- Hydrops fetalis
Name = Hydrops fetalis not due to haemolytic disease/isoimmunization
ICD10 = ICD10|P|83|2|p|80
ICD9 = ICD9|778.0
Hydrops fetalis is a condition in the
fetuscharacterized by an accumulation of fluid, or edema, in at least two fetal compartments, including the subcutaneous tissue, pleura, pericardium, or in the abdomen, which is also known as ascites. The edemais usually seen in the fetal subcutaneoustissue, sometimes leading to spontaneous abortion. It is a prenatal form of heart failure, in which the heart is unable to satisfy the insatiable demand for an unusually high amount of blood flow.
Classification and causes
Hydrops fetalis usually stems from fetal
anemia, when the heart needs to pump a much greater volume of blood to deliver the same amount of oxygen. This anemia can have either an immune or non-immune cause. Non-immune hydrops can also be unrelated to anemia, for example if a tumor or congenital cystic adenomatoid malformationincreases the demand for blood flow.
Rh diseaseis the major cause for immune mediated hydrops fetalis; however, owing to preventative methods developed in the 1970s Rh disease has markedly declined. Rh diseasecan 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.
The non-immune form of hydrops fetalis has many causes including:
Iron deficiency anemia
*Deficiency of the enzyme
beta-glucuronidase. This enzyme deficiency is the cause of the lysosomal storage diseasecalled Mucopolysaccharidosis Type VII.
Parvovirus B19(Fifth Disease) infection of the pregnant woman.
thalassemiacan also cause hydrops fetalis when all four of the genetic loci for α globin are deleted or affected by mutation.
Niemann-Pick DiseaseType C (NPC) can present with hydrops fetalis.
tumor. The most common type of fetal tumor is a teratoma, particularly a sacrococcygeal teratoma.
Hydrops fetalis can be diagnosed and monitored by ultrasound scans.
The treatment depends on the cause.
anemicfetuses can be treated with blood transfusionswhile still in the womb.
Cassidy MD, George. Hydrops Fetalis. eMedicine Online. 2004.
* [http://www.fifthdisease.org/cmsFiles/fifthdisease_patient_information_leaflet_english.pdf www.fifthdisease.org Patient Information]
* [http://www.hydropsfetalis.org www.hydropsfetalis.org Research Information]
* [http://hygen.net/blog/?cat=21 www.hygen.net Case Information & Images]
* [http://www.hygen.net/hydrops-fetalis/ Hydrops Fetalis Community Forums]
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