- Presence of excess extracellular fluid in at least two fetal compartments (ascites, pleural effusion, pericardial effusion, skin edema, polyhydramnios) without any identifiable circulating antibody to red-cell antigens.
- For a discussion on immune-mediated disease, see Chapter 37.
- Prevalence is estimated at 1:1500-4000.
- Highest prevalence in Southeast Asian population.
Approx. % of Cases (in third trimester)
Fetal arrhythmias (bradyarrhythmias and tachyarrhythmias)
Structural cardiac defects causing congestive failure
Extrinsic compression of heart causing low output states → leads to fetal tachyarrhythmia → high-output failure → hydrops
Renal vein thrombosis
Herpes simplex virus
Congenital diaphragmatic hernia
Cystic adenomatoid malformations
Other intrathoracic masses (eg, pulmonary sequestration) that cause compression of thoracic blood vessels → obstructive venous flow → hydrops
Significant cord compression
Umbilical vein thrombosis
True umbilical cord knot
Severe preeclampsia/ eclampsia
In utero midgut volvulus
Trisomies 13, 18, 21
Inborn errors of metabolism
- Increased uterine size for dates
- Decreased fetal movements
- Generalized maternal edema (mirror syndrome)
- Polyhydramnios (AFI >24)
- Infants with nonimmune hydrops are at very high risk for fetal demise.
- Intrauterine therapy is aimed at treating underlying causes (maternal digitalis therapy for fetal tachyarrhythmias); if this is not possible, the risks of intrauterine death versus premature delivery have to be weighed.
- By organ system (see table below).
- Difficult intubation due to severe edema of head/neck/oropharynx. → Consider ENT or anesthesia support for intubation as needed.
- Varying degrees of pulmonary hypoplasia due to large pleural effusions or other extrinsic in utero compression of lungs → May need emergent thoracentesis in delivery area to alleviate lung compression from large effusions.
- Chest tube placement for rapidly reaccumulating pleural effusions.
- Hypotension should be treated with appropriate inotropic support.
- Remember that most hydropic infants are euvolemic intravascularly, often with depressed cardiac function → Avoid large fluid shifts.
- Pericardiocentesis may be necessary if cardiac tamponade from pericardial effusion is suspected → Should ideally be done under US guidance.
- Arterial access is helpful to follow invasive blood pressures.
- Echocardiogram should be obtained to evaluate for structural abnormalities as a cause of hydrops.
Fluids and electrolytes
- Infants are total body fluid overloaded but are usually euvolemic (intravascular status).
- Fluid intake should be based on a “dry” weight (ie, the 50th percentile for gestational age).
- Fluids should be restricted (40–60 mL/kg/day) to avoid further fluid overload and to allow diuresis.
- Vigilant attention to ...
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