PT  - JOURNAL ARTICLE
AU  - A.I. Alomari
AU  - D.B. Orbach
AU  - J.B. Mulliken
AU  - A. Bisdorff
AU  - S.J. Fishman
AU  - A. Norbash
AU  - R. Alokaili
AU  - D.J. Lord
AU  - P.E. Burrows
TI  - Klippel-Trenaunay Syndrome and Spinal Arteriovenous Malformations: An Erroneous Association
AID  - 10.3174/ajnr.A2167
DP  - 2010 Oct 01
TA  - American Journal of Neuroradiology
PG  - 1608--1612
VI  - 31
IP  - 9
4099  - http://www.ajnr.org/content/31/9/1608.short
4100  - http://www.ajnr.org/content/31/9/1608.full
SO  - Am. J. Neuroradiol.2010 Oct 01; 31
AB  - BACKGROUND AND PURPOSE: KTS is a rare limb overgrowth disorder with slow-flow vascular anomalies. This study examines the presumed association between KTS and spinal AVMs. MATERIALS AND METHODS: We performed a MEDLINE search of articles and reviewed textbooks of spinal diseases to study the association between KTS and spinal AVM. Our goal was to ascertain the basis on which the diagnosis of KTS was established and to evaluate the evidence of its association with spinal AVMs. In addition, the data base of the Vascular Anomalies Center at Children's Hospital Boston was queried for patients with KTS, and the association with spinal AVM was investigated. RESULTS: Twenty-four published reports on spinal AVMs in 31 patients with KTS were reviewed. None of these references provided solid evidence of the diagnosis of KTS in any patient. Clinical data were either incompatible with the diagnosis of KTS or were inadequate to establish the diagnosis. Alternative possible diagnoses (CLOVES syndrome and CM-AVM) were suggested by the first author for 9 of the patients reported in these articles. The medical records of 208 patients with the diagnosis of KTS were analyzed; not a single patient had clinical or radiologic evidence of a spinal AVM. CONCLUSIONS: An association between KTS and spinal AVM, as posited in numerous references, is most likely erroneous. The association has neither been reliably proved in the limited published literature nor encountered in a large cohort. AVMarteriovenous malformationCLOVEScongenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal/scoliosis and spinal anomaliesCLVMcapillary-lymphaticovenous malformationCMcapillary malformationCM-AVMcapillary malformation-arteriovenous malformationFfemaleHHThereditary hemorrhagic telangiectasiaKTSKlippel-Trenaunay syndromeLMlymphatic malformationMmaleNAnot available/not applicableVEGFvascular endothelial growth factorVMvenous malformationYyear