Apparent Life Threatening Event–Brief Resolved Unexplained Event




Apparent life-threatening event (ALTE) is a term used to describe an acute, unexpected episode that consists of a change in an infant’s breathing, appearance, or behavior. It is a clinical description, rather than a specific diagnosis, and represents a wide variety of presentations with diverse underlying pathology.


(See Nelson Textbook of Pediatrics , p. 2004.)


Definition


The current definition of ALTE was established at the 1986 National Institutes of Health Consensus Development Conference on Infantile Apnea and Home Monitoring ( Table 5.1 ). ALTE was defined as “an episode that is frightening to the observer, that is characterized by some combination of apnea (central or occasionally obstructive), color change (usually cyanotic or pallid, but occasionally erythematous or plethoric), marked change in muscle tone (usually marked limpness), choking, or gagging.”



TABLE 5.1

Definitions from the 1986 National Institutes of Health Consensus Panel on Infantile Apnea and Home Monitoring
























Apparent life-threatening event (ALTE) An episode that is frightening to the observer and that is characterized by some combination of apnea (central or occasionally obstructive), color change (usually cyanotic or pallid but occasionally erythematous or plethoric), marked change in muscle tone (usually marked limpness), choking, or gagging
Apnea Cessation of respiratory airflow which can be central (i.e., no respiratory effort), obstructive (usually due to upper airway obstruction), or mixed
Pathologic apnea Apnea that is prolonged (20 sec) or associated with cyanosis; abrupt, marked pallor or hypotonia; or bradycardia
Periodic breathing A breathing pattern in which there are three or more respiratory pauses of >3 sec in duration with <20 sec of respiration between pauses
Apnea of prematurity (AOP) Periodic breathing with pathologic apnea in a premature infant that usually ceases by 37 wk of gestation but occasionally persists to several weeks past term
Apnea of infancy (AOI) An unexplained episode of cessation of breathing for 20 sec or longer, or a shorter respiratory pause associated with bradycardia, cyanosis, pallor, and/or marked hypotonia in infants who are >37 wk of gestational age at onset of pathologic apnea
Sudden infant death syndrome (SIDS) The sudden death of any infant or young child, which is unexplained by history and in which a thorough postmortem examination fails to demonstrate an adequate explanation of cause of death


The term ALTE was in part established to replace previously used labels, including “near-miss SIDS” or “aborted crib death,” which inappropriately suggested a clear association between ALTEs and sudden infant death syndrome (SIDS). SIDS is defined as the sudden death of any infant or young child, which is unexplained by history and in which a thorough postmortem examination fails to demonstrate an adequate explanation of cause of death. Studies have failed to establish a clear association between ALTEs and SIDS. Although there are some overlapping risk factors between SIDS and ALTEs, they are separate entities. The vast majority of SIDS victims do not have a preceding ALTE. The American Academy of Pediatrics Task Force on SIDS is clear in its stance that there is no evidence that an ALTE is a precursor to SIDS.


The subjectivity and vagueness of the current definition has made it difficult to standardize the care of these patients. In 2016 the American Academy of Pediatrics (AAP) released a clinical practice guideline related to the care of these patients. This guideline includes a change in terminology to BRUE (brief resolved unexplained event), which is defined as an event lasting <1 min in an infant under one year of age that is associated with at least one of the following: cyanosis or pallor; absent, decreased, or irregular breathing; marked change in muscle tone (hypertonia or hypotonia); altered level of responsiveness in a patient who at the time of examination is otherwise well-appearing and back to baseline, and, on evaluation, has no condition that could explain the event. The guideline recommends limited interventions for patients designated as being at low risk for recurrence. Low-risk patients include those having their first event who are >60 days old, ≥32 weeks’ gestational age, ≥45 weeks postconceptional age, had an event lasting <1 minute and did not require cardiopulmonary resuscitation, and who have no concerning historical or physical exam findings.




