CLINICAL PEARLS AND PITFALLS Retinal hemorrhages are common after labor White or absent red reflex is highly suspicious for retinoblastoma Patients should be referred to an ophthalmologist urgently The lacrimal duct system is not fully functional until weeks of age so dacryostenosis will often appear at that age Neonates not focus at birth Infants start to fixate at to weeks of age Scleral and Subconjunctival Hemorrhage Scleral and subconjunctival hemorrhage are often noted in the newborn as a result of normal delivery or birth trauma Spontaneous resolution within to weeks is the rule When the funduscopic examination is performed, similar hemorrhages may be noted on the retina in about 25% of newborns The presence of retinal hemorrhages should also raise the possibility of intentional trauma Specifically, the shaken baby syndrome has been associated with flame-shaped retinal hemorrhages and subdural hematomas (see Chapters 87 Child Abuse/Assault and 122 Neurosurgical Emergencies ) Leukocoria Leukocoria is defined as the presence of a white pupil ( Fig 96.28 ) A pupillary light reflex is a simple test that should be performed on all newborns In the normal newborn, a “red reflex” is seen when the ophthalmoscope is held to ft in front of the eyes A white pupillary light reflex, or leukocoria, occurs when light reflects off a white surface in the eye It is never normal An absent or black red reflex is also abnormal Both indicate presence of an opacity obstructing the light reflection through the layers of the ocular media Irregular opacities may also produce an irregular reflex Pupillary examination may be difficult if the pupils are small or the neonate will not maintain eyes open In neonates darkening the room and holding up the baby at a 45-degree angle with gentle patting on diaper will help open the baby’s eyes Occasionally placement of one drop of 0.2% cyclopentolate and 1% phenylephrine (Cyclomydril) dilating eye drops may be of help Care should be taken with infants who have a history of prematurity or chronic lung disease when using these drops since some infants may develop cardiovascular instability Leukocoria may be a sign of several conditions of variable severity and prognosis Causes of leukocoria are listed ( Table 96.3 ) Definite evaluation by slit lamp examination is necessary to determine etiology CT may be required for some infants to delineate orbital structures, shape of the globe, and other cranial anomalies Therefore, all infants with an abnormal pupillary light reflex should be referred to an ophthalmologist for a prompt evaluation Treatment depends on the underlying etiology FIGURE 96.28 Unilateral leukocoria in a newborn with congenital rubella This child has congenital rubella syndrome Although full term, the baby is small for his gestational age There is leukocoria due to a cataract in the left eye (Reprinted with permission from Garg S Color Atlas and Synopsis of Clinical Ophthalmology—Wills Eye Institute—Uveitis Philadelphia, PA: Lippincott Williams & Wilkins; 2011.) Retinoblastoma Retinoblastoma is the most common malignant intraocular tumor in neonates, occurring in in 20,000 infants It can be unilateral or bilateral and can be inherited or sporadic in nature Retinoblastoma usually presents by 13 to 18 months if not captured earlier on routine examination (50% of cases are diagnosed by finding an abnormality of red reflex during routine examination or by a parent) Mutation or inactivation of the tumor suppressor gene is thought to contribute to its development Children of affected parents with the inherited or bilateral form have a 45% chance of developing this tumor As the tumor progresses anteriorly, strabismus due to deterioration of vision occurs Fundal examination reveals posterior chamber mass with calcification The globe starts to enlarge because of the large mass or secondary glaucoma Secondary glaucoma also causes photophobia Other manifestations include pseudohypopyon and spontaneous intraocular bleeding CT scan or MRI is necessary for determining the degree of extraocular spread and confirming the diagnosis Early detection and treatment is of utmost importance because 5-year survival is approximately 98% if the tumor is confined to the globe TABLE 96.3 CAUSES OF LEUKOCORIA IN A NEWBORN