An Overview of Neonatal Emergencies

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An Overview of Neonatal Emergencies
Dr. JUNAID M KHAN MBBS.MD.FRCP.FAAP AL-RAHBA
HOSPITAL, ABU DHABI, UAE
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Objectives
1. To discuss some common baby-killers.
2. To discuss some basic terms.
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Introduction

• For this talk, neonates will refer to infants
  aged 0-28 days.
• Just think of them as little children.
• They can have pathology of virtually any organ
  system.

Metabolic
Cardiac
Toxins
Sepsis
GI
Heme
Endo
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Classification

• There are many different ways to organize the
  neonatal emergencies.
• This talk will focus on THE MISFITS approach.

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THE MISFITS A Mnemonic

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism
• Sepsis
• Formula
• Intestinal
• Toxins
• Trisomies
• Seizures

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NAI

• Non-accidental injury (NAI) is the most common
  cause of infant death outside the neonatal
  period.
• The abuser is a related caretaker in 90 of cases.

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NAI

• Signs suggestive of NAI include
• Caregivers who are intoxicated or high.
• Inconsistent or implausible history.
• Multiple (or different aged) fractures.
• Posterior rib fractures.
• Unusually shaped/distributed bruises or burns.
• Bite marks.

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NAI

• Head injury is the leading cause of death in NAI.
• The initial neonatal examination may be
  surprisingly normal.
• Non-specific clues to IC haemorrhage may include
• - altered LOC - vomiting
• - irritability - seizures
• - FTT - decreased mm tone
• Retinal haemorrhages are virtually pathognomonic
  of NAI in the neonate.

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Retina hemorrhages
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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung

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Heart and Lung

• There are numerous cardiac and pulmonary entities
  which cause neonatal morbidity and mortality.
• Many pulmonary issues (eg. RDS, croup,
  bronchiolitis, pneumonia, etc.)
• The focus will be on congenital heart disease.

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Heart

• Congenital heart disease (CHD) occurs in 1/125
  live births.
• Neonates may present with a variety of
  non-specific findings, including -
  tachypnea - cyanosis -
  pallor - lethargy - FTT - sweating
  with feeds
• More specific findings include
• - pathological murmurs - hypertension
• - abnormal pulses - syncope

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Congenital Heart Disease

• Neonates with CHD often rely on a patent ductus
  arteriosus and/or foramen ovale to sustain life.
• Unfortunately for these neonates, both of these
  passages begins to close following birth.
• The ductus normally closes by 72hrs.
• The foramen ovale normally closes by 3 months.

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CHD

• That being said, in the presence of hypoxia or
  acidosis (generally present in ductus-dependent
  lesions), the ductus may remain open for a longer
  period of time.
• As a result, these patients often present to the
  ED during the first 1-3 weeks of life.
• i.e. as the ductus begins to close.

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Classifying CHD

• There are many different classification systems
  for CHD.
• None are particularly good.
• I will be discussing the Pink/Blue/Grey-Baby
  system
• Pink Baby Left to right shunt
• Blue Baby Right to left shunt
• Grey Baby LV outflow tract obstruction

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Pink Baby (L ? R shunt)

• L ? R shunts cause CHF and pulmonary
  hypertension.
• This leads to RV enlargement, RV failure, and cor
  pulmonale.
• These babies present with CHF and respiratory
  distress.
• They are not typically cyanotic.

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Pink Baby (L ? R shunt)

• These lesions include (among others) ASDs,
  VSDs, and persistently patent ductus arteriosus.

VSD
ASD
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Pink Baby (L ? R shunt)
Persistently patent ductus arteriosus
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Pink Baby (L ? R shunt)

• Diagnosing L ? R shunts depends on
• 1. Examination findings
• Non-cyanotic infant in resp distress.
• Crackles, widely-fixed second heart sound,
  elevated JVP, cor pulmonale.
• 2. CXR
• Increased pulmonary vasculature (suggestive of
  CHF).
• RA and/or RV enlargement.
• 3. EKG
• RAE and/or RVH.

