Hydrops fetalis

Hydrops fetalis is excessive extravasation of fluid into the third space in a fetus which could be due to heart failure, volume overload, decreased oncotic pressure, or increased vascular permeability. Hydrops is defined as the accumulation of fluid +/- edema involving at least two fetal components, which may manifest as:

• fetal pleural effusion
• fetal pericardial effusion
• fetal ascites
• generalized body edema: fetal anasarca/nuchal edema/cystic hygroma
• placental enlargement
• polyhydramnios
• hepatomegaly 

Epidemiology

The estimated incidence is at ~1 in 2000 births although this can significantly vary according to different regions.

Pathology

It is considered a prenatal form of cardiac failure. It was traditionally divided into two broad groups:

• immune hydrops fetalis (IHF)
• non-immune hydrops fetalis (NIHF)

Etiology

Immune hydrops

Accounts for a minority (~10%) of cases in present-day pregnancies , although historically accounted for a larger proportion of cases in the early to mid 20 century:

• fetomaternal blood group incompatibility (including rhesus incompatibility): erythroblastosis fetalis

Non-immune hydrops

Accounts for a majority of cases and can arise from protean causes which include:

• chromosomal anomalies
  • Turner syndrome
  • trisomies
    • trisomy 13
    • trisomy 18
    • trisomy 21
• cardiac causes
  • abnormalities in fetal heart rate: e.g. fetal tachyarrhythmias
  • congenital cardiac anomalies
  • fetal cardiac tumors: cardiac rhabdomyoma
• twin pregnancy-related complications
  • twin to twin transfusion syndrome: in the recipient twin
  • twin reversed arterial perfusion sequence: in pump twin
• in utero infection(s)
  • congenital viral infection(s)
    • TORCH group
    • fetal parvovirus B19 infection: considered the most common infectious cause of hydrops; the mechanism is that of anemia
    • coxsackie viral infection
• fetal tumors capable of producing significant AV shunts
  • sacrococcygeal teratoma
  • hepatic hemangioendothelioma
  • placental chorioangioma
• inborn errors of metabolism
  • Gaucher disease
  • Niemann-Pick disease
• fetal hypoproteinemic states
  • congenital nephrotic syndrome
• congenital/fetal anemia(s)
  • hemoglobin Bart's (a.k.a. Hb Bart's hydrops fetalis syndrome) 
    • cause of alpha thalassemia 
    • relatively common cause in Southeast Asia
• fetal skeletal dysplasia(s)
• fetal lymphovascular anomalies
  • cystic hygroma
• high output flow states
  • vein of Galen aneurysmal malformation
  • umbilical vein thrombosis in cases of umbilical vein varix
• thoracic/pulmonary abnormalities: thought to be from a fetal venous obstruction
  • primary fetal hydrothorax
  • congenital pulmonary airways malformation
  • congenital diaphragmatic herniation
  • pulmonary sequestration

Radiographic features

Antenatal ultrasound

Sonographic features can be similar for both immune and non-immune hydrops and include:

• increased amniotic fluid volumes
• increased nuchal translucency (especially 1trimester )
• larger placental size (placentomegaly) / increased placental thickness (placental edema)
• presence of a fetal pleural or pericardial effusion
• generalized fetal body swelling: fetal anasarca and skin thickening
• umbilical venous dilatation

Treatment and prognosis

The overall prognosis can be variable, dependent on the underlying cause.

History and etymology

The term hydrops fetalis comes from the Latin meaning edema of the fetus .

Siehe auch:

• Perikarderguss
• Pätau-Syndrom
• Herzfehler
• Down-Syndrom
• Turner-Syndrom
• nuchal translucency
• Morbus Gaucher
• Trisomie 18
• fetal pleural effusion
• Steißbeinteratom
• trisomies
• in utero infection
• chromosomale Anomalien
• placental thickness
• umbilical vein varix
• fetal anasarca
• fetal tumours
• fetal pericardial effusion
• Rhabdomyom des Herzens
• fetal parvovirus B19 infection
• fetal anaemia
• amniotic fluid volumes
• erythroblastosis fetalis
• primary fetal hydrothorax
• zystisches Lymphangiom
• umbilical venous dilatation
• fetal skeletal dysplasia
• twin reversed arterial perfusion sequence
• twin to twin transfusion syndrome
• fetal tachyarrhythmias
• TORCH group
• nuchal oedema
• Vena Galeni Malformation
• rhesus incompatibility
• congenital pulmonary airways malformation
• kongenitale Zwerchfellhernie
• fetal pleural
• fetaler Aszites
• Plazentamegalie

und weiter:

• Ebstein anomaly
• obstetric curriculum
• Polyhydramnion
• antenatal features of Down syndrome
• fetal hepatomegaly
• fetal toxoplasmosis
• fetal hepatosplenomegaly
• Embryopathia rubeolosa
• fetale Hirntumoren
• Lues connata
• fetal complete atrioventricular block
• fetal tachyarrhythmia
• fetal splenomegaly
• fetal cardiomegaly
• endocardial fibroelastosis
• fetal cardiomyopathy
• fetal bradyarrhythmia
• absent ductus venosus
• feto-maternal rhesus incompatibility
• mirror syndrome
• fetal supraventricular tachycardia
• placental chorioangiomatosis
• pulmonale Lymphangiektasie
• fetal atrial flutter
• erythema infectiosum
• haemolytic disease of the newborn
• fetal atrial fibrillation
• TRAP sequence
• fetal goitre
• connatale Zytomegalie
• fetale Struma
• kongenitale pulmonale Atemwegsmalformation (CPAM)
• kongenitales mesoblastisches Nephrom
• fetales perikardiales Teratom