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*The [[pathophysiology]] of non-immune causes also depend on the underlying conditions, include: | *The [[pathophysiology]] of non-immune causes also depend on the underlying conditions, include: | ||
**Decreased [[ventricular filling]] during [[diastole]] (i.e. [[tachyarrhythmias]]) | **Decreased [[ventricular filling]] during [[diastole]] (i.e. [[tachyarrhythmias]]) | ||
**Increased [[central venous pressure]] due to the increased | **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]]) | **Obstruction of [[lymphatic drainage]] due to a mass (i.e. [[cystic hygroma]]) | ||
==Causes== | ==Causes== | ||
| Line 31: | Line 31: | ||
**[[Antibodies]] may occur due to the exposure to [[non-self]] [[RBC antigens]] during the previous [[pregnancy]] or [[transfusion]]. | **[[Antibodies]] may occur due to the exposure to [[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]]. | **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) | **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.<ref name="pmid16041667">{{cite journal |vauthors=Moise KJ |title=Red blood cell alloimmunization in pregnancy |journal=Semin Hematol |volume=42 |issue=3 |pages=169–78 |date=July 2005 |pmid=16041667 |doi=10.1053/j.seminhematol.2005.04.007 |url=}}</ref> | ||
***[[Rh disease]] is the major cause of immune-mediated hydrops fetalis; however, owing to | ***[[Rh disease]] is the major cause of 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 the administration of | ***[[Rh disease]] can be prevented by the 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)''' | *'''Non-immune hydrops fetalis (NIHF)''' | ||
**Currently, with the decreased [[prevalence]] of Rh disease, non-immune causes are responsible for up to 90% of cases. | **Currently, with the decreased [[prevalence]] 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:<ref name="pmid25712632">{{cite journal |vauthors=Bellini C, Donarini G, Paladini D, Calevo MG, Bellini T, Ramenghi LA, Hennekam RC |title=Etiology of non-immune hydrops fetalis: An update |journal=Am J Med Genet A |volume=167A |issue=5 |pages=1082–8 |date=May 2015 |pmid=25712632 |doi=10.1002/ajmg.a.36988 |url=}}</ref><ref name="pmid22302731">{{cite journal |vauthors=Bellini C, Hennekam RC |title=Non-immune hydrops fetalis: a short review of etiology and pathophysiology |journal=Am J Med Genet A |volume=158A |issue=3 |pages=597–605 |date=March 2012 |pmid=22302731 |doi=10.1002/ajmg.a.34438 |url=}}</ref> | **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:<ref name="pmid25712632">{{cite journal |vauthors=Bellini C, Donarini G, Paladini D, Calevo MG, Bellini T, Ramenghi LA, Hennekam RC |title=Etiology of non-immune hydrops fetalis: An update |journal=Am J Med Genet A |volume=167A |issue=5 |pages=1082–8 |date=May 2015 |pmid=25712632 |doi=10.1002/ajmg.a.36988 |url=}}</ref><ref name="pmid22302731">{{cite journal |vauthors=Bellini C, Hennekam RC |title=Non-immune hydrops fetalis: a short review of etiology and pathophysiology |journal=Am J Med Genet A |volume=158A |issue=3 |pages=597–605 |date=March 2012 |pmid=22302731 |doi=10.1002/ajmg.a.34438 |url=}}</ref> | ||
*** | ***Structural cardiac malformations (especially hypoplastic left heart, [[endocardial cushion defect]]) | ||
***[[Arrhythmias]] | ***[[Arrhythmias]] | ||
***[[ | ***Congenital [[lymphatic dysplasia]] | ||
***[[Chromosomal abnormalities]] ([[Turner Syndrome]], [[trisomy 13]], [[trisomy 18]], [[trisomy 21]]) | ***[[Chromosomal abnormalities]] ([[Turner Syndrome]], [[trisomy 13]], [[trisomy 18]], [[trisomy 21]]) | ||
***[[Alpha-thalassemia]] | ***[[Alpha-thalassemia]] | ||
***Fetomaternal [[transfusion]] | ***Fetomaternal [[transfusion]] | ||
***[[Infections]] ([[ | ***[[Infections]] ([[Parvovirus]]-B19, [[CMV]], [[Adenovirus]], [[Enterovirus]]) | ||
*** | ***Twin to twin transfusion syndrome (both [[donor]] and recipient [[fetus]]) | ||
***Congenital | ***Congenital cystic adenomatoid malformation | ||
***[[Diaphragmatic hernia]] | ***[[Diaphragmatic hernia]] | ||
*** | ***Extrapulmonary sequestration | ||
***[[Hydrothorax]] | ***[[Hydrothorax]] | ||
***[[Chylothorax]] | ***[[Chylothorax]] | ||
***[[Noonan Syndrome]] | ***[[Noonan Syndrome]] | ||
*** | ***Urethral Obstruction | ||
***[[Prune belly syndrome]] | ***[[Prune belly syndrome]] | ||
***[[Lysosomal storage diseases]] | ***[[Lysosomal storage diseases]] | ||
***[[Vascular tumors]] | ***[[Vascular tumors]] | ||
*** | ***Teratoma | ||
***[[Leukemia]] | ***[[Leukemia]] | ||
***[[ | ***[[Liver tumors]] | ||
***[[Neuroblastoma]] | ***[[Neuroblastoma]] | ||
***[[Meconium peritonitis]] | ***[[Meconium peritonitis]] | ||
*** | ***Gastrointestinal obstructions | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
Revision as of 10:56, 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 to 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 of 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 the 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 prevalence 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 (Parvovirus-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 diseases
- Vascular tumors
- Teratoma
- Leukemia
- Liver 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
- ↑ Vanaparthy R, Mahdy H. PMID 33085361 Check
|pmid=value (help). Missing or empty|title=(help) - ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.