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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Klippel-Trenaunay-Weber syndrome &#40;KTS&#47;KTWS&#41; is a congenital disorder defined by the triad of capillary malformations of &#40;haemangiomas or port-wine stains&#41; and venous &#40;varicose veins&#41; and overgrowth of bone and soft tissue with or without lymphatic malformations&#46; It has been described genetic association with the PIK3CA gene encoding the protein p110&#945;&#44; a subunit of the enzyme phosphatidylinositol 3-kinase &#40;PI3K&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> Major complications include coagulation disorders in the form of thrombosis&#44; particularly in the renal vein or the central retinal vein&#44; less common is the occurrence of deep vein thrombosis and pulmonary thromboembolism&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">It has been associated with renal disease in three cases of nephrotic syndrome<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#8211;6</span></a> &#40;one with focal segmental glomerulosclerosis &#91;FSGS&#93;&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> urological malformations &#40;one with upper tract renal haemorrhage requiring nephrectomy<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a>&#41; and one case of unilateral polycystic kidney disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;8</span></a> To our knowledge&#44; there have been no previous reports of patients with KTWS who have received a kidney transplant&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a 39-year-old male patient with a previous medical history of hypertension&#44; insulin-dependent type 2 diabetes mellitus&#44; obesity with a body mass index of 35&#8239;kg&#47;m<span class="elsevierStyleSup">2</span>&#44; hyperuricaemia with nephrolithiasis that required nephrectomy in 2003 and thrombophilia&#44; with positive lupus anticoagulant antibody and antiphospholipid syndrome with thrombosis of the renal vein in 2015 and of the bilateral central retinal vein in 2016&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> on treatment with Sintrom&#174;&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Renally&#44; the patient had clinical and biochemical nephrotic syndrome&#44; diagnosed by renal biopsy in 2015 with perihilar FSGS secondary to obesity in a patient with a single kidney&#44; and treated with anti-proteinuric measures&#46; The chronic kidney disease progressed to stage <span class="elsevierStyleSmallCaps">V</span> and he started haemodialysis in 2020&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In the context of KTS the patient presented with facial haemangiomas &#40;port-wine stain&#41;&#44; lymphoedema&#44; hypertrophy of the lower limbs and vascular malformations&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">For inclusion on the transplant waiting list&#44; a computed tomography angiogram was performed&#44; which revealed neoformed vessels surrounding the left iliac vessels with involvement of the left lower limb&#46; All this was discussed in the renal transplant committee&#44; in conjunction with vascular surgery&#44; urology and radiology&#44; and he was considered suitable for renal transplantation&#46; Finally&#44; the patient received a brain-dead donor cadaveric kidney transplant and as the recipient was at low immunological risk&#44; immunosuppression was based on basiliximab and triple therapy with corticosteroids&#44; tacrolimus and mycophenolate&#46; In view of the patient&#39;s history of thrombophilia and the fact that KTWS could increase the risk of thrombosis&#44; the following thromboprophylaxis strategy was applied&#58; a change of treatment from Sintrom&#174; to acetylsalicylic acid 100&#8239;mg&#47;24&#8239;h since inclusion on the transplant list until transplantation&#46; No blood transfusions were required during surgery&#44; but the patient did need transfusions in the post-transplant period due to a drop in haemoglobin to 6&#46;8&#8239;g&#47;dl related to the presence of perirenal haematomas&#44; which did not require surgical intervention&#46; He was treated with transfusion of two units of red blood cell concentrate and darbepoetin 80 mcg&#47;week&#46; Subsequently&#44; he was started with prophylactic treatment of low-molecular-weight heparin and finally switched to full-dose Sintrom&#174;&#46; The kidney transplant resulted in immediate renal function&#44; with creatinine on discharge &#40;and usual&#41; of 2&#46;3&#8239;mg&#47;dl&#44; glomerular filtration rates of 32&#8239;ml&#47;min&#47;1&#46;73 m<span class="elsevierStyleSup">2</span> &#40;by CKD-EPI&#41; and usual haemoglobin levels of 13&#8239;g&#47;dl without darbepoetin treatment&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The association between KTWS and kidney disease has been described previously&#46; In our patient the kidney disease did not seem to be related to KTWS&#44; rather it was explained by his cardiovascular risk factors in a patient with a single kidney following nephrectomy&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">To our knowledge&#44; this is the first published case of renal transplantation and KTWS&#44; and we should highlight that patients with KTWS are said to have a risk of vascular malformations&#59; from abnormal neoformed vessels predisposing to bleeding to duplication of the vena cava&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;10</span></a> They may also have an increased risk of lymphoedema and lymphoceles&#46; We would therefore stress the importance of carrying out a thorough pre-transplant vascular study of the abdominal vessels&#44; with computed tomography angiogram being recommended&#44; both in arterial and venous phases&#44; as well as a multidisciplinary assessment&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Another important aspect of the case was the strategy of early anticoagulation for thrombophilia&#44; made worse by the KTWS&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">With regard to immunosuppression&#44; the use of mammalian target of rapamycin &#40;MTOR inhibitors&#41; would not be advisable due to their association with lymphatic complications&#44; and a case has been described of a patient with KTWS who received treatment with everolimus &#40;due to severely symptomatic vascular malformations&#41; developing a pulmonary embolism despite being anticoagulated&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">In conclusion&#44; patients with KTWS are not at increased risk of kidney disease&#44; but if they need a kidney transplant&#44; a detailed assessment of the abdominal venous tree must be made in order to plan an adequate surgical approach&#44; and post-transplant use of MTOR inhibitors is not advisable&#44; due to the risk of worsening thrombotic and lymphatic complications&#46;</p></span>"
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