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Pink Baby (L ? R shunt)

• Initial management should be directed at reducing
  the pulm edema.
• Adminster Lasix 1mg/kg IV.
• Peds Cardiology/ PICU should be consulted
  urgently regarding use of
• Morphine
• Nitrates
• Digoxin
• Inotropes

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Blue Baby (R ? L shunt)

• R ? L shunts cause hypoxia and central cyanosis.
• Neither hypoxia or cyanosis tend to improve with
  100 oxygen.
• R ? L lesions include (among others)
• Tetralogy of Fallot (TOF)
• Transposition of the Great Arteries (TGA)

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Tetralogy of Fallot

• Characterized by
• Pulmonary a OTO
• RV hypertrophy
• VSD
• Over-riding aorta
• With severe pulmonary OTO...

bloodflow to the lungs may be highly
ductus-dependent.

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Tetralogy of Fallot

• The classic CXR finding in TOF is the boot-shaped
  heart.

• Pulmonary vasculature is typically decreased.

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Transposition of the Great Arteries

• TGA is the most common cyanotic lesion presenting
  in the first week of life.
• Anatomically
• RV ? aorta
• LV ? pulmonary aa

• To be compatible with life, mixing of the two
  circulations must occur via an ASD, VSD, or PDA.

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Transposition of the Great Arteries

• The CXR findings in TGA are typically less
  dramatic than in TOF.
• Pulmonary vasculature is typically increased.

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Blue Baby (R ? L shunt)

• Hypoxia and cyanosis (unresponsive to oxygen) in
  the neonatal period suggests a ductus-dependent
  lesion.
• Treatment is a prostaglandin-E1 (PGE1) infusion.
• Dosing discussed momentarily
• This should obviously be accompanied by urgent
  Peds Cardiology and consultation.

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Grey Baby (LVOTO)
X

• Left-ventricular outflow tract obstructions
  (LVOTOs) lead to cyanosis, acidosis, and shock
  early in the neonatal period.
• Complete obstruction is universally fatal unless
  shunting occurs through an ASD, VSD, or PDA.
• Examples of these lesions include
• Severe coarctation of the aorta
• Hypoplastic left heart syndrome (HLHS)

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Grey Baby (LVOTO)

• Treatment
• Any neonate presenting with shock unresponsive to
  fluids /- pressors has a LVOTO until proven
  otherwise.
• As with the Blue babies, appropriate management
  is an urgent PGE1 infusion and emergent
  consultation.

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Prostaglandin-E1

• PGE1 promotes ductus arteriosus patency.
• Use an IV infusion at 0.05-0.1 ug/kg/min.
• A response should be seen within 15 min.
• If ineffective, try doubling the dose.
• If effective, try halving the dose.
• The lowest possible dose should be used as
  adverse-effects of PGE1 can include
• - fever - flushing
• - diarrhea - periodic apnea (be ready to
  intubate)

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RDS

• Respiratory Distress Syndrome
• Immaturity of Lungs
• Need Surfactant
• Need Ventilation

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Reticugranular (Ground Glass) Air Bronchogram
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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine

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Endocrine

• Several endocrine emergencies can present in the
  neonatal period.
• We will briefly discuss three
• Hypoglycemia
• Thyrotoxicosis
• Congenital adrenal hyperplasia (CAH)

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Neonatal Hypoglycemia

• This is most commonly seen in the neonates born
  to diabetic mothers.
• During pregnancy, maternal hyperglycemia crosses
  the placenta to cause fetal hyperglycemia.
• The fetal pancreas responds by increasing insulin
  production.
• Following delivery, the hyperglycemic stimulus is
  instantly removedbut insulin production may take
  longer to slow down.

