Reply

Abstract

Drs Dimakakos and Portinos raise several issues regarding the complex clinical presentation and treatment of Klippel-Trénaunay syndrome (KTS). The presence of the classic triad of limb hypertrophy, varicosity, and capillary malformation (port-wine stain) is not always present. In the Mayo Clinic series, published by Jacob et al,1Jacob AG Driscoll DJ Shaughnessy WJ Stanson AW Clay RP Gloviczki P Klippel Trenaunay syndrome: spectrum and management.Mayo Clin Proc. 1998; 73: 28-36Abstract Full Text Full Text PDF PubMed Google Scholar only 159 (63%) of 246 patients had all three features of the syndrome. Lymphatic anomalies have also been noted in KTS, and they are probably much more frequent than reported. Limb hypertrophy and swelling and the mixed vascular malformations in the subcutaneous tissues and muscles almost always have a component of lymphatic abnormality. The frequency of digital anomalies is well-known, and an excellent article on this has been published in the orthopedic literature by Amadio et al.2Amadio PC Dobyns JH Stickler GB Unni KK Anomalies of the fingers and toes associated with Klippel-Trenaunay syndrome.J Bone Joint Surg. 1991; 73A: 1537-1546Google Scholar Syndactyly and polydactyly have been the most frequent digital anomalies in our recent review.1Jacob AG Driscoll DJ Shaughnessy WJ Stanson AW Clay RP Gloviczki P Klippel Trenaunay syndrome: spectrum and management.Mayo Clin Proc. 1998; 73: 28-36Abstract Full Text Full Text PDF PubMed Google Scholar The pathogenesis of such skeletal anomalies is unknown, but it is very likely that the soft tissue and bone hypertrophy are not direct consequences of the hemodynamic changes of the vascular malformations but are independent defects in the limb development. Concomitant mitral valve prolapse or spinal stenosis, mentioned by the authors, are rare. Venous ulcers are usually results of venous hypertension, although erosion of the skin can also be due to extensive superficial vascular malformations. Venous hypertension should be relieved with stripping of the superficial incompetent veins, removal of incompetent avalvular superficial embryonic veins, varicose vein avulsion, or subfascial endoscopic perforator vein ligation, when necessary. The authors are right that the saphenous vein is rarely involved and if competent, should not be stripped. Occasionally, however, the greater saphenous vein is also enlarged and incompetent, and if the deep system is patent, the saphenous vein in these patients can be removed, with good clinical results. We cannot emphasize enough, however, the need for thorough preoperative imaging with contrast or magnetic resonance venography and duplex scanning. High-flow, high-shunt arteriovenous fistulas are not present in KTS, but clinically insignificant microfistulas may occur. One theory of the etiology of KTS suggests that intrauterine injury to the sympathetic ganglia may result in microscopic arteriovenous anastomoses. The clinical significance of this, however, is unknown. When considering deep venous reconstruction in KTS, preoperative pressure measurements, as suggested by the authors, are helpful to confirm that collateral circulation is inadequate. The larger the pressure gradient, the better is the chance of graft patency. Venous bypasses in KT patients are rarely needed. Most patients, as mentioned in the manuscript, are managed conservatively. We must reiterate, as the authors did in their letter, the need for multidisciplinary management of patients with KTS in specialized vascular center. We thank Drs Dimakakos and Portinos for considering these salient points and for their active interest in this fascinating disease. 24/41/117326