Dominic Lucia M.D Assistant Professor of Emergency Medicine Texas A&M Health Science Center Medical Director Emergency Medicine McClane Children’s Hospital Scott and White

 Define ALTE  ALTE Relationship to SIDS  What could this be?  Work-up  Disposition  Take home points  Discussion

 Nurse “skeptically” hands you a chart.  CC: 3 month old coughing spell  VS: p115 rr30 O2sat 98% ra t 37.1  Mother states that 30 minutes ago she had put child down after his evening feeding; she heard a noise (coughing?) and looked at baby and he “didn’t look right”….

 An event that is frightening to the observer accompanied by a combination of color change(cyanosis, pallor, or plethora), apnea, a change in muscle tone (limp or stiff), choking or gagging  …(In some cases the observer fears the infant has died or would have died without significant intervention…)  Many times child is well appearing at time of presentation

 Symptom…not a diagnosis.  Think of it as a warning of sorts…  Adult equivalent: TIA Ambiguity… Concern for future event What will they do in the hospital with this? But with infants….we are more careful!

 Generally talking about under 1 year of age  Peak incidence occurs between 1w-2m  More common in preemies  Male 2:1 Females Am Fam Physician.Am Fam Physician Jun 15;71(12): Evaluation and management of apparent life- threatening events in children. Hall KL, Zalman B.Hall KLZalman B Rocker JA, Israel J: emedicine, Pediatrics, apnea

 The parent is the historian.  Variety of crazy stories out there.  It’s time to get SPECIFIC  History (and Physical) is VERY IMPORTANT  Be firm in explaining how important the details of the situation are to your diagnostic and treatment decisions.

 ~5% of children who die of SIDs have had an ALTE  Causal relationship has not been established ◦ ALTE- heterogenous group of problems ◦ SIDs and ALTE event times vary ◦ Interventions have not decreased incidence of ALTE ◦ Risk factors vary

The sudden and unexpected death of an apparently well, or almost well infant (child< 1 year of age) in which death remains unexplained after the performance of an adequate postmortem investigation including: 1) an autopsy, 2) investigation of the scene and circumstances of the death, and 3) exploration of the medical history of the infant and the family. Pediatric Pathology 1991

 Season: winter  Socioeconomic status: low  Ethnicity: cultural differences  Race: higher in African Americans and Native Americans  Male predominance

 Infant factors and increased risk ◦ low birth weight ◦ short gestational age ◦ IUGR  Maternal factors and increased risk ◦ tobacco use pre and post delivery ◦ opiate and cocaine use ◦ young age ◦ high parity ◦ multiple gestation

 Other factors that increase risk ◦ thermal stress ◦ co-sleeping combined with other factors ◦ soft surfaces ◦ prone position ◦ history of SIDS in prior sibling

Exogenous Stressor Vulnerable infant Development of Homeostatic Control Triple Risk Model for SIDS

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 Duration  Color changes  Respiratory effort  Muscle tone  Intervention required  Activity of infant prior to event  Relationship to feeding  Presence of choking, gasping, emesis  Rhythmic movements, eye movements  Nasal congestion  Fever  Post event appearance and time to norm

 Gastroenterologic (33%)  Idiopathic Apnea of Infancy (23%)  Neurologic (15%)  Respiratory (11%)  Otololaryngologic (4%)  Cardiovascular (1%)  Metabolic/Endocrine  Infectious  Other DeWolfe CC:Apparent Life Threatening Event: a review Pediatr Clin North Am.Pediatr Clin North Am Aug;52(4): , ix.

 Gastroesophageal reflux disease  Gastroenteritis  Esophageal dysfunction  Surgical abdomen  Dysphagias

Pediatr Emerg Care.Pediatr Emerg Care Jan;28(1):  Large children's hospital of infants aged 1 year old and younger hospitalized from January 1, 2004, to March 1, 2007…Three hundred thirteen infants met inclusion. Mean age was 2.1 months; mean length of stay was 2.5 days. A discharge diagnosis of GERD was most common (n = 154, 49%)…  It is important to recognize that gastroesophageal impairments such as GERD or CGV could be responsible for many incidences of ALTE in infancy...including gastroesophageal evaluations in all infants with ALTE…

 Seizure  Central apnea/hypoventilation  Meningitis/encephalitis  Hydrocephalus  Congenital brain malformation  Increased ICP: hydrocephalus, tumor  Neuromuscular disorders  Vasovagal reaction  Head trauma

