly, may cause damage to the nerve going to the levator muscle (because the nerve passes through the superior rectus muscle on its way to the levator muscle). Ptosis thus caused would come on at the time of the surgical procedure, of course, and not months or years later.

MYOTONIC DYSTROPHY
Myotonic dystrophy does not start with ptosis and therefore is not usually a problem in diagnosis. The other muscular signs of myotonia, as well as the age of the patient at onset, make the diagnosis rather easy. Cataracts of all types are seen in association with myotonic dystrophy, but the Christmas tree cataract is pathognomonic. It is seen only with the slit lamp, and it appears as multiple scattered red and green crystalline dots throughout the lens.

Synkinetic Ptosis

In synkinetic ptosis, the change in degree of ptosis is related to some other facial movement, such as that of the nostril, ear, or jaw. Those cases of synkinetic ptosis associated with jaw movement (referred to as the Marcus Gunn, or jaw-winking, phenomenon) are the only ones of clinical significance. The patient exhibiting the Marcus Gunn phenomenon moves the lid up and down by opening and closing the mouth, with associated contraction of the masseter muscle. The same lid action can be achieved by contracting the masseter muscle when the jaw is moved from side to side. The lid movement can be brought out by having the patient chew gum or suck on a hard candy. As long as the masseter muscle is contracted, the ptosis is improved. The jaw-winking component seems to disappear with age in many patients, but the ptosis does not. The reason for this difference is not known.

Cerebral Ptosis
Cerebral ptosis is a rare but recognized clinical entity. The exact anatomic location of the lesion causing this phenomenon is unknown. Other CNS lid problems, such as apraxia of lid opening and blepharospasm, are also a mystery. Cerebral ptosis appears to be more common with right hemisphere strokes. Right conjugate deviation is more commonly seen with bilateral cases of ptotis. In contrast, we see lid retraction, dilation of the pupils, and conjugate deviation of the eyes to the opposite side with stimulation of area eight in the frontal lobes. Blepharospasm is certainly not true ptosis but spasm of the orbicular muscle and can be misinterpreted as ptosis by those who have not seen it before. A full description is covered in the chapter on facial nerve.

Hering's Law of Equal Innervation

Hering in 1868 first proposed the idea of equal innervation to extraocular yoke muscles. This phenomenon allows the two eyes to move smoothly and together in all types of eye movements. It maintains binocular vision during these ocular excursions. Later investigators have applied Hering's equal innervation theory to the levator muscle as well. Unilateral lid retraction is well recognized as occurring when there is contralateral ptotis. This is a frequent result of additive lid support from the frontalis muscle and should be looked for when examining for ptotis. A small ptosis may be overcome by the frontalis support and may be missed by the casual observer. Clinical evidence for equal innervation is the presence of lid retraction in the eye contralateral to a ptosis. Further evidence in support of this innervational hypothesis is obtained when the lower of the two lids is manually lifted. This maneuver reduces innervation to the lifted ptotic lid and also to the retracted lid. Because innervation to the retracted lid is now less, it assumes a normal innervation and position in reference to the globe. This same result can be achieved by covering the eye with the ptotic lid and forcing the patient to fix with the eye that has the retracted lid. If Hering's law of equal innervation can be applied at the nuclear level rather than peripherally, it is equally appealing. This is because of the close proximity of the levator subnuclei to the other subnuclei of the third nerve nucleus. We know that there is some interrelationship between the ocular motor nuclei in the area of the superior rectus muscle. Other nerves also play a role in lid position, including the facial nerve, which controls closing of the lid by innervating the orbicularis muscle. In addition to the levator muscle lifting the lid via oculomotor innervation, the sympathetic system innervates Miller's muscle. All of these muscles are not always subject to Hering's law. In fact, it is easy to open or close one eye, smile on one side of the face, or wink. However, unless we consciously do these things, there is bilateral symmetrical movement such as when we blink or smile. There are many cases of lid retraction that have no relationship to Hering's law. These entities are discussed below.

