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Pulmonary thromboembolism associated with Klippel-Trenaunay syndrome. Pediatrics 2005 Oct;116(4):e596-600 PMID: 16166386

Pubmed ID

16166386

Abstract

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.

Author List

Huiras EE, Barnes CJ, Eichenfield LF, Pelech AN, Drolet BA



Scopus

2-s2.0-33644671630   54 Citations

MESH terms used to index this publication - Major topics in bold

Acute Disease
Adolescent
Child
Humans
Klippel-Trenaunay-Weber Syndrome
Leg
Male
Pulmonary Embolism
Venous Thrombosis
jenkins-FCD Prod-332 f92a19b0ec5e8e1eff783fac390ec127e367c2b5