ptosis [emedicine]

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emedicine.medscape.com

eMedicine Specialties > Ophthalmology > Lid

Ptosis, AdultAdam J Cohen, M D, Eyelid and Facial Aesthetic and Reconstructive Surgery, Diseases and Surgery of the Orbit and Lacr imal System, Cosmetic Laser Surgery

Michael Mercandetti, MD, MBA, FACS, Consulting Staff, Department of Surgery, Doctors Hospital of Sarasota

Updated: Nov 18, 2009

Introduction

Background

Blepharoptosis, also referred to as ptosis, is defined as an abnormal low-lying upper eyelid margin with the eye in primary gaze. The normal adult upper lid lies 1.5 mm below

the superior corneal limbus and is highest just nasal to the pupil.

Left ptosis. Lid crease is absent on the left. The crease is up in the sulcus. Superior sulcus deformity is present on the left and right, and the patient is elevating her brows. The

right upper lid should be checked for an underlying or masked ptosis. If the right lid is ptotic, lifting the left lid causes the right lid to droop.

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Visual field shows functional blockage of superior visual field due to a ptotic lid. Hashed line represents the superior extent of the seen visual field with the lid lifted. Solid line is

with the lid in its natural, ptotic position.

Blepharoptosis can be c lassified as congenital or acquired. This differentiation is based on age. A more comprehensive class ification is based on etiology and includes

myogenic, aponeurotic, neurogenic, mechanical, traumatic, and pseudoptotic. The most common cause of congenital ptosis is myogenic due to the improper development of

the levator muscle.

Congenital ptosis on right. Note the presence of a lid crease.

Most cases of acquired blepharoptosis are secondary to aponeurotic causes, such as involutional changes, a disinsertion, or a dehiscence. Identification of the underlying

pathophysiologic mechanism is paramount in instituting optimal treatment.

Pathophysiology

Blepharoptosis, which is a droopy upper eyelid, is the result of dysfunctioning of one or both upper eyelid elevator muscles. These elevator muscles are the levator palpebrae

superioris and its aponeurosis and the Mueller muscle.

The levator palpebrae superioris is a striated muscle that is innervated by the superior division of the oculomotor nerve (cranial nerve III). This muscle is about 40 mm long and

originates from the lesser wing of the sphenoid. It continues anteriorly, and at the W hitnall ligament, it travels inferiorly as an aponeurosis. This aponeurosis is 14-20 mm long

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and inserts into the anterior aspect of the tarsal plate. It also sends attachments to the skin, forming the upper eyelid crease. The levator muscle and aponeurosis is the major

elevator of the upper eyelid.

The Mueller muscle, a sympathetically innervated smooth muscle, has its origins from the undersurface of the levator superioris. Approximately 12 mm long, it inserts

superiorly on the tarsal border and elevates the upper eyelid by approximately 2 mm.

Mortality/Morbidity

The associated mortality is usually due to anesthetic complications from surgery. Kearns-Sayre disease, a subtype of chronic progressive external ophthalmoplegia, is

a syndrome with associated myogenic ptosis, retinal pigmentary changes, and cardiac conduction abnormalities that can cause death.

Morbidity is associated with blockage of the visual axis in the severely ptotic eyelid. Congenital cases can obst ruct vision and lead to amblyopia. Even without visual

axis obstruct ion, the eyelid may induce refractive errors, especially ast igmatism resulting in amblyopia.

In adults, the morbidity is associated with constriction of the superior visual fields. Patients may complain that they tire easily when reading and experience frontal

headaches as they lift t heir eyebrows in an effort to keep the eyelids open. Patients may state they are dissatisfied with their appearance.

Race

No racial predilection has been described.

Sex

No sexual predilection has been described.

Age

Acquired ptosis can occur at any age, but it is commonly seen in older adults. Congenital ptosis occurs at birth.

Clinical

History

As with any patient, obtain a thorough medical and ophthalmic history.

More specifically, the onset of ptosis, alleviating or aggravating factors, family history of ptosis, and history of trauma or ocular surgery are important clues to the

etiology.

