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SUDDEN INFANT DEATH SYNDROME AND APPARENT LIFE-THREATENING EVENT Collin S. Goto Sing-Yi Feng

SUDDEN INFANT DEATH SYNDROME DEFINITION OF SUDDEN INFANT DEATH SYNDROME (SIDS)
The sudden death of an infant younger than 1 year which remains unexplained after a thorough case investigation, including a complete autopsy, examination of the death scene, and review of the clinical history.

The pathophysiology of SIDS is polygenic and multifactorial with medical, genetic, environmental, and behavioral/sociocultural factors. Prone sleeping is associated with SIDS and has been shown to increase the time infants spend in a state of reduced spontaneous arousability possibly due to the trapped carbon dioxide around the infants face. The SIDS rate in the United States has decreased since the American Academy of Pediatrics first published its recommendation that infants should sleep in a nonprone position in 1992 and started the back to sleep campaign in 1994.

EVALUATION AND MANAGEMENT

Resuscitation efforts should be initiated immediately on the unresponsive infant (Fig. 5-1). Past medical history, present illnesses, current medications, and any history of trauma should be ascertained and the child should be thoroughly examined for any congenital abnormalities, signs of concurrent illness, or evidence of physical abuse. SIDS is a diagnosis of exclusion.

EPIDEMIOLOGY AND PATHOPHYSIOLOGY

SIDS remains the most common cause of death for children aged 1 month to 1 year in developed countries. Approximately 2500 infants die from SIDS every year in the United States. The peak incidence is between 2 and 4 months of age and 90% of SIDS deaths occur in the first 6 months of life. Boys are more likely to die than girls at a ratio of 60:40. Younger maternal age, lack of prenatal care, low birth weight, prone sleeping position, overheating, and preterm birth are all risk factors for SIDS (Table 5-1). In the United States, African-Americans and Native Americans have SIDS rates that are two to three times the national average irrespective of socioeconomic status.

Unresponsive infant

Resuscitation not attempted

Resuscitation attempted

Resuscitation attempted

No return of spontaneous circulation

Return of spontaneous circulation

Cardiovascular deterioration

Multi organ failure

Brain death

Suspected SIDS

TABLE 5-1 Factors Associated with Sudden Infant Death Syndrome MEDICAL/GENETIC
Congenital defects Low birth weight Preterm infant Polymorphisms causing impaired autonomic regulation and arousal

ENVIRONMENTAL/BEHAVIORAL/ SOCIOCULTURAL
Bed sharing Head covering Higher ambient temperature Low socioeconomic status Maternal smoking Multiple layers of clothing Soft sleeping surfaces Smoke exposure Supine sleep position

Family support

Case investigation

SIDS

Non-SIDS death

FIG. 5-1. Management of sudden infant death syndrome.

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The differential diagnosis of SIDS includes sepsis, pneumonia, myocarditis, congenital heart defect, cardiomyopathy, arrhythmia, prolonged QT syndrome, accidental or nonaccidental trauma, suffocation, adrenal hyperplasia, and inherited metabolic disorders. Autopsies and death scene investigations are warranted to help determine the cause of death and provide valuable information and closure for the family. Prevention is the key to reducing mortality secondary to SIDS and risk reduction is the most important measure in preventing SIDS. Risk reduction strategies include nonprone sleeping, avoiding maternal smoking in pregnancy, decreasing environmental smoke exposure, maintaining comfortable ambient temperature, providing a safe sleep environment, and fully immunizing the child.

ETIOLOGY
ALTE is primarily a historic description of the event rather than a single, unifying pathophysiologic process. Apnea is a common presentation but is also the final common pathway for many disease processes seen in infants. A definitive diagnosis of the ALTE is found in only approximately 50% of patients.

INITIAL ASSESSMENT AND STABILIZATION

The initial evaluation of the unstable or ill-appearing infant is directed at identifying and stabilizing immediate life-threatening conditions (Fig. 5-2). A more thorough secondary survey is performed after stabilization to identify any physical findings that may elucidate the etiology of the ALTE.

