NONIMMUNE HYDROPS

Geetha B. Thippeswamy, MD

August 16th 2002

Neonatal presentation

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.

Transition of hydropic babies to extrauterine life

Decreased respiratory compliance and increased resistance:

1. Airway edema2. Chest wall edema3. Pulmonary edema4. RDS5. Pleural effusion, 6. Ascites 7. Pulmonary hypoplasia

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.

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

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.

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.

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.

Resuscitation team responsibilities

Airway/ventilation Circulation Catheters Equipment and medications Data recording Runner

Delivery room and Equipment

Temperature control.

1. Delivery room temp should be at least 75º F

2. Turn overhead warmer to full heater output

3. Clear plastic bag to cover the infant

4. Skin thermistor

5. Warm dry cap

6. Preheat oxygen to be used to 93º to 97ºF

Airway/Ventilation

Endotracheal tube of different sizes Flow inflating bags Flow of heated humidified oxygen at 5 to 8

L/min

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.

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.

Delivery room protocol

Avoid cold stress.1. Position infant under warmer with

servocontrol set to 96º to 98º F.2. Briefly dry and place a cap on the head.3. Cover infant with the clear plastic,

procedures performed by tearing small holes.

4. CR and pulse ox monitors attached.

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.

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.

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.

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.

Monitor

Continuously monitor success of resuscitation by assessing,

1. Adequate breath sounds

2. Heart rate

3. Oxygen saturation If the response is suboptimal, consider

abdominal paracentesis.

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.

Thoracentesis

Proceed to thoracentesis if the response to paracentesis is suboptimal.

Thoracentesis helps only in the presence of normal lungs.

Thoracentesis

Thoracentesis may not be helpful if lung compliance is decreased as in,

1. Pulmonary hypoplasia sec to large and long standing effusion is present.

2. Lung is surfactant deficient.3. Pulmonary edema Pneumothorax in the presence of pulmonary

hypoplasia.

Transfer to ICN

Infants are transferred to ICN only when they are,

1. Stable

2. ET tube and the catheters have been secured.

ICN management

Respiratory: Mechanical ventilation. HFOV and NO therapy may be needed

Chest tubes for persistent pleural effusion.

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.

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.

ICN management

Fluid and electrolyte administration guided by monitoring:

1. Urine and serum sodium levels

2. Strict daily I/O

3. Daily weights Most of these infants loose 15 to 30% of

their body weight.

ICN management

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

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.

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.

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.

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)

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.

NIHF prognosis

Idiopathic variety have the best prognosis. Good prognosis with:

1. Anemia that can be treated in utero or in newborn period; >90% survive.

2. Isolated arrhythmia ; > 50% survive.

NIHF prognosis

Poor prognosis associated with:

1. Prematurity

2. Pleural effusion with pulmonary hypoplasia.

3. Chromosomal disorders

4. Structural malformations.

5. Severe hydrops.

Thank you