This Article Abstract Free Full Text (Free PDF) Free Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Cruise, K. R. L. Articles by Rouse, G. Search for Related Content Social Bookmarking                         What's this?

Klippel-Trenaunay-Weber Syndrome Complicated by Extrafetal Umbilical Vein Varix

Loma Linda University Medical Center, Loma Linda, California; Loma Linda University, 1065 N. La Cadena Drive, #1, Colton, CA 92324.JRKLCruise{at}aol.com

Glenn Rouse, MD

Loma Linda University Medical Center, Loma Linda, California

Klippel-Trenaunay-Weber (KTW) syndrome is a rare vascular malformation including soft-tissue hypertrophy, varicose veins, cutaneous hemangiomas, and arteriovenous fistulas.The majority of reported cases were discovered at birth; only 7 cases have been reported in utero.Varicosities of the umbilical vein in utero involving the extrafetal portion of the cord are rare.Thrombosis of these varicies often re sults in the death of the fetus.No articles, at this time, report a case of KTW associated with an umbilical varix and subsequent thrombosis.The following case describes the sonographic appearance of both these abnormalities.

Key Words: Klippel-Trenaunay-Weber • umbilical vein varix

References

• Klippel M, Trenaunay P: Du noevus variqueux osteohypertrophique. Arch Gen Med 1900;185:641-641.
• Warhit JN, Goldman MA, Sachs L, Weiss LM, Pek H: Klippel-Trenaunay-Weber syndrome: appearance in utero. J Ultrasound Med 1983;2:515–517.[Abstract]
• Weber FP: Angioma formation in connection with hypertrophy of limbs and hemi-hypertrophy. Br J Dermatol1907;19:231-231.
• Hatjis CG, Philip AG, Anderson GG, Mann LI: The in utero ultrasonographic appearance of Klippel-Trenaunay-Weber syndrome. Am J Obstet Gynecol 1981;139:972–974.[Medline] [Order article via Infotrieve]
• Meholic AJ, Freimanis AK, Stucka J, LoPiccolo ML: Sonographiv in utero diagnosis of Klippel-Trenaunay-Weber syndrome. J Ultrasound Med 1991;10:111–114.[Medline] [Order article via Infotrieve]
• Servelle M: La veinographie va-t-elle nous permettre de de membrer le syndrome de Klippel et Trenaunay et l'hemangiectasie hypertophique de Parkes-Weber? Presse Med 1945;53:352-352.
• Servelle M, Albeaux-Fernet M, Laborde S, et al: Lesions des vaisseaux lymphatiques dans les malformations congenitales des veines profondes. Presse Med 1957;65:531-531.[Medline] [Order article via Infotrieve]
• Lindenauer SM: The Klippel-Trenaunay syndrome: varicosity, hypertrophy and hemangioma with no arteriovenous fistula. Ann Surg 1965;162:303-303.[Medline] [Order article via Infotrieve]
• White SP, Kofinas A: Prenatal diagnosis and management of umbilical vein varix of the intra-amniotic portion of the umbilical vein. J Ultrasound Med.1994;13:992–994.[Medline] [Order article via Infotrieve]
• Siddiqi TA, Brendon B, Schultz DM, Miodovnik M: Umbilical artery aneurysm: prenatal diagnosis and management. Obstet Gynecol 1992;18:530–533.
• Wolfman WL, Purohit DM, Self SE: Umbilical vein thrombosis at 32 weeks' gestation with delivery of a living infant. Am J Obstet Gynecol 1983;4:468-468.
• Baxi LV, Daftary A, Loucopoulos A: Single fetal demise in a twin gestation: umbilical vein thrombosis. Gynecol Obstet Invest 1998;4:266-266.
• Benirschke K: Obstetrically important lesions of the umbilical cord. J Reprod Med 1994;4:262-262.
• Heifetz SA: Thrombosis of the umbilical cord: analysis of 52 cases and literature review. Pediatr Pathol 1988;1:37-37.
• Buttner LE, Lippi UG, Borges AF, Andrade AS: Umbilical vein thrombosis. Revista Paulista de Medicina 1988;6:353-353.
• Campagnutta E, Sopracordevole F, Bacoll G, Melloni P, Marchetto G: Massive thrombosis of the umbilical vein with survival of the fetus, in a patient with hypertensive gestosis. Minerva Ginecologica 1987;12:821-821.
• Andersen ES: Thrombosis of the umbilical vein: an unusual cause of intrauterine fetal death. Ugeskrift for Laegar1986;43:2771-2771.
• Hoag RW: Fetomaternal hemorrhage associated with umbilical vein thrombosis. Am J Obstet Gynecol 1986;6:1271-1271.
• Dieminger HJ, Freibel L: Thrombosis of the umbilical vein. Zentralblatt fur Gynokologie.1986;12:765-765.
• Bonilla-Musoles F, Ramirez JV, Correia da Fonseca RJ, Pellicer A, Meyer A: Thrombosis of the umbilical vein in the ultrasound image-pathological findings and review of the literature. Ultraschall in der Medizin 1985;6:346-346.[CrossRef][Medline] [Order article via Infotrieve]
• Schrocksnadel H, Holbock E, Mitterschiffthaler G, Totsch M, Dapunt O: Thrombotic occlusion of an umbilical vein varix causing fetal death. Arch Gynecol Obstet 1991;4:213-213.
• Allen SL, Bagnall C, Roberts AB, Teele RL: Thrombosing umbilical vein varix. J Ultrasound Med 1998;17:189–192.[Medline] [Order article via Infotrieve]
• Rahemtullah A, Lieberman E, Benson C, Norton ME: Out-come of pregnancy after prenatal diagnosis of umbilical vein varix. J Ultrasound Med 2001;20:135–139.[Abstract]
• Vandevijver N, Hermans R, Schrander-Stumpel C, Arends J, Petters L, Moerman P: Aneurysm of the umbilical vein: case report and review of literature. Eur J Obstet Gynecol Reprod Biol 2000;89:85–87.[CrossRef][Medline] [Order article via Infotrieve]
• Ghosh A, Woo J, MacHenry C, Wan C, O'Hoy K, Ma H: Fetal loss from umbilical cord abnormalities'a difficult case for prevention. Eur J Obstet Gynecol Reprod Biol1984;18:183–198.[CrossRef][Medline] [Order article via Infotrieve]

Journal of Diagnostic Medical Sonography, Vol. 18, No. 5, 317-320 (2002)
DOI: 10.1177/875647902236842


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?

This Article Abstract Free Full Text (Free PDF) Free Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Cruise, K. R. L. Articles by Rouse, G. Search for Related Content Social Bookmarking                         What's this?