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Clinical presentation and outcomes after endovascular management in a mixed pediatric and adult Klippel-Trenaunay syndrome population

Published:March 19, 2021DOI:https://doi.org/10.1016/j.jvsv.2021.03.005

      Abstract

      Objective

      We retrospectively studied the clinical presentations and outcomes of endovascular management in a mixed pediatric and adult Klippel-Trenaunay syndrome (KTS) population at a single academic medical center.

      Methods

      We performed a retrospective study of patients with KTS who had been referred for endovascular intervention after evaluation and diagnosis by a multidisciplinary team at a single academic medical center during a 10-year period. The patient demographics, areas affected, presenting symptoms, previous treatments, imaging modalities, endovascular treatment types, number of treatments, and complications were assessed. The technical and clinical success rates were calculated.

      Results

      Twenty-six patients with suspected KTS were evaluated. Of these 26 patients, 20, aged 2 to 75 years, had been diagnosed with KTS using the International Society for the Study of Vascular Anomalies criteria and referred for endovascular management. The left lower extremity was affected most often. The presenting symptoms were pain (80%), edema (70%), bleeding (10%), numbness (25%), and claudication (25%). Of the 20 patients, 16 (80%) had undergone treatment of KTS before presenting to our institution. Magnetic resonance imaging and ultrasound (US) were the most common imaging modalities. Fifteen patients underwent 46 endovascular treatments during the study period. The treatments included 5 endovenous ablations only, 4 US-guided sclerotherapies with endovenous ablation, 5 US-guided sclerotherapies only, and 32 catheter-directed venograms with additional interventions. Localized intravascular coagulopathy was the only procedure-related complication and occurred in one patient after three treatments. The technical success rate was 97.8%, and the clinical success rate was 100%.

      Conclusions

      Endovascular intervention is safe and effective for KTS patients for whom conservative management has failed. Pain and edema were the most common presenting symptoms. Presenting symptoms may be related to pathology of anomalous veins, orthotopic superficial veins or deep veins. Venous claudication can be present in those with KTS despite patency of the deep venous system. Magnetic resonance imaging and duplex US are frequently used modalities for venous assessment. The complications of endovascular treatment are rare but include localized intravascular coagulopathy.