Third Cranial Nerve Misdirection

Synkinetic retractions may be seen in third cranial nerve misdirection between the levator muscle and the inferior rectus muscle; they are referred to as the pseudo-von Graefe's sign. After third cranial nerve paralysis owing to trauma or aneurysm, a misdirection phenomenon may occur between the levator muscle and the inferior rectus or medial rectus muscle. When the eye is moved in the direction of either of these muscles, the lid may be innervated, causing lid retraction.

Lid Retraction
A consideration of lid retraction should be included in any discussion of ptosis. When looking at a patient with ptosis, the physician must decide whether one lid is ptotic or levator muscle and the inferior rectus muscle on down-gaze.

Adversive Seizures
Frontal lobe or adversive seizures have associated ocular signs that are of interest. When one frontal lobe is stimulated, the head and eyes are driven toward the other side and the upper lids retract. Between seizures, the lids and the gaze mechanisms are normal.

Lid Retraction after Operation

Previous operation for ptosis with secondary overcorrection or a surgical procedure for laceration of the lid with contraction of the scar in a vertical direction may result in retraction of the lid. These causes of lid retraction may be identified readily from the history or from simple inspection.

Claude Bernard Syndrome

Claude Bernard syndrome is the, opposite of Horner syndrome. In this rare syndrome, lesions stimulate the sympathetic chain, causing ipsilateral lid retraction. The lesions that cause Horner syndrome also cause Claude Bernard syndrome. Occasionally, a patient voices complaints typical of those made in Claude Bernard syndrome but on examination will be found to have a slight Horner syndrome rather than lid retraction and enlarged pupils. Such a patient may have a peripheral Horner syndrome in which a supersensitivity to his or her own circulating epinephrine has developed. This possibility is confirmed by placing a 1:1000 aqueous solution of epinephrine in both eyes and observing them 30 minutes later. The side the patient has complained about will show a larger pupil and a variable itinount of upper lid retraction.

Voluntary Subluxation of the Globe

In this frightening spectacle, the patient is able to markedly retract the upper and lower lids to near the equator of the globes. At the same time, the patient subluxates the globes enough to get the lids behind the equatorial plane. He or she then squeezes the orbicularis muscle and closes the lids, thus pushing the globes farther out. The patient can usually reverse the process unless the lids go into spasm or secondary swelling occurs. At this point, the patient may require a partial facial nerve block to release the lid spasm and replace the globes.

Myotonic Lid Lag

There are cases of myotonic lid lag in both hypo- and hyperkalemic periodic paralysis that initially look like lid retraction. Myotonic lid lag is more common in the hyperkalemic form but can occur in the hypokalemic form, making the institution of potassium therapy sometimes puzzling.

Tests for Ocular Myasthenia Gravis

Tensilon Test
Any ptosis that is not of obvious origin deserves a Tensilon test, which is given as a 1-mL intravenous injection. Traditionally, it has been advised that an adequate close of atropine be given to the patient prior to injection. A problem involved in giving the atropine dose is that the medication lasts much longer than is required for the test and becomes uncomfortable to the patient. Another problem is that most patients are given the atropine intramuscularly and then almost immediatelybefore it has a chance to workare given the Tensilon test. Many physicians feel that the use of atropine is unnecessary but that it should be available. To do the Tensilon test, give the patient 0.1 mL of Tensilon as a screening dose while looking for any overreactions to the drug. If none occur within 1 minute, then give the rest of the dose in 0.2-mL doses per 30 seconds. An improvement in the ptosis should be seen within 30 seconds; the entire effect will

pass within 3 minutes. The important part of the test is to find the area of maximum ocular deficit, either in extraocular muscle imbalance or as ptosis, before giving the test. Then examine that entity or position during the test period. A positive response reflects significant improvement in these areas and pinpoints the cause as myasthenia gravis. Ptosis is more easily evaluated with the Tensilon test than is diplopia.

Quinine Challenge
Before the Tensilon test came into use, an opposite approach was in vogue, namely, seeing whether one could make the myasthenia worse. The physician gave the patient quinine, which reduced the sensitivity of the motor endplate and thus aggravated the myasthenia. This approach is no longer taken, but the reaction to quinine may still be seen in two other situations: (a) a patient who drinks tonic water may ingest enough quinine to cause a worsening of any myasthenia and (b) quinine given to many older people as a treatment for night leg cramps may at the same time aggravate a myasthenic process.

