Ill appearing neonates

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Dr.Atima Delany

Dr.Atima Delany

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  • presentation after taking certain protein or carb, infection Although IEM may be included in newborn screening tests, infants can present before the results are available.
  • Hypocal - often seen first few days of life, high phosphate level in cow’s milk formula, maternal or congenital hypoparathyroidism, maternal vit D deficiency, hypomagnesemia
  • Lip smacking, bicycling movements of the legs, tongue thrusting, apnea, and staring spells Electrolytes: hypoglycemia, hypo/hypernatremia, hypocalcemia, hypomagnesemia Drug withdrawal: methadone, barbiturates CNS infection: including STORCH
  • Arrest in embryogenic gut rotation resulting in narrow mesenteric base Risk of bowel ischemia Ladd’s band procedure
  • * The DJJ is low and to the right of the normal location In the image on the left, the duodenal bulb is to left of the spine. In the image on the right, with malrotation, the duodenal bulb is overlying spine. * The proximal small bowel (jejunum) is in the right upper quadrant. * The cecum is in the upper and/or left abdomen. * The large bowel is in the left abdomen.
  • anoxic or stress event at birth
  • cyanosis due to R to L shunting Mottle or gray due to outflow obstruction CHF due to L to R intracardiac shunting
  • -cyanotic lesions & obstructive lesions = ductal dependent -The ductal dependent lesions are usually of 2 types: left-sided and right-sided obstructive lesions. -Left-sided obstructive lesions (aortic stenosis, coarctation of the aorta, interrupted aortic arch, hypoplastic left heart syndrome) present with signs of severe systemic hypoperfusion with pallor, mottling, decreased or absent pulses, severe metabolic acidosis, and cardiomegaly with pulmonary congestion on chest radiograph (CXR). -R-sided obstructive lesions (pulmonary atresia, severe pulmonary stenosis, tetralogy of Fallot, tricuspid atresia) present with severe cyanosis, metabolic acidosis, and decreased perfusion of the lung fields on CXR.
  • -Lesions with pulmonary overcirculation (atrial or ventricular septal defects, atrioventricular canal, truncus arteriosus, and partial anomalous pulmonary venous return) can present with evidence of congestive heart failure and respiratory distress; however, the deterioration in these infants is not as dramatic as with ductal dependent lesions. Congestive heart failure may also be due to supraventricular tachycardia
  • Symptoms similar to septic shock!!!!! In septic appearing infants, if there is no clinical improvement after resuscitation and treatment of septic shock … .. think of congenital heart disease !!!!!!!!!!!!!!!!! Diagnosis will be delayed: transferring to another facility for echo
  • home remedies: baking soda for colic, herbal tea for constipation or colic
  • -The most common is 21-hydroxylase deficiency resulting in the inability to convert progesterone to aldosterone or cortisol and causing an accumulation of testosterone. -Adrenal hyperplasia develops as a result of overstimulation by ACTH (which has no negative feedback from cortisol) -Deficiency of aldosterone with urinary salt wasting: classic electrolyte findings and cardiovascular collapse -Female infants classically present with ambiguous genitalia with varying degrees of virilization. Typically recognized in nursery -However, a male infant may only have a hyperpigmented scrotum and no other physical abnormalities and can only be recognized by appropriate laboratory evaluation and results of the newborn screen if available.
  • Earl y onset: 85% presents within 24 hours. Early-onset sepsis syndrome is associated with acquisition of microorganisms from the mother Maternal risk factors: PROM, maternal fever Meningitis: GBS 50%, E. Coli 25%, Rest < 10% each Late-onset sepsis syndrome occurs at 7-90 days of life and is acquired from the caregiving environment Pneumonia is more common in early-onset sepsis, whereas meningitis and bacteremia are more common in late-onset sepsis. Absence of fever or hypothermia doesn ’ t rule out Bulging fontanelle and nuchal rigidity are present in small number of patients
  • enterovirus (coxsackievirus, ECHO virus)
  • (cover gram neg bacilli or late onset)
  • -weight: lost 10% - level off by day 5-7, back to BW day 10-14, gain 20-30 g/day -Poor feeding, improper formula mixing, sweating during the feed (equivalent to stress test - if sweaty, poor suck, stops feeding after few minutes - consider CHF) -fever - although overwhelming infection can present with high, normal or low temperature
  • GA: tone, color, perfusion, work of breathing - V/S BP, HR < 220, RR < 60, periodic breathing temp = rectal temp Broslow tape
  • -most murmurs in neonates are pathologic: VSD, PDA, PS, Aortic stenosis -Abdominal distention nonspecific, tenderness may be difficult to evaluate, rigidity is worrisome, hepatomegaly - CHF
  • Give O2
  • -poor feeding, small glycogen storage, high metabolic demands in critically ill infants
  • Ampicillin treats gram positive, listeria and enterococcus. Gentamin treats gram negative and synergistic effect. Ceftriaxone in younger infants can cause biliary sludging. S. pneumo meningitis - add vanco to cover resistant strain
  • stress dose hydrocortisone
  • infants may not demonstrate pyuria when UTI is present, always obtain urine culture