      Keywords

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      References

      1. ISSVA Classification for Vascular Anomalies.
        (Available at:) (Accessed September 12, 2020)
        • John P.R.
        Klippel-Trenaunay syndrome.
        Tech Vasc Int Radiol. 2019; 22: 100634
        • Le Cras T.D.
        • Boscolo E.
        Cellular and molecular mechanisms of PIK3CA related vascular anomalies.
        Vasc Biol. 2019; 28: H33-H40
        • Bertino F.
        • Braithwaite K.A.
        • Hawkins C.M.
        • Gill A.E.
        • Briones M.A.
        • Swerdlin R.
        • et al.
        Congenital limb overgrowth syndromes associated with vascular anomalies.
        Radiographics. 2019; 39: 491-515
        • Delis K.T.
        • Gloviczki P.
        • Wennberg P.W.
        • Rooke T.W.
        • Driscoll D.J.
        Hemodynamic impairment, venous segmental disease, and clinical severity scoring in limbs with Klippel-Trenaunay syndrome.
        J Vasc Surg. 2007; 45: 561-567
        • Noel A.
        • Gloviczki P.
        • Cherry K.J.
        • Rooke T.W.
        • Stanson A.W.
        • Driscoll D.J.
        Surgical treatment of venous malformations of Klippel-Trenaunay syndrome.
        J Vasc Surg. 2000; 32: 840-847
        • Wang S.K.
        • Drucker N.A.
        • Gupta A.K.
        • Marshalleck F.E.
        • Dalsing M.C.
        Diagnosis and management of the venous malformations of Klippel-Trenaunay syndrome.
        J Vasc Surg Venous Lymphat Disord. 2017; 5: 587-595
        • Sreekar H.
        • Dawre S.
        • Petkar K.S.
        • Shetty R.B.
        • Lamba S.
        • Naik S.
        • et al.
        Diverse manifestations and management options in Klippel-Trenaunay syndrome: a single centre 10-year experience.
        J Plast Surg Hand Surg. 2013; 47: 303-307
        • Sermsathanasawadi N.
        • Hongku K.
        • Wongwanit C.
        • Ruangsetakit C.
        • Chinsakchai K.
        • Mutirangura P.
        Endovenous radiofrequency thermal ablation and ultrasound-guided foam sclerotherapy in treatment of Klippel-Trenaunay syndrome.
        Ann Vasc Dis. 2014; 7: 52-55
        • Nassiri N.
        • Crystal D.
        • Huntress L.A.
        • Murphy S.
        Transcatheter embolization of persistent embryonic veins in venous malformation syndromes.
        J Vasc Surg Venous Lymphat Disord. 2017; 5: 749-755
        • Fereydooni A.
        • Nassiri N.
        Evaluation and management of the lateral marginal vein in Klippel-Trenaunay and other PIK3CA-related overgrowth syndromes.
        J Vasc Surg Venous Lymphat Disord. 2020; 8: 482-493
        • Intersocietal Accreditation Commission
        IAC standards and guidelines for vascular testing accreditation.
        (Available at:) (Accessed January 3, 2021)
        • Khalilzadeh O.
        • Baerlocher M.O.
        • Shyn P.B.
        • Connolly B.L.
        • Devane A.M.
        • Morris C.S.
        • et al.
        Proposal of a new adverse event classification by the Society of Interventional Radiology Standards of Practice Committee.
        J Vasc Interv Radiol. 2017; 28: 1432-1437
        • Parker V.E.R.
        • Keppler-Noreuil K.M.
        • Faivre L.
        • Luu M.
        • Oden N.L.
        • De Silva L.
        • et al.
        Safety and efficacy of low-dose sirolimus in the PIK3CA-related overgrowth spectrum.
        Genet Med. 2019; 21: 1189-1198
        • Adams D.M.
        • Trenor C.C.
        • Hammill A.M.
        • Vinks A.A.
        • Patel M.N.
        • Chaudry G.
        • et al.
        Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies.
        Pediatrics. 2016; 137: e20153257
        • Killewich L.A.
        • Martin R.
        • Cramer M.
        • Beach K.W.
        • Strandness D.E.
        Pathophysiology of venous claudication.
        J Vasc Surg. 1984; 1: 507-511
        • Johnson C.M.
        • Navarro O.M.
        Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations.
        Pediatr Radiol. 2017; 47: 1196-1208
        • Alomari A.I.
        Diversion venography—a modified technique in Klippel-Trenaunay syndrome: initial experience.
        J Vasc Interv Radiol. 2010; 21: 685-689
        • Bittles M.
        • Jodeh D.S.
        • Mayer J.L.R.
        • Gallant M.
        • Rottgers S.A.
        Laser ablation of embryonic veins in children.
        Pediatr Int. 2019; 61: 358-363
        • Patel P.A.
        • Barnacle A.M.
        • Stuart S.
        • Amaral J.G.
        • John P.R.
        Endovenous laser ablation therapy in children: applications and outcomes.
        Pediatr Radiol. 2017; 47: 1353-1363
        • Hung J.W.S.
        • Leung M.W.Y.
        • Liu C.S.W.
        • Fung D.H.S.
        • Poon W.L.
        • Yam F.S.D.
        • et al.
        Venous malformation and localized intravascular coagulopathy in children.
        Eur J Pediatr Surg. 2017; 27: 181-184
        • Zhuo K.
        • Russell S.
        • Wargon O.
        • Adams S.
        Localised intravascular coagulation complicating venous malformations in children: associations and therapeutic options.
        J Pediatr Child Health. 2017; 53: 737-741
        • Dompmartin A.
        • Acher A.
        • Thibon P.
        • Tourbach S.
        • Hermans C.
        • Deneys V.
        • et al.
        Association of localized intravascular coagulopathy with venous malformations.
        Arch Dermatol. 2008; 144: 873-877
        • Binet Q.
        • Lambert C.
        • Hermans C.
        Dabigatran etexilate in the treatment of localized intravascular coagulopathy associated with venous malformations.
        Thromb Res. 2018; 168: 114-120