Primary congenital glaucoma is glaucoma that occurs in children. Fortunately, although the onset of glaucoma at a young age is not very common, persistence of high eye pressure for long periods can be disastrous. What are the causes and symptoms of primary congenital glaucoma? What is the treatment?

Primary congenital glaucomais glaucoma that occursin children.Although fortunately the occurrence of glaucoma at a young age is not very common, the persistence of high pressure in the eyes for a long time can be disastrous. This condition, if left untreated, leads to irreversible changes in the entire eyeball and the associated blindness.

Primary congenital glaucoma - causes

The cause of primary congenital glaucoma is abnormal development of the anterior chamber angle, also known as the drainage angle or the corneal iris angle. As a result of congenital anomalies in this structure, the outflow of aqueous humor from the eye is impeded and the intraocular pressure increases significantly.

Most cases of congenital glaucoma are not genetically determined, but can sometimes be related to certain genes, so all children with glaucoma should be referred to a genetic counselor.

It is common for congenital glaucoma to accompany congenital abnormalities. The most common are:

  1. congenital lack of iris (various degrees of underdevelopment of the iris)
  2. Sturge-Weber syndrome (the so-called strawberry mark, or "red wine" stain)
  3. neurofibromatosis
  4. Marfan's syndrome
  5. Pierre Robin's band
  6. homocystinuria
  7. Axenfeld-Reiger syndrome (abnormal development of the drainage angle)
  8. Peters anomaly with corneal endosperm
  9. Lowe's syndrome (oculocerebral renal syndrome)
  10. rubella
  11. wide thumb syndrome
  12. persistent hyperplastic vitreous body

See what you don't know about glaucoma

Worth knowing

Childhood glaucoma is divided into four groups:

1. primary congenital glaucoma 2. adolescent glaucoma 3. glaucoma accompanying congenital developmental abnormalities4. secondary glaucoma

Primary congenital glaucoma - symptoms

WDepending on the severity of the congenital disorder, primary congenital glaucoma can develop in the first two years after birth and is then referred to as true congenital glaucoma, or up to the age of 10 and is then called childhood glaucoma. In young children, this disease manifests itself as:

  • photophobia
  • tearing
  • squeezing the eyelids
  • rubbing my eyes

As a result of the increased pressure, the wall of the eyeball may stretch and the so-called " voloina " may appear, i.e. the eyes become large, like an ox.

GOOD TO KNOW>>Which eye diseases can be genetically determined?

Primary congenital glaucoma - diagnosis

The examination usually shows increased intraocular pressure, corneal edema, damaged optic nerve, and often an abnormal tearing angle.

Primary congenital glaucoma - treatment

Treatment of this type of glaucoma with drops is of limited use. Surgical methods are more effective. Due to the development of effective surgical techniques, the prognosis of sick children has improved significantly. Nevertheless, treatment is always long and difficult and should be carried out by an experienced ophthalmologist specialized in this field.

Surgical treatment is very complicated. Procedures are performed to improve the outflow of aqueous humor from the eyeball - goniotomy, trabeculectomy, procedures involving the creation of a fistula that drains the aqueous humor - trabeculectomy, fistula surgeries with the use of setons and treatments reducing the production of aqueous humor in the globe - cyclodestructive procedures. The goal of all these treatments is to lower the intraocular pressure and stop the damage to the optic nerve. The sooner the pressure is lowered, the better the vision prognosis becomes. Such a patient, however, must remain under ophthalmological care throughout his life.

About the authorBarbara Polaczek-Krupa, MD, PhD, specialist in eye diseases, Centrum Opulystowa Targowa 2, Warsaw

Dr. Barbara Polaczek-Krupa, MD, initiator and founder of the T2 Center. She specializes in modern diagnostics and treatment of glaucoma - this was also the subject of her PhD thesis defended with honors in 2010.

Dr. med. Polaczek-Krupa has been gaining experience for 22 years, since she started working at the Ophthalmology Clinic of CMKP in Warsaw, with which she was associated in 1994-2014. During this period, she obtained two degrees of specialization in ophthalmology and the title of doctor of medical sciences.

In the years 2002-2016 she worked at the Institute of Glaucoma and Eye Diseases inWarsaw, where she gained knowledge and medical experience by consulting patients from all over Poland and from abroad.

For years, as part of cooperation with the Medical Center of Postgraduate Education, he has been a lecturer at courses and trainings for doctors specializing in ophthalmology and primary he alth care.

He is the author or co-author of numerous publications in scientific journals. Member of the Polish Ophthalmology Society (PTO) and the European Glaucoma Society (EGS).

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Eye diseases and vision disorders