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Definition

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  • 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.

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Affected System

Approx. % of Cases (in third trimester)

Examples

Cardiac

20%

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

Myocarditis

Renal

5%

Nephrosis

Renal hypoplasia/aplasia

Renal vein thrombosis

Obstructive uropathies

Infection

8%

Toxoplasmosis

Herpes simplex virus

Syphilis

Adenovirus

Rubella

CMV

Hepatitis

Parvovirus

Pulmonary

5%

Congenital chylothorax

Pulmonary lymphangiectasia

Congenital diaphragmatic hernia

Cystic adenomatoid malformations

Other intrathoracic masses (eg, pulmonary sequestration) that cause compression of thoracic blood vessels → obstructive venous flow → hydrops

Placenta/cord

Rare

Chorangioma

Arteriovenous malformation

Significant cord compression

Umbilical vein thrombosis

True umbilical cord knot

Maternal conditions

5%

Diabetes mellitus

Severe preeclampsia/ eclampsia

Hyperthyroidism

Gastrointestinal

5%

In utero midgut volvulus

Bowel atresias

Chromosomal

10%

Turner syndrome

Aneuploidy

Trisomies 13, 18, 21

Noonan Syndrome

Miscellaneous

10%

Congenital myopathies

Inborn errors of metabolism

CNS malformations

Skeletal dysplasias

Abdominal neoplasms

Unknown

20%

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Diagnosis

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  • Increased uterine size for dates
  • Decreased fetal movements
  • Generalized maternal edema (mirror syndrome)
  • Polyhydramnios (AFI >24)
  • Placentomegaly

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Management

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  • 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).

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System

Potential Difficulties/Management

Pulmonary

  • 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.

Cardiovascular

  • 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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