Anne Jensen
Gil Binenbaum

BASICS

DESCRIPTION

• Child abuse and neglect as defined by the Child Abuse Prevention and Treatment Act (CAPTA) is any act or failure to act on the part of a parent or caretaker, which results in death or serious physical or emotional harm. It includes sexual abuse or exploitation. State-specific guidelines are based on this definition. (1)

• Child neglect involves the omission of fundamental resources and support, including food, clothing, supervision, shelter, medical care, and education.

• Shaken Baby Syndrome (SBS), a subset of abusive head trauma, refers to a constellation of findings (retinal hemorrhage, subdural or subarachnoid hemorrhage, and brain injury, with or without fractures, usually with minimal apparent external trauma) thought to arise from repetitive acceleration–deceleration forces, with or without head impact. (2).

EPIDEMIOLOGY

Incidence

• In 2008, there were 3.3 million referrals in the US to child protection services alleging abuse or neglect of 6 million children. Of the 63% investigated, 772,000 cases were confirmed.

• About 2,000 deaths annually result from child maltreatment. (1)

• Over 80% of all offenses and over 70% of all child fatalities in 2008 were caused by parents of the victim. (1)

RISK FACTORS

• Age: Almost 33% of all victims in 2008 were under 4 years old. Younger children are at much greater risk, with 22% of all cases and over 40% of all fatalities occurring in children under 1 year of age. (1)

• Gender: Risk for victimization is essentially equal between both boys and girls except for sexual abuse where girl victims predominate. (1)

• Race: Though almost half of all victims are Caucasian, African American children, Native American children, and children of mixed ethnicity are most at risk per 1,000 children of the same race. (1)

GENERAL PREVENTION

Parental education upon discharge from the hospital, in the form of videos, pamphlets, and signed contracts regarding the risks associated with shaking as well as suggestions for coping with a crying child are thought to decrease rates of SBS.

PATHOPHYSIOLOGY

• Rotational acceleration: Leads to axonal injury and tearing of bridging veins. This leads to the formation of subdural hematoma (SDH), subarachnoid hemorrhage (SAH), and cerebral edema. (3)

• Tractional ocular injury: The typical pattern of retinal hemorrhage in SBS coincides with areas of maximal vitreoretinal adhesion: Vessels, macula, and peripheral retina. Vigorous repeated acceleration–deceleration produces translational movement of the vitreous, which can lead to characteristic macular tractional retinoschisis with or without surrounding retinal folds. (6) Trauma may also occur to orbital tissues.

ETIOLOGY

• SBS involves an act of shaking so violent that observers would easily recognize it as dangerous. The injuries from this type of trauma are distinct from injuries sustained from short falls. Seizures, coughing, and vaccinations do not simulate these injury patterns. (2)

• In infants the most common catalyst for abuse is crying or issues around feeding. In toddlers, toilet training is a common inciting event.

COMMONLY ASSOCIATED CONDITIONS

• Caretaker substance/domestic abuse

• Neglect and failure to thrive

• Poverty

• Sexual abuse

• Mental/physical/behavioral disability

• Chronic medical conditions

DIAGNOSIS

• Approximately 85% of SBS cases involve retinal hemorrhage, which in two-thirds are too numerous to count, multilayered, and extending to the ora serrata.

• Initial presentation will vary widely based on the type of abuse that has occurred. Suspect abuse with unexplained delay in seeking medical care.

• Physicians are required by law to report all suspected cases of child abuse and/or neglect. Abuse need not be proven by the reporting physician.

HISTORY

• A detailed history including a specific timeline should be recorded to assess plausibility of injuries. (2)

• A diagnosis of abuse is suggested by a clinical history that is changing or incongruent with the current presentation.

• Evaluation of developmental history is especially pertinent.

PHYSICAL EXAM

• General: Complete exam should be performed. Any external evidence of injury should be well-documented with forensic photographs, labeled with patient name and date. (2)

• Funduscopic: A dilated eye examination should be performed by a trained ophthalmologist, preferably within 24 hours but no later than 72 hours when abusive head injury is suspected or a child sustains and unexplained acute life threatening event (ALTE). Care should be taken to view the peripheral retina. Hemorrhages, present in 50–80% of SBS cases, may be preretinal, subretinal, or within the retina itself. Thorough documentation of the number, type, asymmetry/unilaterality, extent, and pattern of hemorrhage should be carefully recorded. These patterns can be used to narrow the differential diagnosis. Retinoschisis, with blood accumulation between retinal layers, with/without an associated hypopigmented circumlinear retinal pleat or fold at its edge is strongly suggestive of abuse. Blood in the retinoschisis cavity may leak into the vitreous over several days and require surgical intervention for vitreous hemorrhage. Therefore, patients with retinoschisis should be assessed serially.

• Other ocular injuries: Signs of direct ocular trauma, such as bruising, hyphema, lens dislocation, and so on, should raise the suspicion of abuse in the absence of a plausible history of accidental trauma.

• Sexually Transmitted Infections (4)

– Ocular manifestations of sexually transmitted diseases may represent a congenitally acquired infection or a “red flag” for sexual abuse. Incubation after perinatal transmission may be delayed, especially for chlamydia.

