A Case of Prenatally Diagnosed Klippel-Trenaunay-Weber Syndrome.
- Author:
So Young KWON
1
;
Yun Ah KIM
;
Jong Won LEE
;
Su Jin KIM
;
Kyoung Jin LEE
;
Ji Hyeun PARK
;
Chang Jo CHUNG
Author Information
1. Department of Obstetrics and Gynecology, College of Medicine, Pochon CHA University, Seoul, Korea. k-soyung@hanmail.net
- Publication Type:Case Report
- Keywords:
Klippel-Trenaunay-Weber syndrome;
Ultrasonography;
Antenatal Diagnosis
- MeSH:
Ascites;
Blood Platelets;
Extremities;
Female;
Heart Failure;
Hemangioma;
Hepatomegaly;
Humans;
Hydrops Fetalis;
Infant, Newborn;
Klippel-Trenaunay-Weber Syndrome*;
Pregnancy;
Pregnancy Trimester, Second;
Prenatal Diagnosis;
Thrombocytopenia;
Ultrasonography
- From:Korean Journal of Perinatology
2003;14(4):416-421
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Klippel-Trenaunay-Weber syndrome is a rare sporadically occurring congenital soft tissue anomaly characterized by cutaneous hemangiomata, hemihypertrophy and occasionally arteriovenous malformations(AVMs). No definite genetic defect has been identified. The appearance is a soft tissue mass of an extremity, usually affecting the adjacent trunk, hydrops fetalis, ascites, abdominal hemangiomatous masses, and hepatomegaly. When diagnosed prenatally, the disorder may be severe. Thrombocytopenia due to platelet consumption within the hemangioma and high output cardiac failure may complicate the outcome. Termination of pregnancy can be offered in severe forms, otherwise no alteration of management in expected. The management of newborns is primarily nonoperative, but some may benefit from surgical intervention. We report a case of Klippel-Trenaunay-Weber syndrome diagnosed prenatally by ultrasonogram in the second trimester and subsequently was terminated, with a brief review of literature.
