Sandbox:Ezici

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 an increased rate of fluid transudation from the vascular compartment or decreased lymphatic return to the circulation, chiefly because of the developmental defects in microcirculation and lymphatic system, respectively.
• While these conditions may be both immune and non-immune, 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.
• It results in decreased albumin, increased ADH, and increased activity of RAAS.
• Through these changes, the central venous pressure increases, which further results in decreased lymphatic return.
• As a result, severe and progressive edema occurs in a fetus.
• 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.
    • 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 common causes of non-immune hydrops fetalis include:
    • 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

Causes by Organ System

Cardiovascular	Arteriovenous fistula, Budd-Chiari syndrome, Cardiac amyloidosis, Cardiomegaly, Cardiomyopathy, Cholesterol pericarditis, Congestive heart failure, Constrictive pericarditis, Congestive heart failure, Constrictive pericarditis, Tuberculous pericarditis, Chylous ascites, Cor pulmonale, Diffuse neonatal hemangiomatosis, Endomyocardial fibrosis, Heart failure, Inferior vena cava web, Lymphatic obstruction, Lymphangioma, Kaposiform hemangio-endothelioma, Inferior vena cava syndrome, Pericarditis, Right heart failure, Tricuspid insufficiency, Tricuspid regurgitation, Tricuspid stenosis, Tricuspid valve disease, Veno-occlusive disease, Ventriculoperitoneal shunt, Waldmann disease.
Chromosomal	No underlying causes
Hematologic	Cutis marmota telangictatica congenita
Lymphatic Dysplasia	Acyclovir, Mercaptopurine, Pramipexole, Thalidomide
Infections	No underlying causes
TTTF-Placental	Hypothyroidism, Thyroxine deficiency, Ovarian hyperstimulation syndrome
Thoracic	No underlying causes
Syndromic	Acute liver failure, Acute pancreatitis, Alcoholic hepatitis, Alcoholic liver disease, Ascending cholangitis, Biliary atresia, Biliary fistula, Budd-Chiari syndrome, Cholangiocarcinoma, Cholecystitis, Cholelithiasis, Chronic hepatitis,Cirrhosis, Colorectal cancer, Congenital liver fibrosis, End stage liver failure, Eosinophilic gastroenteritis, Esophageal cancer, Fitz-Hugh-Curtis syndrome, Fulminant liver failure, Granulomatous peritonitis, Hemochromatosis, Hepatic failure, Hepatic venoocclusive disease with immunodeficiency, Hepatitis, Hepato-biliary diseases, Idiopathic liver cirrhosis, Krukenberg tumor, Liver cancer, Liver damage, Ménétrier's disease, Mosse syndrome, Neonatal hepatitis, Nodular regenerative hyperplasia of the liver, Nutmeg liver, Obliterative portal venopathy, Obstructive jaundice, Pancreatic cancer, Pancreatic fistula, Peritonitis, Portal hypertension, Portal vein occlusion, Portal vein thrombosis, Primary biliary cirrhosis, Primary sclerosing cholangitis, Pseudomyxoma peritonei, Secondary biliary cirrhosis, Severe hepatitis, Stomach cancer, Tuberculous peritonitis, Waldmann disease, Whipple disease
Urinary Tract Malformations	Niemann-Pick type C
Inborn Storage Diseases	Angioimmunoblastic T-cell lymphoma, Banti's Syndrome, Budd-chiari syndrome, Diffuse neonatal hemangiomatosis, Eosinophilic gastroenteritis, Erythroblastosis fetalis , Extramedullary haemopoiesis, Hemolytic disease of the newborn, Mastocytosis, Mosse syndrome, Tang Hsi Ryu syndrome
Extrathoracic Tumors	Alveolar hydatid disease, Amebiasis, Bilharzia, Chronic hepatitis, Chronic viral hepatitis, cytomegalovirus, Echinococcosis, Epstein Barr virus, Filariasis, Fitz-Hugh-Curtis syndrome, acute hepatitis A, Acute hepatitis B, Acute hepatitis C, Acute Hepatitis D, Acute hepatitis E, Herpes simplex infection, Hookworm disease, Kaposiform hemangio-endothelioma, Leptospirosis, Malaria, Portal hypertension in schistosomiasis, Poststreptococcal glomerulonephritis, Rubella virus infection,Schistosomiasis, Hepatosplenic schistosomiasis, Sleeping sickness, Strongyloidiasis, Stuartbras disease, liver disease in syphilis, Toxoplasmosis, Tuberculosis, Tuberculous peritonitis, Tularemia, Whipple disease, Yellow fever virus infection
Gastrointestinal	No underlying causes References