DISPOSITION
The loss of a child is a devastating event. The parents should be allowed to see and hold the baby and details of the resuscitation should be explained. Immediate social work and pastoral support will help the family to cope with the difficult and confusing situation. Surviving siblings and other family members need age-appropriate support.

HISTORY
The history often provides the most important information in the evaluation of an ALTE. Information about the details of the event should be ascertained, including the infants respiratory effort, skin color, mental status, muscle tone, the duration of the event, and the degree of resuscitation required prior to evaluation in the emergency department.

THE APPARENT LIFE-THREATENING EVENT DEFINITION

Possible ALTE

Definition of ALTE: an episode that is frightening to the observer and is characterized by some combination of apnea (central or obstructive), color change (cyanosis, pallor, erythema, or plethora), marked change in muscle tone (rigidity or limpness), or unexplained choking or gagging. The majority of patients will appear well and the challenge for the emergency physician is to determine whether a true life-threatening event has occurred.

ALTE

Non-ALTE

Ill, Cardiopulmonary compromise

Wellappearing

Periodic breathing or other benign diagnosis

EPIDEMIOLOGY
ALTE is estimated to occur in 0.5% to 6% of all children during the first year of life and It is more common in boys and premature infants. The peak incidence is in infants younger than 2 months. The exact relationship between ALTE and SIDS is not clear.

ED stabilization

ED evaluation

ED evaluation

Admit for further management (PICU if patient is unstable)

Admit for further management

Discharge home

FIG. 5-2. Management of apparent life-threatening event.

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Central apnea is characterized by an absence of respiratory effort while obstructive apnea is usually associated with choking, gasping, or gagging. An infant who remains awake and alert during an event is unlikely to have suffered prolonged hypoxia or an acute neurologic event. Hypotonia associated with apnea or color change implies significant hypoxia or decreased cerebral perfusion, while hypertonicity is characteristic of seizures. A history of apnea that required vigorous physical stimulation or cardiopulmonary resuscitation implies a true life-threatening event. Information should be gathered concerning past medical history and any recent illness that may have contributed to the ALTE. A history of a sibling with SIDS is a recognized risk factor for sudden death. The possibility of factitious ALTE because of Munchausens syndrome by proxy must be considered in the infant who repeatedly presents with an ALTE or other unexplained illnesses.

TABLE 5-2 Differential Diagnosis of an Apparent Life-Threatening Event

Cardiovascular System Anemia Cardiomyopathy Congenital heart disease Dysrhythmia (prolonged QT syndrome, Wolff-Parkinson-White syndrome) Hemorrhage (child abuse) Myocarditis Vascular rings and slings Central Nervous System Apnea of prematurity Congenital brain malformation Head trauma (child abuse) Idiopathic central apnea Increased intracranial pressure (congenital hydrocephalus, tumor) Meningitis/encephalitis Seizure Respiratory System Breath-holding spell Bronchiolitis (respiratory syncytial virus) Congenital malformation (choanal atresia, laryngeal cleft, tracheoesophageal fistula) Foreign body Laryngomalacia/tracheomalacia Laryngospasm (choking spell, gastroesophageal reflux) Obstructive sleep apnea Periodic breathing of infancy Pertussis Pneumonia Smothering (intentional or unintentional) Upper airway obstruction (nasal congestion) Systemic/Metabolic/Other Dehydration Electrolyte abnormality (hyponatremia, hypocalcemia, congenital adrenal hyperplasia) Factitious (Munchausens syndrome by proxy) Hypoglycemia Hypothermia Inborn errors of metabolism Sepsis Toxins/drugs

PHYSICAL EXAMINATION
A thorough head-to-toe examination of the infant may provide clues to the etiology of the ALTE. Particular attention should be paid to the respiratory, cardiovascular, and neurologic systems. Continuous monitoring in the emergency department may provide the opportunity to observe events such as gastroesophageal reflux, choking, cyanosis, or apnea.

