Objective:
To study the clinical profile of Klippel–Trenaunay syndrome (KTS) and the management of venous malformations (VMs) with ultrasound-guided foam sclerotherapy using the compass technique.
Methods:
In this open prospective series, 11 consecutive patients with KTS seeking therapy at a phlebology group practice were included. Seven (64%) of them were post-surgical recurrences.
Results:
Pain was the commonest presenting complaint (100%), accompanied with VMs/ varicose veins in all except one (91%). Chronic venous insufficiency (CVI) was seen in seven (64%). Soft tissue and bone deformity was present in all (100%). Limb lengthening was frequent and detected in eight (73%), thickening in six (54%); unusually, one patient (9%) presented with severe thinning and shortening of the leg. Venous malformation was the primary vascular problem in nine patients (82%), with capillary malformation (CM)/port wine stain in seven (64%) and isolated lymphatic malformation (LM) in two (18%). The lateral embryonic/Klippel–Trenaunay (KT) vein was detected in 10 patients (91%). Deep vein deformity was not detected in any of them. All the deformities were of the lower limb (100%), while two (18%) had deformities of the thorax and one (9%) had gastrointestinal (GI) malformation. None of the patients gave family history of KTS, while five (45%) had history of varicosities in the family. Eight patients (73%) completed the therapeutic plan with a mean follow-up of 5±3.9 years. An excellent to good type result could be documented in six of the eight cases (75%). These patients had an event-free, good quality of life. Follow-up duplex studies revealed the evolving refluxing tracts requiring sequential therapies.
Conclusions:
The results in this series demonstrate the utility of foam sclerotherapy using the compass technique in the management of KTS. Chronicity of the VMs in KTS necessitates strict clinical monitoring and sequential therapies.
