Case 25

Question 1

What are key history findings with shaken baby syndrome?

Answer 1

Apnea, neurological depression, difficult home situation

Question 2

What are key physical exam findings with shaken baby syndrome?

Answer 2

Hypothermic, tachycardic, bradypneic, hypertensive, abnormal neurological exam (poor suck, tense full fontanelle, intermittent crying, decreased tone, inability to fix and follow visually)

Question 3

What is the differential diagnosis for a patient with shaken baby syndrome?

Answer 3

Meningitis, encephalitis, GERD, closed-head injury (shaken baby), cardiac arrhythmia, respiratory infection

Question 4

What are the key findings from testing for shaken baby syndrome?

Answer 4

Subdural hematoma on CT and MRI.

Question 5

What is the epidemiology of shaken baby syndrome?

Answer 5

• 10-12 percent of all deaths among children who are victims of child abuse are due to shaken baby syndrome
• Mortality rate is 25 percent and 20-40 percent have poor outcomes due to neurological injury

Question 6

What is the pathophysiology of shaken baby syndrome?

Answer 6

• Injuries, including close head injury (CHI) and retinal hemorrhages, are due to violent shaking or throwing (with resulting blunt trauma to the head). Bridging vessels tear when infant is shaken - or shaken and thrown - achieving an extreme rotational acceleration force to the brain and diffuse axonal injury to neurons
• Victims of shaken baby syndrome often have no other signs of physical abuse

Question 7

Presentation/Signs and Symptoms of shaken baby syndrome may include:

Answer 7

• Constant crying
• Stiffness
• Excessive sleeping
• Difficulty to arouse
• Seizures
• Dilated pupils
• Decreased appetite
• Retinal hemorrhages (retinal hemorrhages outside the newborn period are pathognomonic for shaken baby syndrome and are found in 65-90 percent of victims)

Question 8

Outcomes of shaken baby syndrome:

Answer 8

Increased risk for intellectual disability, developmental delays, motor delay or extreme motor deficit, difficulty with vision (including blindness) and seizures

Question 9

What is apnea?

Answer 9

Cessation of inspiratory gas flow for 20 sec, or for a shorter period of time if accompanied by bradycardia (heart rate less than 100 bpm), cyanosis or pallor.

Question 10

What is an apparent life-threatening event (ALTE)?

Answer 10

Not a diagnosis, but a description of an event. Caregiver usually describes apnea, color change, change in tone, and possibly choking or gagging. The observer may think the infant has died. Recovery occurs only after stimulation or resuscitation. Incidence is 0.05-1 percent in population-based studies.

Question 11

What are potential causes of an ALTE?

Answer 11

1. Central nervous system
2. Cardiac
3. Pulmonary
4. Gastrointestinal
5. Systemic

Question 12

What are CNS causes of ALTE?

Answer 12

• Seizures (due to bleeding, infection, structural abnormalities, metabolic disorders, electrolyte imbalances, genetic syndromes, epilepsy)
• Breath-holding spells (pallid or cyanotic)
• Increased intracranial pressure (due to bleed, trauma, tumor, infection)

Question 13

What are Cardiac causes of ALTE?

Answer 13

• Arrhythmia (bradycardia, long QT syndrome)

- Congenital heart disease (ductal dependent lesions, unrepaired Tetralogy of Fallot)

Question 14

What are respiratory causes of ALTE?

Answer 14

Respiratory infections (including RSV, pertussis)

Question 15

What are gastrointestinal causes of ALTE?

Answer 15

• GERD (may cause choking and laryngospasm)

- Swallowing abnormalities/tracheoesophageal fistula (coughing, difficulty with feeds)

Question 16

What are systemic causes of ALTE?

Answer 16

• Infection (sepsis due to Group B strep, E. coli, or Listeria infection, among others)
• Metabolic disorders (inborn errors of metabolism)
• Intoxication (ingestions of mediations or toxins)
• Infant botulism (exposure to botulinum toxin in soil or honey)
• Environmental exposure (carbon monoxide)

Question 17

Vital signs in shaken baby syndrome:

Answer 17

1. Tachycardia: Indicates a deterioration in CV status and may be related to sepsis or increased ICP.
2. Respiratory rate: A low respiratory rate suggests CNS depression of respiratory center rather than sepsis or a respiratory infection, which generally present with increased respiratory rate
3. Blood pressure: Elevated blood pressure indicates a response from the CV system either to pain, compensated shock, or increased ICP.

