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Hydrops fetalis

from Section 3 - Classic and rare scenarios in the neonatal period

Published online by Cambridge University Press:  05 March 2012

Andrea Zimmermann
Affiliation:
Technical University Munich
Georg Hansmann
Affiliation:
Children's Hospital Boston, Harvard Medical School
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Summary

Definition

Generalized edema (anasarca) and accumulation of fluid in one or two visceral cavities. Pleural/pericardial effusion(s) or ascites alone is insufficient to establish the diagnosis “hydrops fetalis.” Hydrops may have an immunological (IHF) or a non-immunological (NIHF) cause. See also chapters on “hemolytic disease of the newborn” (HDN, p. 423) and “twin–twin (feto–fetal) transfusion syndrome” (TTTS, p. 240).

Epidemiology

NIHF occurs in approx. 1:1500 to 1:4000 (0.25–0.67/1000) pregnancies.

Etiology/pathophysiology

Immunological hydrops fetalis (IHF)

  • Feto–maternal Rhesus incompatibility leads to fetal anemia due to hemolytic antibodies and results in hypoxia (central apnea), heart failure, and capillary leak syndrome causing generalized edema, fluid accumulation in visceral cavities, hypoproteinemia, and hepatomegaly of various degrees. Hemolysis and anemia induce or enhance extramedullary erythropoiesis (→ hepatosplenomegaly)

Non-immunological hydrops fetalis (NIHF)

  • Fetal anemia, hypoxia, hypoproteinemia, and/or heart failure due to multiple possible etiologies lead to generalized edema and effusions

  • Anemia (hemolytic, non-hemolytic): virus infection (e.g., parvovirus B 19); α-thalassemia; glucose-6-phosphate-dehydrogenase deficiency (G6PDD), fetal leukemia, aplastic anemia, feto–maternal or twin–twin (=feto–fetal) transfusion in monochorial twin pregnancies (p. 240)

  • Pulmonary causes: chylothorax (p. 419), hydrothorax, congenital diaphragmatic hernia (CDH; p. 404), congenital cystic adenomatoid malformation (CCAM; p. 417), lymphangiectasia, and tumor

  • Cardiac causes: fetal tachycardia or bradycardia (p. 325), heart failure associated with congenital cardiovascular malformations (p. 340) or myocardial disease (e.g., myocarditis, mitochondrial defects, endomyocardial fibrosis), arteriovenous (AV) and other cardiovascular malformations (p. 340), premature closure of the foramen ovale, thrombosis, and tumor

  • Infections: toxoplasmosis, rubella, cytomegalovirus, syphilis, hepatitis, adenovirus, leptospirosis, respiratory syncytial virus (RSV), Coxsackie virus, and possibly also hepatitis viruses and HIV (see Table 3.5)

  • […]

Type
Chapter
Information
Neonatal Emergencies , pp. 427 - 431
Publisher: Cambridge University Press
Print publication year: 2009

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