An unusual cause of postmenopausal vaginal haemorrhage: a case report.

BACKGROUND: Post-menopause vaginal haemorrhage is typically related to gynaecological malignancies. Bleeding from vaginal varices rarely occurs, especially in nonpregnant women. Moreover, nonpregnancy-related causes of vaginal varicosities include portal hypertension, especially that caused by liver cirrhosis, pelvic congestion syndrome and Klippel-Trenaunay syndrome or Parkes-Weber syndrome. Here, we report an unusual cause of nonpregnancy-associated vaginal variceal bleeding. CASE PRESENTATION: A 55-year-old postmenopausal woman presented in our outpatient department with complaints of recurrent bloody vaginal discharge. A group of varicose veins and several haemorrhagic spots were found on her vaginal wall during a vaginal speculum examination. Genital cancers were excluded by colposcopy and transvaginal ultrasonography; furthermore, a pelvic arteriovenous fistula was not found on a pelvic computed tomography (CT) scan. However, congenital varicosities and deep arteriovenous shunts were observed in her left leg on arterial angiography. Moreover, vaginal bleeding was improved after resolution of the underlying deep arteriovenous shunts in her left leg. Therefore, congenital arteriovenous shunts and elevated inferior vena cava pressure might be responsible for her recurrent vaginal varicose bleeding. CONCLUSION: Haemorrhage due to vaginal varices is easily detected with a vaginal speculum examination. However, diagnosis and treatment of the original disease are important after bleeding is controlled.

Klippel-Trénaunay syndrome: a case report of a rare vascular disorder identified in a rural Canadian hospital.

CONTEXT: Klippel-Trénaunay syndrome (KTS) is a very rare congenital vascular anomaly. It is characterized by the presence of capillary malformation, venous malformation as well as limb overgrowth, generally affecting one extremity. Although clinical characteristics of KTS are well known, the epidemiology and pathophysiology still remain to be defined. Awareness of these disorders is important for rural physicians for managing potential complications. ISSUE: A 60-year-old male presented with symptomatic varicosities and chronic venous insufficiency of the left leg. The patient had a history of several episodes of ulceration and thrombophlebitis on the left side. Physical examination revealed extensive varicosities and a large port-wine stain on the lateral side of the left leg, despite a previous ligation of the saphenofemoral junction. The left leg was circumferentially larger than the right leg. Additional investigations were conducted to rule out arterial disorders and confirm the venous nature of the abnormalities. The patient was clinically diagnosed with KTS and his varicosities were treated surgically using phlebectomy with stab avulsion technique. LESSONS LEARNED: KTS is a distinct entity that belongs to the vascular malformations group. In a rural practice, identification and accurate diagnosis are challenging, as these patients often require a multidisciplinary approach and do not present until later in the course of the condition. An understanding of the correct nomenclature and associated complications is imperative for proper assessment and management in rural and remote settings.

Haploinsufficiency of Klippel-Trenaunay syndrome gene Aggf1 inhibits developmental and pathological angiogenesis by inactivating PI3K and AKT and disrupts vascular integrity by activating VE-cadherin.

Aggf1 is the first gene identified for Klippel-Trenaunay syndrome (KTS), and encodes an angiogenic factor. However, the in vivo roles of Aggf1 are incompletely defined. Here we demonstrate that Aggf1 is essential for both physiological angiogenesis and pathological tumour angiogenesis in vivo. Two lines of Aggf1 knockout (KO) mice showed a particularly severe phenotype as no homozygous embryos were observed and heterozygous mice also showed embryonic lethality (haploinsufficient lethality) observed only for Vegfa and Dll4. Aggf1+/- KO caused defective angiogenesis in yolk sacs and embryos. Survived adult heterozygous mice exhibit frequent haemorrhages and increased vascular permeability due to increased phosphorylation and reduced membrane localization of VE-cadherin. AGGF1 inhibits VE-cadherin phosphorylation, increases plasma membrane VE-cadherin in ECs and in mice, blocks vascular permeability induced by ischaemia-reperfusion (IR), restores depressed cardiac function and contraction, reduces infarct sizes, cardiac fibrosis and necrosis, haemorrhages, edema, and macrophage density associated with IR. Mechanistically, AGGF1 promotes angiogenesis by activating catalytic p110α subunit and p85α regulatory subunit of PI3K, leading to activation of AKT, GSK3ß and p70S6K. AKT activation is significantly reduced in heterozygous KO mice and isolated KO ECs, which can be rescued by exogenous AGGF1. ECs from KO mice show reduced capillary angiogenesis, which is rescued by AGGF1 and AKT. Tumour growth/angiogenesis is reduced in heterozygous mice, which was associated with reduced activation of p110α, p85α and AKT. Together with recent identification of somatic mutations in p110α (encoded by PIK3CA), our data establish a potential mechanistic link between AGGF1 and PIK3CA, the two genes identified for KTS.