Epidemiology


The exact incidence of ALTEs is unknown because the definition of an ALTE is subjective, and not all children with ALTEs present for evaluation. Reported figures may underestimate the true incidence of patients presenting with ALTEs since studies may not include those cases where the underlying cause is ultimately identified. The incidence is estimated to be between 0.46 and 2.46 per 1000 live births, accounting for 0.6-1% of all emergency department visits by patients younger than 1 year and 2% of pediatric hospitalizations. Most occur in infants less than 1 year of age, with a peak incidence between 1 week and 3 months. Occurrence is equal between males and females. The mortality rates reported to be associated with ALTEs vary widely depending on the definition used and the population studied. Due to the diversity of the potential underlying etiology of an ALTE, clinicians should be cautious in the application of these rates to the ALTE population as a whole ( Table 5.2 ).



TABLE 5.2

Conditions That Can Cause Apparent Life-Threatening Events or Sudden Unexpected Infant Death

























































































































Central Nervous System
Arteriovenous malformation
Subdural hematoma
Seizures
Congenital central hypoventilation *
Neuromuscular disorders (Spinomuscular atrophy)
Chiari crisis
Leigh syndrome *
Cardiac
Subendocardial fibroelastosis *
Aortic stenosis
Anomalous coronary artery
Myocarditis
Cardiomyopathy *
Arrhythmias (prolonged Q-T syndrome, Wolff-Parkinson-White syndrome, and congenital heart block) *
Pulmonary
Nasal obstruction
Pulmonary hypertension
Vocal cord paralysis
Aspiration
Laryngotracheal obstructive diseases
Gastrointestinal
Diarrhea and/or dehydration
Gastroesophageal reflux
Volvulus
Endocrine–Metabolic
Congenital adrenal hyperplasia *
Malignant hyperpyrexia *
Long- or medium-chain acyl coenzyme A deficiency *
Hyperammonemias (urea cycle enzyme deficiencies) *
Glutaric aciduria *
Carnitine deficiency (systemic or secondary) *
Glycogen storage disease type I *
Maple syrup urine disease *
Congenital lactic acidosis *
Biotinidase deficiency *
Infection
Sepsis
Meningitis
Encephalitis
Brain abscess
Pyelonephritis
Bronchiolitis (respiratory syncytial virus)
Infant botulism
Pertussis
Trauma
Child abuse *
Accidental or intentional suffocation
Physical trauma
Factitious syndrome (formerly Munchausen syndrome) by proxy *
Poisoning (Intentional or Unintentional)
Boric acid
Carbon monoxide
Salicylates
Barbiturates
Ipecac
Cocaine
Insulin
Others

Modified from Hunt CE, Hauck FR. Sudden infant death syndrome. In: Nelson Textbook of Pediatrics , 20th ed. Philadelphia: Elsevier; 2016:1999.

* May be seen with recurrences in siblings.





Etiology


Because ALTE is a descriptive category based on broad symptomatology, the differential diagnosis is large. Comorbid conditions are frequently identified, but it can be challenging to identify true causation. Thus caution must be used in implicating a specific diagnosis as the true cause of an ALTE. A suspected diagnosis is found in approximately 50% of ALTEs. These diagnoses encompass a wide range of etiologies and systems.


The most commonly cited diagnoses include gastroesophageal reflux (GER), seizures, and lower respiratory tract infections. However, numerous less common but potentially dangerous and/or treatable conditions can also present as an ALTE (see Table 5.2 ). These need to be carefully considered in order to provide prompt life saving or outcome-altering treatment. A thorough and thoughtful history and physical examination is extremely important in the evaluation of a patient with an ALTE, as it provides essential clues to help narrow the differential. It is often helpful to consider the differential diagnosis by a systems-based approach, considering both common and rare but concerning diagnoses in each category. Key systems-based historical and physical examination findings may help discriminate among possible etiologies ( Table 5.3 ).



