ALTE, SIDS, and Diseases of Prematurity

1. ALTE, SIDS, and Diseases of Prematurity • Chris McCrossin, PGY 3 • Thanks to: Kelly Millar, Bella Sztukowski, Ian Wishart, and Jay Green

2. objectives • Understand the underlying etiologies for ALTE’s, what to look for on history and physical exam • Gain an appreciation for what we understand as the natural history and future risk to those presenting with ALTE’s and how it compares and contrasts to SIDS • Know what constitutes an appropriate ED evaluation, work-up, and disposition of ALTE cases • SIDS (definitions, addressing parental questions, appropriate recommendations, identify risk in future siblings) • Diagnoses associated with prematurity: • Bronchopulmonary dysplasia • SAH • NEC

3. Cases • 3 week old infant, chokes on a vitamin D tablet, turns purple. Parents saw her go limp and describe a period of apnea, mom gives 5-6 assisted respirations, baby looks well on arrival in ED • 6 week old male, asleep in his car seat, dad glanced down at him and noticed him to be bluish and not breathing. Parents give assisted respirations while waiting for EMS. Baby still listless on EMS arrival but vitals stable in ED • 3 month old female, referred in to ED because twin sister dies that AM from SIDS. She’s entirely asymptomatic

4. Definitions • ALTE (Apparent Life Threatening Event) • An episode that is frightening to the observer and is characterized by a combination of: • Apnea • Color change • Marked change in muscle tone • Choking • Gagging

5. Definitions • SIDS • Sudden death of an infant < 1 year old • Remains unexplained after investigations including • Complete autopsy • Examination of the death scene • A review of the clinical history

6. SIDS Epidemiology • 95% < 6-8 mo • Peaks 2-4 months • 1% < 1 mo, 2% > 2 years • How effective is the “Back to sleep campaign?” • Before: ~ 7000 deaths/year in the U.S. (1.3/1000) • After: ~ 2300 deaths/year in the U.S. (0.57/1000) Nelson’s

7. SIDS Risk Factors • Modifiable • Cigarette smoking • Sleep Environment • Drug and Ethanol Use • Pregnancy Related • Nutritional • Non-Modifiable • Socioeconomic status • Prematurity • Genetic Nelson’s

8. Recurrent SIDS in a Sibling Nelson’s

9. ALTE Epidemiology • What % of patients who die from SIDS have had a previous ALTE? • 7-8% • % of ALTE’s who subsequently die unexpectedly? • 10% in ALTE’s occuring during sleep + require CPR • What time do ALTE’s tend to occur? • During Waking hours • At what age do children with ALTE’s present? • 2 months on average • Risk Factors for ALTE? • Maternal smoking, single parents, nocturnal diaphoresis • What % are eventually considered idiopathic? • 50% • What is the recurrence rate for severe ALTE? • Up to 68% in some studies!

10. ALTE Outcomes • Retrospective cohort • N = 471 • Urban setting • Excluded patients with probable etiologies at time of first presentation • 5 year ave follow-up (range: 2-7 years) Pediatrics 2008; 122(1): 125-31

11. ALTE vs SIDS • Common Risk Factor: Smoking during pregnancy • Differences: Most ALTE’s occur in peds < 2 months, compared with a 2-4 month peak for SIDS • 52% of ALTE’s occur during wakefulness • SIDS occurs during sleep • No change in ALTE incidence with back to sleep program J Pediatr 2009; 154(3): 317-19

12. WhIch ALTE’s are At Higher Risk? • Infants born prematurely • Infants < 43 weeks post-conceptional age • ALTE’s that occur with symptoms of URTI’s J Pediatr 2009; 154(3): 332-37

13. Critical Diagnosis • Bronchiolitis (12%) • Seizures (8%) • Sepsis (7%) • ICH (3%) • Meningitis (2%) • Dehydration (2%) • Anemia (2%) Ann Emerg Med 2004: 43:711-17

