Shaken baby syndrome

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	Shaken baby syndrome;
ICD-9	995.55
MedlinePlus	000004

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD [2]

Synonyms and keywords: abusive head trauma, the battered-child syndrome, the whiplash shaken infant syndrome, nonaccidental head injury, nonaccidental head trauma, inflicted traumatic brain injury, shaken impact syndrome

Overview

Abusive head trauma (AHT), commonly known as shaken baby syndrome (SBS), is the injury to the skull/intracranial structures due to violent shaking and/or abrupt impact in children aged less than 5 years. AHT is caused by shaking injuries from repetitive and rapid flexion, extension, and rotation of the head and neck usually following parental frustration over an infant that does not stop crying. Retinal hemorrhages and subdural hematomas associated with spine injuries and/or bone raise high suspision for shaken baby syndrome. Noncontrast head CT is the initial radiologic workup for evaluating intracranial injury due to abusive head trauma (AHT). The prognosis of AHT is associated with the extent of damage seen on CT scan and MRI. The initial management of AHT is to maintain the patient's airway and circulation. Vital signs, oxygenation, and intracranial pressure (ICP) should be monitored. Increased intracranial pressure (IICP) and seizures should be managed. Decompressive craniectomy may be required.

Historical Perspective

In 1945, Dr. John Caffey reported pediatric cases with chronic subdural hematoma and long bone fractures.^[1]
Later, Dr. Caffey discovered the association between traumatic shaking, subdural hematoma, and retinal hemorrhage.^[2]
In 1962, Henry Kempe used the term 'the battered-child syndrome'.^[3]
In 1974, Caffey used 'the whiplash shaken infant syndrome' for infants with injures from shaking the extremities with whiplash induced bleeding inside the brain and eye.^[4]
'Shaken baby syndrome' (SBS) has been used for decades to describe abusive head trauma (AHT) or inflicted traumatic brain injury on infants and young pediatric patients.^[5]
Other terms for SBS include: nonaccidental head injury or trauma, inflicted traumatic brain injury, or shaken impact syndrome.^[6]^[7]
The American Academy of Pediatrics and the Centers for Disease Control and Prevention (CDC) recommend the term 'abusive head trauma' (AHT).^[8]^[9]

Classification

The modified grading system of abusive head trauma (AHT) is as the following:^[10]


Modified Grading System of Abusive Head Trauma (AHT) (Modified Table from Khan et al.: Pediatric abusive head trauma and stroke. J Neurosurg Pediatr 2017;20:183e90.)^[10]
Grade	Description	Brain Infarction Seen on CT or MRI
1	Skull fracture alone +/- associated craniofacial soft-tissue injury	-
2a	Intracranial hemorrhage not requiring surgical intervention or Cerebral edema not requiring surgical intervention	No
2b	Intracranial hemorrhage not requiring surgical intervention or Cerebral edema not requiring surgical intervention.	Yes
3a	Intracranial hemorrhage requiring surgery or procedure or Cerebral edema requiring surgery or procedure or Death due to intracranial injuries.	No
3b	Intracranial hemorrhage requiring surgery or procedure or Cerebral edema requiring surgery or procedure or Death due to intracranial injuries.	Yes

Pathophysiology

AHT is caused by shaking injuries from repetitive and rapid flexion, extension, and rotation of the head and neck and may result in:^[11]^[12]

Tearing of the vessels due to the rapid striking of the brain on the skull result in bleeding. Consequently, the enlarging hematoma may cause pressure within the skull and lead to increased intracranial pressure (IICP) and additional injury to the brain.

Sheering forces across the brain may injure nerve axons and lead to diffuse axonal disruption.

Greater parenchymal movement

The head may hit an object and result in lacerations, bruises, and fractures.