Autonomic Neuroscience 2010 Aug 25;156(1-2):90-5. Epub 2010 Apr 18.  Abstract  BACKGROUND:  Apparent life-threatening events (ALTE) and/or prolonged apnoea have been well- documented during respiratory syncytial virus (RSV) infection in infants less than 2 months of age but fundamental mechanisms remain unclear. The possibility of a central origin for the development of severe cardiac and respiratory events encouraged us, to explore the autonomic nervous system (ANS) profile of infected infants, since ANS activity may contribute to the constellation of symptoms observed during severe forms of RSV bronchiolitis.  METHODS:  Eight infants (2 preterm and 6 full-term) less than 2 months of age and presenting with severe and apnoeic forms of RSV infection were evaluated using non-invasive electrophysiological monitoring obtained simultaneously for approximately 2 consecutive hours, including a quiet sleep period. Eight control subjects, paired for gestational and postnatal age, were also evaluated. ANS status was monitored using electrocardiogram recordings and quantified through a frequency-domain analysis of heart rate variability (HRV). This included sympathetic (VLF and LF) and parasympathetic (HF) indices as well as a measure of baroreflex sensitivity (BRS) obtained using non-invasive continuous arterial pressure.  RESULTS:  Regardless of gestational and postnatal age, heart rate variability components (Ptot, VLF, LF, and HF) and baroreflex components (alpha LF, alpha HF and sBR) were found to be significantly lower in the RSV-infected group than in the control group (p<0.05).  CONCLUSION:  RSV infection in neonates is associated with profound central autonomic dysfunction. The potentially fatal consequence stresses the importance of maintaining prolonged cardiopulmonary monitoring.

 Respiratory syncytial virus  Pertussis  Congenital malformation  Laryngospasm (choking/GERD)  Aspiration  Respiratory tract infection (obligate nasal breathers)  Reactive airway disease  Foreign body

 Laryngomalacia  Choanal atresia  Laryngeal cleft  Tracheoesophageal fistula  Subglottic and/or laryngeal stenosis  Obstructive sleep apnea

 Anemia  Congenital heart disease  Cardiomyopathy  Vascular rings and slings  Cardiac arrhythmias/prolonged QT syndrome; WPW  Myocarditis

 Hypoglycemia  Electrolyte disturbance  Inborn error of metabolism

 Sepsis  Urinary tract infection

 Emerg Infect Dis Sep;15(9): Emerg Infect Dis.  Role of rhinovirus C in apparently life-threatening events in infants, Spain.  Calvo C, Garcia ML, Pozo F, Reyes N, Pérez-Breña P, Casas I. Calvo CGarcia MLPozo FReyes NPérez-Breña PCasas I  Source  Hospital Severo Ochoa, Leganes, Madrid, Spain.  Abstract  To assess whether infants hospitalized after an apparently life-threatening event had an associated respiratory virus infection, we analyzed nasopharyngeal aspirates from 16 patients. Nine of 11 infants with positive virus results were infected by rhinoviruses. We detected the new genogroup of rhinovirus C in 6 aspirates.

 Child maltreatment syndrome  Shaken baby syndrome  Breath-holding spell  Choking  Drug or toxin reaction  Dehydration  Unintentional smothering  Periodic breathing  Munchhausen-by-proxy syndrome

 Children < 24mo with an ALTE btwn 3/97-2/99  opthalmologic exam as well as, lab tests  Results: ◦ 128 with ALTE ◦ mean age 2.1 mo, 44.5% boys; 67.2% caucasian ◦ 26/128: 20.3% hx of ALTE ◦ 4/117: 3.4% fam hx of ALTE ◦ 15/117: 12.8% fam hx of SIDs  dilated fundoscopic exam done in 73/128: 1 (1.4%) ret hemorrhage  4 children including above underwent CPS investigation  3/128 (2.3%) were found to have been abused Pediatrics September 2002

 Periodic Breathing  Breath holding spells (older)  Standard reflux  *Be sure your history physical and subsequent documentation firmly support your diagnosis

 Never trust a neonate…  “Well appearing”  Social Smile…?  Feeding issues…  Significant episode Under 6 weeks, consider Full sepsis workup (even if afebrile…)

 Monitoring while in the department  Being very specific to caretaker that if the event or similar symptoms occur while in the ED to immediately notify the staff.  The child should be visually re-checked in the department by a physician at least every 30 minutes (document)  BVM and other resuscitative equipment should be readily available.

 Younger…more aggressive  Punky…listless…full court press  Let the history and physical exam guide your work-up.

 Blood glucose*  CBC with diff  CMP  Mg, Phos, Ca  Blood Culture  UA  Urine Culture  UDS  VBG  RSV  Respiratory Virus Panel

 Chest X-Ray  EKG  Abuse? CT, skeletal survey, UDS

 All children who have suffered an ALTE should be transported for evaluation by a physician (EMS)  Admission for observation at the least ◦ Anyone with cyanosis ◦ Apnea ◦ Loss of tone ◦ Required CPR  Home only if clearly NOT an ALTE

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 Benefit  Pushback  What can an inpatient stay offer?