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Neonatal Hypoglycemia

• Neonatal hypoglycemia therefore typically arises
  in the nursery, but could still be seen in the
  ED.
• As with any patient, check a chem strip in any
  neonate presenting with
• - decreased LOC - weak tone
• - seizures - hypotension
• - resp distress - cardiac failure

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Neonatal Hypoglycemia

• Glucose lt 40(in a symptomatic neonate) or lt 30
  (in an asymptomatic neonate) should be treated.
• Can use 2cc/kg of D10W (repeated prn).
• This should be followed by
• Searching for an etiology (if not obvious)
• Repeated glucose monitoring

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Neonatal Thyrotoxicosis

• This is a very uncommon conditionoccurring in
  about 1 of pregnancies of mothers with Graves
  disease.
• The risk in babies born to euthryoid mothers is
  negligible.
• Caused by the placental passage of stimulating
  TSH-receptor antibodies from the mother to the
  fetus.

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Neonatal Thyrotoxicosis

• All neonates are screened for thyroid function at
  birth.
• As such, it would be unusual for neonatal
  thyrotoxicosis to present to the ED.
• That being said, potential findings could
  include
• - goiter - proptosis
• - HR gt 160 - dysrhythmias
• - shock - CHF
• - hyperthermia - pallor

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Neonatal Thyrotoxicosis

• Appropriate treatment (preferably in consultation
  with Peds Endo) includes
• Beta-blockade
• Propanolol 0.1mg/kg IV
• Blocking thyroxine production
• PTU 5-10mg/kg PO
• Blocking thyroxine release
• Potassium-iodide 1-4 drop PO
• Only give gt 1 hr after giving PTU
• Decreasing T4 ? T3 conversion
• Dexamethasone 0.1mg/kg IV

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Congenital Adrenal Hyperplasia

• CAH refers to any one of several
  autosomally-inherited disorders.
• These disorders involve a defect in the adrenal
  production of cortisol, mineralocorticoid, or
  both.
• These defects are caused by a missing or
  malfunctioning enzyme.
• 21-hydroxylase deficiency accounts for 90-95
  of all cases
• It wil be the focus of this section.

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Epidemiology

• 21-hydroxylase deficiency is present in 1 in
  3500 births.
• Because of variable penetrance, clinical effects
  in the neonate are probably seen in only 1 in
  10,000-20,000 births.
• The incidence of CAH can be 10-100 fold higher in
  certain populations.
• e.g. Ashkenazi Jews

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CAH

• Lack of 21-hydroxylase causes
• A build-up of the immediate precursor
  (17-hydroxyprogesterone).
• Instead of being converted into cortisol, this
  precursor is converted into androgens.
• 2. Another precursor metabolite, progesterone,
  is prevented from being converted into
  aldosterone.

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21-hydroxylase deficiency

• A highly-simplified schematic is shown here.

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21-hydroxylase deficiency

• Because of variable penetrance of the disease, a
  neonate with 21-hydroxylase deficiency may
  present with any or all of the following

1. Cortisol deficiency ? hypoglycemia, hypotension,
   and shock.

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21-hydroxylase deficiency

• Because of variable penetrance of the disease, a
  neonate with 21-hydroxylase deficiency may
  present with any or all of the following
• 2. Aldosterone deficiency ? hyponatremia,
  hyperkalemia, and dehydration.

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21-hydroxylase deficiency

• Because of variable penetrance of the disease, a
  neonate with 21-hydroxylase deficiency may
  present with any or all of the following
• 3. Androgen excess ? virilization of female
  genitalia.

- Male genitalia are typically unaffected at
birthbut may be unusually small.
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CAH

• Other forms of CAH may present similarly to
  21-hydroxylase deficiency
• However, because of different defects, findings
  may also include
• Androgen deficiency
• ? potentially causing lack of virilization
  of male genitalia.
• Mineralocorticoid excess
• ? potentially causing hypertension and
  hypokalemia.

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Treatment of CAH

• Patients suspected of 21-hydroxylase deficiency
  should have the following bloodwork sent
• Electrolytes
• Glucose
• 17-hydroxyprogesterone levels
• Cortisol levels
• Aldosterone and renin levels.

