The Ill-Appearing Neonates

Atima Delaney, MDDivision of Emergency Medicine

Children’s Hospital Boston

Case

• 10-day-old infant presents with poor feeding for 3 days, today noted to be lethargic

• PE: Limp, cyanotic, mottledV/S T 37, HR 80, RR 12, BP 52/38, O2 Sat 80% RAHEENT: open, soft AF, pupils 3->2 mmHeart: RRR, no murmurLungs: no retractions, clear BSAbdomen: soft, nontender, no HSMExt: cap refills > 5 secondsNeuro: decreased tone throughout

Case

• What is initial management?

• What history should be taken?

• What are the differential diagnoses?

Unique Features of the Neonates

Ill-appearing Infants

• Clinical FeaturesDepressed or altered mental statusLethargyGrunting respirationHead bobbingIncreased work of breathingBradypnea, apneaPoor muscle tone or floppySkin changes: pallor, cyanosis, poor cap refills,

Differential Diagnoses• Most common causes for catastrophic illness in

the neonates

ID: GBS, Gram-neg bacilli, HSV, enterovirus, RSVDuctal-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 atresiaMetabolic Disorder: Inborn error of metabolism, CAHGI: volvulus, NEC, Hirschprung’s disease, incarcerated herniaNeurologic: Seizure, CNS hemorrhageRespiratory: bronchiolitis, pneumonia

Differential Diagnoses

• “NEO SECRETS”

iNborn error of metabolismElectrolyte abnormalityOverdoseSeizuresEnteric emergenciesCardiac abnormalityRecipe (formula, additives)Endocrine crisisTraumaSepsis

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

Electrolytes Abnormality

• Hyponatremia: Water intoxication, SIADH, CAH

• Hypernatremia:Breastfeeding difficulty (dehydration), DI

• Hypoglycemia

• Hypocalcemia

Overdose/Toxic Exposure

• Methhemoglobinemia-Newborns are at risk -Reports of association with Prilocaine and occasionally EMLA use

• Carbon monoxide poisoning

Seizures

• May not present as tonic-clonic activity

• Hypoxic-ischemia injuryIntracranial hemorrhageCNS infectionsElectrolyte abnormalitiesInborn error of metabolismCongenital abnormality of brainDrug withdrawal

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

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

Enteric Emergencies

• UGI series-Dilated duodenum-Abnormal duodeno-jejunal junction

Malrotation with or without volvulus

uptodate

Normal Malrotation

“corkscrew”Volvulus

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

Cardiac

• CHD often presents during first month of life

• Presentations1) cyanosis2) mottle or gray appearance3) CHF

Cardiac• DUCTAL DEPENDENT LESIONS

• Right-to-Left ShuntCyanosis, metabolic acidosis, decreased perfusion or CHF on CXRTransposition of great arteriesTetralogy of FallotTruncus arteriosusTotal anomalous venous return

• Left-sided Obstructive LesionsSevere systemic hypoperfusion, decreased or absent pulses, metabolic acidosis, cardiomegaly with pulmonary congestion on CXRHypoplastic left heart, Coarctation of aorta, interrupted aortic arch, AS

Cardiac

• Presents with CHF

• Left-to-Right Shunt-Large VSD-Complete AV canal defect-Large PDA

• SVT

Cardiac

• Ill-appearing neonates not responding to initial resuscitation, consider ductal- dependent CHD

• Hyperoxia Test100% O2 for 10 minutes

Left-to-right lesions: PO2 > 60-70 mmHg (Sat > 90-95%)

Cyanotic lesions: PO2 < 60 mmHg (Sat < 85-90%)

Recipe

• Incorrect formula preparation

• Home remedies

• Botulism-Infant botulism-Peak 2-4 mo.-Hypotonia, constipation, descending flaccid paralysis, autonomic instability, CN deficits

Endocrine

• 21-hydroxylase deficiencySalt-losing or Non-salt losing formsGirls: ambiguous genitaliaBoys: salt-losing adrenal crisis (vomiting, hyptension, hyponatremia, hyperkalemia, metabolic acidosis, hypoglycemia)

uptodate

Congenital Adrenal Hyperplasia(CAH)

Trauma

• Inflicted head injury

-Lethargy-Altered mental status-Seizures-Respiratory distress, apenea

emedicine

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

HSV

• Birth- 1 month (peak 10-17 days)

• 1. Localized skin, eye, mouth (SEM)2. CNS ± SEM3. 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

Other Overwhelming Viral Infections

• Enterovirus-Myocarditis-Hepatitis

• Bronchiolitis with apneaRisk factors: age < 6 weeks, preemie, low O2 Sat

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

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

Physical Exam

• General Appearance

• Vital signs, pulse oxBP: neonates - SBP 60 mmHg, infants - SBP 70 mmHgHR: >220/min consider SVTRR: tachypnea, apnea, periodic breathingT: lack of fever does not exclude serious infection

• 4-Ext BPDiminished pulse and BP -> left-ventricular outflow obstruction

Physical Exam

• Head: fontanelle, scalp

• HEENT: pupils, neck

• Chest: nasal flaring, grunting, retractions, breath sounds, murmur

• Abdomen: distention, rigidity, hepatomegalyNormal exam doesn’t exclude abdominal pathology

• Skin: cap refills, petechiae

• Neuro: mental status, muscle tone, abnormal movement

Management

• ABCD

• Obtain V/S, pulse oxymetry, glucose

• Monitoring

• Treat hypovolemia and electrolyte abnormality

• Treat hypothermia & maintain body temperature

Airway

• Intubation

• Preemie: 2.5 mmFull-term: 3 - 4 mm1 yo- toddler: 4-4.5 mm

• Broselow tape

• Cuffed or uncuffed tubesCuffed tube: high inspiratory pressure

• RSI: atropine recommended

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

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

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

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 daysMucocutaneous vesiclesSeizureCSF pleocytosis with negative gram stainCSF RBC from atraumatic LP

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

Diagnostic studies

• Bedside:glucose, urine dipstick, EKG

• LabsCBC, blood cultureElectrolytes, Ca, Mg, PhosBlood gasAmmonia, lactate, pyruvate, blood & urine ketoneUA, urine cultureLP for CSF, CSF culture (if stable) CSF HSV PCR or enterovirus (if indicated)Bilirubin level

Radiology Tests

• CXR

• Abdominal plain film

• Head CT

• Upper GI series

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