– Some genital infections may rarely be transmitted to the eye by infected secretions on contaminated fingers.

– Syphilis uniquely travels to the eye through the bloodstream and always indicates sexual transmission.

– Infections associated with ocular manifestations include Chlamydia trachomatis, Phthirus pubis, syphilis, gonorrhea, HIV, and herpes simplex virus (usually nonsexual transmission).

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Diagnostic tests should be performed at the discretion of the primary care provider or child abuse pediatrician.

Imaging

• Radiographic findings: Skeletal survey, including all bones (not a «babygram»), should be performed to evaluate for rib, skull, and long bone fractures, particularly in children less than 2 years old. Neuroimaging (CT or MRI) may reveal subdural hematoma, skull fractures, axonal injury, and edema. Subdural hemorrhages involving bilateral convexities, posterior interhemispheric fissure, or posterior fossa are particularly worrisome as signs of abuse in the absence of a clear history of accidental trauma. (3)

• Retinal photography: Retinal imaging is not a necessary component of a thorough work-up. A diagram with detailed written descriptions is acceptable. When available, fundus photos can be useful for documentation. Photography should not replace the clinical examination.

Pathological Findings

• Pathological brain findings include hemorrhage, cerebral edema, cerebral contusions, and diffuse axonal injury, atrophy, or infarction. (2)

• Postmortem, a full autopsy must be performed with en bloc removal and evaluation of the eyes and orbital tissues.

DIFFERENTIAL DIAGNOSIS

Nonabuse causes of retinal hemorrhage are almost always few in number and confined to the posterior pole, or are associated with abnormal laboratory findings, physical examination findings, or historical information that readily makes the diagnosis.

• Retinal hemorrhage

– Shaken baby syndrome

– Hypertension

– Coagulopathy/leukemia

– Meningitis/sepsis/endocarditis

– Vasculitis

– Cerebral aneurysm

– Anemia

– Hypoxia/hypotension

– Papilledema (peripapillary hemorrhages)

– Glutaric aciduria type 1, Osteogenesis imperfecta

– Retinopathy of prematurity

– Extracorporeal membrane oxygenation

– Hypo- or hypernatremia

– Birth

– Vitamin K deficiency

– Carbon monoxide poisoning

– Drowning

TREATMENT

ADDITIONAL TREATMENT

General Measures

• Hospitalization may be indicated for protection and diagnosis.

• Reporting suspected cases to child protective services in a timely manner is crucial.

• Multidisciplinary treatment plan is needed to address medical, social, and psychological needs of the patient and family, including family counseling and mental health therapy.

• Removal of the child from the current home or social situation is a matter determined by child protective service agencies.

• State laws require medical staff to report suspected cases to a child protective service. (1)

SURGERY/OTHER PROCEDURES

Retinal hemorrhages generally resolve without intervention. Vitrectomy should be considered in young patients when the risk of amblyopia arises secondary to prolonged visual axis obstruction. (6)

IN-PATIENT CONSIDERATIONS

Initial Stabilization

• Should address acute respiratory or neurological decompensation, though notably some victims will present with little or no external signs of trauma and nonspecific symptoms

• Systemic stabilization takes priority over eye examination.

Admission Criteria

The American Academy of Pediatrics recommends that in communities without specialized crisis intervention centers, all children who present with concern for abuse be hospitalized until they are medically stable and safe placement options are identified.

Discharge Criteria

Patient may be discharged when medically stable to safe care as identified by child protective services.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Retinal hemorrhages generally resolve without intervention. Close monitoring may be required to ensure appropriate management of amblyopia, refractive changes, optic atrophy, and central visual impairment.

• Serial fundus exams may be necessary as blood accumulation within a retinoschisis cavity may leak out into the vitreous over the course of several days and obstruct the visual axis.

• Skeletal surveys should be repeated 2 weeks after presentation to identify callus around areas of previously undetectable fractures.

PATIENT EDUCATION

• Patient education should include strategies for coping with a crying child, as well as information regarding the danger of violent shaking even in the absence of impact.

• Discuss family stressors, especially with families caring for a disabled child.

PROGNOSIS

• Overall reported mortality rates in SBS range between 15–38%. (2)

• Younger age at injury is associated with poorer prognosis, with the highest mortality rates in children under 2 years old. This effect may in part be due to some disruption of normal development.

• Severity of injury is also a strong predictor of poor outcomes, as lower Glasgow Coma Scale scores and longer periods of unconsciousness are associated with subsequent cognitive, motor, and behavioral deficits.

• Long term deficits may include cortical visual impairment, spasticity, seizure disorders, microcephaly, chronic subdural fluid collections, cerebral atrophy, and encephalomalacia. (2)

• There is a direct relationship between the severities of brain and eye injury. (5)

• More common mechanisms of permanent vision loss in abusive head injury include occipital cortical damage, optic atrophy, and less commonly, retinal detachment.

COMPLICATIONS

• Death

• Developmental delay

• Cerebral palsy

• Seizures

• Central visual impairment

• Optic atrophy

• Amblyopia

• Myopia

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