EVALUATION AND MANAGEMENT

The many diagnostic studies to be considered in the evaluation of an ALTE reflect the diverse differential diagnosis (Table 5-2). For suspected serious bacterial infection, a complete blood count, blood culture, urinalysis with urine culture, and lumbar puncture should be performed. Nasopharyngeal swabs for viral identification should be considered when a viral respiratory infection is suspected. A chest radiograph should be obtained for any infant with respiratory or cardiac abnormalities. Electrocardiogram is useful to assess for cardiac pathology, including prolonged QT syndrome, Wolff ParkinsonWhite syndrome, myocarditis, or anomalous left coronary artery with myocardial ischemia. Serum electrolytes, glucose, blood urea nitrogen, serum creatinine, calcium, magnesium, and phosphorus are obtained to evaluate seizures.

Computed axial tomography scan of the head, skeletal survey, and drug screen should be obtained if evaluating child abuse. Further evaluation after hospitalization may include sleep studies, pH probe testing, electroencephalography, and other studies that are beyond the scope of the emergency department.

DISPOSITION
An infant may be discharged from the emergency department if a detailed history and physical examination do not indicate that a true ALTE has occurred. A reasonable period of observation in the emergency department is important, but does not prove that the prior event was insignificant and does not rule out recurrence.

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After parental education, the infant without true ALTE may be discharged home with specific instructions for follow-up in 24 hours with a primary care provider or return to the emergency department sooner if any problems occur. Any infant with a history of apnea, pallor, cyanosis, limpness, or unresponsiveness requiring vigorous physical stimulation, or cardiopulmonary resuscitation is excluded from outpatient consideration. It is best to admit the infant for observation and monitoring if there is any question about the nature of the event, the parents ability to care for the infant at home, or the adequacy of follow-up. Any infant who is unstable should be admitted to the pediatric intensive care unit.

BIBLIOGRAPHY
American Academy of Pediatrics: Changing concepts of sudden infant death syndrome: Implications for infant sleeping environment and sleep position. Task Force on Infant Sleep Position and Sudden Infant Death Syndrome. Pediatrics 105:650656, 2000. American Academy of Pediatrics: Task Force on Sudden Infant Death Syndrome: The changing concept of sudden infant death syndrome: diagnostic coding shifts, controversies regarding the sleeping environment, and new variables to consider in reducing risk. Pediatrics 116:12451255, 2005. Bajanowski T, Vege A, Byard RW, et al: Sudden infant death syndrome (SIDS)standardised investigations and classification: recommendations. Forensic Sci Int 165:129143, 2007. Beeber B, Cunningham N: Fatal child abuse and sudden infant death syndrome (SIDS): A critical diagnostic decision. Pediatrics 93:539540, 1994. Esani N, Hodgman JE, Ehsani N, Hoppenbrouwers T: Apparent life-threatening events and sudden infant death syndrome: comparison of risk factors. J Pediatr 152:365370, 2008. Moon RY, Fu LY: Sudden infant death syndrome. Pediatr Rev 28:209214, 2007. Moon RY, Horne RS, Hauck FR: Sudden infant death syndrome. Lancet 370:15781587, 2007. Reece RM: Fatal child abuse and sudden infant death syndrome: a critical diagnostic decision. Pediatrics 91:423429, 1993. Valdes-Dapena M, Gilbert-Barness E: Cardiovascular causes for sudden infant death. Pediatr Pathol Mol Med 21:195211, 2002. Willinger M, James LS, Catz C: Defining the sudden infant death syndrome (SIDS): deliberations of an expert panel convened by the National Institute of Child Health and Human Development. Pediatr Pathol 11:677684, 1991.