TABLE 5.3

System-Based Approach to ALTEs/BRUEs




















































Diagnostic Categories Common and/or Concerning Causes to Consider Suggestive Historical Findings Suggestive Physical Examination Findings Testing to Consider
Gastrointestinal GER
Intussusception
Volvulus
Swallowing abnormalities
Coughing, vomiting, choking, gasping
Feeding difficulties
Recent preceding feed
Irritability following feeds
Milk in mouth/nose
Bilious emesis
Pulling legs to chest
Bloody/mucousy stool
Lethargy following event
Gastric contents in the nose and mouth
Abdominal distention
Abdominal tenderness
Upper GI to assess for anatomic anomalies
Swallow evaluation
Abdominal ultrasound
pH probe
Infectious Upper and lower respiratory tract infection (RSV, pertussis, pneumonia)
Bacteremia
Meningitis
Urinary tract infection
Preceding URI symptoms
Multiple events on the day of presentation
Sick exposures
Foul-smelling urine
Fever/hypothermia
Lethargy
Ill appearance
Coryza
Cough
Wheeze
Tachypnea
NP swab for RSV, pertussis
Chest radiograph
CBC and Blood culture
Cerebrospinal fluid analysis and culture
Urinalysis and culture
Neurologic Seizures
Breath holding spells
Congenital central hypoventilation syndrome
Neuromuscular disorders
Congenital malformations of the brain and brainstem
Malignancy
Intracranial hemorrhage
Multiple events
Loss of consciousness
Change in tone
Abnormal muscular movements
Eye deviation
Preceding triggers
Papilledema
Abnormal muscular movements
Hypertonicity or flaccidity
Abnormal reflexes
Micro- or macrocephaly
Dysmorphic features
EEG
Neuroimaging
Respiratory/
ENT
Apnea of prematurity
Apnea of infancy
Periodic breathing
Airway anomaly
Aspiration
Foreign body
Obstructive sleep apnea
Prematurity
Foreign body
Aspiration
Noisy breathing
Wheezing
Stridor
Crackles
Rhonchi
Tachypnea
Chest radiograph
Neck radiograph
Laryngoscopy
Bronchoscopy
Esophagoscopy
Polysomnography
Child maltreatment Non-accidental head trauma
Smothering
Poisoning
Factitious syndrome (formerly Munchausen syndrome) by proxy
Multiple events
Unexplained vomiting or irritability
Recurrent ALTEs
Historical discrepancies
Family history of unexplained death, SIDS, or ALTEs
Single witness of event
Delay in seeking care
Bruising (especially in a non-mobile child)
Ear trauma, hemotympanum
Acute abdomen
Painful extremities
Oral bleeding/trauma
Frenulum tears
Unexplained irritability
Retinal hemorrhages
Depressed mental status
Skeletal survey
Computed tomography of the head
Dilated funduscopic examination
Toxicology screen
Cardiac Dysrhythmia (prolonged QT syndrome, Wolff-Parkinson-White syndrome)
Cardiomyopathy
Congenital heart disease
Myocarditis
Abrupt onset
Feeding difficulties
Failure to thrive
Diaphoresis
Prematurity
Abnormal heart rate/rhythm
Murmur
Decreased femoral pulses
4 extremity blood pressure
Pre- and post-ductal oxygen saturation measurements
ECG
Echocardiogram
Serum electrolytes, calcium, magnesium
Metabolic/genetic Inborn errors of metabolism
Electrolyte abnormalities
Genetic syndromes including those with craniofacial malformations
Severe initial event
Multiple events
Event associated with period of stress or fasting
Developmental delay
Associated anomalies
Failure to thrive
Severe/frequent illnesses
Family history of ALTE, consanguinity, seizure disorder, or SIDS
Dysmorphic features
Microcephaly
Hepatomegaly
Serum electrolytes; glucose, calcium, and magnesium levels
Lactate
Ammonia
Pyruvate
Urine organic and serum amino acids
Newborn screen

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Apr 4, 2019 | Posted by in PEDIATRICS | Comments Off on Apparent Life Threatening Event–Brief Resolved Unexplained Event

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