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non-immune hydrops fetalis

Wednesday 3 January 2007

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. NIHF is observed in 7.5% of neonates who underwent autopsies.

Etiology

- cardiovascular disease (50%) with low output cardiac failure

  • structural congenital heart disease
  • cardiomyopathies: oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency
  • myocarditis
  • cardiac rhabdomyomas

- congenital infections

  • cytomegalovirus
  • bacteria
  • parvovirus

- genitourinary diseases

  • congenital nephrotic syndrome
  • VACTER association
  • prune-belly syndrome
  • urogenital sinus malformation

- intrathoracic lesions

  • pulmonary sequestration
  • diaphragmatic hernia

- genetic metabolic diseases

  • Gaucher disease

- volume overload due to twin transfusion syndrome

References

- Rodriguez MM, Bruce JH, Jimenez XF, Romaguera RL, Bancalari E, Garcia OL, Ferrer PL. Nonimmune hydrops fetalis in the liveborn: series of 32 autopsies. Pediatr Dev Pathol. 2005 May-Jun;8(3):369-78. PMID: 16010481