Patients usually complain of a bedroom-eye appearance, always appearing sleepy or tired, and constriction of the visual fields.

Physical

If the patient has not been under the care of an ophthalmologist, a complete ocular examination is required.

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Quantification and qualification of the blepharoptosis is essential for proper diagnosis and treatment. All quantitative eyelid and eyebrow measurements should be taken before

the use of dilating drops.

The palpebral fissure is the distance between the upper and lower eyelid in vertical alignment with the center of the pupil.

The marginal reflex distance-1 (MRD-1) is the distance between the center of the pupillary light reflex and the upper eyelid margin with the eye in primary gaze. A

measurement of greater than 2.5 mm is considered normal.

The marginal reflex distance-2 (MRD-2) is the distance between the center of the pupillary light reflex and the lower eyelid margin with the eye in primary gaze. Ameasurement greater than 5 mm is considered normal.

The margin crease distance is the distance from the upper eyelid margin to the lid crease. In white women, a central measurement of 10-11 mm is considered normal,

and in white men, 8-10 mm is considered normal.

Levator function is the distance the eyelid travel from downgaze to upgaze while the frontalis muscle is held inactive at the brow. A measurement of greater than 10 mm

is considered excellent, whereas 0-5 mm is considered poor.

The presence of proptosis, lagophthalmos, tear dysfunction, absence of a Bell response, and lower eyelid laxity or scleral show should be appreciated and may alter

the amount of ptosis repair.

Pseudoptosis can result from mic rophthalmos, enophthalmos or anophthalmos, acquired hypotropia after a blowout fracture (orbital floor fracture), superior sulcusdeformity, or contralateral vertical lid retraction.

Eyelid retraction may warrant thyroid function studies and the consideration of dysthyroid orbitopathy.

Parinaud syndrome should be considered if convergence-retraction nystagmus and pupillary light-near disassociation is found in conjunction with eyelid

retraction; neuroimaging should be obtained.

The margin fold distance is the distance from the upper eyelid margin to the fold of skin.

Causes

Ptosis can be caused by problems with the elevator muscles of the eyelid, the aponeurosis of the levator, nerve abnormalities either central or peripheral, trauma,inflammation, or lesions of the lid or orbit.

Aponeurotic ptosis is the most common cause of acquired ptosis.

Senescence, involutional changes, dehiscence, or disinsertion of the levator aponeurosis are common.

Chronic inflammation or intraocular surgery (eg, cataract surgery) can incite stretching of the levator aponeurosis and dehiscence from the anterior surface of the

tarsal plate.

Long-term use of contact lenses has also been implicated. Patients maintain normal or near-normal levator function, with a high upper eyelid crease. The

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attachments from the levator to the skin remain intact, and this forms the crease.

Neurogenic blepharoptosis may be congenital or acquired in origin. Congenital neurogenic ptosis is usually due to Horner syndrome or a third nerve palsy. Acquired

neurogenic ptosis causes include Horner syndrome, third nerve palsy, or myasthenia gravis.

Congenital Horner syndrome can result in mild ptosis associated with ipsilateral miosis, iris and areola hypopigmentation, and anhidrosis. The cause is paresis

of the Mueller muscle, secondary to an embryologic les ion of the sympathetic pathway.

Congenital third nerve palsy has a variety of causes. Patients can present with aberrant regeneration and a small pupil. Often, parents believe that this is

secondary to birth trauma.

Acquired Horner syndrome can be secondary to trauma, neoplastic insult, or vascular disease of the sympathetic pathway. All stigmata of congenital Horner

syndrome, exc luding iris and areola hypopigmentation, are present. Raeder paratrigeminal syndrome occurs in middle-aged men with daily ipsi lateral headaches

and the stigmata of acquired Horner syndrome.

Dysfunction of the third cranial nerve can result from a myriad of acquired insults. Trauma, multiple sclerosis, vasculopathy, and infection are all potential

etiologies. Extraocular muscle dysfunction, pupillary abnormalities, and the presence of aberrant regeneration may aid in establishing the correct diagnosis.

