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A Journal on Pediatrics, Neonatology, Adolescent Medicine,
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Minerva Pediatrica 2000 May-June;52(5-6):307-12

language: English, Italian

A case of non-immune hydrops fetalis with congenital cystic adenomatoid malformation of the left lung in a twin

Arena S., Liberatore A., Carrera G., Riboni G.


Hydrops fetalis is a rare condition that occurs in one out of every 1.400-4.000 pregnancies. There are two types, immune and non-immune. It can be caused by a maternal-fetal incompatibility due to the Rh factor or, more rarely, the Kell factor or due to other antigens to red corpuscles. Non-immune types of hydrops have a complex multiform pathogenesis and in 50% of the cases they are of idiopathic nature. The causes that could determine non-immune hydrops are manifold and in 50% of the cases they are of an idiopathic nature. Independently of the clinical condition, the presented case could be classified in those forms of pathogenesis in which there is the presence of a congenital cystic adenomatoid malformation (CAM), not encountered in the fetal ultrasound performed at the sixteenth week of pregnancy. The particularity of our case is due to the association of the fetal hydrops of the CAM type in only one of the two twins. The presented case is clasified in those forms of hydrops that are expressed with a clinical trend towards a worse prognosis, characterized by the intrauterine death of the fetus or the birth of a hydropic fetus that does not survive because of respiratory insufficiency and cardiocirculatory collapse.

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