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Treatment of CAH

• After drawing appropriate bloodwork
• Pts with dehydration, hyponatremia, or
  hyperkalemia should receive a bolus of isotonic
  crystalloid to restore volume.
• Hypoglycemic patients should receive a dextrose
  bolus /- infusion.
• Pts suspected of adrenal insufficiency should be
  treated with steroids empirically (i.e. rather
  than waiting for the results of confirmatory
  studies).

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Treatment of CAH

• When administering steroids
• Use an initial dose of HC 1-2 mg/kg IV (followed
  by q6h dosing)
• The disadvantage of hydrocortisone is that it
  will confound any ACTH-Stim testing.
• The advantage of hydrocortisone is that it is a
  complete steroidwith both glucocorticoid and
  mineralocorticoid activity.

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances

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Metabolic disturbances

• Remember to send the following bloodwork in any
  toxic neonate
• LFTs and coagulation parameters
• Lipase
• Ammonia
• Lactate levels
• pH
• Okay... moving on.

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism

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Inborn Errors of Metabolism

• The inborn errors of metabolism are a group of
  diverse disordersusually caused by the lack of a
  specific enzyme.
• They include
• Urea Cycle Defects
• Organic Acidurias
• Galactosemias
• These disorders are rareaffecting only 1 in
  100,000-200,000 live births.
• Unfortunately, they typically present during the
  neonatal periodand cause irreparable brain
  injury if missed.

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism
• Sepsis
• Formula
• Intestinal
• Toxins
• Seizures

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Sepsis

• Much too broad a topic to tackle here.
• I will discuss five guidelines pertinent to the
  ED resuscitation of pediatric severe sepsis
  and/or septic shock.
• Based on Pediatric recommendations from the 2003
  Surviving Sepsis Campaign.

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Sepsis

• Early intubation.
• Neonates with severe sepsis may benefit from
  early mechanical ventilation.
• Similar lung protective strategies apply to the
  neonate once he or she is on the ventilator.
• High FiO2s should be avoided in premies (if at
  all possible) to prevent retinopathy

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Sepsis

• Aggressive fluid resuscitation.
• This is fundamental to survival in pediatric
  septic shock.
• There is no strong evidence for the use of
  colloid over crystalloid (or vice-versa)
• Use 10mL/kg IV bolus(es) of crystalloid prn given
  over 5-10mins.

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Sepsis

• Vasopressors/inotropes should be used in septic
  shock only after appropriate volume
  resuscitation.
• If required, the appropriate vasopressor
  /inotrope will depend on assessment of CO and SVR.

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Sepsis

• Endpoints to resuscitation
• Normal LOC
• Cap refill lt 2 sec
• Normal pulses
• Warm extremities
• Urine output gt 1mL/kg/hr
• Decreased lactate
• Increased pH

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Sepsis

• 5) Steroids.
• No clear consensus for the role of steroids in
  pediatric septic shock.
• Should be reserved for refractory hypo-tension or
  known adrenal insufficiency.
• Use Hydrocortisone 1-2mg/kg IV (followed by q6h
  dosing as per Peds Endo).

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism
• Sepsis
• Formula and Feeding

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Formula and Feeding Mishaps

• Neonates are ideally breastfed.
• The neonatal breastfeeding schedule should be
  on-demand.
• 10mins/breast, q3h, but variable.
• The neonate should be allowed ample time to fully
  drain each breast.
• The final dregs of breastmilk (or hind-milk)
  are felt to contain more fat.
• The mothers breasts should feel empty
  following a full feed.

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Formula and Feeding Mishaps

• If formula is used, the feeding schedule is
  approximately the same
• ie. on demand, q3h, highly variable.
• Hard to make mistakes with jarred baby food (i.e.
  insert into baby).
• With powdered formula, make sure it is being
  mixed appropriately.
• Typically a 11 ratio.

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Formula and Feeding Mishaps

• Irrespective of the type of feeding, neonatal
  signs of satiety should be sought after a meal.
• These can include
• Decreased irritability.
• Falling asleep.
• No longer rooting or sucking.

