NONIMMUNE HYDROPS

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NONIMMUNE HYDROPS

• Geetha B. Thippeswamy, MD
• August 16th 2002

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Neonatal presentation
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Transition of hydropic babies to extrauterine life

• Understanding this is very important in planning
  the resuscitation of the hydropic newborn
• Hydropic babies often display signs of
  intrapartum asphyxia at birth
• No respiratory effort or have a poor effort.

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Transition of hydropic babies to extrauterine life

• Decreased respiratory compliance and increased
  resistance
• Airway edema
• Chest wall edema
• Pulmonary edema
• RDS
• Pleural effusion,
• Ascites
• Pulmonary hypoplasia

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Transition of hydropic babies to extrauterine life

• Hypoxia and acidosis sec to gas exchange
  compromise.
• Hypoxia decreases cardiac function.
• PPHN sec to hypoxia.
• PPHN worsens vent perfusion matching and
  hypoxemia that is minimally responsive to
  supplemental oxygen.

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Things to do when consulted

• Review antepartum and intrapartum history
• 1. Maternal history
• 2. Past obstetric history
• 3. Present pregnancy history
• 4. Diagnostic evaluations
• 5. Labor

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Counsel parents

• Meet with parents before delivery.
• Explain in the language they understand.
• Inform them about the fetal condition and the
  prognosis.
• Explain the delivery room resuscitation and
  potential procedures to be performed.
• Genetic consult.

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Delivery and resuscitation

• Hydropic babies should be delivered in Tertiary
  care centers.
• Coordinated and aggressive delivery room
  resuscitation is very important.
• Personnel and equipment required for
  resuscitation exceeds the general Neonatal
  resuscitation recommendations of AAP and AHA.

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Resuscitation team

• Size of the resuscitation team is considerably
  larger.
• Six to seven people with a variety of tasks
  assigned form the team and will be present in the
  delivery room.
• An experienced neonatologist should orchestrate
  all resuscitation activities.

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Resuscitation team responsibilities

• Airway/ventilation
• Circulation
• Catheters
• Equipment and medications
• Data recording
• Runner

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Delivery room and Equipment

• Temperature control.
• Delivery room temp should be at least 75º F
• Turn overhead warmer to full heater output
• Clear plastic bag to cover the infant
• Skin thermistor
• Warm dry cap
• Preheat oxygen to be used to 93º to 97ºF

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Airway/Ventilation

• Endotracheal tube of different sizes
• Flow inflating bags
• Flow of heated humidified oxygen at 5 to 8 L/min

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Catheters

• Prepare for umbilical artery and umbilical vein
  catheterization.
• Transducers for arterial and venous pressure
  monitoring.
• Equipment for drawing and transporting blood
  gases.
• A sterile tray for paracentesis, thoracentesis.

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Other..

• Blood O neg, PRBCs cross matched against
  mothers blood.
• Cardio respiratory monitor.
• Pulse ox monitor.
• Portable radiography equipment.
• Defibrillator or equipment for ventricular pacing.

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Delivery room protocol

• Avoid cold stress.
• Position infant under warmer with servocontrol
  set to 96º to 98º F.
• Briefly dry and place a cap on the head.
• Cover infant with the clear plastic, procedures
  performed by tearing small holes.
• CR and pulse ox monitors attached.

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Airway/Ventilation

• Respiratory efforts are depressed or ineffective.
• Suction the mouth and nose. Tracheal suctioning
  if amniotic fluid is meconium stained.
• Bag and mask ventilation is extremely difficult.

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Airway/Ventilation

• IMMEDIATE INTUBATION IS RECOMMENDED in all
  hydropic infants.
• Depth of ET tube insertion based on position of
  the tube at the vocal cords and symmetry of
  breath sounds on auscultation.

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Airway/Ventilation

• Positive pressure ventilation is initiated using
  peak pressures.
• Pressures used should provide sufficient tidal
  volume.
• Tidal volume is assessed by chest wall motion and
  breath sounds.
• Surfactant administered in premature infants.