Question 18

At two months of age a typically developing infant:

Answer 18

1. Can fix and follow easily with his eyes, smiles to voices; has a strong suck and is beginning to coo
2. Lies flexed at hips with good tone and moves all four extremities well
3. Lacks good head control when held upright and cannot roll over
4. When prone, can raise head and move it from side to side

Question 19

What is on the differential diagnosis for Closed-head injury (CHI)?

Answer 19

Meningitis, Gastroesophageal (GE) reflux, cardiac arrhythmia, respiratory infection

Question 20

Closed-head injury (CHI):

Answer 20

• Apnea, seizure, and altered neurological status are common in closed-head injuries
• Subdural hematomas result from trauma, either accidental or non-accidental. They can be seen in newborns, particularly after vacuum extraction deliveries. Not seen as result of cardiopulmonary resuscitation or seizures and do not occur from short falls (eg, from a height of less than 4 feet). Subdural hematomas are often found in infants who have experienced violent shaking.

Question 21

Meningitis:

Answer 21

• Meningitis in young infants causes increased ICP and may present with apnea
• Infants are irritable, do not eat well, vomit, and usually have fever greater than 38.2C
• Infants are inconsolable and lethargic

Question 22

Gastroesophageal (GE) reflux:

Answer 22

• May cause emesis, gagging and aspiration pneumonia
• Seldom presents as apnea without associated symptoms
• Infants with GE reflux would have normal vital signs; may be irritable at times, but would not present with abnormal neurological findings

Question 23

Cardiac arrhythmia:

Answer 23

• Arrhythmia may cause apnea, but would not lead to persistent alteration of mental status
• A long QT syndrome or other arrhythmia may be identified on electrocardiogram

Question 24

Respiratory infection:

Answer 24

• Respiratory infection may present as apnea; other sx include tachypnea, fever, decreased feeding, and change in activity.
• Respiratory rate may be increased
• Infants may not cough or wheeze initially

Question 25

Coagulation studies:

Answer 25

Necessary to rule out a bleeding disorder

Question 26

Skeletal survey:

Answer 26

X-rays of the entire body to evaluate for old or new fractures. Involvement of a pediatric radiologist skilled in interpreting these films can be critical. Skeletal survey findings that raise suspicion for abuse include: - Fractures or injuries that are inconsistent with reported mechanism of injury and/or the developmental stage or abilities of the child - Multiple fractures or injuries at different stages of healing - Fracture of the femur or tibia in a non-walking child - Posterior rib fractures - frequently associated with shaken baby syndrome due to squeezing of the thorax by the perpetrator's hands during shaking. - Skull fracture in an infant is also very suspicious for abuse.

Question 27

CT of the head:

Answer 27

CT is often the first and definitive study to confirm subdural hematomas. Repeat CT should be considered if there is a concern about clinical deterioration.

Question 28

MRI:

Answer 28

MRI requires sedation and is not done until the child is stable. MRI is superior to CT for defining ischemia, visualizing shearing injuries to neurons. MRI can act as confirmation for findings identified on a head CT.

Question 29

Initial emergency management of head trauma:

Answer 29

1. Assess circulation, airway, and breathing (CABs) before beginning resuscitation efforts. The airway should be clear and stable. 2. Assess heart rate, respiratory rate, pulse ox, and strength of pulses 3. If CABs or vital signs are abnormal, immediate intervention is required. 4. Obtain IV access 5. Obtain head CT. 6. Admit to critical care unit 7. Consult neurosurgery/neurology

Question 30

Management of Shaken Baby Syndrome:

Answer 30

1. Consult with child advocacy specialist: Investigate for other signs of child abuse: A skeletal surgery should be performed if there is a suspicion of child abuse. 2. Refer to ophthalmology: A ophthalmology consultation is required to look for retinal hemorrhages (highly suggestive of shaken baby syndrome) 3. Social worker