1.
The Ill-Appearing Neonates <ul><li>Atima Delaney, MD </li></ul><ul><li>Division of Emergency Medicine </li></ul><ul><li>Children’s Hospital Boston </li></ul>

2.
Case <ul><li>10-day-old infant presents with poor feeding for 3 days, today noted to be lethargic </li></ul><ul><li>PE: Limp, cyanotic, mottled V/S T 37, HR 80, RR 12, BP 52/38, O2 Sat 80% RA HEENT: open, soft AF, pupils 3->2 mm Heart: RRR, no murmur Lungs: no retractions, clear BS Abdomen: soft, nontender, no HSM Ext: cap refills > 5 seconds Neuro: decreased tone throughout </li></ul>

3.
Case <ul><li>What is initial management? </li></ul><ul><li>What history should be taken? </li></ul><ul><li>What are the differential diagnoses? </li></ul>

4.
Unique Features of the Neonates

5.
Ill-appearing Infants <ul><li>Clinical Features Depressed or altered mental status Lethargy Grunting respiration Head bobbing Increased work of breathing Bradypnea, apnea Poor muscle tone or floppy Skin changes: pallor, cyanosis, poor cap refills, </li></ul>

6.
Differential Diagnoses <ul><li>Most common causes for catastrophic illness in the neonates ID: GBS, Gram-neg bacilli, HSV, enterovirus, RSV Ductal-Dependent CHD: Left-sided lesions: Coarctation of the aorta, Interrupted aortic arch, aortic stenosis, Hypoplastic left heart syndrome Right-sided lesions: Pulmonary atresia or stenosis Tetralogy of Fallot Tricuspid atresia Metabolic Disorder: Inborn error of metabolism, CAH GI: volvulus, NEC, Hirschprung’s disease, incarcerated hernia Neurologic: Seizure, CNS hemorrhage Respiratory: bronchiolitis, pneumonia </li></ul>

7.
Differential Diagnoses <ul><li>“ NEO SECRETS” i N born error of metabolism E lectrolyte abnormality O verdose S eizures E nteric emergencies C ardiac abnormality R ecipe (formula, additives) E ndocrine crisis T rauma S epsis </li></ul>

8.
Inborn Error of Metabolism <ul><li>Presentation 2-7 days of age </li></ul><ul><li>Several categories of IEM (amino acid disorders, organic acidemias, urea cycle disorders, disorders of carbohydrate metabolism, fatty acid oxidation defects, and mitochondrial disorders) </li></ul><ul><li>GI: Poor feeding, poor suck, vomiting, FTT, hepatosplenomegaly, jaundice </li></ul><ul><li>CNS: Irritability, lethargy, coma, death </li></ul><ul><li>Hyperammonemia, profound acidosis </li></ul><ul><li>Consult metabolism </li></ul>

9.
Electrolytes Abnormality <ul><li>Hyponatremia: Water intoxication, SIADH, CAH </li></ul><ul><li>Hypernatremia: Breastfeeding difficulty (dehydration), DI </li></ul><ul><li>Hypoglycemia </li></ul><ul><li>Hypocalcemia </li></ul>

10.
Overdose/Toxic Exposure <ul><li>Methhemoglobinemia -Newborns are at risk -Reports of association with Prilocaine and occasionally EMLA use </li></ul><ul><li>Carbon monoxide poisoning </li></ul>

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Seizures <ul><li>May not present as tonic-clonic activity </li></ul><ul><li>Hypoxic-ischemia injury Intracranial hemorrhage CNS infections Electrolyte abnormalities Inborn error of metabolism Congenital abnormality of brain Drug withdrawal </li></ul>

12.
Seizures <ul><li>Lorazepam </li></ul><ul><li>Phenobarbital or phynetoin/fosphynetoin </li></ul><ul><li>Consider giving 10% Calcium gluconate if seizure persists after standard therapy </li></ul><ul><li>Consider giving MgSO4 </li></ul><ul><li>Pyridoxine (Vit B6) if seizure persists after above </li></ul>

13.
Enteric Emergencies <ul><li>True surgical emergency </li></ul><ul><li>Volvulus - twisting around mesenteric artery </li></ul><ul><li>Bilious emesis (>90%), maybe well-appearing </li></ul><ul><li>Shock if bowel is ischemic or necrotic </li></ul>Malrotation with or without volvulus emedicine

14.
Enteric Emergencies <ul><ul><li>UGI series -Dilated duodenum -Abnormal duodeno-jejunal junction </li></ul></ul>Malrotation with or without volvulus uptodate Normal Malrotation “ corkscrew” Volvulus

