Portal vein dilation in Klippel-Trenaunay and CLOVES syndromes.

Background: Klippel-Trenaunay Syndrome (KTS) and congenital lipomatous overgrowth, vascular anomalies, epidermal nevi and skeletal abnormalities (CLOVES) syndrome are rare conditions associated with overgrowth and vascular anomalies more frequently involving the lower extremities. Although uncommon, these syndromes can be associated with portal vein (PV) abnormalities, including PV thrombosis, and PV hypertension. In this study, we describe additional findings of PV dilation in five patients with KTS or CLOVES.

Methods: A single institution 20 year retrospective review was performed to evaluate PV findings in patients with vascular anomalies and overgrowth of the lower extremities. Two radiologists reviewed the imaging examinations (US, CT and MRI) and recorded type and location of vascular alterations.

Results: Of 35 patients (25 females, 24 KTS, 7 CLOVES and 4 other, median age: 8.5 years) with available abdominal imaging, 5 patients had PV dilation and one PV thrombosis. All patients presented additional abdominal vascular anomalies in association.

Conclusions: Systematic evaluation of the abdominopelvic region in patients with KTS and CLOVES can identify vascular anomalies, including PV dilation, which may indicate a prethrombotic state. Early detection and monitoring of these findings could have clinical implications for preventing complications such as portal hypertension and thrombosis.