Synkinetic neurogenic ptosis is the product of innervational anomalies. Marcus-Gunn jaw winking and posttraumatic ptosis are 2 examples of this interesting

etiology. Microvascular diabetic neuropathies never result in synkinetic neurogenic ptosis.

Myogenic blepharoptosis usually is congenital, but it can be associated with acquired disease processes.

Congenital myogenic ptosis is secondary to levator dysgenesis.

Acquired myogenic ptosis can be found in myasthenia gravis, chronic progressive external ophthalmoplegia, oculopharyngeal dyst rophy, and myotonic

dystrophy.

Traumatic blepharoptosis can ensue after an eyelid laceration with transection of the upper eyelid elevators or disruption of the neural input.

Mechanical ptosis can stem from the presence of eyelid neoplasms, for example, neurofibromas or hemangiomas or cicatrization secondary to inflammation or surgery.

Differential Diagnoses

Anophthalmos Hemangioma, Capillary

Apraxia of Lid Opening Horner SyndromeBell Palsy Laceration, Eyelid

Blepharospasm, Benign Essential Lyme Disease

Cellulitis, Orbital Marcus Gunn Jaw-winking Syndrome

Cellulitis, Preseptal Multiple Sclerosis

Chalazion Myasthenia Gravis

Chronic Progressive External Ophthalmoplegia Neuro-ophthalmic History

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Conjunctivitis, Giant Papillary Neurofibromatosis-1

Corneal Abrasion Oculomotor Nerve Palsy

Corneal Foreign Body Orbital Fracture, Apex

Dermatitis, Atopic Orbital Fracture, Floor

Duane Syndrome Ptosis, Congenital

Exophthalmos Thyroid Ophthalmopathy

Other Problems to Be ConsideredCraniofacial syndromes

Socket contraction

Poor-fitting ocular prosthesis

Hemifacial spasm

Blepharophimosis

Blepharochalasia

Double elevator palsy

Orbital and lid tumors

Cavernous sinus syndrome

Superior orbital fissure syndrome

Malingering

Workup

Laboratory Studies

If myasthenia gravis is suspected, a serum assay for acetylcholine receptor antibodies and an edrophonium chloride (Tensilon) test or single-fiber electromyography

may be needed.

CSF analysis can aid in the diagnosis of multiple sclerosis. Mild lymphocytosis or increased protein levels in the CSF levels may be present. In addition, elevated

immunoglobulin G (IgG) levels and oligoclonal bands often are found.

In patients with chronic progressive external ophthalmoplegia, an electrocardiogram, electroretinogram, elect romyography, and mitochondrial assay should be

considered.

Patients with suspected thyroid abnormalities should undergo tests for thyroid function, including triiodothyronine (T3), thyroxine (T4), and thyroid-stimulating hormone

(TSH).

Imaging Studies

MRI of the brain with gadolinium enhancement is the imaging modality of choice if multiple sclerosis is suspected.

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If blepharoptosis is present with other neurologic deficits, imaging of the brain, orbits, or cerebrovascular system should be performed.

CT scanning can be used to evaluate dysthyroid orbitopathy.

In acquired Horner syndrome, MRI or CT of the brain, CT or radiography of the spine, and CT or radiography of the chest (especially of the apex of the lung) are

warranted.

Other Tests

Sympathomimetic agents can be used to stimulate the Mueller muscle, as follows:

2.5% phenylephrine

10% phenylephrine: Be aware of cardiac complications.

0.5% apraclonidine (Iopidine): This is an alpha-adrenergic agonist.

1.0% apraclonidine (Iopidine): This is an alpha-adrenergic agonist.

Instill 2 drops on the eye under the eyelid (have the patient look down), wait 5 minutes, and assess any change in the palpebral fissure and the marginal reflex distance.

If no response is observed or if elevation is not adequate, external levator resection or advancement may be needed to correct the blepharoptosis.

If a good response is observed, the ptosis can be repaired by advancing the internal levator (Mueller muscleconjunctival resection).

Treatment

Medical Care

If myasthenia gravis is diagnosed, treatment may involve the use of pyridostigmine (Mestinon).