AHT includes primary and secondary injuries: ^[13]

Primary injuries is the consequence of the initial direct trauma and may include:
- Skull fracture
- Epidural, subdural, subarachnoid, and intraparenchymal hemorrhages
- Cortical contusion
- Diffuse axonal injury
Secondary injuries are inflammatory changes causing impairments in neurons and the microcirculation of the brain and include the complications of the primary injuries:
- Diffuse brain edema
- Herniation
- Infarction or cerebrovascular accidents

The neurometabolic cascade of AHT is similar to traumatic brain injury:^[14]

Depolarization
Release of excitatory neurotransmitters
Potassium efflux
Increased activity of membrane pumps
Hyperglycolysis
Lactate accumulation
Calcium influx
Decreased production of ATP
Calpain activation and initiation of apoptosis
Axolemmal disruption and calcium influx
Neurofilament compaction
Microtubule disassembly
Axonal swelling and axotomy

Children are more susceptible to head injuries at younger ages because:

The skull is easily compressed and therefore causes injuries to the underlying brain tissue.
The head is larger (in proportion to the rest of the body) and as they fall it is usually injured first.
The brain is more likely to suffer acceleration-deceleration injuries due to higher water content compared to adults.

Causes

AHT is caused by shaking injuries from repetitive and rapid flexion, extension, and rotation of the head and neck.^[11]

Differentiating Shaken Baby Syndrome from Other Diseases

Shaken baby syndrome must be differentiated from the following conditions:^[15]^[16]^[17]^[18]^[19]^[20]^[21]

Accidental head trauma that may cause:
- Epidural hemorrhage
- Subdural hemorrhage
- Subarachnoid hemorrhage
- Cerebellar hemorrhage
- Parenchymal hemorrhage
Bleeding diathesis
Arteriovenous malformation
Stroke
Neoplastic conditions
Metabolic disorders
Glutaric aciduria
Vitamin K deficiency
Connective tissue diseases
Osteogenesis imperfecta

Epidemiology and Demographics

The exact incidence of shaken baby syndrome/abusive head trauma (AHT) is unknown.^[12]
In a population-based study, the incidence of AHT was reported to be 29.7 cases per 100,000 children younger than one year in the United States.^[22]
In another study, the incidence of AHT was reported to be 24.6 cases per 100,000 children younger than one year in Scotland.^[23]
Shaken baby syndrome/AHT is the leading cause of death due to head injuries in children younger than 2 years, worldwide.^[12]

Risk Factors

Common risk factors in the development of shaken baby syndrome/AHT include factors that increase the risk of child abuse and may include:^[24]^[25]^[26]^[27]^[28]^[29]^[30]^[31]

Infantile colic
Inconsolable cry
Child Disability
Lack of parental frustration tolerance
Lack of prenatal and childcare experience
Low education level
Low socioeconomic status
Single-parent families
Young parents without support
Community isolation
Limited recreational facilities
Poverty

Screening

There is insufficient evidence to recommend routine screening for shaken baby syndrome.

Natural History, Complications, and Prognosis

Children with AHT are more likely to experience the following compared to children with accidental head trauma:^[32]^[33]

Worse outcomes
Cardiorespiratory compromise
Diffuse cerebral hypoxia-ischemia
Deeper brain injuries
Death

Complications of AHT may include:^[34]^[32]^[35]^[12]

Blindness
Seizures
Attention deficit
Behavior difficulties
Learning difficulties
Sensory impairment
Motor dysfunction
Severe handicaps
Decreased quality of life
Death

The prognosis of AHT is associated with the extent of damage seen on CT scan and MRI.^[36]

Diagnosis

Retinal hemorrhages and subdural hematomas associated with spine injuries and/or bone raise high suspision for shaken baby syndrome.^[37]

Clinical Evaluation

The clinical work-up of shaken baby syndrome should include:^[12]

Comprehensive history
Physical examination
Laboratory tests
Imaging
Consultation with specialists

History and Symptoms

History

The following should be considered in the history of shaken baby syndrome/AHT:^[12]

Open-ended questions should be asked by physicians
Caretakers should be interviewed separately
An inconsistent or changing history may suggest AHT or child abuse
The most common history suggestive of abusive head trauma (AHT) are:
- History of non-accidental trauma
- History of falling from a low height

Symptoms

Symptoms of shaken baby syndrome/AHT may include:^[12]

Physical Examination

The following signs should be considered on the physical examination of shaken baby syndrome/AHT:^[12]

Decreased level of consciousness
Cardiovascular collapse
Bulging fontanel
Hydrocephalus
Microcephaly
Subdural hematoma
Retinal hemorrhages
Fractures in long bone, metaphysis, and rib
Lack of external injury
Bruises (ears, neck, or trunk)

Laboratory Findings

Laboratory tests for shaken baby syndrome should include:^[12]

Complete blood cell count (CBC) with platelet count
Prothrombin time (PT)
Partial thromboplastin time (PTT)
Chemistry panel
Aspartate aminotransferase (AST)
Alanine aminotransferase (ALT)
Amylase
Lipase
Urinalysis

Electrocardiogram

There are no ECG findings associated with shaken baby syndrome.

