2001-03-19-12 Skin Edema Secondary to Anti Kell Isoimmunization in Early Pregnancy© Malinger www.thefetus.net/
Skin Edema Secondary to Anti Kell Isoimmunization in Early Pregnancy.
Gustavo Malinger, MD, Dorit Lev, MD, Nirit Greenberg, MD.
Department of Obstetrics and Gynecolgy, and Genetics Institute. The Edith Wolfson Medical Center. The Sackler School of Medicine. Tel Aviv University. Holon, Israel
A 35 year old women, G5P4, was referred for US examination at 13 weeks.
2 and 3D US showed severe and diffuse scalp edema and pleural effusion, without cardiomegaly.(Figures)
The medical history was negative except for a blood transfusion performed after her third delivery because of post-partum hemorrhage. The subsequent pregnancy was uneventful.
The blood group was A Rh positive, but the indirect Coombs was positive in a titer of 1:256 for anti Kell antibodies. Fetal demise occurred at 17 weeks. The karyotype, obtained from skin fibroblast, was 46XX.
Shulman et al.1 described 30 cases of fetuses with pan-body hydrops giving the appearance of a "space-suit". Our case exemplifies, using the 3D approach, the similarity between fetuses with severe skin edema and an astronaut inside his space-suit. Most of the cases are due to non-immune hydrops fetalis and associated with chromosome abnormalities1,2, other possible causes of early hydrops include parvovirus B19 infection and cardiovascular defects.
Immune hydrops, usually due to Rh isoimmunization, develops in the most severe cases around 17-18 weeks. The occurrence of severe hydrops at 13 weeks due to anti Kell isoimmunization is difficult to explain based in current concepts in fetal immunology. Placental transfer of maternal IgG starts early in pregnancy, but only during the second trimester sufficient amounts are present to produce fetal hemolysis.
In couples desiring an other child, paternal determination of Kell genotype followed by prenatal determination of fetal genotype is indicated3.
1. Shulman LP, Phillips OP, Emerson DS, Felker DS, Tharapel AT. Fetal "space-suit" hydrops in the first trimester: differentiating risk for chromosome abnormalities by delineating characteristics of nuchal translucency. Prenat Diagn 2000 20:30-32.
2. Isakros J, Jauniaux E, Rodeck C. Outcome of nonimmune hydrops fetalis diagnosed during the first half of pregnancy. Obstet Gynecol 1997 90:321-325.3. Spence WC, Maddalena A, Demers DB, Bick DP. Prenatal determination of genotypes Kell and Cellano in high risk pregnancies. J Reprod Med 1997 42:353-357.