MACULAR HOLE AND PUCKER

The information provided in this section was developed by the National Eye Institute (NEI) to help patients and their families search for general information about macular hole.    An ophthalmologist  who has examined the patient's eyes and is familiar  with his or her medical history is the best person to answer specific  questions.

Other Names  

Macular cyst, retinal hole, retinal tear, and retinal perforation.

What is a macular hole?  

A macular hole is a small break in the  macula, located in the center of the eye's light-sensitive tissue called  the retina. The macula provides the sharp, central vision we need for  reading, driving, and seeing fine detail.

A macular hole can cause blurred and  distorted central vision. Macular holes are related to aging and usually  occur in people over age 60.

Is a macular hole the same as age-related macular degeneration?  

No. Macular holes and age-related  macular degeneration are two separate and distinct conditions, although  the symptoms for each are similar. Both conditions are common in people  60 and over. An eye care professional will know the difference.

What causes a macular hole?  

Most of the eye's interior is filled  with vitreous, a gel-like substance that fills about 80 percent of the  eye and helps it maintain a round shape. The vitreous contains millions  of fine fibers that are attached to the surface of the retina. As we  age, the vitreous slowly shrinks and pulls away from the retinal  surface. Natural fluids fill the area where the vitreous has contracted.  This is normal. In most cases, there are no adverse effects. Some  patients may experience a small increase in floaters, which are little  "cobwebs" or specks that seem to float about in your field of vision.

However, if the vitreous is firmly  attached to the retina when it pulls away, it can tear the retina and  create a macular hole. Also, once the vitreous has pulled away from the  surface of the retina, some of the fibers can remain on the retinal  surface and can contract. This increases tension on the retina and can  lead to a macular hole. In either case, the fluid that has replaced the  shrunken vitreous can then seep through the hole onto the macula,  blurring and distorting central vision.

Macular holes can also occur from eye  disorders, such as high myopia (nearsightedness), macular pucker, and  retinal detachment; eye disease, such diabetic retinopathy and Best's  disease; and injury to the eye.

What are the symptoms of a macular hole?  

Macular holes often begin gradually. In  the early stage of a macular hole, people may notice a slight  distortion or blurriness in their straight-ahead vision. Straight lines  or objects can begin to look bent or wavy. Reading and performing other  routine tasks with the affected eye become difficult.

Are there different types of a macular hole?  

Yes. There are three stages to a macular hole:

• Foveal detachments (Stage I). Without treatment, about half of Stage I macular holes will progress.  
• Partial-thickness holes (Stage II). Without treatment, about 70 percent of Stage II macular holes will progress.  
• Full-thickness holes (Stage III). 

The size of the hole and its location  on the retina determine how much it will affect a person's vision. When a  Stage III macular hole develops, most central and detailed vision can  be lost. If left untreated, a macular hole can lead to a detached  retina, a sight-threatening condition that should receive immediate  medical attention.

How is a macular hole treated?  

Although some macular holes can seal  themselves and require no treatment, surgery is necessary in many cases  to help improve vision. In this surgical procedure--called a  vitrectomy--the vitreous gel is removed to prevent it from pulling on  the retina and replaced with a bubble containing a mixture of air and  gas. The bubble acts as an internal, temporary bandage that holds the  edge of the macular hole in place as it heals. Surgery is performed  under local anesthesia and often on an out-patient basis.

Following surgery, patients must remain  in a face-down position, normally for a day or two but sometimes for as  long as two-to-three weeks. This position allows the bubble to press  against the macula and be gradually reabsorbed by the eye, sealing the  hole. As the bubble is reabsorbed, the vitreous cavity refills with  natural eye fluids.

Maintaining a face-down position is  crucial to the success of the surgery. Because this position can be  difficult for many people, it is important to discuss this with your  doctor before surgery.

What are the risks of surgery?  

The most common risk following macular  hole surgery is an increase in the rate of cataract development. In most  patients, a cataract can progress rapidly, and often becomes severe  enough to require removal. Other less common complications include  infection and retinal detachment either during surgery or afterward,  both of which can be immediately treated.

For a few months after surgery,  patients are not permitted to travel by air. Changes in air pressure may  cause the bubble in the eye to expand, increasing pressure inside the  eye.

How successful is this surgery?  

Vision improvement varies from patient  to patient. People that have had a macular hole for less than six months  have a better chance of recovering vision than those who have had one  for a longer period. Discuss vision recovery with your doctor before  your surgery. Vision recovery can continue for as long as three months  after surgery.

What if I cannot remain in a face-down position after the surgery?  

