Prenatal sonographic appearances of Klippel-Trenaunay-Weber syndrome

Management of Pregnancy with Klippel-Trenaunay-Weber Syndrome: A Case Report and Review

“…The presentation may be in the form of multiple echo lucent areas suggestive of cutaneous vascular malformations [ 15 ] and/or hemihypertrophy of a lower extremity [ 16 ]. Roberts [ 17 ] described a case where by serial sonography was used to follow the in utero progression of the case and helped to decide the mode of delivery as a C-section, given the risk of labour dystocia and excessive fetal bleeding. In the second trimester the dominant abnormality was the cutaneous vascular lesions, while in the third trimester limb hypertrophy was the predominant finding.…”

Prenatal sonographic diagnosis of Klippel‐Trénaunay‐Weber syndrome with cardiac failure

“…It is characterized by large cutaneous hemangiomas, varices, soft tissue and bone hypertrophy, and occasionally arteriovenous malformations. 1,2 Prenatal diagnosis of KTWS by sonography has been reported sporadically, [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] and the fetal features have appeared to vary according to gestational age and extent of the lesions. In this report, we describe a prenatally diagnosed case of KTWS characterized by strikingly extensive involvement that led to fetal congestive heart failure and Kasabach-Merritt syndrome.…”

“…Since Hatjis et al 29 provided the first sonographic description of a fetus affected by KTWS in 1981, various reports of prenatal diagnoses of KTWS have been published. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] The hallmarks of the prenatal diagnosis of KTWS are cutaneous or subcutaneous cystic areas and limb hypertrophy. [3][4][5][6][7][8][9][10][11] Uncommonly, other features may be seen prenatally, such as complex soft tissue masses that affect unusual areas (eg, head, neck, or trunk), nonimmune hydrops fetalis, cardiomegaly, and polyhydramnios.…”

“…[3][4][5][6][7][8][9][10][11] Uncommonly, other features may be seen prenatally, such as complex soft tissue masses that affect unusual areas (eg, head, neck, or trunk), nonimmune hydrops fetalis, cardiomegaly, and polyhydramnios. [12][13][14][15][16][17][18] Three-dimensional sonography has been applied in some instances. 19,20 We believe this case of KTWS was remarkable because of the extent of the lesions and the early detection of cardiomegaly, which could be related to the unusual occurrence of hemangiomaassociated diffuse intravascular coagulation and intravascular hemolysis (Kasabach-Merritt syndrome).…”

Prenatal Sonographic Diagnosis of Klippel-Trenaunay-Weber Syndrome Associated with Umbilical Cord Hemangioma

“…Klippel-Trenaunay-Weber (KWT) syndrome is a rare disorder and its prenatal diagnosis has been reported in sporadic cases. [1][2][3][4] It is characterized by the presence of cutaneous hemangiomata, asymmetric limb and/or trunk hypertrophy, and varicosities. Unilateral leg hypertrophy is the most common finding.…”