Patient with myasthenia gravis. Right lid is more ptotic than the left lid.

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Same patient as in Media file 8, 3 months later. Note how the ptosis has changed and is more on the left than the right.

In certain cases, a patient may not want to undergo surgery. Glasses can be made with a crutch attachment that can hold up the lid.

Glasses with a crutch attached (arrow) that can be used to lift the lid if the patient does not desire surgery.

Surgical Care

Many surgical techniques have been well described for blepharoptosis correction. A surgeon may prefer one technique to another. This brief discussion is merely a guide and

not dogma for approaching ptosis correction.

If levator function is poor (


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A levator advancement or resection is a technique that results in shortening of the levator aponeurosis and muscle, depending on the amount of correction needed. The

levator can be approached from an anterior or posterior direction.

In the anterior approach, an external eyelid incision is made by using the natural lid crease, if present, to allow for direct visualization of the aponeurosis. Once

the levator aponeurosis is identified, it is disinserted from the tarsus, advanced and/or resected, and reattached. The amount of advancement depends on the

degree of blepharoptosis being t reated. The aponeurosis also is attached to the sk in to reform the crease.

Anterior approach to the levator. White band is the levator aponeurosis (arrow).

In posterior levator resection, the eyelid is everted, and the conjunctiva is separated from the Mueller muscle and the levator aponeurosis. Double-armed sutures

are placed in the conjunctiva. The Mueller muscle and levator are separated from the septum and clamped. Then, the preplaced sutures in the conjunctiva are

passed through the levator, and the excess t issue is excised. The sutures are passed through the skin with 1 arm of the double-armed suture taken a bit t hrough

the tarsus, and these sutures are tied reforming the eyelid crease.

If the levator is disinserted or dehisced, the anterior or posterior approach can be used, and the dehiscence or disinsertion repaired.

In the Fasanella-Servat ptosis procedure, the conjunctiva and tarsus and the Mueller muscle are resected. Two hemostats are placed across the superior tarsal border.

The tissue below the hemostats is sutured, and then the tissue is resected.

The internal levator advancement, known more commonly as the Mueller muscleconjunctival resection, is performed on the underside of the lid, as in a Fasanella-

Servat procedure.

This surgery is chosen if the eyelid has had a good response to phenylephrine.

The conjunctiva and the Mueller muscle are marked off, clamped with a specialized clamp, sutured, the tissues are resected.

The conjunctival layer is then closed.

This procedure is believed to advance the levator aponeurosis, thereby elevating the ptotic lid.

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Patient with bilateral ptosis before surgery. Note the high lid creases.

Same patient as in Media file 1 after bilateral internal levator advancement. No skin incision was made, and no crease reformation was performed.

Patient with bilateral ptosis before surgery.

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Same patient as in Media file 10 after internal levator advancement. Patient has excessive sk in (dermatochalasia) after the lid was lifted, with a pseudoptotic effect more on the

left than the right. The dermatochalasia was present before surgery but is more significant afterward. Patient also has brow ptosis.

Consultations

If a specific etiology of blepharoptosis is identified and has related systemic manifestations, consultation with other specialists is necessary.

If myasthenia gravis or multiple sclerosis is diagnosed, appropriate follow-up care with a neurologist is warranted.

If dysthyroid orbitopathy is found, an endocrinologist should be consulted to address the thyroidopathy.

Patients with Kearns-Sayre disease can have cardiac conduction abnormalities that should be managed by an internist or a cardiologist.

If the etiology of the ptosis is unclear and associated with ophthalmoplegia, consultation with a neuro-ophthalmic specialist is prudent.

Follow-up

Further Outpatient Care

If surgical correction of blepharoptosis is undertaken, the patient should be observed on days 1-7 after surgery.

Inpatient & Outpatient Medications

After blepharoptosis surgery, a topical antibiotic ointment (with or without a steroid) should be applied twice daily for 5-7 days.

An oral antibiotic, that is , a penicillin derivative or a cephalosporin, may be given for 5-7 days as well.