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Formula and Feeding Mishaps

• Always ask about potentially dangerous feeding
  behaviours
• Feeding the neonate inappropriate substances
  (i.e. pop, juice, Coffeemate).
• Feeding the neonate water.
• Neonates should not get any
• supple-mentary water until 6 months (or older)
• Water lacks nutritional content but causes
  satiety ? decreased food intake.
• Lack of money to afford proper food.

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism
• Sepsis
• Formula
• Intestinal

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Intestinal Emergencies

• At last count, there were 1 million pediatric
  intestinal emergencies.
• I will focus on one such emergency specific to
  the neonate necrotizing enterocolitis (NEC).
• NEC is characterized by the mucosal or
  transmucosal necrosis of part of the intestine.
• The exact etiology is unknown.

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Necrotizing Enterocolitis

• NEC is the most common intestinal
  medical/surgical neonatal emergency.
• It affects 1-2 neonates per 1,000 births.
• Most commonly seen in preemiesbut can also be
  seen in term neonates.
• Mortality ranges from 0-100 depending on the
  stage of prematurity and severity of disease.

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Necrotizing Enterocolitis

• Premies remain at risk for NEC for several weeks
  after birth.
• Onset is typically while the neonate is on
  advancing enteral feeds.
• With term babies, the onset of NEC is generally
  in the first 1-3 days of life.
• Term neonates who get NEC are usually already ill
  from another condition.
• e.g. congenital heart disease, lung disease,
  metabolic abnormalities, etc.

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Necrotizing Enterocolitis

• Initial symptoms can include
• feeding intolerance
• abdominal distension
• /- hematochezia
• This can progress to
• lethargy
• apnea
• DIC
• shock and cardiovascular collapse

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Necrotizing Enterocolitis

• Lab findings can include
• abnormal WBC
• thrombocytopenia
• hyponatremia
• elevated PT/PTT
• metabolic acidosis

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Necrotizing Enterocolitis

• The mainstay of ED imaging studies is the
  abdominal X-ray.
• It can reveal a variety of non-specific findings
  including
• dilated bowel loops.
• thickened bowel walls.
• However, the finding of pneumatosis intestinalis
  is pathognomonic for NEC.

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• Pneumatosis intestinalis is the presence of air
  bubbles within the bowel wall (caused by
  extravasation of air from the lumen.)
• It has a characteristic train-track lucency
  appearance on AXR.

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Necrotizing Enterocolitis

• Free air is not pathognomonic for NEC.
• In the context of NEC, however, free air
  indicates the need for urgent surgery.
• Free air in the neonate may be best detected with
  a left lateral decubitus (ie. left side down)
  view.
• Look for free air above the liver shadow.

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Intestinal Emergencies

• Management of NEC depends on the severity of
  illness.
• The Bell staging criteria have been developed to
  help guide therapy.
• Stage 1 Suspected disease
• Mild, non-specific clinical and radiological
  findings.
• /- grossly bloody stool.
• Treatment is NPO and antibiotics for 3d.

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Intestinal Emergencies

• Management of NEC depends on the severity of
  illness.
• The Bell staging criteria have been developed to
  help guide therapy.

• Stage 2 Definite disease (Medical NEC)
• Pt is mildly to moderately ill
• Findings can include abdominal pain, mild
  acidosis, pneumatosis intestinalis
• - Treatment is NPO and antibiotics for 7 - 14
  days

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Intestinal Emergencies

• Management of NEC depends on the severity of
  illness.
• The Bell staging criteria have been developed to
  help guide therapy.

• Stage 3 Severe disease (Surgical NEC)
• Pt has severe findings that can include gross
  peritonitis, hematological abnormalities, free
  air, et cetera.
• - Treatment is NPO x 14d, ICU support, /-
  paracentesis or open laparotomy.