14. Differential Diagnosis

15. Apnea • Most common sign associated with ALTE • Apnea can be normal or pathologic • Various Terms: • Apnea of infancy - unexplained pathologic apneic event occurs for the first time in an infant older than 37 weeks (e.g. unexplained ALTE) • Apnea of prematurity - occurs prior to 37 weeks at which point it resolves • Periodic breathing - three or more resp pauses of greater than 3 seconds duration with less than 20 seconds of respiration between pauses. Common and physiologic in preterm infants. considered pathologic if associated with cardiorespiratory instability

16. Pathologic Apnea • no air movement for > 20 seconds* OR • any period of no air movement associated with physiologic compromise (bradycardia, pallor, hypotonia, cyanosis) • Central • No drive from resp centers, neuromuscular insufficiency • e.g. head trauma, Ondine’s curse, apnea of prematurity • Obstructive • Breathing through an occluded airway • e.g. masses, adenotonsillar hypertrophy, OSA, foreign body, laryngomalacia, intralumial cysts • Mixed • Two conditions; e.g. premature infant with central apnea and nasal congestion from URTI, • One condition with features of both; e.g. GERD

17. Infections • One retrospective analysis suggested that SBI’s occurred in close to 3% of well appearing infants presenting with ALTE (risk is higher for premature infants) • Newborns may show little in terms of signs/symptoms of serious bacterial infections so go looking for it! Ped Emerg Care 2009; 25(1):19-25

18. GERD • One of the most common diagnoses made in the setting of ALTE • Unclear if GERD is purely and associative, responsive, or triggering factor in ALTE’s Ped Clin N Am 2005; 52(4): 1127-46/ J Pediatr 2000; 137(3): 298-300

19. History • Identify central apnea vs obstructive apnea if possible (respiratory pause vs choking, gagging, or gasping) • Try to recreate a technicolor image in your mind what the caregiver saw, what the infant was doing before the event, what happened immediately after, and the infant’s position at the time • Read the EMS report: What did the infant look like when they arrived?

20. History • A good feeding history will identify cardiac causes, TEF, and swallowing dysfunction • Ask about snoring, look for obstructions, query about foreign body aspiration • Family history: ask about siblings

21. Physical Exam • Good CVS, Resp, Abdo, and Neuro Exams • Look for signs of trauma • Don’t miss the fundoscopic exam

22. Investigations • Basic screening tests • CBC, CH6, ABG, Ammonia level, CXR, ECG • Consider septic work-up • Consider Tox screen • Targeted testing based on history and symptoms

23. Cases • 3 week old infant, chokes on a vitamin D tablet, turns purple. Parents saw her go limp and describe a period of apnea, mom gives 5-6 assisted respirations, baby looks well on arrival in ED • 6 week old male, asleep in his car seat, dad glanced down at him and noticed him to be bluish and not breathing. Parents give assisted respirations while waiting for EMS. Baby still listless on EMS arrival but vitals stable in ED • 3 month old female, referred in to ED because twin sister dies that AM from SIDS. She’s entirely asymptomatic

24. Disposition • Choking episodes • Clear choking episodes are not usually life-threatening • Assuming no hx of chronic feeding problems monitor for a few hours, ensure a normal feed occurs, then discharge home • ALTE • If true apnea or significant resuscitation in field most will admit for monitoring (PICU) • Consider septic work-up

25. Sids in the ED • Twins of SIDS deaths are admitted for monitoring • Rare that a SIDS death will be brought to ED in active resuscitation • Labs in this case may be helpful for prevention of future siblings

26. Minimizing SIDS • Canadian Pediatric Society Recommendations: • Babies should sleep on their back for the first year of life (or until they can turn over on their own) • Firm surface • Soft material out of baby’s sleep environment • Make sure baby is not too warm • Keep baby away from cigarette smoke • No bed sharing • Risk of SIDS with bed sharing is increased if parent has had alcohol, taken any drug with sedating side effects

27. Take-Home Points • SIDS and ALTE’s are not the same • ALTE’s are primarily a result of Apnea • Most children with ALTE’s do fine but severe ALTE’s are at higher risk • Although ALTE’s are idiopathic 50% of the time, remember your differential • Think about Sepsis • Think about child abuse • The most important modifiable risk factor for SIDS that we know about is ensuring a safe sleep environment (back to sleep)

