[en] We report the prenatal diagnosis of Wolf-Hirschhorn syndrome (4p-) in a 24-week-old fetus. Echographic features included cystic hygroma, a complex heart defect with right ventricular hypoplasia, and a large placental chorioangioma. We suggest that chorioangioma may be associated with chromosomal imbalance and that systematic careful morphologic examination of the fetus and karyotyping of any pregnancy in which large chorioangioma is detected is advisable. Jugular lymphatic obstruction sequence has not been reported so far in association with 4p-syndrome.
Disciplines :
Genetics & genetic processes
Author, co-author :
Verloes, Alain ; Université de Liège - ULiège > Génétique générale et humaine
Schaaps, Jean-Pierre ; Université de Liège - ULiège > Département des sciences biomédicales et précliniques > Embryologie
Herens, Christian ; Centre Hospitalier Universitaire de Liège - CHU > PLAN COS
Soyeur, Daniel ; Université de Liège - ULiège > Département des sciences cliniques > Cardiologie
Hustin, J.
Dodinval, P.
Language :
English
Title :
Prenatal Diagnosis of Cystic Hygroma and Chorioangioma in the Wolf-Hirschhorn Syndrome
Publication date :
February 1991
Journal title :
Prenatal Diagnosis
ISSN :
0197-3851
eISSN :
1097-0223
Publisher :
John Wiley & Sons, Hoboken, United States - New Jersey
Chervenak F.A., Isaacson G., Blakemore K.J., Berg W.R., Hobbins J.C., Berkowitz R.L., Tortora M., Mayden K., Mahoney M.J. (1983) Fetal cystic hygroma. Causes and natural history. N. Engl. J. Med. 309:822-825.
Clark E.B. (1984) Neck web and congenital heart defects: a pathogenic association in 45,XO Turner syndrome. Teratology 29:355-361.
Cowchock F.S., Wapner R.J., Kurtz A., Chatzkel S., Barnhart J.S., Lesnick D.C. (1982) Brief clinical report: not all cystic hygromas occur in the Ullrich‐Turner syndrome. Am. J. Ned. Genet. 12:327-331.
de Grouchy J., Turleau C. Atlas des Maladies Chromosomiques, 2nd ed., Paris, LExpansion Scientifique; 1982.
Fox H. Pathology of the Placenta , London, W. B. Saunders; 1978, 343-367.
Froehlich L.A., Fujikura T., Fisher P. (1971) Chorioangiomas and their clinical implication. Obstet. Gynecol. 37:51-59.
Miyabara S., Sugihara H., Maehara N., Shouno H., Tasaki H., Yoshida K., Saito N., Kayama F., Ibara S., Suzumori K. (1989) Significance of cardiovascular malformations in cystic hygroma: a new interpretation of the pathogenesis. Am. J. Med. Genet. 34:489-501.
Mulliez N., Gonzales M., Migne G., Roume J., Perot C., Razavi F., Roux C. (1986) Les hygroma kystiques foetaux. Revue de la littérature. A propos de Lexpérience du centre de foetopathologie de LHopital Saint Antoine. Soir. Echo. Gyn. Obst. 43:29-33.
(1988) Cystic hygroma. Prenatal Diagnosis of Congenital Anomalies , Romero, R., Pilu, G., Jeanty, P., Ghidini, A., Hobbins, J., (Eds), Norwalk, CT, Appleton & Lange; 115-118.
Schinzel A. Catalogue of Unbalanced Chromosome Aberrations in Man, Berlin, W. de Gruyter; 1984.
Wurster D.E., Hoefnagel D., Bernischke K., Allen F.J. (1969) Placental chorioangiomata and mental deficiency in a child with 2/15 translocation: 46, XX, t(2q; 15q+). Cytogenetics 8:389-399.