Neonatal Emergencies

1. Neonatal Emergencies Lazaro Lezcano, MD Director, Division of Neonatology August 18, 2009

2. Neonatal Emergencies • Neonates often present with non-specific or a history of symptoms that may or may not be benign • In order to recognize which neonates will require life-saving interventions, clinicians need to remain current on these life-threatening illnesses and their management

3. The Misfits Movie

4. Neonatal Emergencies “THE MISFITS” • T- Trauma (accidental & nonaccidental) • H- Heart Disease/Hypovolemia/Hypoxia • E- Endocrine(congenital adrenal hyperplasia, thyrotoxicosis) • M- Metabolic(electrolyte imbalance) • I- Inborn Errors of Metabolism:metabolic emergencies • S- Sepsis (meningitis, pneumonia, UTI) • F- Formula mishaps (under or overdilution) • I- Intestinal catastrophes (volvulus, intususception, NEC) • T- Toxins/poisons • S- Seizures

5. Trauma(accidental & non-accidental) • May be a difficult process • Non-accidental subtle historical findings and no physical exam findings • Presenting symptoms may be nonspecific • Early diagnosis of an occult head injury may prevent significant long-term morbidity • An ALTE is often an unrecognized presenting symptom of abusive head injuries

6. Trauma(accidental & non-accidental) • Infants with ALTE w/o an immediate obvious cause should be evaluated for head trauma with neuroimaging • CT scan, HUS or MRI • Skull x-rays may not be helpful- significant head injury w/o skull fracture • Consider neuroimaging in any non-accidental injury for other skeletal injuries regardless of physical examination of the head

7. Trauma(accidental & non-accidental) • 37% of abused children < 2 y/o had an occult traumatic injury • In addition, the ophthalmologic evaluation did not demonstrate retinal hemorrhages in most of the patients Pediatrics 6/2003 CHOP 74%  No retinal hemorrhages

8. Trauma(accidental & non-accidental) • Management: • Evaluation and stabilization of the ABC’s • Bedside glucose evaluation • Appropriate temperature regulation • If bruising or known intracranial bleed: • CBC • Platelet count • PT/PTT • Neuroimaging after stabilization

9. Trauma(accidental & non-accidental) • Admit the patient • Report injury to appropriate state department for abuse • Skeletal survey • Ophthalmologic exam

10. Heart Disease and HypoxiaCyanotic Heart Disease • Cyanosis requires immediate attention and evaluation • Differential diagnosis: • Respiratory causes • Infectious causes • CNS abnormalities • Toxins • Cyanotic heart disease

11. Heart Disease and HypoxiaCyanotic Heart Disease • Terrible T’s: • Transposition of the great arteries (TGA) • Tetralogy of Fallot (TOF) • Tricuspid atresia (TA) • Total anomalous pulmonary venous return (TAPVR) • Truncus arteriosus (TA)

12. Heart Disease and HypoxiaCyanotic Heart Disease • May not be detected in the WBN • Adequately oxygenated blood  PDA  systemic circulation • PDA functionally closes in the first 10-14 hrs of life • Several factors can delay its closure • Prematurity • Respiratory distress • Acidosis • Hypoxia

13. Heart Disease and HypoxiaCyanotic Heart Disease • PDA is anatomically closed by 2 weeks of age, contributing to a delayed detection of cyanotic heart disease • 100% FiO2: • Non-cardiac disease • At least 10% increase in O2 saturation • Cyanotic heart disease • Minimal change in O2 saturation

14. Heart Disease and HypoxiaCyanotic Heart Disease • Hyperoxia test: • Initial ABG on R/A • Repeat ABG after 10-20 minutes of 100% O2 • Cyanotic heart disease PaO2 will not increase significantly • If PaO2 rises above 150 mm Hg, cardiac disease can generally be excluded • Failure of PaO2 to rise above 150 mm Hg suggests a cyanotic cardiac malformation

15. Heart Disease and HypoxiaCyanotic Heart Disease • During stabilization the physical exam should include B/P’s in all 4 extremities and careful cardiac exam • A murmur may be audible • Absence of a murmur does not exclude a cardiac defect • CXR & EKG should be included in the evaluation • ECHO is diagnostic

16. Heart Disease and HypoxiaCyanotic Heart Disease • Management: • PGE1 • Bolus of 0.05 mcg/Kg IV • Drip of 0.05-0.1 mcg/Kg/min • Secure airway • Profound apnea is a non-dose dependent complication of PGE1

17. Hypoplastic Left Heart Syndrome • 25% of cardiac deaths during first week of life • Occurs in both cyanotic and acyanotic forms • In 15% of cases the FO is intact preventing mixing at the atrial level • Infants with mixing at the atrial level are acyanotic