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Vascular Access

• Place UVC and UAC.
• Attach pressure transducers.
• Obtain blood sample for blood gas and hematocrit
  analysis.
• Infuse glucose at 8 to 10 mg/kg/min to avoid
  hypoglycemia.
• A-P radiograph obtained to confirm ET tube and
  catheter placement.

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Monitor

• Continuously monitor success of resuscitation by
  assessing,
• Adequate breath sounds
• Heart rate
• Oxygen saturation
• If the response is suboptimal, consider abdominal
  paracentesis.

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Abdominal paracentesis

• This improves cardiac and respiratory functions.
• Just remove enough fluid to improve chest wall
  motion.
• Excess fluid removal could precipitate
  hypovolemic shock.
• 18 to 20 gauge iv catheter with stylet is
  preferred.

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Thoracentesis

• Proceed to thoracentesis if the response to
  paracentesis is suboptimal.
• Thoracentesis helps only in the presence of
  normal lungs.

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Thoracentesis

• Thoracentesis may not be helpful if lung
  compliance is decreased as in,
• Pulmonary hypoplasia sec to large and long
  standing effusion is present.
• Lung is surfactant deficient.
• Pulmonary edema
• Pneumothorax in the presence of pulmonary
  hypoplasia.

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Transfer to ICN

• Infants are transferred to ICN only when they
  are,
• Stable
• ET tube and the catheters have been secured.

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ICN management

• Respiratory Mechanical ventilation. HFOV and NO
  therapy may be needed
• Chest tubes for persistent pleural effusion.

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ICN management

• Fluid and Electrolytes Primary goal is
  resolution of hydrops.
• Maintenance fluids should be restricted.
• Bolus fluids for inadequate intravascular volume.
• Avoid sodium initially.

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ICN management

• Fluids and electrolytes cont..
• Initiate diuresis with 25 albumin or diuretics.
• Albumin infused only if CVP is low or normal.
• Diuretics given only when CVP is high.

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ICN management

• Fluid and electrolyte administration guided by
  monitoring
• Urine and serum sodium levels
• Strict daily I/O
• Daily weights
• Most of these infants loose 15 to 30 of their
  body weight.

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ICN management

• Cardiovascular Shock sec to hypovolemia.
• Maintain adequate intravascular volume.
• Ionotropic support.

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ICN management

• Hyperbilirubinemia in anemic infants.
• Develops within 30 to 60 mins after birth.
• Phototherapy and exchange transfusion based on
  bilirubin levels.
• Anemia PRBC transfusion or partial exchange
  transfusion.

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ICN management

• Supportive care as appropriate, especially for
  the premature infants.
• Evaluation of the newborn if cause of NIH is not
  known.
• Specific therapy based on underlying etiology of
  NIHF when possible.
• Asses parental needs and encourage them to
  participate in the care.

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Evaluation of NIH infant with unknown cause

• CVS Echo and electrocardiogram
• Pulm CXR, pleural fluid analysis
• Hemat cord blood studies and PBS
• GIU/S of abdomen, LFTs, peritoneal fluid
  analysis.

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Evaluation of NIH infant with unknown cause

• Renal UA, BUN, Cr.
• Genetic Chromosomal analysis, skeletal
  radiographs, genetic consultation.
• Cong infections Viral cultures or serology
• Pathologic Placental examination, autopsy(in
  case of neonatal death)

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NIHF Prognosis

• Prognosis is very poor with high rate of
  morbidity and mortality
• Perinatal mortality 50 to 90
• 50 of cases diagnosed in utero result in fetal
  death.
• 50 of all live born infants die.

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NIHF prognosis

• Idiopathic variety have the best prognosis.
• Good prognosis with
• Anemia that can be treated in utero or in newborn
  period gt90 survive.
• Isolated arrhythmia gt 50 survive.

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NIHF prognosis

• Poor prognosis associated with
• Prematurity
• Pleural effusion with pulmonary hypoplasia.
• Chromosomal disorders
• Structural malformations.
• Severe hydrops.

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Thank you