1. Shaken Baby Syndrome
Child Abuse
Although most eye injuries in childhood are accidental or innocently caused by other children, a significant portion results
from physical abuse by adults. Child abuse is a pervasive problem in our society, with an estimated 2 million victims per
year in the United States. Abusive behavior in a parent or other caregiver usually reflects temporary loss of control during
a period of anger or stress rather than premeditated cruelty. Lack of knowledge of the proper way to care for or discipline
a child is also a frequent contributing factor. In the relatively rare Munchausen syndrome by proxy, the child is physically
harmed by a psychopathic parent to create signs of illness in an effort to manipulate medical care providers.
A reliable history is often difficult to obtain when child abuse has occurred. Suspicion should be aroused when
repeated accounts of the circumstances of injury or histories obtained from different individuals are inconsistent or when
the events described seem to conflict with the extent of injuries (eg, bruises on multiple aspects of the head after a fall) or
with the child’s developmental level (eg, a 2-month-old rolling off a bed or a 6-month-old climbing out of a high chair).
Any physician who suspects that child abuse might have occurred is required by law in every US state and Canadian
province to report the incident to a designated governmental agency. Once this obligation has been discharged, the
ophthalmologist is probably best advised to leave full investigation of the situation to appropriate specialists or authorities.
The presenting sign of child abuse involves the eye in approximately 5% of cases, and ocular manifestations are
detected in the course of evaluating many others. Blunt trauma inflicted with fingers, fists, or implements such as belts or
straps is the usual mechanism of nonaccidental injury to the ocular adnexa or anterior segment. Periorbital ecchymosis,
subconjunctival hemorrhage, and hyphema should raise suspicion of recent abuse if the explanation provided is less than
completely plausible. Cataract and lens dislocation may be signs of repeated injury or trauma inflicted more remotely in
the past. A majority of rhegmatogenous retinal detachments that occur in childhood have a traumatic origin; abuse should
be suspected when such a finding is encountered in a child without a history of injury or an apparent predisposing factor
such as high myopia.
Shaking Injury
A unique complex of ocular, intracranial, and sometimes other injuries occurs in infants who have been abused by violent
shaking. Because the essential features of what is now generally known as shaken baby syndrome were identified in the
early 1970s, it has become widely recognized as one of the most important manifestations of child abuse.
Victims of shaking injury are always under 3 years old and usually under 12 months. When a reliable history is
available, it typically involves a parent or other caregiver who shook an inconsolably crying baby in anger and frustration.
Often, however, the only information provided is that the child’s mental status deteriorated or that seizures or respiratory
difficulty developed; or the involved caregiver may relate that an episode of relatively minor trauma occurred, such as a fall
from a bed. Even without a supporting history, the diagnosis of shaken baby syndrome can still be made with confidence on
the basis of characteristic clinical findings. It must be kept in mind, however, that answers to important questions concerning
the timing and circumstances of injury and the identity of the perpetrator frequently cannot be inferred from medical
evidence alone.
Intracranial injury in shaken infants almost always includes subdural hematoma, typically bilateral over the cerebral
convexities or in the interhemispheric fissure. Evidence of subarachnoid bleeding is also often apparent. Although initial
scans may be normal in many cases, cerebral parenchymal damage is manifest on neuroimaging, acutely as edema,
ischemia, or contusion and in later stages as atrophy. These findings are thought to result from repetitive abrupt
deceleration of the child’s head as it whiplashes back and forth during the shaking episode. Some authorities, citing the
frequency with which shaken baby syndrome victims also show evidence of having received blows to the head, think that
impact is an essential component. Displacement of the brain in relation to the skull and dura mater ruptures bridging
vessels, and compression against the cranial bones produces further damage. The infant’s head is particularly vulnerable
to such effects because of its relatively large mass in relation to the body and poor stabilization by neck muscles.
Ocular involvement
The most common ocular manifestation of shaking injury, present in a large majority of cases, is retinal hemorrhage.
Preretinal, nerve fiber layer, deep retinal, or subretinal localization may be seen. Hemorrhages tend to be concentrated in
or near the macular region but sometimes are so extensive that they occupy nearly the entire fundus (Fig 30‑ 1). Vitreous

2. hemorrhage may also develop, usually as a secondary phenomenon resulting from migration of blood that was initially
intraretinal. Occasionally, the vitreous becomes almost completely opacified by dispersed hemorrhage within a few days
of injury. Retinal hemorrhages in shaken infants resolve over a period ranging from 1 or 2 weeks to several months.