If you cannot remain in a face-down  position for the required period after surgery, vision recovery may not  be successful. People who are unable to remain in a face-down position  for this length of time may not be good candidates for a vitrectomy.  However, there are a number of devices that can make the "face-down"  recovery period easier on you. There are also some approaches that can  decrease the amount of "face-down" time. Discuss these with your doctor.

Is my other eye at risk?  

If a macular hole exists in one eye,  there is a 10-15 percent chance that a macular hole will develop in your  other eye over your lifetime. Your doctor can discuss this with you.

Research  

Research studies are being conducted to  determine other treatments for macular holes. Currently the research is  looking at using silicon oil to close the macular hole instead of the  gas bubble that is being used now. No definite conclusions have been  reached at this time.

Other name

Epiretinal membrane, preretinal  membrane, cellophane maculopathy, retina wrinkle, surface wrinkling  retinopathy, premacular fibrosis, and internal limiting membrane  disease.

What is a macular pucker?  

A macular pucker is scar tissue  that has formed on the eye's macula, located in the center of the eye's  light-sensitive tissue called the retina. The macula provides the sharp,  central vision we need for reading, driving, and seeing fine detail. A  macular pucker can cause blurred and distorted central vision.

Most of the eye's interior is filled  with vitreous, a gel-like substance that fills about 80 percent of the  eye and helps it maintain a round shape. The vitreous contains millions  of fine fibers that are attached to the surface of the retina. As we  age, the vitreous slowly shrinks and pulls away from the retinal  surface. This is called a vitreous detachment, and is normal. In most  cases, there are no adverse effects, except for a small increase in  floaters, which are little "cobwebs" or specks that seem to float about  in your field of vision.

However, sometimes when the vitreous  pulls away from the retina, there is microscopic damage to the retina's  surface (Note: This is not a macular hole). When this happens, the  retina begins a healing process to the damaged area and forms scar  tissue, or an epiretinal membrane, on the surface of the retina. This  scar tissue is firmly attached to the retina surface. When the scar  tissue contracts, it causes the retina to wrinkle, or pucker, usually  without any effect on central vision. However, if the scar tissue has  formed over the macula, our sharp, central vision becomes blurred and  distorted.

What causes a macular pucker?  

Most macular puckers are related to  vitreous detachment, which usually occurs in people over age 50. As you  age, you are at increased risk for macular pucker.

A macular pucker can also be triggered  by certain eye diseases and disorders, such as a detached retina and  inflammation of the eye (uveitis). Also, people with diabetes sometimes  develop an eye disease called diabetic retinopathy, which can cause a  macular pucker. A macular pucker can also be caused by trauma from  either surgery or an eye injury.

What are the symptoms of a macular pucker?  

Vision loss from a macular pucker can  vary from no loss to severe loss, although severe vision loss is  uncommon. People with a macular pucker may notice that their vision is  blurry or mildly distorted, and straight lines can appear wavy. They may  have difficulty in seeing fine detail and reading small print. There  may be a gray area in the center of your vision, or perhaps even a blind  spot.

Is a macular pucker the same as age-relaed macular degeneration?  

No. A macular pucker and age-related  macular degeneration are two separate and distinct conditions, although  the symptoms for each are similar. An eye care professional will know  the difference.

Can macular pucker get worse?  

For most people, vision remains stable  and does not get progressively worse. Usually macular pucker affects one  eye, although it may affect the other eye later.

Is a macular pucker similar to a macular hole?  

A macular pucker and a macular hole are  different conditions, although they both result from the same reason:  The pulling on the retina from a shrinking vitreous. When the "pulling"  causes microscopic damage, the retina can heal itself; scar tissue, or a  macular pucker, can be the result. If the shrinking vitreous pulls too  hard, it can tear the retina, creating a macular hole, which is more  serious. Both conditions have similar symptoms - distorted and blurred  vision. Also, a macular pucker will not "develop" into a macular hole.  An eye care professional will know the difference.

How is a macular pucker treated?  

A macular pucker usually requires no  treatment. In many cases, the symptoms of vision distortion and  blurriness are mild, and no treatment is necessary. People usually  adjust to the mild visual distortion, since it does not affect  activities of daily life, such as reading and driving. Neither eye  drops, medications, nor nutritional supplements will improve vision  distorted from macular pucker. Sometimes the scar tissue--which causes a  macular pucker--separates from the retina, and the macular pucker  clears up.

