Is there a link between infantile cataract and glaucoma?
Yes, there are several links between these two conditions. All children who undergo cataract surgery, especially in infancy, are at life long risk for developing glaucoma after surgery. Although this risk is not very high, the serious visual effects of glaucoma can be detected even at the earliest stage. This is why regular follow-up with the pediatric ophthalmologist is important, even after the surgery. Some eyes with congenital cataract are also at risk for glaucoma even if no surgery is done yet. This only means that there are other problems in the eye other than just the cataract. Some eye conditions that are associated with glaucoma would include eye and body syndromes such as aniridia (absent iris), anterior segment dysgenesis, congenital rubella syndrome, and persistent hyperplastic primary vitreous (PHPV). These eye conditions sometimes present initially with cataract and glaucoma.
Video | Congenital Glaucoma
Does congenital cataract present with glaucoma?
The following conditions present with infantile cataract and glaucoma:
Aniridia literally means absence of an iris. However, this is somewhat a misnomer because there is always some amount of iris tissue present. This condition commonly presents with other ocular problems that involve the other parts of the eye such as cataract, lens subluxation, glaucoma, optic nerve hypoplasia, and foveal hypoplasia. The latter two results in poor vision
- Anterior Segment Dysgenesis
Anterior segment dysgenesis is a spectrum of eye disorders that involves the anterior part of the eye. These diseases present abnormal development of the cornea, iris and lens during embryogenesis. The structural abnormalities that result from the dysgenesis produce corneal opacity, glaucoma, and cataract.
- Persistent Hyperplastic Primary Vitreous (PHPV)
PHPV represents an abnormal regression of the primitive hyaloid vascular system in the eye. This produces a fibrovascular stalk that connects the optic disc and the posterior part of the lens (posterior cataract). A retrolental membrane forms at the posterior part of the lens and it may extend to the ciliary processes. Over time, the membrane can contract, pulling the ciliary processes centrally. If left untreated, severe forms of PHPV can lead to shallowing of the anterior chamber and angle closure glaucoma
- Congenital Rubella Syndrome
Systemic findings of congenital rubella syndrome include congenital heart defects, hearing loss, and mental retardation. Ocular findings include pigmentary retinopathy (25%), cataract (15%), strabismus (20%), microphthalmos (15%), optic atrophy (10%), corneal haze (10%), glaucoma (10%), and phthisis bulbi (2%). The retinopathy is stable and usually does not affect vision. Rubella cataracts are caused by invasion of the lens by the rubella virus, and are bilateral in 80% of all cases. These cataracts may present with a hazy cornea caused by either congenital glaucoma or keratitis. Treatment of the cataract involves removing the entire lens cortex, since the patient’s tendency to postoperative inflammation is increased if residual cortex is left after the surgery
Lowe syndrome (oculocerebrorenal syndrome)
This is an x-linked disorder that presents with bilateral congenital cataracts and often with bilateral congenital glaucoma. Infants show severe developmental delay, hypotonia, and renal failure with aminoaciduria. The visual prognosis is poor since there is progression of neurological and renal deterioration. Death occurs in late childhood. A dilated slit lamp examination of the patient’s mother shows multiple punctate white snowflake opacities of the lens periphery