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==Historical Perspective==
==Historical Perspective==
Hydrops fetalis was first discovered by Dr. John William Ballantyne, a Scottish physician and obstetrician, in 1892.
Hydrops fetalis was first discovered by Dr. John William Ballantyne, a Scottish physician and [[obstetrician]], in 1892.


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


*'''Immune Hydrops Fetalis'''
*'''Immune Hydrops Fetalis'''

Revision as of 10:35, 24 April 2021

Hydrops Fetalis

Overview

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.
  • This is shown to be originated from developmental defects in microcirculation and lymphatic system, respectively.[1]
  • The potential causes may be immune or non-immune, and they often result in anemia and further hypoxia.
  • The sympathetic system becomes activated due to hypoxia, and it causes blood redistribution with decreased blood flow to the liver and kidneys.
  • Decreased blood flow to the liver and kidneys, results in decreased albumin, increased ADH, and increased activity of RAAS.
  • Following these changes, the central venous pressure increases, which further results in decreased lymphatic return.
  • As a result, hydrops fetalis (the accumulation of fluid, or edema, in at least two fetal compartments)[2] occurs.
  • The pathophysiology of non-immune causes also depend on the underlying conditions, include:
    • Decreased ventricular filling during diastole (i.e. tachyarrhythmias)
    • Increased central venous pressure due to the increased right heart pressure (i.e. cardiac tumors and subendocardial fibroelastosis)
    • Obstruction of lymphatic drainage due to a mass (i.e. cystic hygroma)

Causes

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

  • Immune hydrops fetalis
    • Antibodies may occur due to the exposure of non-self RBC antigens during the previous pregnancy or transfusion.
    • In the next pregnancy, these antibodies may attack the fetal erythrocytes if the fetus has that antigen.
    • Following the red blood cell destruction, hemolytic disease of the fetus and newborn (HDFN) may occur with a wide range of clinical outcome from only mild anemia to high output heart failure and hydrops fetalis.[3]
      • Rh disease is the major cause for immune-mediated hydrops fetalis; however, owing to preventative methods developed in the 1970s, the incidence of Rh disease has markedly declined.
      • Rh disease can be prevented by administration of anti-D IgG (Rho (D) Immune Globulin) injections to RhD-negative mothers during pregnancy and/or within 72 hours of the delivery.
  • Non-immune hydrops fetalis (NIHF)
    • Currently, with the decreased prevelance of Rh disease, non-immune causes are responsible for up to 90% of cases.
    • The most common causes of non-immune hydrops fetalis are hematologic diseases, and chromosomal abnormalities, followed by lymphatic anomalies, and cardiovascular diseases. Causes of NIHF include:[4][5]
      • Structural cardiac malformations (especially hypoplastic left heart, endocardial cushion defect)
      • Arrhythmias
      • Congenital lymphatic dysplasia
      • Chromosomal abnormalities (Turner Syndrome, trisomy 13, trisomy 18, trisomy 21)
      • Alpha-thalassemia
      • Fetomaternal transfusion
      • Infections (Parvo-B19, CMV, Adenovirus, Enterovirus)
      • Twin to twin transfusion syndrome (both donor and recipient fetus)
      • Congenital cystic adenomatoid malformation
      • Diaphragmatic hernia
      • Extrapulmonary sequestration
      • Hydrothorax
      • Chylothorax
      • Noonan Syndrome
      • Urethral Obstruction
      • Prune belly syndrome
      • Lysosomal storage disease
      • Vascular tumors
      • Teratoma
      • Leukemia
      • Hepatic tumors
      • Neuroblastoma
      • Meconium peritonitis
      • Gastrointestinal obstructions

Epidemiology and Demographics

  • In developed countries, the incidence of non-immune hydrops fetalis (NIHF) is 25-79 per 100.000 live born infants worldwide.[6][7]
  • The median gestational age (GA) at diagnosis of NIHF is 23 weeks.
  • Gestational age is predictive of mortality, as preterm infants with this condition are more likely to die.
  • The case-fatality rate of NIHF is ranged from 43.2% to 78.2%.[8] [9]

Risk Factors

References


  1. Vanaparthy R, Mahdy H. PMID 33085361 Check |pmid= value (help). Missing or empty |title= (help)
  2. Kontomanolis EN, Fasoulakis Z (2018). "Hydrops Fetalis and THE Parvovirus B-19". Curr Pediatr Rev. 14 (4): 239–252. doi:10.2174/1573396314666180820154340. PMID 30124157.
  3. Moise KJ (July 2005). "Red blood cell alloimmunization in pregnancy". Semin Hematol. 42 (3): 169–78. doi:10.1053/j.seminhematol.2005.04.007. PMID 16041667.
  4. Bellini C, Donarini G, Paladini D, Calevo MG, Bellini T, Ramenghi LA, Hennekam RC (May 2015). "Etiology of non-immune hydrops fetalis: An update". Am J Med Genet A. 167A (5): 1082–8. doi:10.1002/ajmg.a.36988. PMID 25712632.
  5. Bellini C, Hennekam RC (March 2012). "Non-immune hydrops fetalis: a short review of etiology and pathophysiology". Am J Med Genet A. 158A (3): 597–605. doi:10.1002/ajmg.a.34438. PMID 22302731.
  6. Meng, Dahua; Li, Qifei; Hu, Xuehua; Wang, Lifang; Tan, Shuyin; Su, Jiasun; Zhang, Yue; Sun, Weijia; Chen, Biyan; He, Sheng; Lin, Fei; Xie, Bobo; Chen, Shaoke; Agrawal, Pankaj B.; Luo, Shiyu; Fu, Chunyun (2019). "Etiology and Outcome of non-immune Hydrops Fetalis in Southern China: report of 1004 cases". Scientific Reports. 9 (1). doi:10.1038/s41598-019-47050-6. ISSN 2045-2322.
  7. Steurer MA, Peyvandi S, Baer RJ, MacKenzie T, Li BC, Norton ME, Jelliffe-Pawlowski LL, Moon-Grady AJ (August 2017). "Epidemiology of Live Born Infants with Nonimmune Hydrops Fetalis-Insights from a Population-Based Dataset". J Pediatr. 187: 182–188.e3. doi:10.1016/j.jpeds.2017.04.025. PMID 28533037.
  8. Ota S, Sahara J, Mabuchi A, Yamamoto R, Ishii K, Mitsuda N (April 2016). "Perinatal and one-year outcomes of non-immune hydrops fetalis by etiology and age at diagnosis". J Obstet Gynaecol Res. 42 (4): 385–91. doi:10.1111/jog.12922. PMID 26712114.
  9. Turgal, Mert; Ozyuncu, Ozgur; Boyraz, Gokhan; Yazicioglu, Aslihan; Sinan Beksac, Mehmet (2015). "Non-immune hydrops fetalis as a diagnostic and survival problems: what do we tell the parents?". Journal of Perinatal Medicine. 43 (3). doi:10.1515/jpm-2014-0094. ISSN 1619-3997.