Overview

Idiopathic macular pucker is an eye condition, that is also known as epiretinal membrane. The epiretinal membrane is scar tissue formed at the vitreoretinal interface. It appears as a greyish semi-translucent membrane over the internal membrane on the surface of the retina. The formation of this tissue can cause disturbances and distortion of vision.¹ 

These lesions may arise for unknown reasons, or be secondary to eye diseases, injuries, and retinal surgeries. The cause of idiopathic macular pucker is largely unknown, but it is often associated with ageing and vitreoretinal interface changes.² 

Surgery is the main treatment for severe cases. This review aims to provide an overview of idiopathic macular pucker, including its development, symptoms, diagnosis and treatment.

What is an idiopathic macular pucker?

Macular pucker is an eye condition affecting the macula of the retina that is particularly common among older people, where a membrane grows over the macula of the retina. It is also commonly known as epiretinal membrane. It usually affects only one eye. The condition can significantly impact an individual’s quality of life due to its negative effect on central vision. While the exact cause is unknown, it is thought that it may occur due to age-related changes in the vitreous and the retinal surface.² 

Idiopathic is a term that refers to any condition that occurs without a known cause. Idiopathic macular pucker is therefore a condition of unknown cause. It affects approximately 95% of patients diagnosed with macular pucker, and it is directly related to cellular proliferation resulting from posterior vitreous detachment.² The terms epimacular membrane, cellophane maculopathy and preretinal macular fibrosis have also been used to describe this condition.³ 

Risk factors for macular pucker

The main risk factors for macular pucker are age and extent of posterior vitreous detachment. This condition in the vitreous humour is observed in two-thirds of patients with evidence of macular pucker. Macular pucker usually appears after the age of 50 years, and the risk of its appearance is exacerbated with increasing age. Other factors contributing to the development of macular pucker are obesity, type 2 diabetes, hypertension, hypercholesterolaemia, and hypertriglyceridemia.^2,4,5 These associations indicate that a healthy diet and lifestyle may help prevent or manage idiopathic macular pucker. Although, there have been no studies looking at this specifically, yet. 

Incidence of macular pucker

Macular pucker becomes more common from 50 years of age onwards and its prevalence increases with age onwards. Approximately 20% of patients over the age of 75 years have a macular pucker and some reports show a slightly higher incidence in females.¹ It has been estimated that about 30 million people in the United States have macular pucker in at least one eye.⁶ The Royal College of Ophthalmologists estimate that approximately 34% of people over 60 years of age may have macular pucker in one eye.

Ethnicity may also play a role in the incidence of macular pucker. One American study found that people of Chinese origin had the highest incidence, followed by Latin American, Caucasian and African descent.⁵ 

How does the macular pucker develop?

The vitreous is a clear gel in the eye that gives it a round shape. With age, the vitreous shrinks and pulls away from the retina. This is known as a vitreous detachment.^1,2

The formation of idiopathic macular pucker might be caused by increased cell growth on the inner retinal surface. The epiretinal membrane shrinks, causing retinal wrinkling and distortion. In some cases, a membrane forms on the surface of the retina. This membrane can create what looks like wrinkles or puckers. If the membrane forms over a part of the retina called the macula, it can cause a macular pucker.^1,2

Cellular involvement in idiopathic macular pucker

Various types of cells within the eye are involved in the development of macular pucker:¹

• Glial cells are involved in the response to retinal injury or stress contributing to membrane formation
• Fibroblasts move to the retinal surface causing shrinkage of the epiretinal membrane
• Myofibroblasts move to the retinal surface, contributing to the shrinking of the epiretinal membrane
• Growth factors play vital roles in cell growth and membrane formation

Symptoms of macular pucker

Patients with idiopathic macular pucker typically arriev withtheir optician or doctor with symptoms that include:¹

• Metamorphopsia: distortion of vision where straight lines appear wavy or bent 
• Reduced visual acuity: gradual decline in central vision affecting reading and recognising faces
• Micropsia: perception of objects appearing smaller 
• Blurred vision

The severity of symptoms can vary, with some patients having no symptoms, and others experiencing significant visual impairment. In some cases, the condition might be picked up on just a routine eye examination by the optician.¹ 

Diagnosis of macular pucker

There are different ways that your optician or ophthalmologist can diagnose the idiopathic macular pucker. We will analyse some of them below.

Ophthalmoscopy

During a fundoscopic examination, idiopathic macular pucker appears as a glistening, semi-translucent membrane on the retinal surface. Retinal folds or distortion of the macular architecture may also be visible.^1,2

Optical coherence tomography (OCT)

Optical coherence tomography (OCT) provides high-resolution cross-sectional images of the retina that can show:^1,2

• Epiretinal membrane: This is a hyperreflective band on the retinal surface
• Retinal thickening: Increased retinal thickness due to membrane contraction
• Macular oedema: Fluid accumulation in severe cases
• Macular distortion: Changes in the foveal contour and retinal layers

Fluorescein angiography

Fluorescein angiography can also help assess retinal vascular changes, and exclude other conditions in the macula, but it is not routinely used. This involves the injection of a dye into your arm, which will travel to the veins in your eye causing them to shine, and allowing a special camera to take pictures of the eye.^1,2

Treatment for macular pucker

The management of idiopathic macular pucker ranges from observation, in mild cases, to surgery in more severe cases.^1,2

Observation

Regular monitoring is often recommended for patients with minimal symptoms and stable visual acuity. Follow-up examinations include visual acuity testing, and optical coherence tomography, to detect any progression.^1,2 

Medication

There are no effective medicinal treatments for idiopathic macular pucker as yet. Research is ongoing to explore the potential of anti-inflammatory drugs, and growth factor inhibitors. Studies have shown that treating intraocular inflammation after surgery allows faster visual recovery, less macular oedema, and a decrease in complications with the use of corticosteroids.⁷ 

Surgery

Surgical removal of macular pucker is currently the only treatment, improvements in vitrectomy procedures have enabled less invasive treatment. Pars plana vitrectomy (PPV) with membrane peeling is the main surgical treatment for idiopathic macular pucker. However, there are currently no standardised criteria for the surgery, and not all patients show satisfactory postoperative recovery of visual function. Thus, further research is needed to determine the criteria for surgery and methods to improve postoperative outcomes.^2,3

Complications of surgery

The potential complications of surgery include:^1,2

• Cataract formation
• Retinal detachment
• Recurrent macular pucker
• Macular hole

Prognosis after surgery

The prognosis for patients with idiopathic macular pucker varies. Progressive visual improvement during the first 3 years after PPV-MP, has been found alongside stable vision thereafter, with very long-term monitoring.⁸ Regular follow-up and timely management are crucial for maintaining optimal vision. This is why it is important to visit your optometrist regularly, for routine eye tests, at intervals of usually every one to two years, as advised. In the UK, adults over 60 years of age can avail of free sight tests.

Patient education and support

Any condition that limits vision can consequently negatively affect quality of life. An individual with macular pucker can enlist support from family, friends, support groups, and specialist groups, such as RNIB, the Macular Society or Guide Dogs for the Blind Association to name but a few. 

Summary

Idiopathic macular pucker is also commonly known as epiretinal membrane, and is a cause of visual impairment, particularly in the elderly. The term idiopathic in the name tells us that the condition has an unknown origin. Advances in diagnostic imaging have improved the management of this condition. Surgical intervention is still the main treatment for symptomatic cases, but ongoing research may lead to future non-surgical options. Early diagnosis, appropriate management, and support are key to preserving visual function and enhancing the quality of life for affected individuals.