15.
Enteric Emergencies <ul><li>Necrotizing enterocolitis -More common in preemies -Term infants with risk factors -Ill-appearing, distended abdomen, bloody stools </li></ul>pneumatosis intestinalis Portal vein gas uptodate

16.
Cardiac <ul><li>CHD often presents during first month of life </li></ul><ul><li>Presentations 1) cyanosis 2) mottle or gray appearance 3) CHF </li></ul>

17.
Cardiac <ul><li>DUCTAL DEPENDENT LESIONS </li></ul><ul><li>Right-to-Left Shunt Cyanosis, metabolic acidosis, decreased perfusion or CHF on CXR Transposition of great arteries Tetralogy of Fallot Truncus arteriosus Total anomalous venous return </li></ul><ul><li>Left-sided Obstructive Lesions Severe systemic hypoperfusion, decreased or absent pulses, metabolic acidosis, cardiomegaly with pulmonary congestion on CXR Hypoplastic left heart, Coarctation of aorta, interrupted aortic arch, AS </li></ul>

18.
Cardiac <ul><li>Presents with CHF </li></ul><ul><li>Left-to-Right Shunt -Large VSD -Complete AV canal defect -Large PDA </li></ul><ul><li>SVT </li></ul>

19.
Cardiac <ul><li>Ill-appearing neonates not responding to initial resuscitation, consider ductal- dependent CHD </li></ul><ul><li>Hyperoxia Test 100% O2 for 10 minutes Left-to-right lesions: PO2 > 60-70 mmHg (Sat > 90-95%) Cyanotic lesions: PO2 < 60 mmHg (Sat < 85-90%) </li></ul>

20.
Recipe <ul><li>Incorrect formula preparation </li></ul><ul><li>Home remedies </li></ul><ul><li>Botulism - Infant botulism -Peak 2-4 mo. -Hypotonia, constipation, descending flaccid paralysis, autonomic instability, CN deficits </li></ul>

21.
Endocrine <ul><li>21-hydroxylase deficiency Salt-losing or Non-salt losing forms Girls: ambiguous genitalia Boys: salt-losing adrenal crisis (vomiting, hyptension, hyponatremia, hyperkalemia, metabolic acidosis, hypoglycemia) </li></ul>uptodate Congenital Adrenal Hyperplasia (CAH)

22.
Trauma <ul><li>Inflicted head injury -Lethargy -Altered mental status -Seizures -Respiratory distress, apenea </li></ul>emedicine

23.
Sepsis & Meningitis <ul><li>All ill-appearing infants should be considered sepsis until proven otherwise </li></ul><ul><li>Irritability, lethargy, poor feeding, ± fever, hypothermia, apnea, cyanosis, poor perfusion </li></ul><ul><li>Early onset: First few days - 6 days Associated with perinatal risk factors </li></ul><ul><li>Late onset: ≥ 7 days </li></ul><ul><li>Common organisms: GBS, E.coli, Gram neg rods, Listeria monocytogenes, Strep pneumoniae </li></ul>

24.
HSV <ul><li>Birth- 1 month (peak 10-17 days) </li></ul><ul><li>1. Localized skin, eye, mouth (SEM) 2. CNS ± SEM 3. Disseminated </li></ul><ul><li>HSV cultures of vesicles, oropharynx, conjunctiva, urine, blood, stool or rectum, and CSF HSV PCR and LFTs - Mucocutaneous vesicles - Seizure - CSF pleocytosis with negative gram stain - Mother known to have HSV </li></ul>uptodate

25.
Other Overwhelming Viral Infections <ul><li>Enterovirus -Myocarditis -Hepatitis </li></ul><ul><li>Bronchiolitis with apnea Risk factors: age < 6 weeks, preemie, low O2 Sat </li></ul>

26.
Sepsis <ul><li>CBC, UA, blood & urine culture, CRP </li></ul><ul><li>LP </li></ul><ul><li>Start Ampicillin + Gentamicin (early onset) or Ampicillin + Cefotaxime/Ceftazidime </li></ul><ul><li>Start Acyclovir IV -CSF pleocytosis & negative gram stain -CSF pleocytosis & vesicular rash, focal neurologic signs, pneumonitis, hepatitis, maternal hx -CSF elevated RBC </li></ul>

27.
History <ul><li>Maternal Hx: GBS </li></ul><ul><li>Birth Hx: delivery, complications, birth weight </li></ul><ul><li>Neonatal course </li></ul><ul><li>Symptoms </li></ul><ul><li>Feeding, UOP and stooling, emesis - bilious? </li></ul><ul><li>Inflicted injury: no clear hx or hx inconsistent with findings </li></ul>

