Published online September 15, 2005
PEDIATRICS Vol. 116 No. 4 October 2005, pp. e596-e600 (doi:10.1542/peds.2004-1607)
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ELECTRONIC ARTICLE

Pulmonary Thromboembolism Associated With Klippel-Trenaunay Syndrome

Erin E. Huiras, MD*, Cheryl J. Barnes, MD{ddagger}, Lawrence F. Eichenfield, MD{ddagger}, Andrew N. Pelech, MD§ and Beth A. Drolet, MD*

* Department of Pediatric Dermatology
§ Department of Pediatric Cardiology, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin
{ddagger} Department of Pediatric and Adolescent Dermatology, Children’s Hospital, San Diego, and University of California, San Diego, San Diego, California

Klippel-Trenaunay syndrome (KTS) is a rare congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations (port wine stains) of the affected limb or limbs. Large venous malformations such as those observed in KTS are rare, and many physicians are unfamiliar with the potential complications, which include hypercoagulability, thrombosis, and pulmonary embolism (PE). As a result, patients may suffer from delayed diagnosis of a potentially life-threatening thromboembolic event. We present 2 cases of children with KTS complicated by PE, and we review the English-language literature regarding pathophysiologic features, interventions, and outcomes of PE in the setting of KTS among both pediatric and adult patients, with emphasis on issues relevant to pediatricians.

Key Words: Klippel-Trenaunay syndrome • Proteus syndrome • venous malformation • hypercoagulable state • deep venous thrombosis • pulmonary embolism • Kasabach-Merritt syndrome

Abbreviations: KTS, Klippel-Trenaunay syndrome • DVT, deep venous thrombosis • PE, pulmonary embolism • IVC, inferior vena cava

Accepted Apr 19, 2005.


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