Hydrops fetalis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Adnan Ezici, M.D[2]

Overview

Hydrops Fetalis indicates an excessive accumulation of interstitial fluid in extravascular compartments and body cavities which is characterized by generalized skin edema, ascites, pleural, or pericardial effusion, and placental enlargement. It may be classified into two groups based on the presence or absence of rhesus iso-immunization. Although Rh disease is the major cause of immune-mediated hydrops fetalis, with the decreased prevalence of Rh disease, non-immune causes (eg, cardiovascular diseases, chromosomal abnormalities, lymphatic anomalies, hematologic diseases, etc.) are responsible in the majority of cases. Screening for Rh(D) incompatibility by Rh(D) blood typing and antibody testing are strongly recommended for all pregnant women during their first visit for pregnancy-related care. Also repeated Rh(D) antibody testing for all unsensitized Rh(D)-negative women at 24 to 28 weeks gestation is recommended unless the biological father is known to be Rh(D) negative. Prognosis is generally poor, and the mortality rate of patients with non-immune hydrops fetalis (NIHF) is approximately 43.2% at 1 year of age, and the presence of either large birth weight, polyhydramnious, or prematurity are associated with a particularly poor prognosis among patients. Ultrasound may be helpful in the diagnosis of hydrops fetalis. Findings on ultrasound suggestive of hydrops fetalis include increased skin thickness (indicative of generalized skin edema), increased placental thickness (indicative of placental edema), polyhydramnios, ascites, pleural effusion, and pericardial effusion.

Historical Perspective

Hydrops fetalis was first discovered by Dr. John William Ballantyne, a Scottish physician and obstetrician, in 1892.

Classification

Hydrops Fetalis may be classified into two groups based on the presence or absence of rhesus iso-immunization:

  • Immune Hydrops Fetalis
  • Non-Immune Hydrops Fetalis (NIHF)

Pathophysiology

It is thought that hydrops fetalis is caused by conditions with either increased rate of fluid transudation from the vascular compartment or decreased lymphatic return to the circulation.

Causes

Hydrops Fetalis is caused by either immune or non-immune conditions.

Differential Diagnosis

Epidemiology and Demographics

Risk Factors

Maternal risk factors in the development of non-immune hydrops fetalis (NIHF) include:[9]

Screening

  • According to the U.S. Preventive Services Task Force (USPSTF), screening for Rh(D) incompatibility by Rh(D) blood typing and antibody testing are strongly recommended for all pregnant women during their first visit for pregnancy-related care.
  • The USPSTF recommends repeated Rh(D) antibody testing for all unsensitized Rh(D)-negative women at 24 to 28 weeks gestation, unless the biological father is known to be Rh(D) negative.[10]

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

There are no established criteria for the diagnosis of hydrops fetalis.

History and Symptoms

The hallmark of hydrops fetalis is an abnormal accumulation of fluid within the fetal body compartments. History and symptoms may differ among patients based on the etiology.

  • A positive history of Rh(D) incompatibility is suggestive of immune hydrops fetalis.

Physical Examination

The physical examination findings in the neonatal period may indicate the underlying diseases.

Laboratory Findings

Electrocardiogram

Ultrasound

An ultrasound showing a fetus with hydrops fetalis
An ultrasound showing a fetus with cystic hygroma and hydrops fetalis. Arrows pointing to bilateral pleural effusion

Echocardiography

Genetic Testing

Other Diagnostic Studies

Treatment

The treatment depends on the cause.

Severely anemic fetuses can be treated with blood transfusions while still in the womb.

Template:Certain conditions originating in the perinatal period

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References


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  18. Sparks TN, Lianoglou BR, Adami RR, Pluym ID, Holliman K, Duffy J; et al. (2020). "Exome Sequencing for Prenatal Diagnosis in Nonimmune Hydrops Fetalis". N Engl J Med. 383 (18): 1746–1756. doi:10.1056/NEJMoa2023643. PMC 7650529 Check |pmc= value (help). PMID 33027564 Check |pmid= value (help).