Complications

Uncorrected congenital ptosis can result in amblyopia secondary to deprivation or uncorrected astigmatism.

An abnormal eyelid position can have negative psychosocial effects, especially in young children and teenagers.

Ostracism can lead to poor academic performance, loss of self-esteem, and alienation.

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In some cases, uncorrected acquired blepharoptosis results in decreased field of vision and frontal headaches.

The decreased visual field can affect one's ability to perform activities of daily life.

Driving, reading, and navigating a flight of steps can be particularly difficult.

If correction of blepharoptosis is undertaken, complicat ions related to the surgery can ensue.

Because most ptosis surgery is performed with the patient under local anesthesia and with monitored anesthesia care, reactions to anesthetic agents are

possible complications.

Bleeding and poor response to anesthetic agents are potential intraoperative complicat ions.

Bleeding and infection can be devastating complications in the early postoperative period. Prolonged bruising, edema, undercorrection or overcorrection of the

ptosis, eyelid asymmetry, and corneal foreign body sensation can be later complications.

Patient Education

Inform patients that symmetry is difficult, if not impossible, to achieve (see Medical/Legal Pitfalls).

Miscellaneous

Medicolegal Pitfalls

Correction of blepharoptosis without an appropriate examination or exclus ion of medically t reatable etiologies can result in poor outcomes.

Aesthetic and functional complications can lead to dissatisfied patients, a reduced referral base, and litigation.

Patients must be informed that symmetry is difficult, if not impossible, to achieve.

If a patient presents with unilateral ptosis, t he other eyelid must be evaluated to ensure that contralateral ptosis is not present.

Even if contralateral ptosis is not discovered on examination, informing the patient that the uninvolved side might manifest ptosis after surgery may be prudent.

Also, when an eyelid is lifted, the amount of dermatochalasia may appear to be increased. The patient should be forewarned of this outcome and of the need for

possible blepharoplasty.

Multimedia

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Media file 1: Patient with bilateral ptosis before surgery. Note the high lid creases.

Media file 2: Same patient as in Media file 1 after bilateral internal levator advancement. No skin incision was made, and no crease reformation was performed.

Media file 3: Anterior approach to the levator. White band is the levator aponeurosis (arrow).

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Media file 4: Left ptosis. Lid crease is absent on the left. The crease is up in the sulcus. Superior sulcus deformity is present on the left and right, and the patient is elevating her

brows. The right upper lid should be checked for an underlying or masked ptosis. If the right lid is ptotic, lifting the left lid causes the right lid to droop.

Media file 5: Visual field shows functional blockage of superior visual field due to a ptotic lid. Hashed line represents the superior extent of the seen visual field with the lid lifted.

Solid line is with the lid in its natural, ptotic position.

Media file 6: Congenital ptosis on right. Note the presence of a lid crease.

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Media file 7: Glasses with a crutch attached (arrow) that can be used to lift the lid if the patient does not desire surgery.

Media file 8: Patient with myasthenia gravis. Right lid is more ptotic than the left lid.

Media file 9: Same patient as in Media file 8, 3 months later. Note how the ptosis has changed and is more on the left than the right.

Media file 10: Patient with bilateral ptosis before surgery.

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Media file 11: Same patient as in Media file 10 after internal levator advancement. Patient has excessive skin (dermatochalasia) after the lid was lifted, with a pseudoptotic effect

more on the left than the right. The dermatochalasia was present before surgery but is more significant afterward. Patient also has brow ptosis.

References

1. Arslan E, Demirkan F, Unal S, et al. Enhanced frontalis sling with double-fixed, solvent-dehydrated cadaveric fascia lata allograft in the management of eye ptosis. J

Craniofac Surg. Nov 2004;15(6):960-4; discussion 965-6. [Medline].

2. Beard C. Types of ptosis . In: Beard C, ed. Ptosis. 3rd ed. St. Louis: Mosby; 1981:39-76.

3. Carter SR, Meecham WJ, Seiff SR. Silicone frontalis slings for the correction of blepharoptosis: indications and efficacy. Ophthalmology. Apr 1996;103(4):623-

30. [Medline].