 Prolonged monitoring  Culture monitoring  Sleep studies  pH probe testing  EEG  Parental calming…

More scholarly reading….

Journal of Pediatrics Mar;152(3): Epub 2007 Nov 5. Apparent life-threatening events and sudden infant death syndrome: comparison of risk factors. Esani NEsani N, Hodgman JE, Ehsani N, Hoppenbrouwers T.Hodgman JEEhsani NHoppenbrouwers T  OBJECTIVE:  To compare the risk factors of 153 cases of apparent life-threatening event (ALTE) enrolled in the multicenter Collaborative Home Infant Monitoring Evaluation (CHIME) from 1994 to 1998 with the published risk factors for sudden infant death syndrome (SIDS).  STUDY DESIGN:  Trained CHIME interviewers gathered histories of infants with ALTE who met the criteria. The following risk factors were analyzed: male predominance, gestational age, low birth weight, very low birth weight, incidence of small for gestational age (SGA), age at the event, multiparity, maternal age, and smoking. Population-based SIDS studies with >100 deaths, focusing on 1 or more pertinent risk factors and carried out during the decade in which CHIME data were collected, were chosen for comparison.  RESULTS:  One of the 153 infants with ALTE in this study died during follow-up (0.6%). CHIME ALTE differed significantly from SIDS in 4 respects: fewer infants with low birth weight and SGA at birth, fewer teenage pregnancies, and a younger infant age at ALTE.  CONCLUSIONS:  Although a number of risk factors for ALTE are similar to those for SIDS, the differences warrant a separate focus on ALTE beyond that on SIDS

Curr Opin Pediatr.Curr Opin Pediatr Jun;19(3):  PURPOSE OF REVIEW:  Apparent life-threatening events are an ongoing diagnostic dilemma for clinicians. Since most apparent life-threatening event episodes occur in infants under 6 months of age, they can generate considerable anxiety in parents and providers. This review will discuss issues to consider in the evaluation of infants after an apparent life-threatening event. To ensure proper management, a systematic approach should be taken to attempt to determine the cause of the event.  RECENT FINDINGS:  More recent literature suggests that infants with apparent life-threatening events frequently present without signs or symptoms of illness. Obtaining a careful history and physical examination is essential in determining the cause of the event. In this article, we will review the most current literature and discuss the American Academy of Pediatrics new recommendations on sudden infant death syndrome prevention.  SUMMARY:  After a careful review of the literature, prone sleeping is one of the biggest risk factors for sudden infant death syndrome. The association between apparent life-threatening events and sudden infant death syndrome remains to be explored further, but current evidence suggests minimal risk after an apparent life-threatening event episode. This article will help clinicians prepare for this difficult challenge by providing up-to-date information and identifying problems to be addressed in future research.

Arch Dis Child.Arch Dis Child Nov;89(11): Causes of apparent life threatening events in infants: a systematic review. McGovern MCMcGovern MC, Smith MBSmith MB  AIMS:  To determine the most likely diagnoses when infants first present with an apparent life threatening event (ALTE).  METHODS:  Medline ( ), Embase ( ), and Cinahl ( ) were searched. Primary authors and content experts were contacted to identify further studies. Bibliographies from studies, reviews, and textbooks were searched. Foreign language studies were translated. Articles were included if the ALTE was clearly defined and if the evaluation recorded was from the initial contact. Case reports and studies focusing on single conditions or non-clinical data were excluded.  RESULTS:  From an initial 2912 papers, eight studies involving 643 infants (aged 0-13 months) were included. All studies were non-randomised and methodological quality varied. All diagnoses were made after evaluation in hospital but investigation protocols varied widely. There were 728 diagnoses assigned overall. Some infants had multiple diagnoses. The most common diagnoses were gastro-oesophageal reflux (n = 227), seizure (n = 83), lower respiratory tract infection (n = 58), and "unknown" (n = 169). Five deaths were noted in total.  CONCLUSIONS:  There is a wide range of diagnoses reported after evaluation of an ALTE. Differing management protocols contributed to variations in the frequency of the diagnoses. The development and validation of an evidence based management plan may contribute to the care of this common condition.

 Know definition of ALTE  H&P very important  Be careful on the non-ALTES documentation  Family history of SIDS?  Work-up should be guided by H&P  When in doubt…admission.

 Natalie Lane MD  Marisa, Derian, Malaya, and Lillian