Rarely, vision deteriorates to the  point where it affects daily routine activities. However, when this  happens, surgery may be recommended. This procedure is called a  vitrectomy, in which the vitreous gel is removed to prevent it from  pulling on the retina and replaced with a salt solution (Because the  vitreous is mostly water, you will notice no change between the salt  solution and the normal vitreous). Also, the scar tissue which causes  the wrinkling is removed. A vitrectomy is usually performed under local  anesthesia.

After the operation, you will need to  wear an eye patch for a few days or weeks to protect the eye. You will  also need to use medicated eye drops to protect against infection.

How successful is this surgery?  

Surgery to repair a macular pucker is  very delicate, and while vision improves in most cases, it does not  usually return to normal. On average, about half of the vision lost from  a macular pucker is restored; some people have significantly more  vision restored, some less. In most cases, vision distortion is  significantly reduced. Recovery of vision can take up to three months.  Patients should talk with their eye care professional about whether  treatment is appropriate.

What are the risks of surgery?  

The most common complication of a  vitrectomy is an increase in the rate of cataract development. Cataract  surgery may be needed within a few years after the vitrectomy. Other,  less common complications are retinal detachment either during or after  surgery, and infection after surgery. Also, the macular pucker may grow  back, but this is rare.

Research  

Research studies are being conducted to  determine other treatments for macular pucker. Please note that both of  the procedures described below need additional clinical testing. We  suggest you share this information with your eye care professional.

Some physicians are researching the use  of a surgical procedure in which scar tissue is peeled off without  performing the vitrectomy.

Other doctors are researching a new  surgical technique to remove the internal limiting membrane (a layer of  the retina) for patients with both macular pucker and macular hole. This  surgical technique is called Fluidic Internal Limiting Membrane  Separation (FILMS). After a vitrectomy, fluid is injected between the  membrane and the retina that causes the membrane, along with the scar  tissue, to lift away. It is then removed with forceps.

The following organizations may be able to provide additional information on macular pucker and  holes. 

 American Academy of Ophthalmology
P.O. Box 7424
San Francisco, CA 94120-7424
(415) 561-8500
http://www.aao.org
Distributes a fact sheet on macular hole for patients. 

Association for Macular Diseases
210 E. 64th Street
New York, NY 10021
(212) 605-3719
http://www.macula.org
Offers  education and information on macular disease through seminars,  newsletters, and a hotline. Offers counseling to patients and their  families.

(The) Foundation Fighting Blindness
Executive Plaza 1, Suite 800
11435 Cronhill Drive
Owings Mills, MD 21117-2220
1-888-394-3937
1-800-683-5555 (TDD)
(410) 568-0150
(410) 363-7139 (TDD)
http://www.blindness.org.  Acts  as a clearinghouse and distributor of self-help program information.  Sponsors research on the cause, prevention, and treatment of retinitis  pigmentosa, Usher's syndrome, macular degeneration, and other retinal  degenerative conditions. Conducts education programs for those affected  by the disorders as well as professionals and the general public.  Coordinates a national information and referral service and the Retinal  Donor program. Publishes newsletters and other publications. 

For additional information, you may wish to contact a local library.

Below is a sample of the citations  available in MEDLINE, a comprehensive medical literature database  coordinated by the National Library of Medicine (NLM). MEDLINE contains  information on medical journal articles published from 1966 to the  present. You can conduct your own free literature search by accessing  MEDLINE through the Internet at http://medlineplus.nlm.nih.gov. You can also get assistance with a literature search at a local library.

To obtain copies of any of the articles  listed below, contact a local community, university, or medical  library. If the library you visit does not have a copy of a particular  article, you may usually obtain it through an inter-library loan.

Please keep in mind that articles in  the medical literature are usually written in technical language. We  encourage you to share any articles you order with a health care  professional who can help you understand them.

Surgical management of macular  holes: a report by the American Academy of Ophthalmology. Benson WE,  Cruickshanks KC, Fong DS, Williams GA, Bloome MA, Frambach DA, Kreiger  AE, Murphy RP. Ophthalmology 2001; 108(7):1328-1335
This document  describes macular hole surgery and examines the available evidence to  address questions about the effectiveness of the procedure for different  stages of macular hole, complications during and after surgery, and  modifications to the technique. The evidence does not support surgery  for patients with stage 1 holes. Properly conducted, well-designed  randomized trials support surgery for stage 2 holes to prevent  progression to later stages of the disease and further visual loss.  Additional evidence shows that surgery improves the vision in a majority  of patients with stage 3 and stage 4 holes. There is no strong evidence  that adding another form of therapy at the time of surgery results in  improved surgical outcomes. Patient inconvenience, patient preference,  and quality of life issues have not been studied.

