|“My child has bumps on the colored part of the eye.”|
|Small iris cysts at the pupillary border|
|Large iris cysts|
|Congenital iris ectropion|
|Intraocular tumor (diktyoma)|
|Small lesions on the iris are usually asymptomatic and do
not affect vision. A history of eye pain, corneal clouding, or decreased vision suggests a possible tumor. Juvenile xanthogranuloma (JXG)
may be associated with small orange-brown papules on the head or
face. Iris JXG lesions may bleed, and the resultant hyphema may cause
|Without a slit lamp, evaluation of iris lesions may be difficult,
particularly in a noncooperative toddler. Some lesions may be visible
with a penlight. Benign iris cysts are often seen best by examining
the pupil margin when evaluating the red reflex with a direct ophthalmoscope.|
Small irregular lesions at the pupil margin in an infant do not
require further evaluation. Small iris nevi are common and also
do not require evaluation unless abnormal growth occurs. Children with
other iris abnormalities should be referred to a pediatric ophthalmologist.
Lisch nodules are almost pathognomonic of neurofibromatosis,
and evaluation for other abnormalities associated with neurofibromatosis
should be performed.
Although extremely rare, large iris cysts or iris distortion
due to intraocular tumors (diktyoma) may cause glaucoma, eye pain,
redness, and corneal clouding. This requires immediate evaluation.
- 1. Iris cysts. Cysts of the
iris are not common, but may occur in otherwise normal children.
Small, scalloped irregularities at the pupil margin are almost always
benign (Figure 17–1). Large iris cysts are very rare. They
may cause vision loss (Figure 17–2).
- 2. Lisch nodules. Lisch nodules
almost always occur in children with neurofibromatosis. They are
usually not present in infancy. The incidence and number of lesions
increase with age. By age 20, more than 95% of patients
with neurofibromatosis have Lisch nodules. Lisch nodules are small,
tan, and slightly elevated from the iris surface (Figure 17–3A
and B). The Lisch nodules do not cause any vision problems. They
play an important role in establishing a diagnosis.
- 3. Iris nevi. Iris nevi present
as areas of irregular pigment on the surface of the iris. They are
most easily noticed when the nevi are brown and the underlying iris
pigment is fair (Figure 17–4). These are flat (rather than
elevated like Lisch nodules), but this feature cannot be accurately
assessed without a slit lamp. Iris nevi in children are almost always
Small iris pigment epithelial cysts (arrow) at pupil
margin. These cause no visual problems.