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Definition and Incidence

  • Hydrops fetalis is a condition of excess fluid accumulation.
  • It is characterized by two or more abnormal fetal fluid collections.
  • NIHF occurs in every 1/1500 to 1/4000 births.
  • Chromosomal abnormalities are the most common cause.
  • The mortality rate for fetuses diagnosed with NIHF is 50–80%.
  • The mortality rate for infants born with NIHF is 50%.

Etiologies of NIHF

  • More than 80 conditions are known to cause NIHF.
    • Cardiovascular abnormalities
    • Placental malformations
    • Hematologic problems
    • Congenital infections
    • Non-cardiac congenital abnormalities
    • Chromosomal abnormalities
    • Genetic syndromes
    • Miscellaneous or idiopathic causesEtiologies include:

Cardiovascular Abnormalities

  • Congenital heart malformations
    • Atrioventricular septal defects
    • Hypoplastic left and right heart
    • Tetralogy of Fallot
    • Ebstein’s anomaly
    • Truncus arteriosis
  • Fetal arrhythmias
    • Atrial flutter
    • Long QT interval
    • Supraventricular tachycardia
    • Bradyarrythmias (heart block)
    • Wolff-Parkinson-White Syndrome
  • Premature closure of the foramen ovale
  • Premature closure of the ductus arteriosis
  • Arteriovenous malformations
  • Cardiac or vascular hemangiomas or tumors

Placental Malformations

  • Acardiac twin
  • Twin-to-twin transfusion syndrome

Hematological Problems

  • Hemolytic anemia
  • Alpha-thalassemia
  • Fetomaternal transfusion
  • In utero hemorrhage
  • Red cell enzyme deficiencies
  • Pyruvate kinase deficiency

Congenital Infections

  • Parvovirus
  • Cytomegalovirus
  • Toxoplasmosis
  • Herpes Simplex
  • Syphilis
  • Rubella
  • Coxsackievirus
  • Leptospirosis
  • Listeria

Non-Cardiac Congenital Abnormalities

  • Thoracic abnormalities
    • Diaphragmatic hernia
    • Cystic adenomatoid mass
    • Pulmonary sequestration
  • Lymphatic disorders
    • Congenital chylothorax
    • Cystic hygromas
  • Neurological disorders
    • Fetal intracranial hemorrhage
    • Fetal hydrocephalus
    • Arnold-Chiari malformations
  • Gastrointestinal malformations
    • Volvulus
    • Malrotation
    • Intussusception
    • Meconium ileus
    • Polycystic kidney disease
    • Renal vein thrombosis
    • Posterior urethral valves
    • Prune belly syndromeGenitourinary malformations

Chromosomal Abnormalities

  • Trisomy 21
  • Trisomy 18
  • Trisomy 13
  • Monosomy 45, X
  • Duplicated 11p

Genetic Syndromes

  • Achondrogenesis
  • Chondrodysplasia
  • Arthrogryposis
  • Inborn errors of metabolism
  • Myotonic dystrophy
  • Noonan’s syndrome
  • Osteogenesis imperfecta
  • Tuberous sclerosis

Idiopathic Causes

  • Miscellaneous causes of NIHF include:
    • Fetal tumors
    • Intrauterine growth restriction
    • Fetal hypomotility
  • The etiology of 50–70% of NIHF cannot be determined.
  • NIHF not associated with a specific cause is called idiopathic.

Pathophysiology

  • The primary factor in the development of NIHF is abnormal fluid movement between fetal plasma and tissues.
  • Fluid accumulates in fetal tissues or body cavities.
    • Production of fluid exceeds rate of reabsorption.
    • Production of fluid exceeds rate of removal by capillary and lymphatic systems.
  • Four theories have been identified as possible causes:
    • Increase in hydrostatic capillary pressure
    • Reduction in plasma osmotic pressure
    • Obstruction of lymphatic flow
    • Damage to peripheral capillary integrity

Increase in Hydrostatic Capillary Pressure

  • Hydrostatic pressure within capillaries rises:
    • In response to increased central venous pressure
    • In response to increased arterial pressure
    • In response to obstructed venous drainage
  • Results from primary or secondary heart failure.
  • Results from obstruction of venous return.
  • Increased capillary pressure causes excessive filtration of fluid into the interstitial tissues.

Reduction in Plasma Osmotic Pressure

  • Plasma or intravascular pressure is decreased:
    • In response to an inadequate volume of fluid
    • In response to a loss of filtration pressure
    • In response to decreased fluid reabsorption
  • Results from decreased albumin production.
  • Results from increased albumin loss:
    • Capillary leak
    • Chylothorax
    • Nephrotic syndrome
  • Decreased plasma or intravascular pressure causes significant loss of fluid volume into interstitial spaces

Obstruction of Lymphatic Flow

  • Lymphatic flow is obstructed:
    • In response to external compression
    • In response to elevated central venous pressure
    • In response to reduced lymph flow
  • Results from congenital malformations or masses.
  • Results from congestive heart failure.
  • Results from fetal hypomotility.
  • Obstruction or reduction of lymph flow causes an ineffective clearance of excess interstitial fluid.