Transcript

  • 1. The Ill-Appearing Neonates
    • Atima Delaney, MD
    • Division of Emergency Medicine
    • Children’s Hospital Boston
  • 2. Case
    • 10-day-old infant presents with poor feeding for 3 days, today noted to be lethargic
    • 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
  • 3. Case
    • What is initial management?
    • What history should be taken?
    • What are the differential diagnoses?
  • 4. Unique Features of the Neonates
  • 5. Ill-appearing Infants
    • 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,
  • 6. Differential Diagnoses
    • 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
  • 7. Differential Diagnoses
    • “ 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
  • 8. Inborn Error of Metabolism
    • Presentation 2-7 days of age
    • Several categories of IEM (amino acid disorders, organic acidemias, urea cycle disorders, disorders of carbohydrate metabolism, fatty acid oxidation defects, and mitochondrial disorders)
    • GI: Poor feeding, poor suck, vomiting, FTT, hepatosplenomegaly, jaundice
    • CNS: Irritability, lethargy, coma, death
    • Hyperammonemia, profound acidosis
    • Consult metabolism
  • 9. Electrolytes Abnormality
    • Hyponatremia: Water intoxication, SIADH, CAH
    • Hypernatremia: Breastfeeding difficulty (dehydration), DI
    • Hypoglycemia
    • Hypocalcemia
  • 10. Overdose/Toxic Exposure
    • Methhemoglobinemia -Newborns are at risk -Reports of association with Prilocaine and occasionally EMLA use
    • Carbon monoxide poisoning
  • 11. Seizures
    • May not present as tonic-clonic activity
    • Hypoxic-ischemia injury Intracranial hemorrhage CNS infections Electrolyte abnormalities Inborn error of metabolism Congenital abnormality of brain Drug withdrawal
  • 12. Seizures
    • Lorazepam
    • Phenobarbital or phynetoin/fosphynetoin
    • Consider giving 10% Calcium gluconate if seizure persists after standard therapy
    • Consider giving MgSO4
    • Pyridoxine (Vit B6) if seizure persists after above
  • 13. Enteric Emergencies
    • True surgical emergency
    • Volvulus - twisting around mesenteric artery
    • Bilious emesis (>90%), maybe well-appearing
    • Shock if bowel is ischemic or necrotic
    Malrotation with or without volvulus emedicine
  • 14. Enteric Emergencies
      • UGI series -Dilated duodenum -Abnormal duodeno-jejunal junction
    Malrotation with or without volvulus uptodate Normal Malrotation “ corkscrew” Volvulus
  • 15. Enteric Emergencies
    • Necrotizing enterocolitis -More common in preemies -Term infants with risk factors -Ill-appearing, distended abdomen, bloody stools
    pneumatosis intestinalis Portal vein gas uptodate
  • 16. Cardiac
    • CHD often presents during first month of life
    • Presentations 1) cyanosis 2) mottle or gray appearance 3) CHF
  • 17. Cardiac
    • DUCTAL DEPENDENT LESIONS
    • 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
    • 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
  • 18. Cardiac
    • Presents with CHF
    • Left-to-Right Shunt -Large VSD -Complete AV canal defect -Large PDA
    • SVT
  • 19. Cardiac
    • Ill-appearing neonates not responding to initial resuscitation, consider ductal- dependent CHD
    • 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%)
  • 20. Recipe
    • Incorrect formula preparation
    • Home remedies
    • Botulism - Infant botulism -Peak 2-4 mo. -Hypotonia, constipation, descending flaccid paralysis, autonomic instability, CN deficits
  • 21. Endocrine
    • 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)
    uptodate Congenital Adrenal Hyperplasia (CAH)
  • 22. Trauma
    • Inflicted head injury -Lethargy -Altered mental status -Seizures -Respiratory distress, apenea
    emedicine
  • 23. Sepsis & Meningitis
    • All ill-appearing infants should be considered sepsis until proven otherwise