X-ray

There are no specific x-ray findings associated with shaken baby syndrome, however, in order to identify child abuse in children aged less than two years with unexplained trauma, a skeletal survey should be performed with radiographs of the:^[12]

Echocardiography or Ultrasound

There are no echocardiography or ultrasound findings associated with shaken baby syndrome.

CT scan

Noncontrast head CT is the initial radiologic workup for evaluating intracranial injury due to abusive head trauma (AHT).^[12]^[38] CT is helpful in detecting:^[38]

MRI

MRI may be helpful in:^[39]

Detecting subacute and chronic subdural bleeding
Evaluating the extent of injuries in the parenchyma
Differentiating chronic subdural from subarachnoid collections

Other Imaging Findings

There are no other imaging findings associated with shaken baby syndrome.

Other Diagnostic Studies

There are no other diagnostic studies associated with shaken baby syndrome.

Treatment

Medical Therapy

Vital signs should be monitored.

The initial care of AHT is to maintain the patient's airway, breathing, and circulation.^[12]

Patients without impairment of consciousness may be managed with supportive care.^[12]

Hypotension is treated with fluids.

Intubation and mechanical ventilation are required in patients with moderate impairment of consciousness, severe respiratory insufficiency, or hemodynamic instability.^[12]

Oxygenation should be monitored with a pulse oximeter.

Intracranial pressure (ICP) should be monitored.^[12]
- Maintain ICP below 20 mmHg and minimal cerebral perfusion pressure (CPP) over 40 mmHg.^[40]
- The age-dependent CPP is recommended as the following:
  - 50 mmHg for 2-6 years
  - 55 mmHg for 7-10 years
  - 60 mmHg for 11-16 years

Increased intracranial pressure (IICP) should be decreased in order to prevent secondary brain injury by:^[12]
- Hyperventilation
  - Maintain the PaCO2 at 25-30 mmHg (since excessive hyperventilation and hypocapnia result in vasoconstriction and decreased cerebral perfusion, capnography is recommended to monitor end-tidal CO2)
- Raise the head to 30 degree
  - Improves venous return without affecting cerebral blood flow
- Hypertonic agents may be used in moderate IICP.
  - 3% hypertonic saline bolus 2-6 ml/kg, followed by 0.1-1 ml/kg/hour till the upper limit of serum osmolarity 360 or sodium level 155 g/dl.
- Mannitol
  - 0.25-1 gm/kg IV every 4-6 hours with the upper limit of serum osmolarity 320.
- Sedation with barbiturates may be needed in persistent IICP.
  - Barbiturates decrease cerebral metabolism and in turn decrease cerebral blood flow and therefore, reduce ICP.
  - Thiopental or pentobarbital

Continuous EEG (cEEG) monitoring should be performed and antiepileptic drug may be used to reduce the risk for early posttraumatic seizures (EPTS).^[41]^[42]
- Seizure may occur at any stage.

Stepwise hypothermia keeps the body temperature between 32 C and 33 C for 48 hours and neuromuscular blockade may be helpful in preventing shivering.
- Therapeutic hypothermia may reduce:^[43]
  - Inflammation
  - Cell death
  - Excitotoxicity
  - Acute seizures
  - Cerebral metabolic demands

Surgery

Decompressive craniectomy (limits secondary brain injury by removing part of the skull and allowing brain swelling which ) is indicated when there is:^[44]

No response to prior treatments
Neurologic deterioration
Signs of herniation

Primary Prevention

Prevention of AHT includes:^[12]^[25]

Decrease in child abuse and maltreatment
Parental education with public service announcements, family resource centers, and home visit programs in:
- Parenting
- Child development
- Dealing with a child's cry
- Parental resilience
- Danger of shaking a baby
- Social support

Secondary Prevention