Vitrectomy should be considered if amblyopia is likely.
Figure 30-1 Extensive retinal hemorrhages, left eye, in a 2-month-old infant thought to have been violently shaken.
Temporal portion of the disc is visible near the left edge of the photograph.
Some eyes of shaken infants show evidence of retinal tissue disruption in addition to hemorrhage. Full-thickness
perimacular folds in the neurosensory retina, typically with circumferential orientation around the macula that creates a
craterlike appearance, are highly characteristic. Splitting of the retina (traumatic retinoschisis), either deep to the nerve
fiber layer or superficial (involving only the internal limiting membrane), may create partially blood-filled cavities of
considerable extent, also usually in the macular region (Fig 30-2). Full-thickness retinal breaks and detachment are rare.
Retinal folds usually flatten out within a few weeks of injury, but schisis cavities can persist indefinitely.
Figure 30-2 Traumatic retinoschisis. A, Deep splitting of the retina, typically associated with severe permanent visual
impairment and loss of ERG b-wave. B, Superficial splitting, with separation of the internal limiting membrane and a full-
thickness perimacular fold. Recovery of good vision is common. (Reproduced by permission from Greenwald MJ. The
shaken baby syndrome. Semin Ophthalmol. 1990;5:202–213. Illustrations by S. Gordon.)
A striking feature of shaken baby syndrome is the typical lack of external evidence of trauma. The ocular adnexa and
anterior segments appear entirely normal. Occasionally, the trunk or extremities show bruises representing the imprint of
the perpetrator’s hands. In a minority of cases, broken ribs or characteristic metaphyseal fractures of the long bones result
from forces generated during shaking. It must be kept in mind, however, that many shaken babies are also victims of other
forms of abuse. In particular, signs of impact to the head must be carefully sought.
When extensive retinal hemorrhage accompanied by perimacular folds and schisis cavities is found in association
with intracranial hemorrhage or other evidence of trauma to the brain in an infant, shaking injury can be diagnosed with
confidence regardless of other circumstances. Extensive retinal hemorrhage without other ocular findings strongly
suggests that intracranial injury has been caused by shaking, but alternative possibilities such as a coagulation disorder
must be considered as well. Severe accidental head trauma (eg, sustained in a fall from a second-story level or a motor
vehicle collision) is infrequently accompanied by retinal hemorrhage, which is virtually never extensive. Retinal
hemorrhage is rare and has never been documented to be extensive following cardiopulmonary resuscitation by trained
personnel. Spontaneous subarachnoid hemorrhage occurs rarely in young children and may be associated with some
degree of intraocular bleeding. Retinal hemorrhages resulting from birth trauma are common in newborns but seldom
persist beyond age 1 month.
Emerson MV, Pieramici DJ, Stoessel KM, et al. Incidence and rate of disappearance of retinal hemorrhage in
newborns. Ophthalmology. 2001;108:36–39.
Prognosis
In one large study, 29% of children with shaken baby syndrome died from their injuries. Poor visual and pupillary
response were correlated with a higher risk of mortality. Survivors often suffered permanent impairment ranging from
severe retardation and quadriparesis to mild learning disability and motor disturbances. Visual loss from traumatic
retinoschisis, optic nerve damage, or cortical injury occurred in 20% of patients, but nearly complete recovery of vision
was common. Dense vitreous hemorrhage, usually associated with deep traumatic retinoschisis, carried a poor prognosis
for both vision and life. Vitrectomy should be deferred if bright-flash electroretinography shows loss of the b-wave.
McCabe CF, Donahue SP. Prognostic indicators for vision and mortality in shaken baby syndrome. Arch
Ophthalmol. 2000;118:373–377.
Morad Y, Kim YM, Armstrong DC, et al. Correlation between retinal abnormalities and intracranial abnormalities
in the shaken baby syndrome. Am J Ophthalmol. 2002;134:354–359.
Pierre-Kahn V, Roche O, Dureau P, et al. Ophthalmologic findings in suspected child abuse victims with subdural
hematomas. Ophthalmology. 2003;110:1718–1723.