High levels of serum sclerostin and DKK1 in a case of Klippel-Trénaunay syndrome.

Klippel-Trénaunay syndrome (KTS) is described as a complex syndrome characterized by various combinations of capillary, venous, and lymphatic malformations associated with bone and soft tissue hypertrophy. We report a case of a 67-year-old postmenopausal Caucasian women with KTS that shows elevated levels of sclerostin and Dickkopf-related protein 1 (DKK1). Dual-energy X-ray absorptiometry (DXA) BMD T-scores at lumbar spine and femur were normal. Serum calcium and phosphorus levels were consistently normal, 25-hydroxyvitamin D (25OHD) < 30 ng/mL, and normal parathyroid hormone (PTH). Turnover markers (serum osteocalcin [OCN], and carboxy-terminal cross-linking telopeptide of type 1 collagen [CTx]) were in the reference limits. It is interesting to note that the serum levels of sclerostin and DKK-1 were significantly higher in our patient with KTS than in a healthy volunteer (control), without impact on bone mineral density and bone formation markers. In fact, in our patient, the BMD at lumbar spine and femur was normal, and osteocalcin was not suppressed. Based on what is known, we would have expected to find low levels of the inhibitors of the Wnt system, perhaps we can explain the data as a response to the compensation for ß-catenin hyper-transformation.

Complications of pregnancy and labour in women with Klippel-Trénaunay syndrome: a nationwide cross-sectional study.

OBJECTIVE: To evaluate complications of pregnancy, including thromboembolism, in women with extensive vascular malformations associated with Klippel-Trénaunay syndrome (KTS). DESIGN: Nationwide cross-sectional study. SETTING: Two tertiary expert centres and the Dutch Klippel-Trénaunay patient organisation. SAMPLE: Adult women with KTS. METHODS: Patients with KTS were invited to participate in a comprehensive online survey about their obstetric history. Reference data on pregnancy outcomes and complications of non-diseased women were collected from population-based cohorts from the literature. MAIN OUTCOME MEASURES: Prevalence of complications, specifically venous thromboembolism and postpartum haemorrhage. RESULTS: Sixty women completed the survey. Seventeen patients did not conceive, of whom three refrained from pregnancy because of KTS. A total of 97 pregnancies and 86 deliveries were reported in 43 patients. KTS-related symptoms were aggravated during pregnancy in 43% of patients. Deep vein thrombosis was present in 5.8% and pulmonary embolism was present in 2.3% of pregnancies, which was extremely high compared with the reference population (P < 0.0001), with a relative risk of 108.9 (95% confidence interval, 95% CI 46.48-255.03) and 106.2 (95% CI 26.97-418.10), respectively. Severe postpartum haemorrhage (PPH) occurred in 11% of KTS pregnancies, compared with 5.8% of pregnancies in the reference population (relative risk, RR 1.81, 95% CI 0.97-3.37, P = 0.06). CONCLUSIONS: Our data suggest that women with KTS have a significant risk of venous thromboembolic events, severe postpartum haemorrhage, and aggravation of KTS symptoms during pregnancy, and in early postpartum period. Obstetricians should counsel patients about these risks in the preconception phase. Antithrombotic prophylaxis should be considered in the obstetric management of patients with KTS. TWEETABLE ABSTRACT: High risk of complications during pregnancy and labour in women with Klippel-Trénaunay syndrome.