Macular hole surgery in 2000.  Margherio AR. Michigan State University College of Human Medicine, Grand  Rapids, MI. Current Opinion in Ophthalmology 2000; 11(3):186-90.
This  article begins by describing possible causes of macular holes. It then  discusses the different stages of macular holes, the surgeries being  used to close the holes, and different procedures that combined with  surgery can get the best possible results.

Complications of macular hole  surgery. Javid CG; Lou PL. Massachusetts Eye and Ear Infirmary, Boston,  MA. International Ophthalmology Clinics 2000; 40(1):225-32.
This  article begins with a brief overview of macular holes. The article  discusses why macular holes form, conditions that can be mistaken for  macular holes, and the different stages of macular holes. The article  continues on to describe how a vitrectomy is done and talks about the  different complications that can follow vitrectomy. Six possible  complications are discussed in detail. Possible reasons for  complications are mentioned, as well as how common each complication is.

Macular hole. University of  Pennsylvania Scheie Eye Institute, Retina Service, Philadelphia, PA.  Survey of Ophthalmology 1998; 42(5):393-416.
This article reports  on different theories that have been proposed over the years to explain  the development of macular holes and then describes the current theory.  The different stages of macular holes are explained, along with  different diagnostic tests for detecting them. The article then lists  various diseases that can be confused with a macular hole and details  how macular holes progress. Risk factors for developing a hole in the  unaffected eye are also noted. The article ends with a discussion of the  success of different treatments used to manage macular holes and  describes the complications that can result from these treatments.

Postoperative complications of  epiretinal membrane surgery. Graham K, D'Amico DJ. Retina Service,  Massachusetts Eye and Ear Infirmary, Boston 02114, USA. International Ophthalmology Clinics 40(1):215-223, Winter 2000.
Since  the initial description by Machemer, surgical removal of an epiretinal  membrane (ERM) in nondiabetic eyes has become a routine procedure after  which the majority of patients experience visual improvement. Of  patients undergoing ERM surgery, 74 percent to 87 percent can expect  visual improvement of at least two lines on the Snellen chart. In  general, surgical removal of ERMs that are idiopathic (no known cause)  is more successful than removal of membranes that are secondary to  retinal tears or detachments. Poor visual outcome from ERM surgery is  most commonly due to cataract development, retinal breaks, and retinal  detachments. Other postoperative complications include cystoid macular  edema (CME), retinal phototoxicity, endophthalmitis, subretinal  neovascularization, and recurrent ERMs. These complications should be  considered when evaluating a patient with poor vision after ERM surgery.

Macular epiretinal membranes.  Pournaras CJ, Donati G, Brazitikos PD, Kapetanios AD, Dereklis DL,  Stangos NT. Department of Clinical Neurosciences, University Hospitals  of Geneva, Geneva, Switzerland. Seminars in Ophthalmology 15(2):100-107,  June 2000.
Epiretinal membranes (ERM) are a common finding in  older patients. Although they may be associated with numerous clinical  conditions, most epiretinal membranes occur in the absence of ocular  pathology (disease). Patients' symptoms range from asymptotic to  complaints of severe vision loss and metamorphopsia (distortion). Pars  plana vitrectomy has been found to be effective in removing ERM from the  macula, improving the visual acuity and decreasing metamorphopsia. Both  idiopathic and secondary ERMs do well after surgery, although secondary  ERMs showed a greater amount of improvement than idiopathic ones.  Complications are frequent including accelerated postoperative nuclear  sclerosis (cataracts), retinal breaks and retinal detachment (RD),  macular edema, retinal pigment epithelium (RPE) changes, and,  occasionally, macular hole and hypotony (low eye pressure). However,  only RD involving the macula has a worsening prognosis on final outcome.

Epiretinal membranes. Jacobsen CH. School of Optometry, University of California-Berkeley. Optometry Clinics 5(1):77-94, 1996.
Epiretinal  membranes are a common finding in older patients but are rare in young  patients. Although they may be associated with other ocular conditions,  most epiretinal membranes occur in the absence of ocular disease.  Patient symptoms range from asymptomatic to complaints of severe vision  loss and distortion. Epiretinal membranes are commonly classified  according to their density and contractile characteristics. In this  review, cellophane maculopathy refers to thin, glistening membrane,  surface wrinkling maculopathy is characterized by fine, superficial  retinal folds and macular pucker is associated with a dense,  grayish-white membrane causing a characteristic pattern of severe  retinal distortion. With sufficient visual disturbance, epiretinal  membranes may be treated by pars plana posterior vitrectomy and  epiretinal membrane peeling.

The information in this section was compiled from the NEI website www.nei.nih.gov.