Damage to Peripheral Capillary Integrity

  • Peripheral capillary integrity is damaged:
    • In response to hypoxic damage to cell walls
    • In response to inflammatory mediator release
    • In response to endotoxin release due to sepsis
  • Results from fetal anemia.
  • Results from uteroplacental insufficiency.
  • Results from maternal infectious disease.
  • Damage to capillaries increases capillary wall permeability allowing excess fluid to leak into interstitial spaces.

Clinical Presentation and Diagnosis

  • A fetus or infant with NIHF presents with abnormal fluid accumulations.
  • Diagnosis of NIHF is based on ultrasound.
  • It is identified by the presence of two or more findings:
    • Ascites
    • Pleural effusions
    • Pericardial effusions
    • Skin edema
    • Polyhydramnios
    • Placentomegaly
  • Uterine size may be large for dates.
  • Decreased fetal movement may be present.

Laboratory Evaluation

  • Maternal evaluation
    • CBC with RBC indices
    • Hemoglobin electrophoresis
    • Blood type and antibody screen
    • Serologies
      • Cytomegalovirus
      • Toxoplasmosis
      • Rubella
      • Parvovirus
      • Syphilis
    • Kleihauer-Betke acid elution
    • CBC with RBC indices
    • Hemoglobin electrophoresis
    • Blood type and antibody screen
    • TORCH studies via PCR
    • Parvovirus B19 via PCR
    • Karyotype
    • Genetic microarray
    • Metabolic evaluationFetal (or infant) evaluation

Prognosis and Outcome

  • NIHF is associated with a high mortality rate.
    • Overall 50–80% rate of mortality
  • Prognosis depends on the underlying etiology.
  • The earlier NIHF occurs, the poorer the outcome.
  • Pleural effusions and polydramnios <20 weeks
    • Poor outcomes
    • Pulmonary hypoplasia
    • Increased preterm labor
  • Risk or recurrence depends on underlying etiology.

Treatment and Clinical Management

  • Prenatal diagnosis allows adequate preparation.
    • Maternal–Fetal Medicine consultation
    • Neonatology consultation
    • Fetal echocardiogram if needed
    • Intrauterine therapies
    • Complex resuscitation planning
    • Improved infant outcomes
  • Initial infant management is aimed at decreasing effects of excess interstitial fluid on body function.
    • Supporting adequate ventilation
    • Supporting adequate oxygenation
    • Supporting adequate perfusion
  • Infants often present in respiratory failure.
  • Infants often present in hypovolemic shock.
  • Pleural effusions require drainage with chest tubes.
  • Ascites requires drainage via abdominal taps or drains.
  • Umbilical catheters should be placed.
  • Fluid resuscitation is critical.
    • Intravascular volume is depleted.
    • Immediate normal saline replacement
    • Inotropic and vasopressor support
  • Blood or exchange transfusions may be required.
    • If severe anemia is suspected
    • O-negative unmatched blood
  • Hyperbilirubinemia is common with severe anemia.
    • In utero anemia leads to massive RBC formation
    • Bilirubin levels rise as RBCs are destroyed
    • In utero, maternal circulation manages RBCs
    • At birth, hyperbilirubinemia occurs within 60 minutes
  • Severe hypoglycemia is common.
    • Occurs as soon as 15 minutes
    • Aggressive dextrose support may be needed
  • Fluids and medications should be based on dry weight.
    • Estimated at 50th percentile for gestational age
  • An immediate echocardiogram may be required.
  • Comfort measures are essential.
  • Pain management is also essential.
    • To support family coping mechanisms
    • To facilitate parent-infant bondingParent and family support should be a priority:

Intrauterine Therapies

  • Several fetal therapies have been developed.
    • Anemia – fetal intravascular blood transfusions
    • Arrhythmias – transplacental antiarrhythmics or pacing
    • Cardiac defects – in utero fetal heart surgery
    • Hypoalbuminemia – fetal intravascular albumin transfusions
    • Infection – transplacental antiviral or antibiotic therapy
    • Obstructive uropathy – fetal bladder shunt or ex utero surgery
    • Pleural effusions – fetal intrathoracic decompression
    • Pulmonary cysts – fetal intrathoracic shunt or ex utero surgery
    • Thoracic congenital anomaly – fetal ex utero surgery
      • Ex utero intrapartum treatment
      • Also known as EXIT procedure
    • Tumors – fetal in utero resection
    • Twin-to-twin transfusion syndrome
      • Serial amnioreduction
      • Fetal septostomy
      • Fetoscopic laser photocoagulation of vascular anastomoses

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