18. Hypoplastic Left Heart Syndrome • PE: • Pallor • Tachypnea • Poor perfusion • Poor to absent peripheral pulses • Loud single S2 • Gallop rhythm w/o murmur • Hepatomegaly • Metabolic acidosis

19. Hypoplastic Left Heart Syndrome • EKG: • Small or absent (L) ventricular forces • CXR: • Moderate cardiomegaly • Large PA shadow • ECHO: • Small or slit-like (L) ventricle • Hypoplastic ascending aorta

20. Hypoplastic Left Heart Syndrome • Treatment: • PGE1- systemic blood flow is ductal dependent • Surgical correction • Surgical correction • 1st stage • Norwood procedure • 2nd stage • Fontan procedure • Neonatal cardiac transplantation • Compassionate care may be appropriate in some instances

21. Acyanotic Heart DiseaseCongestive Heart Failure • Typically presents with symptoms of CHF • Tachypnea • Tachycardia • Hepatomegaly • History of poor or slow feeding • Sweating or color change with feeding • Poor weight gain • More gradual clinical decompensation • May not present until after the first 2-3 weeks of age

22. Acyanotic Heart Disease Congestive Heart Failure • Causes of CHF in Neonates: • Acyanotic heart disease (VSD, ASD, PDA, CoA) • Severe anemia • Trauma • Sepsis • SVT • Metabolic abnormalities • SLE • Thyrotoxicosis

23. Acyanotic Heart DiseaseCongestive Heart Failure • Initial management: • Stabilization of the ABC’s • CXR • EKG • Labs: • CBC • BMP • ABG • ECHO- diagnostic of heart defect • Furosemide • 1 mg/Kg IV

24. Acyanotic Heart DiseaseCongestive Heart Failure • Pressors: • Dopamine • 5-15 mcg/Kg/min IV • Dobutamine • 2.5-15 mcg/Kg/min IV • Careful with fluid overloading • Peds. Cardiology consult

25. Acyanotic Heart DiseaseSupraventricular Tachycardia • SVT is the most common neonatal dysrhythmia (1/25,000 births) • Signs/symptoms: • Tachycardia • Poor feeding • Irritability • Heart Failure • Shock • Heart rate sustained at >220 bpm with a QRS < 0.08 seconds

26. Acyanotic Heart DiseaseSupraventricular Tachycardia

27. Acyanotic Heart DiseaseSupraventricular Tachycardia • Management: • Stable patient: • Vagal maneuvers • Ice to face avoiding the nares • If unsuccessful: • Adenosine • 50 mcg/Kg rapid IVP (1-2 secs.), increase dose in 50mcg/Kg increments Q2 mins. until return of sinus rhythm, maximum dose 250 mcg/Kg

28. Acyanotic Heart DiseaseSupraventricular Tachycardia • Unstable patient w/o IV access: • Synchronized cardioversion • 0.5-1 J/Kg • Initial cardioversion should be attempted pharmacologically if IV access is established and adenosine is readily available • If unresponsive to adenosine & cardioversion • Amiodorone • 5mg/Kg IV over 30-60 mins.

29. Acyanotic Heart DiseaseSupraventricular Tachycardia • Procainamide- alternative to amiodorone • 15 mg/Kg IV over 30-60 mins. • The administration of procainamide and amiodorone together can lead to hypotension and widening of the QRS complex • Lidocaine • 1mg/Kg IV • Final option for a wide QRS and should only be used in consultation with a pediatric cardiologist

30. Acyanotic Heart DiseaseSupraventricular Tachycardia • 12-lead EKG prior to and after conversion from SVT to NSR • Useful diagnostic tool for the cardiologists to help determine further management • Consult pediatric cardiologist for further evaluation

31. Heart Disease and HypoxiaBronchiolitis • Viral lower-airway disease caused by RSV 80% of the time • Other etiologies include adenovirus, influenza, or parainfluenza • RSV is responsible for 50-90% of bronchiolitis hospital admissions • More common in winter and spring seasons, may present at any time • In NY from October-April

32. Heart Disease and HypoxiaBronchiolitis • Signs/Symptoms: • Rhinorrhea • Cough • Congestion • Wheezing • Significant respiratory distress • Apnea may be the only initial symptom

33. Heart Disease and HypoxiaBronchiolitis • Management: • Infants with severe, prolonged apnea with bradycardia unresponsive to O2 therapy may need intubation • Nebulized racemic epinephrine or • Beta-agonist • The adjunct use of corticosteroids has not been shown to improve symptoms • A fever or sepsis evaluation may be part of the management

34. Heart Disease and HypoxiaBronchiolitis • Controversy over the incidence of severe bacterial infections in infants who have RSV • The presence of a viral infection doesn’t exclude the possibility of a concomitant UTI • Consider hospitalization for all RSV(+) neonates, especially preemies or all neonates with other comorbidities

