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SHAKEN BABY SYNDROME
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SHAKEN BABY SYNDROME
PEARLS
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Any intracranial injury in the infant under 1 year of age is presumed to be child abuse until proven otherwise.
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The Shaken Baby Syndrome is characterized by the absence of external visible injury in association with retinal and intracranial hemorrhages.
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The syndrome may be often overlooked when only mild cerebral trauma has been caused.
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Prevention may occur through anticipatory guidance by discussing the risks of shaking and providing written information.
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What are the clinical features of the Shaken Baby Syndrome?
 The classic clinical findings include a
 constellation of retinal hemorrhages,
 subdural and surarachnoid hemorrhages with little or no evidence of external cranial trauma.
 Retinal hemorrhages are thought to occur in 75 to 90% of cases and may be unilateral or bilateral but may be missed unless the child is examined by a pediatric ophthalmologist.
 The symptoms and signs of the Shaken Baby Syndrome may be very subtle if
 only mild ocular or cerebral trauma has occurred.
 Infants may have a history of poor feeding,
 vomiting,
 lethargy and/or irritability occurring intermittently for days or weeks prior to the time of the initial health care examination. T
 he subtle symptoms are often minimized by physicians or attributed to mild viral illnesses, feeding dysfunction or infant colic. Most often one caretaker is aware of the true etiology of injuries, the others are not.
 The caretaker who violently shakes the young infant causing unconsciousness may put the infant to bed hoping that the baby will later recover.
 In the extreme form of the Shaken Baby Syndrome the infant typically will present with seizures, in a coma, not sucking or swallowing, unable to follow movements and not vocalizing.
 Some infants will have respiratory difficulty with apnea or complete respiratory arrest.
 In the severe form of Shaken Baby Syndrome the diagnosis may be confused with
 meningitis,
 sepsis,
 or late hemorrhagic disease of the newborn due to vitamin K deficiency.
What investigations should be done?
 Computed tomography (CT) is generally the method of choice for demonstrating subarachnoid hemorrhage.
 Magnetic resonance imaging is considered complementary to CT.
 Skull fractures that are multiple, bilateral, or cross suture lines are more likely to be nonaccidental in origin than simple linear fractures.
 A skeletal survey including the long bones, skull, spine and ribs should be obtained as soon as the infant's medical condition permits, in order to exclude multiple injuries.
 Single or multiple fractures of the midshaft or metaphysis of the long bones or rib fractures would confirm the suspicion of non-accidental injury in the young infant under 6 months of age.
What steps should be taken by physicians who suspect or diagnose nonaccidental head injuries?
 Suspicion of nonaccidental head injury must be reported immediately to the appropriate authorities in order for them to institute a thorough investigation before the issues become muddied by time and the comparison of explanations by the infants' responsible caretakers.
 A team approach is the only reasonable way in which the management of child abuse can be successfully accomplished.
Why do some parents shake their babies?
What are the risk factors for inflicting cerebral trauma?
 Violent shaking of an infant is the caretaker's response to the tension and frustration frequently generated by a baby's incessant crying or irritability.
 Caretakers who are at risk for such abusive behavior generally have unrealistic expectations of their children and may exhibit a role reversal, whereby the parents expect their needs to be met by the child.
 Parents with psychiatric difficulties or those experiencing stress as a result of environmental, social, biologic or financial situations may also be more prone to impulsive and aggressive behavior.
 In some cases there is careless disregard for the child's safety and in other cases it is less clear whether there was an intent to inflict serious harm on the infant.
 What can be expected following the diagnosis of the Shaken Baby Syndrome?
 There is a high incidence of morbidity and mortality. If the infant presents in a coma, up to 60% may die, have profound mental retardation, spastic quadriplegia or severe motor dysfunction.
 Those infants who present with seizures, irritability, or lethargy with no lacerations or infarctions of brain tissue may have subtle neurologic sequelae or persistent seizures.
 The consequences of shaking infants who do not come to medical attention is unknown.
 What can physicians do to prevent the occurrence of the Shaken Baby Syndrome?
 As part of an anticipatory guidance, physicians should ask about parental stresses and their responses to the crying infant. Parents should be advised regarding the risks of shaking babies.
 Reading material on Shaken Baby Syndrome should be provided to all parents.
 An open channel of communication for stressed out parents should be made available.
 If baby-sitters are used, careful checking of references and employment history is mandatory since many of these caretakers have no experience and may simply be unemployed individuals looking for temporary work until a job situation presents itself
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Caffey described a constellation of subdural hematoma, brain injury, retinal hemorrhages, and metaphyseal fractures in 1972. He noted that these infants did not have signs of external cranial trauma and postulated that the injuries were caused by shaking of the infant, hence the term shaken baby syndrome. In this syndrome, there is often a vague clinical presentation with minimal history of trauma, yet there may be substantial intracranial bleeding or intracranial injury that may lead to permanent neurologic deficit and mental retardation. The clinical spectrum may vary tremendously with some patients presenting with poor feeding, vomiting, failure to thrive and others presenting with lethargy, seizures or cardiopulmonary arrest.
