Pediatric emergency medicine trisk 629

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 3 weeks of age so
dacryostenosis will often appear at that age.
Neonates do not focus at birth. Infants start to fixate at 5 to 6 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 1 to 2 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 2 to 3 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 1 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