NONIMMUNE HYDROPS

1. NONIMMUNE HYDROPS Geetha B. Thippeswamy, MD August 16th 2002

2. Neonatal presentation

3. 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.

4. 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

5. 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.

6. 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

7. 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.

8. 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.

9. 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.

10. Resuscitation team responsibilities • Airway/ventilation • Circulation • Catheters • Equipment and medications • Data recording • Runner

11. 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

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

13. 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.

14. Other.. • Blood: O neg, PRBCs cross matched against mother’s blood. • Cardio respiratory monitor. • Pulse ox monitor. • Portable radiography equipment. • Defibrillator or equipment for ventricular pacing.

15. 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.

16. 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.

17. 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.

18. 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.

19. 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.

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

21. 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.

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

23. 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.

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

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

26. 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.

27. 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.

28. 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.

29. ICN management • Cardiovascular: Shock sec to hypovolemia. • Maintain adequate intravascular volume. • Ionotropic support.

30. 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.

31. 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.

32. Evaluation of NIH infant with unknown cause • CVS: Echo and electrocardiogram • Pulm: CXR, pleural fluid analysis • Hemat: cord blood studies and PBS • GI:U/S of abdomen, LFTs, peritoneal fluid analysis.

33. 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)

34. 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.

35. NIHF prognosis • Idiopathic variety have the best prognosis. • Good prognosis with: • Anemia that can be treated in utero or in newborn period; >90% survive. • Isolated arrhythmia ; > 50% survive.

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

37. Thank you