28. You thought you were having a bad day...

29. ...and then it got worse

30. Prematurity

31. Prematurity • Definitions • Late preterm - GA greater than 34, less than 37 weeks • Very preterm - GA less than 32 weeks • Low birth weight - Less than 2500g • Very low birth weight - Less than 1500g • Extremely low birth weight - Less than 1000g • Complications with prematurity • RDS 44% • PDA 30% • BPD 20% • Late onset sepsis 20% • IVH 12% • NEC 7% • Periventricular leukomalacia 5% www.uptodate.com

32. CASE • 16 day old infant presents to the ED with 2-3 days of vomiting. Had been doing well with feedings prior to that. Parents now feel that the vomiting is becoming increasingly forceful • Vomiting becoming dark brown/maroon in color • Last few stools have become darker than usual • No BM, No wet diapers in past 8 hours • No fevers, no sick contacts • Went to WIC yesterday, dx with “overfeeding” • PMHx • born @ 34 weeks gestational age • 1 week in NICU requiring phototherapy for hyperbilirubinemia • No pulmonary or cardiovascular issues

33. Necrotizing enterocolitis

34. NEC • Etiology • Unknown, multifactorial (ischemic/infectious insults/feeding related) • Spectrum of presentation • I - Early or suspected NEC based on feeding intolerance, vomiting, or ileus • II - NEC proven on AXR with abdominal dilation and pneumatosis intestinalis • III - Advanced disease with perforation, metabolic acidosis, DIC, shock

35. Who’s at Risk? • Prematurity • Low birth-weight • Timing of presentations: • Term infants less than 1 week old • Within first 3 weeks of life in infants born at 29-32 weeks GA • Between 2-4 weeks of life in infants born at 24-28 weeks GA

36. Clinical Presentation • May appear well if early or may present in a profound state of shock • Non-specific signs/symptoms • feeding intolerance • blood in vomit or stool • apnea • respiratory distress • abdominal distention

37. Investigations • Labs are non specific but serve as markers of severe disease and follow trends • Thrombocytopenia • Neutropenia • Coagulopathy • CRP • Lactate • Blood Gases

38. X-Ray Findings • Dilated loops of bowel • Air fluid levels • Free air • Pneumatosis intestinalis • Portal venous gas

39. X-Ray Findings

40. Management

41. Who Needs Surgery? • Only hard indication is bowel perforation • “Soft” indications: • Radiological signs • persistent fixed loop • portal venous gas • ascites • Lab features • severe acidosis

42. Bronchopulmoary Dysplasia

43. Definition • Often used as a “catchall” term to describe chronic lung disease in the neonatal population • Clinical definition: requiring O2 @ 36 weeks postmenstrual age • Defining who needs supplemental O2 therapy is not black and white and practice varies widely J Perinat 2008; 28(12): 837-840

44. Pathogenesis May have severe or mild respiratory disease Initially vented with low pressures and FiO2 Honeymoon Period After weeks may show progressive deterioration in lung function BPD Sem Neonat 2003; 8(1): 63-71

45. Who is at Risk? • Most infants with BPD are born prematurely • 75% weigh less than 1 kg at birth • 20% of ventilated newborns Lancet 2006; 367(9520): 1421-31

46. Natural History • Most infants with BPD will show progressive improvement in pulmonary function and wean from supplemental O2 as their lungs grow and remodel • 50% of all infants with BPD will need to be readmitted to hospital during early childhood for respiratory distress often exacerbated by RSV • High rate of admission falls during the second year of life • Strong association between BPD and growth retardation and cognitive delays Lancet 2006; 367(9520): 1421-31

47. Radiographic Findings • Hyperinflation • Non-homogeneity of pulmonary tissues • Densities extending to the periphery • Diffuse haziness Sem Neonat 2003; 8(1): 63-71

48. Management • Treat as per asthmatic pathway • Ventolin • Atrovent • Dexamethasone www.uptodate.com

49. Intraventricular Hemorrhage

50. intraventricular hemorrhage • Bleeding originates from the germinal matrix • Occurs most frequently in infants born before 32 weeks or less than 1500g • Virtually all IVH occurs in the first 5 postnatal days