28.
Physical Exam <ul><li>General Appearance </li></ul><ul><li>Vital signs, pulse ox BP : neonates - SBP 60 mmHg, infants - SBP 70 mmHg HR: >220/min consider SVT RR: tachypnea, apnea, periodic breathing T: lack of fever does not exclude serious infection </li></ul><ul><li>4-Ext BP Diminished pulse and BP -> left-ventricular outflow obstruction </li></ul>

29.
Physical Exam <ul><li>Head: fontanelle, scalp </li></ul><ul><li>HEENT: pupils, neck </li></ul><ul><li>Chest: nasal flaring, grunting, retractions, breath sounds, murmur </li></ul><ul><li>Abdomen: distention, rigidity, hepatomegaly Normal exam doesn’t exclude abdominal pathology </li></ul><ul><li>Skin: cap refills, petechiae </li></ul><ul><li>Neuro: mental status, muscle tone, abnormal movement </li></ul>

30.
Management <ul><li>ABCD </li></ul><ul><li>Obtain V/S, pulse oxymetry, glucose </li></ul><ul><li>Monitoring </li></ul><ul><li>Treat hypovolemia and electrolyte abnormality </li></ul><ul><li>Treat hypothermia & maintain body temperature </li></ul>

31.
Airway <ul><li>Intubation </li></ul><ul><li>Preemie: 2.5 mm Full-term: 3 - 4 mm 1 yo- toddler: 4-4.5 mm </li></ul><ul><li>Broselow tape </li></ul><ul><li>Cuffed or uncuffed tubes Cuffed tube: high inspiratory pressure </li></ul><ul><li>RSI: atropine recommended </li></ul>

32.
Circulation <ul><li>IV, umbilical vein, IO </li></ul><ul><li>IO access early if failed IV attempts </li></ul><ul><li>Give 20 ml/kg NSS bolus (unless CHF) </li></ul><ul><li>Septic shock: - Requires several fluid boluses - Children who received > 40 ml/kg in the first hour do better than those receiving < 20 ml/kg (Carcillo, JAMA 1991) </li></ul><ul><li>Maintenance fluid D5 1/4NSS @ 4 ml/kg/hr </li></ul>

33.
Circulation <ul><li>Anemia or trauma: 10 ml/kg PRBC </li></ul><ul><li>If no improvement in BP, mental status, skin perfusion after 60-80 ml/kg of NSS -> Dopamine starting 6-10 mcg/kg/min -> Consider central line </li></ul>

34.
Hypoglycemia <ul><li>Presumed hypoglycemia in all critically ill infants until proven otherwise </li></ul><ul><li>Treat if blood glucose < 50 mg/dL </li></ul><ul><li>Give D10 W 5-10 ml/kg </li></ul>

35.
Antibiotics & Acyclovir <ul><li>Presumed septic until proven otherwise </li></ul><ul><li>Early antibiotic (within 30-40 min) in most ill-appearing infants </li></ul><ul><li>≤ 28 days: Ampicillin + gentamicin or Ampicillin + Cefotaxime </li></ul><ul><li>Older infants: Ceftriaxone </li></ul><ul><li>Acyclovir: infants ≤ 28 days Mucocutaneous vesicles Seizure CSF pleocytosis with negative gram stain CSF RBC from atraumatic LP </li></ul>

36.
Other Specific Treatment <ul><li>Prostaglandin E1 - Cyanotic or obstructive heart disease with hemodynamic instability - Temporarily restore pulmonary and systemic blood flow - Consult cardiologist & neonatologist - 0.05 mcg/kg IV infusion, titrate to lowest dose Side effects: apnea, tachycardia, hypotension </li></ul><ul><li>Hydrocortisone -Usually male patient with adrenal crisis -Draw blood work before if possible </li></ul>

37.
Diagnostic studies <ul><li>Bedside: glucose, urine dipstick, EKG </li></ul><ul><li>Labs CBC, blood culture Electrolytes, Ca, Mg, Phos Blood gas Ammonia, lactate, pyruvate, blood & urine ketone UA, urine culture LP for CSF, CSF culture (if stable) CSF HSV PCR or enterovirus (if indicated) Bilirubin level </li></ul>

38.
Radiology Tests <ul><li>CXR </li></ul><ul><li>Abdominal plain film </li></ul><ul><li>Head CT </li></ul><ul><li>Upper GI series </li></ul>

39.
Summary <ul><li>Treat sepsis in all ill-appearing neonates </li></ul><ul><li>Check bedside glucose in all ill-appearing neonates </li></ul><ul><li>DDx: NEO SECRETS </li></ul><ul><li>Neonates with bilious emesis needs work-up to rule out volvulus which is true surgical emergency </li></ul><ul><li>Monitor glucose and temperature throughout ED stay </li></ul>