QUESTIONS
1. You are peer reviewing a case of SIDS, which was seen in the emergency department several

weeks ago. The child was a two-month-old female infant, full term, small for gestational age with a birth weight of 2 kg. The 30-year-old mother had placed the infant on her back in the crib the night before. The next morning the infant was found unresponsive and apneic and she was brought to the emergency department where she was declared dead about an hour and a half later. Which of the following characteristics of this patient is a known risk factor for SIDS? A. Female B. Full term birth C. Supine sleeping position D. 30-year-old mother E. 2 kg birth weight 2. You have been asked to be an expert discussant on SIDS at a conference. You have been asked specifically to give a statement about the recommended infant sleeping position. Which of the following statements is true regarding sleeping positions and SIDS? A. The American Academy of Pediatrics advocates the prone sleeping position to prevent SIDS. B. Prone sleeping has been shown to increase the time infants spend in a state of reduced spontaneous arousability. C. Prone sleeping is believed to cause nitrogen to be trapped around the infants nose and mouth. D. Prone sleeping position has not been found to be associated with SIDS. E. Supine sleeping position has been strongly associated with SIDS. 3. During a recent small group discussion, a medical student asked the question of what exactly defines SIDS in children. Which of the following characteristics is needed to qualify a death as SIDS? A. Age greater than 1 year of age B. Cause of death unexplained despite investigation C. Male infant D. Patient with diagnosed chronic illness E. Previous history of an apparent life-threatening event (ALTE) 4. You are educating new parents about SIDS. One of the parents asks you for examples of risk-reducing strategies. You respond with one of the following answers: A. Avoiding maternal smoking during pregnancy B. Increased temperature of the sleeping environment C. Increasing environmental smoke exposure D. Limiting immunizations in children E. Prone sleeping position

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5. You are helping to develop a multidisciplinary team to help families grieve when a child dies of SIDS. Your team includes social workers, chaplains, and child life specialists. Which of the following is an appropriate grieving method? A. Age-appropriate support for family members B. Antidepressant therapy for surviving family members C. Counseling the family as a group D. Encouraging the temporary use of substances to help in grieving E. Use of stimulant medication to enhance mental focus 6. A 2-month-old female infant is brought to the emergency department by her parents after a choking spell at home. Which of the following statements best fits the definition of an ALTE? A. The infants skin was pink and warm during the choking spell. B. The muscle tone was normal throughout the event. C. The parents appear unconcerned about the event. D. The patient was breathing well throughout the episode. E. The patient was found choking and gagging during a nap. 7. The ALTE is a common reason for families to bring children to the emergency department for evaluation. However when considering the epidemiology of ALTE, which of the following statements is true? A. An ALTE is a reliable predictor of SIDS B. An ALTE occurs in 50% of all infants C. ALTE is more common in female infants rather than male infants D. ALTE is more common in full term infants rather than premature infants E. The peak incidence is in children under 2 months of age 8. A 2-month-old male infant is being evaluated in the emergency department for a possible ALTE. Which of the following statements is true regarding the evaluation of an infants breathing during an ALTE? A. A history of apnea that required cardiopulmonary resuscitation suggests a true life-threatening event. B. An infant who remains awake and alert during an event has likely suffered severe, prolonged hypoxia. C. During central apnea the infant appears to be distressed and struggling to breathe.

D. During obstructive apnea the infant appears to be limp with absence of respiratory effort. E. Normal periodic breathing is often associated with cyanosis. 9. A 4-month-old infant is being evaluated for a possible apneic episode. Which of the following scenarios is most compatible with safe discharge from the emergency department and close out-patient follow-up? A. A stable infant with no pediatrician and a single 16-year-old mother with no private transportation. B. A well-appearing infant with periodic breathing whose detailed history and physical examination do not indicate that a true ALTE has occurred. C. An infant with apnea to whom the parents administered mouth-to-mouth resuscitation. D. An infant with limpness that required vigorous stimulation. E. An infant with prolonged apnea associated with cyanosis. 10. When evaluating an infant for ALTE, which of the following tests is most important to perform in the emergency department prior to hospital admission? A. An electroencephalogram to evaluate for seizures. B. Esophageal pH probe study to evaluate for gastroesophageal reflux. C. Serum electrolytes and glucose to evaluate for acute life-threatening metabolic derangements. D. Sleep study or polysomnogram to determine the type of apnea that is occurring. E. Urine organic acid and serum amino acid profile to evaluate for inborn errors of metabolism.