Electromyograph
In my experience, electromyograms for ocular myasthenia gravis are not useful. The electrical pattern obtained may be typical of myasthenia gravis but not entirely diagnostic. Furthermore, the technique is inadequate because the recording needle is frequently inserted in the levator aponeurosis rather than in the muscle, which is farther back in the orbit. Testing other muscles, such as the biceps muscle, may give false-negative results if the patient has only the ocular form of myasthenia gravis.

Biopsy
Biopsy is also of limited diagnostic value because, first, one has to get some muscle and not the aponeurosis, and second, the specific features that are diagnostic of myasthenia gravis are not commonly seen in biospy specimens. The basic defect is not in the muscle anatomy; it is a biochemical defect in the number of packets of acetylcholine in the motor endplate. The biochemical defect is evaluated by the Tensilon test. The pathologic changes are indirect evidence of the disease. None of the other myopathic processes involve significant enough differential pathologic changes to warrant a biopsy. Drachman points out that one cannot differentiate a neuropathic process from a myopathic one by a biopsy. In addition, good experimental evidence now exists that an antigen-antibody reaction at the motor endplate also interferes with the motor endplate function.

Treatment of Ptosis
Treatment of ptosis depends on the problems involved. If the ptosis causes skeletal deformity owing to head position or if the lid covers the pupil in a child in whom amblyopia may develop, repair must be undertaken early. If the ptosis is caused by recent trauma, delay and careful observation for many months are indicated before embarking on a surgical procedure. If the condition is a myopathy and up-gaze is affectedor may be affected in the futuresurgical caution is the word because severe corneal exposure (owing to inadequate Bell's phenomenon and the associated orbicularis muscle weakness during sleep) becomes a problem. Cautious neglect may be the treatment of choice in these cases.

Surgical Treatment
The type of operation varies according to the degree of ptosis, the amount of levator muscle function, and the judgment of the surgeon. In-depth discussion of the possibilities is best left to surgical texts. The two approaches I recommend are the tarsal-conjunctival approach of Iliff for a small ptotis (3 mm or less) and the skin approach of Berke for a larger one. For ptosis associated with the Marcus Gunn phenomenon, the frontalis sling, along with the cutting of the innervation to the lid so as to prevent the recurrence of jawwinking, is recommended. Crutch glasses can be used, but they seldom are tolerated by the patient.

Corticosteroid Therapy
Oral corticosteroids have been used in the treatment of ocular myasthenia gravis for several years. Despite the lack of universal agreement as to the efficacy of this treatment in purely ocular cases, certainly it may be worthwhile in a number of cases, particularly when the patient does not have an adequate response to anticholinesterase drugs. In addition, some evidence suggests that long-term

anticholinesterase therapy may destroy some of the smaller nerve terminals and therefore may not be the best treatment over long periods of time.

Treatment of Lid Retraction

The treatment of lid retraction is the treatment of the underlying process. In the case of thyroid disease, the lid retraction usually does not go away completely with control of the hyperthyroidism, but it can be further Improved by lateral and, if necessary, a small medial tarsorrhaphy. In extreme cases, a reverse ptosis procedure can be performed, in which part or all of the tarsal attachments of the smooth and striate muscles are detached. The use of topical guanethidine to create a chemical Horner syndrome was first reported by Gay and his associates and subsequently popularized in the British literature. It has not been useful in my hands and has a history of ocular irritation. Recent studies with topical 0.5% thymoxamine have been more promising but also have the same irritating effects when applied topically. The thymoxamine is an alphaadrenergic blocker and, therefore, ideal to effect abnormal stimulation of Miller's muscle. Thymoxamine does relieve about 2 to 3 mm of lid retraction even if thickened muscles are demonstrable on computerized tomography. It does not work on stable, longstanding thyroid eye disease. It also does not affect normal persons, nor does it work in a variety of other neuromuscular diseases. It is in this latter category, as a diagnostic agent to confirm thyroid disease and rule out other neuromuscular diseases that thymoxamine may find its greatest use.

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