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism
• Sepsis
• Formula
• Intestinal
• Toxins

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Toxins

• Theres lots of them.
• It takes less of them to kill a neonate.
• Remember to consider
• Maternal toxins (ie. if breastfeeding).
• Environmental toxins (eg. cigarette smoke, carbon
  monoxide).
• Abuse (ie. Munchausen by proxy).

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THE MISFITS

• Trauma/Abuse (NAI)
• Heart and Lung
• Endocrine
• Metabolic disturbances
• Inborn errors of metabolism
• Sepsis
• Formula
• Intestinal
• Toxins
• Seizures

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Seizures

• There have been few striking new developments in
  the world of acute neonatal seizure management.
• First line medications remain BZPs.
• Phenobarb is preferred as a 2nd-line agent over
  phenytoin in patients under the age of 1-2 yrs.
• Phenytoin has a myocardial depressant effect and
  unpredictable metabolism in the neonate.

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Seizures

• Other investigations and management strategies
  include
• Stat ABG/VBG (with lytes, Hb, lactate).
• Full set of labwork including LFTs, ammonia,
  urine and blood cultures.
• Empiric antibiotics /- Acyclovir prn.
• Head U/S or CT

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Seizures

• There are several forms of benign neonatal
  convulsionsbut these are unlikely to be
  diagnosed.
• Remember that the differential of seizures in a
  neonate essentially includes all of the other
  MISFITS.

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Trisomies

• Trisomy 21 (Downs Syndrome)
• Trisomy 18 (Edwards Synd)
• Trisomy 13 (Pataus Synd)

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Putting It All Together

• Basics.
• IV, O2, monitors, vitals, foley, /- NG
• 2. Stat chem strip and ABG/VBG (with the works).
• 3. Stat EKG and CXR.

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Putting It All Together

• 4. Labwork.
• If possible, draw bloodwork at the start of the
  resuscitation.
• Remember blood and urine cultures.
• Remember ammonia and lactate levels.
• Both of these must be stored on ice and rushed to
  lab.
• Remember to draw a red-top tube if an inborn
  error of metabolism is suspected.

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Putting It All Together

• Administer empiric antibiotics.
• Make sure all cultures are drawn first.
• LPs are typically postponed in the acute phase.
• A reasonable empiric regimen might be
• Ampicillin 100mg/kg IV
• Cefotaxime 50/mg kg IV
• Acyclovir 20mg/kg IV

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Putting It All Together

• Examine EKG.
• If a life-threatening rhythm is present, treat as
  per PALS guidelines.
• Examine sats.
• If sats are lt85 despite 100 O2 (especially if
  centrally cyanotic), start a PGE1 infusion at
  0.05-0.1ug/kg/min.

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Putting It All Together

• CHF suspected?
• If CXR or clinical examination reveal evidence of
  CHF, give Lasix 1mg/kg IV.
• Consult Peds Cardio regarding
• Use of digoxin, nitrates, inotropes.
• Admission.

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Putting It All Together

• NAI suspected?
• Order a skeletal survey /- CT of the
  head/ab/pelvis.
• 10. Clinical jaundice or bilirubin gt 340?
• Arrange admission for phototherapy.

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Putting It All Together

• Inborn error of metabolism suspected?
• If not already done, draw a red-top tube.
• Begin IV fluids and glucose.
• Consult Peds Metabolics.

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Putting It All Together

• CAH suspected?
• Draw cortisol, 17-hydroxyprogesterone,
  aldosterone, and renin levels.
• Begin IV fluids and glucose.
• Treat elevated K as necessary.
• Give hydrocortisone 1-2mg/kg IV.

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Putting It All Together

• Suspicion of NEC or history of bilious
  vomiting?
• Order an abdominal x-ray.
• Consult Peds GI or Peds Surgery.

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Putting It All Together

• 14. Continue resuscitation as necessary.
• Consider additional fluid bolus(es).
• Consider inotrope/pressor support.
• Consider PRBC 5-10mg/kg (if blood loss is
  suspected).
• Consult as appropriate.

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THANKS A LOT