Pigmentary Glaucoma

Pigmentary glaucoma is caused by the blockage of the trabeculum (the drainage system of the eye) by pigment-bearing cells released from the iris (the colored tissue of the eye). Initially, the release of these cells causes the so-called pigment dispersion syndrome. In other words, a release of the pigment-bearing cells is observed, which results in thinning of the iris and their deposition on the inner side of the cornea (endothelium) and in the drainage angle of the eye. With the obstruction of the angle, the drainage of the aqueous humor is reduced, the intraocular pressure increases, which can lead to glaucomatous damage to the optic nerve. After a period of 15 years, approximately 15% of patients with pigment dispersion syndrome may develop increased intraocular pressure or pigmentary glaucoma. It is inherited in an autosomal dominant manner but with variable penetrance. So if you suffer from pigment dispersion syndrome you are likely to pass it on to your children.

What is the etiology of pigment dispersion?

Pigment dispersion usually results from the configuration of the iris, which is concave so that its back side is in contact with the crystalline lens. Due to friction at the point of contact with the lens, pigment cells are released. These cells may be gathered on the inner side of the cornea (in the endothelium), in the anterior capsule of the lens or in the trabecular meshwork (in the drainage system of the eye).

What are the symptoms of pigment dispersion?

Most of the time patients have no symptoms. Blurred vision or bright halos around lights may rarely be seen.

Who is at risk of pigment dispersion syndrome?

It usually manifests itself in young people, aged 20 to 30 years. It most often affects Caucasian men who suffer from myopia. As mentioned, it is inherited in an autosomal dominant manner. So you are more likely to suffer from pigment dispersion syndrome if one of your parents has it.

How is the diagnosis made?

The diagnosis of pigment dispersion syndrome as well as pigment dispersion glaucoma is made by the clinical examination by the ophthalmologist. The main findings during the clinical examination are the following:

– Krukenberg’s spindle, i.e. the deposition of the pigment released by the iris in the endothelium (the inner layer of cells) of the cornea

– The concavity of the iris towards the crystalline lens

– The transillumination of the iris (small areas in the middle circumference of the iris in which the pigment is completely missing)

– The pigment deposition on the anterior capsule of the crystalline lens

If the ophthalmologist notices any of these signs, the diagnosis of pigment dispersion syndrome is made. The clinical examination is complemented by the measurement of intraocular pressure, gonioscopy (examination of the drainage angle of the eye in which intense pigment deposition is observed), pachymetry and fundoscopy. In addition, it is recommended to carry out other examinations such as visual field test and optical coherence tomography of the optic nerve that help to diagnose glaucomatous damage as well as to monitor its progression.

What is the treatment?

In some cases where the concavity of the iris towards the crystalline lens is detected before the dispersion of the pigment, it is recommended to perform YAG laser iridotomy (link) in order to change the configuration of the iris and prevent its contact with the lens.

In cases of pigment dispersion syndrome without the existence of glaucomatous damage, it is recommended to monitor the patients frequently 2 to 3 times a year to measure the intraocular pressure, as well as to carry out annual tests such as visual field test and optical coherence tomography (OCT).

In pigmentary glaucoma, the treatment consists of lowering the intraocular pressure either with anti-glaucoma drops or with YAG laser trabeculoplasty (link). In cases where drug therapy or laser are not sufficient, we proceed with surgical operations such as trabeculectomy (link) or the insertion of aqueous drainage device (link)

Pigment dispersion syndrome and physical exercise

Studies show that vigorous physical exercise can cause an acute release of pigment in people with pigment dispersion syndrome or pigment dispersion glaucoma. This can cause a significant increase in intraocular pressure manifested by symptoms such as sudden blurring of vision or a halo around lights and, more rarely, pain. It is therefore recommended that patients suffering from pigment dispersion syndrome or pigment dispersion glaucoma avoid vigorous physical exercise.

Remember, early diagnosis and early treatment are essential to managing pigmentary glaucoma. We are always at your disposal for any further question as well as for the thorough evaluation of your case.