    • Irritability, lethargy, poor feeding, ± fever, hypothermia, apnea, cyanosis, poor perfusion
    • Early onset: First few days - 6 days Associated with perinatal risk factors
    • Late onset: ≥ 7 days
    • Common organisms: GBS, E.coli, Gram neg rods, Listeria monocytogenes, Strep pneumoniae
  • 24. HSV
    • Birth- 1 month (peak 10-17 days)
    • 1. Localized skin, eye, mouth (SEM) 2. CNS ± SEM 3. Disseminated
    • 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
    uptodate
  • 25. Other Overwhelming Viral Infections
    • Enterovirus -Myocarditis -Hepatitis
    • Bronchiolitis with apnea Risk factors: age < 6 weeks, preemie, low O2 Sat
  • 26. Sepsis
    • CBC, UA, blood & urine culture, CRP
    • LP
    • Start Ampicillin + Gentamicin (early onset) or Ampicillin + Cefotaxime/Ceftazidime
    • Start Acyclovir IV -CSF pleocytosis & negative gram stain -CSF pleocytosis & vesicular rash, focal neurologic signs, pneumonitis, hepatitis, maternal hx -CSF elevated RBC
  • 27. History
    • Maternal Hx: GBS
    • Birth Hx: delivery, complications, birth weight
    • Neonatal course
    • Symptoms
    • Feeding, UOP and stooling, emesis - bilious?
    • Inflicted injury: no clear hx or hx inconsistent with findings
  • 28. Physical Exam
    • General Appearance
    • 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
    • 4-Ext BP Diminished pulse and BP -> left-ventricular outflow obstruction
  • 29. Physical Exam
    • Head: fontanelle, scalp
    • HEENT: pupils, neck
    • Chest: nasal flaring, grunting, retractions, breath sounds, murmur
    • Abdomen: distention, rigidity, hepatomegaly Normal exam doesn’t exclude abdominal pathology
    • Skin: cap refills, petechiae
    • Neuro: mental status, muscle tone, abnormal movement
  • 30. Management
    • ABCD
    • Obtain V/S, pulse oxymetry, glucose
    • Monitoring
    • Treat hypovolemia and electrolyte abnormality
    • Treat hypothermia & maintain body temperature
  • 31. Airway
    • Intubation
    • Preemie: 2.5 mm Full-term: 3 - 4 mm 1 yo- toddler: 4-4.5 mm
    • Broselow tape
    • Cuffed or uncuffed tubes Cuffed tube: high inspiratory pressure
    • RSI: atropine recommended
  • 32. Circulation
    • IV, umbilical vein, IO
    • IO access early if failed IV attempts
    • Give 20 ml/kg NSS bolus (unless CHF)
    • 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)
    • Maintenance fluid D5 1/4NSS @ 4 ml/kg/hr
  • 33. Circulation
    • Anemia or trauma: 10 ml/kg PRBC
    • 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
  • 34. Hypoglycemia
    • Presumed hypoglycemia in all critically ill infants until proven otherwise
    • Treat if blood glucose < 50 mg/dL
    • Give D10 W 5-10 ml/kg
  • 35. Antibiotics & Acyclovir
    • Presumed septic until proven otherwise
    • Early antibiotic (within 30-40 min) in most ill-appearing infants
    • ≤ 28 days: Ampicillin + gentamicin or Ampicillin + Cefotaxime
    • Older infants: Ceftriaxone
    • Acyclovir: infants ≤ 28 days Mucocutaneous vesicles Seizure CSF pleocytosis with negative gram stain CSF RBC from atraumatic LP
  • 36. Other Specific Treatment
    • 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
    • Hydrocortisone -Usually male patient with adrenal crisis -Draw blood work before if possible
  • 37. Diagnostic studies
    • Bedside: glucose, urine dipstick, EKG
    • 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
  • 38. Radiology Tests
    • CXR
    • Abdominal plain film
    • Head CT
    • Upper GI series
  • 39. Summary
    • Treat sepsis in all ill-appearing neonates
    • Check bedside glucose in all ill-appearing neonates
    • DDx: NEO SECRETS
    • Neonates with bilious emesis needs work-up to rule out volvulus which is true surgical emergency
    • Monitor glucose and temperature throughout ED stay