ANSWERS
1. E. The best choice for the question would be the low birth weight of 2 kg. Other known risk factors for SIDS are young maternal age, lack of prenatal care, prone sleeping position, overheating, and preterm birth. Also, boys are more likely to die from SIDS than girls at a ratio of 60:40. 2. B. Prone sleeping is associated with SIDS and has been shown to increase the time infants spend in a state of reduced spontaneous arousability. The mechanism is not completely understood but it is believed that carbon dioxide becomes trapped around the infants nose and mouth. The American

CHAPTER 6 ALTERED MENTAL STATUS AND COMA

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3.

4.

5.

6.

7.

8.

Academy of Pediatrics advocates a supine sleeping position as a SIDS risk reduction strategy. B. The definition of SIDS is the sudden death of an infant younger than 1 year of age, which remains unexplained after a thorough case investigation, including a complete autopsy, examination of the death scene, and review of the clinical history. Although male infants seem to have greater incidence of SIDS, the sex of the infant is not needed to diagnose SIDS. Also, chronic illness and previous ALTE are not necessary for the diagnosis of SIDS. A. The risk reduction strategies include avoiding maternal smoking in pregnancy, nonprone sleeping, decreasing environmental smoke exposure, maintaining comfortable ambient temperature, providing a safe sleep environment, and fully immunizing the child. A. Age-appropriate therapy should be provided to siblings and family members by the members of your multidisciplinary team. During the initial phase of grieving, medications and substances should not be encouraged. Although counseling the family as a group may be helpful, it does not meet all the needs of each individual family member. E. An ALTE is defined as an episode that is frightening to the observer and is characterized by some combination of apnea (central or obstructive), color change (cyanosis, pallor, erythema, or plethora), marked change in muscle tone (rigidity or limpness), or unexplained choking or gagging. E. The peak incidence is in infants younger than 2 months. Although the true incidence of ALTE is unknown, it is estimated to occur in 0.5% to 6% of all children during the first year of life and is more common in boys and premature infants. Although there is some overlap of epidemiologic risk factors, the exact relationship between ALTE and SIDS is not clear. A. A history of apnea that required vigorous physical stimulation or cardiopulmonary resuscitation is ominous and implies a true life-threatening event. Normal periodic breathing is not associated with skin color changes. Central apnea is characterized by an absence of respiratory effort, whereas during obstructive apnea, the infant typically appears to be struggling to breathe with choking, gasping, or gagging. The infants mental status during the event is also important. An infant who remains awake and alert during an event is unlikely to have suffered prolonged hypoxia or an acute neurologic event such as a seizure.

9. B. An infant may be discharged from the emergency department if a detailed history and physical examination do not indicate that a true ALTE has occurred, provided the infant continues to do well and the parents are comfortable with the situation and capable of observing the infant at home. Examples of such a situation include periodic breathing mistaken for apnea or a minor coughing or gagging episode. Any infant with a history of apnea, pallor, cyanosis, limpness, or unresponsiveness requiring vigorous physical stimulation or cardiopulmonary resuscitation is excluded from outpatient consideration. If there is any question about the nature of the event, the parents ability to care for the infant at home or the adequacy of follow-up, it is best to err on the side of caution and admit the infant for observation and monitoring. 10. C. Of the available choices, the serum electrolytes and glucose are most likely to impact immediate decision making, including determining the severity of the event and stability of the infant. For example, severe hyponatremia, hypoglycemia, or metabolic acidosis may be diagnosed and treated. The studies should also help to determine the disposition of the patient (ie, admit to monitored ward versus intensive care setting). Further studies can be performed after admission including sleep studies, pH probe testing, electroencephalography, and other studies that are beyond the scope of the emergency department. Results of urine organic acid and serum amino acid testing will not return for several days and will not affect emergency department decision making.

ALTERED MENTAL STATUS AND COMA Susan Fuchs

INTRODUCTION
Altered mental status refers to an aberration in a patients level of consciousness. It always implies serious pathology and mandates an aggressive search for the underlying disorder. More precise terminology describes the degree of altered mental status and has important implications for differential diagnosis and management: + Lethargy is a state of reduced wakefulness in which the patient displays disinterest in the environment