35. Heart Disease and Hypoxia Apnea/ALTE • Apnea • cessation of respiration for 20 secs. or more, associated with color change (cyanosis or pallor) or bradycardia • ALTE • poorly defined term used to describe any event that is “frightening to the observer and is characterized by some combination of apnea, color change, marked change in muscle tone, choking or gagging”

36. Heart Disease and HypoxiaApnea/ALTE • Management depends on history provided by observers and PE • Hospitalization for observation and monitoring • Common differential diagnosis: • Sepsis • Pneumonia • RSV • Hypothermia • Anemia

37. Heart Disease and HypoxiaApnea/ALTE • Botulism • Dysrhythmias • Acid/base disturbances • Intracranial hemorrhage • Meningitis/encephalitis • Pertussis • Hypoglycemia • Seizures • GER • Child abuse • Inborn errors of metabolism • Electrolyte abnormalities

38. Endocrine EmergenciesCongenital Adrenal Hyperplasia • Most patients diagnosed by newborn screening • Occasionally diagnosis is missed because of inadequate blood sample, laboratory error, or inability to contact the family

39. Endocrine EmergenciesCongenital Adrenal Hyperplasia • Autosomal recessive • Most common is 21-hydroxylase deficiency- 95% of affected patients • Inadequate cortisol levels • Excessive ACTH stimulation • Adrenal hyperplasia • Excessive production of adrenal androgens and testosterone  virilization

40. Endocrine EmergenciesCongenital Adrenal Hyperplasia • Two forms • Virilizing form • Relative aldosterone deficiency • Mild salt loss • Adrenal insufficiency tends not to occur unless under stressful situations • Salt-losing form • Absolute aldosterone deficiency • Adrenal insufficiency under basal conditions • Manifests in the neonatal period or soon after as an adrenal crisis

41. Endocrine EmergenciesCongenital Adrenal Hyperplasia • 11- hydroxylase deficiency • Less common- 5-8% of cases • Salt retention • Volume expansion • Hypertension

42. Endocrine EmergenciesCongenital Adrenal Hyperplasia • Management: • Labs: • Blood glucose • Hypoglycemia • Serum electrolytes • Hyponatremia • Hyperkalemia • Hypotension unresponsive to fluids or inotropes heightens suspicion of CAH

43. Endocrine EmergenciesCongenital Adrenal Hyperplasia • Hydrocortisone • 25-50mg/m2 IV • Treat hypoglycemia • Hyperkalemia usually responds to fluid therapy • If patient is symptomatic or with EKG changes • Calcium chloride • NaHCO3 • Insulin and glucose • Polystyrene sulfonate (Kayexalate)

44. Endocrine EmergenciesCongenital Adrenal Hyperplasia • Pediatric critical care management • Endocrinology consultation

45. Endocrine EmergenciesThyrotoxicosis • Hypermetabolic state resulting from excessive thyroid hormone activity in the newborn • Usually results from transplacental passage of thyroid-stimulating immunoglobulin from a mother with Graves’ disease • Rare disorder • Occurs in ~1/70 thyrotoxic pregnancies • Incidence of maternal thyrotoxicosis in pregnancy is 1-2/1000 pregnancies

46. Endocrine EmergenciesThyrotoxicosis • Clinical presentation • Fetal tachycardia in the 3rd trimester may be the first manifestation • Signs usually apparent within hours from birth • If mother is on antithyroid medications presentation may be delayed 2-10 days • Thyrotoxic signs • Irritability • Tachycardia • Flushing • Tremor • Poor weight gain • Trombocytopenia • Arrhythmias

47. Endocrine EmergenciesThyrotoxicosis • Initial diagnosis difficult w/o clear history of Graves’ disease from mother • Goiter usually present  tracheal compression • Labs • Increased T4, FT4 & T3 • Suppressed levels of TSH • Treatment • Mild • Close observation

48. Endocrine EmergenciesThyrotoxicosis • Moderate • Lugol’s solution (iodine) • 1 drop PO Q8H • Propylthiouracil • 5-10mg/Kg/day in 3 divided doses • Methimazole • 0.5-1mg/Kg/day in 3 divided doses • Severe • In addition to above meds • Prednisone • 2mg/Kg/day • Propanolol – for tachycardia • 1-2mg/Kg/day in 2-4 divided doses • Digitalis may be used to prevent cardiovascular collapse

49. Inborn Errors of Metabolism

50. Inborn Errors of Metabolism • Urea cycle defects • Ornithine-transcarbamylase deficiency • Carbamyl phosphate synthetase deficiency • Transient hyperammonemia of the neonate (unclear cause) • Argininosuccinate synthetase deficiency (citrulinemia) • Argininosuccinate lyase deficiency • Arginase deficiency • N-acetylglutamate synthetase deficiency