4. Collin JRO. Ptosis. In: Manual of Systematic Eyelid Surgery. Oxford, England: Butterworth-Heinemann; 1999:41-72.

5. Dinges WL, Witherspoon SR, Itani KM, Garg A, Peterson DM. Blepharoptosis and external ophthalmoplegia associated with long-term antiretroviral therapy. Clin Infect

Dis. Sep 15 2008;47(6):845-52. [Medline].

6. Dutton JJ.Atlas of Clinical and Surgical Orbital Anatomy . Philadelphia: WB Saunders; 1994:120-5.

7. Emsen IM. A new ptosis correct ion technique: a modification of levator aponeurosis advancement. J Craniofac Surg. May 2008;19(3):669-74. [Medline].

8. Frueh BR, Musch DC, McDonald H. Efficacy and efficiency of a new involutional ptos is correct ion procedure compared to a traditional aponeurotic approach. Trans Am

Ophthalmol Soc. 2004;102:199-206; discussion 206-7. [Medline].

9. Frueh BR, Musch DC, McDonald HM. Efficacy and efficiency of a small-incision, minimal dissection procedure versus a traditional approach for correcting aponeurotic

ptosis. Ophthalmology. Dec 2004;111(12):2158-63. [Medline].

10. Goldey SH, Baylis HI, Goldberg RA, et al. Frontalis muscle flap advancement for correction of blepharoptosis. Ophthal Plast Reconstr Surg. Mar 2000;16(2):83-

93. [Medline].

11. Levine MR. Manual of Oculoplastic Surgery. Oxford, England: Butterworth-Heinemann; 1996:75-105.

12. Park DH, Baik BS. Advancement of the Mller muscle-levator aponeurosis composite flap for correction of blepharoptosis. Plast Reconstr Surg. Jul 2008;122(1):140-

2. [Medline].

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13. Putterman AM. Cosmetic Oculoplastic Surgery Eyelid, Forehead, and Facial Techniques . London: WB Saunders; 1999:137-59.

14. Sakol PJ, Mannor G, Massaro BM. Congenital and acquired blepharoptosis. Curr Opin Ophthalmol. Oct 1999;10(5):335-9. [Medline].

15. Tsa CC, Li TM, La CS, et al. Use of orbicularis oculi muscle flap for undercorrected blepharoptosis with previous frontalis suspension. Br J Plast

Surg. Sep 2000;53(6):473-6. [Medline].

Keywords

adult ptosis, blepharoptosis, droopy lid, droopy eyelid, drooping eyelid, upper eyelid ptosis, lazy eye, bedroom eyes

Contributor Information and Disclosures

Author

Adam J Cohen, MD, Eyelid and Facial Aesthetic and Reconstructive Surgery, Diseases and Surgery of the Orbit and Lacrimal System, Cosmetic Laser Surgery

Adam J Cohen, MD is a member of the following medical societies: American Academy of Ophthalmology and American College of Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Michael Mercandetti, MD, MBA, FACS, Consulting Staff, Department of Surgery, Doctors Hospital of Sarasota

Michael Mercandetti, MD, MBA, FACS is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy

of Ophthalmology, American College of Surgeons, American Society for Laser Medicine and Surgery, American Society of Ophthalmic Plast ic and Reconstructive Surgery,

Association of Military Surgeons of the US, and Sarasota County Medical Society


Medical Editor

Ron W Pelton, MD, PhD, Private Practice, Colorado Springs, Colorado

Ron W Pelton, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of

Ophthalmic Plastic and Reconstructive Surgery, Colorado Medical Society, Utah Medical Association, and Wilderness Medical Society


Pharmacy Editor

Simon K Law, MD, PharmD, Assis tant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans

Affairs Healthcare Center, West Los Angeles

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research

in Vis ion and Ophthalmology


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Managing Editor

J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American

Medical Association, Association for Research in Vis ion and Ophthalmology, Florida Medical Associat ion, Pan-American Association of Ophthalmology, Sigma Xi, and

Southern Medical Association


CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri


Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of

Ophthalmology


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