Klippel-Trenaunay Syndrome and Spinal Arteriovenous Malformations: An Erroneous Association

Abstract

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.

Klippel-Trénaunay Syndrome and Spinal Arteriovenous Malformations: An Erroneous Association

“…I read with interest the article entitled "Klippel-Trénaunay Syndrome and Spinal Arteriovenous Malformations: An Erroneous Association" by Alomari et al 1 The classification of Klippel-Trénaunay syndrome (KTS) in the complex group of segmental angiomatous phakomatoses based on data from meta-analyses and the findings of 208 patients with the diagnosis KTS is commendable and important with respect to the diagnostic lack of clarity in the classification of overgrowth disorders.…”

Parkes Weber Syndrome and Spinal Arteriovenous Malformations

“…e read with great interest the article written by Alomari et al, 1 and agree with the authors that Klippel-Trenaunay syndrome is not associated with spinal cord AVM. However, in their discussion, in relationship to spinal cord AVM with Parkes Weber syndrome, they comment briefly that "Spinal AVM has not been reported to be a feature of Parkes Weber syndrome."…”

“…However, in their discussion, in relationship to spinal cord AVM with Parkes Weber syndrome, they comment briefly that "Spinal AVM has not been reported to be a feature of Parkes Weber syndrome." 1 Here, we demonstrate a case of Parkes Weber syndrome harboring a concomitant spinal AVM and raise the argument that spinal AVM may be a feature of Parkes Weber syndrome. A baby girl was born with multiple skin capillary malformations on her right buttock, thigh, shin, and left axilla, as well as overgrowth of the affected right leg.…”

Conus Medullaris-Cauda Arteriovenous Malformation and Klippel-Trenaunay Syndrome: What is the Treatment Goal?

“…1,5,11,28,30) However, these assumptions were recently contested, suggesting that the presence of spinal AVM is not a feature of KTS and this association might be erroneous. 3) In addition to the controversy about the terminology and pathological associations, there is no consensus about the optimal treatment for symptomatic spinal AVMs, and few KTS patients have been treated for spinal AVMs (Table 1). 2,4,[6][7][8][9][10][14][15][16][17][18][19][20][21][22][23][24][25][26][27]29) Here we describe the treatment of a 29-year-old man who was diagnosed with KTS at the age of 8 years at a different institution and presented with symptomatic conus medullaris-cauda AVM treated by multisession endovascular embolization.…”

“…16) Recent observations have clearly differentiated Klippel-Trenaunay syndrome (KTS) from Parker-Weber syndrome (PWS): KTS is characterized by the presence of slow flow vascular malformations that may involve capillaries and lymphatics of an overgrown limb, whereas PWS involves fast flow vascular lesions. 3) KTS may exceptionally be associated with spinal arteriovenous malformations (AVMs) that could be related to the overexpression of angiogenic factors. 1,5,11,28,30) However, these assumptions were recently contested, suggesting that the presence of spinal AVM is not a feature of KTS and this association might be erroneous.…”