Primary Congenital Glaucoma
Autorzy: Joanna Zawistowska, Alina Bakunowicz-Łazarczyk
Klinika Okulistyki Dziecięcej z Ośrodkiem Leczenia Zeza, Uniwersytecki Dziecięcy Szpital Kliniczny im. L. Zamenhofa w Białymstoku
Kierownik: prof. dr hab. n. med. Alina Bakunowicz-Łazarczyk
Primary congenital glaucoma is a rare disease that is genetically-dependant. It is a significant problem of paediatric ophthalmology. It is estimated to cause blindness in 2–15% of cases. Abnormal development of the structures of the anterior segment of the eye and the irido-corneal angle result in impaired outflow of aqueous humor and, consequently, increased intraocular pressure. In an immature, susceptible eyeball with a flexible sclera and cornea, characteristic anatomical and functional disorders occur in fetuses, newborns or infants.
They threaten the proper vision development, and an untreated disease in most cases leads to irreversible blindness. Taking into account the long life expectancy of the paediatric population, it is essential to promptly diagnose and undertake appropriate treatment if disturbing symptoms are observed. The triad of symptoms may occur: lacrimation, photophobia and blepharospasm, as well as increased corneal diameter and buphthalmos. The treatment of choice is angle surgery: goniotomy, trabeculotomy or trabeculectomy. As the second-line treatment drainage devices surgery and cyclodestructive procedures are performed.
Medical therapy has a preparatory function prior to planned operating procedure or helps maintaining satisfactory levels of intraocular pressure after. All cases of primary congenital glaucoma, even despite effective surgical intervention, require long-term outpatient care with regular tonometry, measurement of refractive status, assessment of the morphology of the optic nerves and corneal diameter, and, after the child reaches the appropriate age, visual field tests. The paper presents overview of the classification of congenital glaucoma, diagnostic approach and symptomatology, as well as its treatment options.