The shaken baby syndrome has been postulated to result from the effect of non-impact acceleration-deceleration forces. It has been suggested that the back and forth movement of the head alone is sufficient to cause tearing of the bridging veins, resulting in subdural hematoma and death. The relatively large size of an infant's head, weakness of the neck musculature, softness of the skull, relatively large subarachnoid space, and high water content of the brain have been postulated to contribute to the susceptibility of shaking injuries in infants. However recent studies have questioned whether shaking alone is sufficient to produce the intracranial lesions. In fact, recent work that included analysis of a laboratory biochemical model, indicated that a cranial impact trauma is an essential component of the pathophysiology of the injury and therefore the advocates of this theory like to use the term "shaken impact syndrome."
The most common scenario of the impact may be a child who is shaken, then thrown into or against the crib or other surface, striking the back of the head and thus undergoing a large brief deceleration. However, independent of the actual mechanism of injury inflicted there is no disagreement that the combination of the injuries described by Caffey is associated strongly with child abuse. In fact, several studies so far have confirmed that simple falls from the heights (even up to 5 feet) rarely, if ever, result in significant primary brain injury. In fact, 36% of all head injuries are due to abuse, and 95% of serious intracranial injuries are due to abuse.
In these patients the ocular exam is very important. The most common ocular complication of non-accidental injury is retinal hemorrhage that may occur in up to 50-80% of shaken babies. The mechanism of retinal hemorrhage remains speculative. Historically, it has been attributed to violent shaking. Other postulated mechanisms include increased pressure to the central retinal vein from increased intracranial, increased intrathoracic pressure or some effect to direct head trauma.
Traumatic retinoschesis resulting from acceleration/deceleration forces applied to the eyes has also been postulated as a possible mechanism. This may be particularly relevant in very young children because of the more solid consistency of the vitreous body in the infant and the stronger adhesions at the vitreoretinal interface. It is important to note that known accidental trauma very rarely causes retinal hemorrhage. The incidence of retinal hemorrhages is estimated to be as high as 40% of all neonates after vaginal delivery. These hemorrhages are venous in origin and usually resolve within 10 days after delivery, and recently a study that looked at full term neonate with normal Apgar scores and uneventful births retinal hemorrhages showed that there was no associated intracranial injuries similar to those seen in shaken infants. However, another study has shown significant correlation between the severity of retinal hemorrhages and the acute neurologic injury in children with shaken baby syndrome. The long term sequelae of retinal hemorrhage would vary from normal vision to no light perception. The retinal function may be obscured for a significant portion of the development that deprivational amblyopia may occur. Finally, these patients may develop cortical blindness.
Other injuries in shaken infants include fractured ribs, fractured scapula, fractured spinous process, fractured sternal process, chest wall contusion, intrabdominal injuries including pancreatic injury as well as duodenal hematoma. The long bone injuries usually include metaphysical avulsion, epiphyseal separation, subperiosteal hemorrhage or new bone formation and spiral fractures. Cervical cord injuries occur rarely but are associated with bad outcome. Finally, tin ear syndrome with unilateral ear bruising also occur in this syndrome.
In evaluating these patients, skull x-ray and unenhanced CT with bone window are important. Some radiologists advocate that MRI is extremely sensitive for detection of cortical brain injury and subdural hematoma and it is also thought that it can precisely date the subdural hematoma and provide better evaluation of posterior fossa bleeds. However, these are much less common and MRI is more expensive, less accessible and the bones are usually seen as a negative signal. Finally, ultrasound is usually done for follow-up if needed rather than in the initial evaluation.
Recently, the issue of misdiagnosing of osteogenesis imperfecta, mainly type IV as child abuse has been brought up and remains controversial. However, because at least in the United States child abuse is so much more common than osteogenesis imperfecta especially type IV, it is essential that child abuse be considered in children who have unexplained fractures, particularly if metaphysical corner fracture or bucket handle lesions can be identified. In addition, fractures of the ribs on skeletal survey in the absence of a history of major chest trauma is strongly suggestive of abuse.
Shaken baby syndrome is not only associated with increased mortality rate (7-33%) and increased morbidity with significant poor neurologic outcome as well as blindness in some patients but also it is associated with development antisocial behavior. In fact, there is a growing body of empirical literature on the immediate and long term effects of childhood physical abuse. In addition to the fact that former abuse victims may become abusive themselves, studies of the long term effect of physical maltreatment suggest that adults who were abused as children have a greater tendency to commit criminal acts, as well as to display more psychological symptoms. The abused subjects will also have relative absence of verbal expressions of disagreement which suggest that such individuals may characteristically choose physical over verbal methods of conflict resolution.
Child abuse and neglect, in their various forms, constitute major unsolved problems in individual and public health. While recognition and reporting have improved lately, intervention, treatment and prevention remain premature. One approach to preventing maltreatment and its sequelae is to provide preventive sources to families of infants who are at risk for such problems. Therefore, it is our duty to recognize these risk factors. Considerable research has suggested that low income is consistently associated with the reporting of child abuse, in addition, premature infants are more likely to be abused but poverty is also associated with prematurity, making it difficult to determine how these associations work.
Recently, data suggested that various serious cases of physical abuse are caused by unrelated male caretakers of young infants. Also, parents who were abused themselves are more likely to abuse their children. Older male children who are being sexually abused as children often begin to abuse younger children while they, themselves, are still victims. Women who were sexually abused as children have higher incidence of abuse in their own children than do others. Finally, handicapped children are somewhat more likely to be abused in a number of ways than are their intact peers. An important element of the victim of abuse is helplessness, and the lack of ability to interrupt the process. Young infants tend to receive the most serious physical injuries when abused, and virtually all the deaths from abuse of young children occur under 3 years of age.
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