Functional characterization of Klippel-Trenaunay syndrome gene AGGF1 identifies a novel angiogenic signaling pathway for specification of vein differentiation and angiogenesis during embryogenesis.

Specification of arteries and veins is a key process for establishing functional vasculature during embryogenesis and involves distinctly different signaling mechanisms. Vascular endothelial growth factor-A (VEGFA) is required for differentiation of arteries; however, the upstream angiogenic factor for vein specification is unknown. Klippel-Trenaunay syndrome (KTS) is a congenital vascular disease associated with capillary and venous malformations (VMs), but not with arterial defects. We have previously reported that upregulation of angiogenic factor AGGF1 is associated with KTS, but the molecular mechanism is not clear. Here, we show that AGGF1 is involved in establishing venous identity in zebrafish embryos. Overexpression of AGGF1 led to increased angiogenesis and increased lumen diameter of veins, whereas knockdown of AGGF1 expression resulted in defective vasculogenesis and angiogenesis. Overexpression of AGGF1 increased expression of venous markers (e.g. flt4), but had little effect on arterial markers (e.g. notch5). Knockdown of AGGF1 expression resulted in a loss of venous identity (loss of expression of flt4, ephb4 and dab2), but had no effect on the expression of arterial development. We further show that AGGF1 activates AKT, and that decreased AGGF1 expression inhibits AKT activation. Overexpression of constitutively active AKT rescues the loss of venous identity caused by AGGF1 downregulation. Our study establishes AGGF1 as an angiogenic factor with an important role in the specification of vein identity and suggests that AGGF1-mediated AKT signaling is responsible for establishing venous cell fate. We propose that increased AGGF1 expression leads to increased vein differentiation by inducing activation of AKT signaling, resulting in VMs s in KTS patients.

[Vascular anomalies]. / Anomalies vasculaires: exemple du syndrome de Klippel-Trenaunay.

Vascular anomalies are rare conditions that could be observed at all ages. They are classified, according to their histology, in vascular tumors or vascular malformations. The general practitioner plays a significant role in diagnosis and patient management, diagnosis being suspected on clinical history. In case of vascular anomaly, ultrasound-Doppler assessment is helpful to characterize morphologic and hemodynamic changes of the lesion and permits to monitor the evolution and to detect complications. Further investigations are often necessary prior to multidisciplinary management. In this article, a brief overview of vascular anomalies, their multidisciplinary management and the exemple of Klippel-Trenaunay syndrome are presented.

Klippel­Trenaunay­Weber syndrome (KTWS) and spontaneous spinal CSF leak: coincidence or link.

OBJECTIVE: To highlight the occurrence of spontaneous cerebrospinal fluid (CSF) leak in the setting of Klippel­Trenaunay­Weber syndrome (KTWS). BACKGROUND: KTWS is a congenital multicomponent disorder of angiogenesis plus limb asymmetry. The cause of spontaneous CSF leaks often remains unknown, but the notion of a pre-existing dural weakness related to a disorder of connective tissue matrix is gaining momentum. REPORT OF CASES AND METHODS: Two women with KTWS developed spontaneous CSF leaks. Each underwent extensive head and spine imaging studies. One patient underwent surgery to treat the CSF leak and later an epidural blood patch upon partial recurrence of her symptoms. The other patient, who had intermittent CSF leak, developed cerebral venous thrombosis requiring several months of anticoagulation therapy. Both patients have histories of visceral bleeding: gastrointestinal in 1 patient and genitourinary in the other. RESULTS: The predominant site of vascular anomaly was the left lower limb in 1 patient and the right upper limb in the other, while the involved limb was larger in 1 patient and smaller in the other. Each patient presented with orthostatic headaches. One had additional choreiform movements and cognitive difficulties that responded to the treatment of the leak. Head magnetic resonance imaging in both patients showed diffuse pachy meningeal enhancement and evidence of sinking of the brain. Computed tomography myelography in 1 patient disclosed the site of the leak; and she underwent surgery to treat the leak, and later an epidural blood patch upon partial recurrence of her symptoms to which she responded well. The other patient had intermittent leak with history of long remission and was reluctant to go through invasive diagnostic or therapeutic measures. CONCLUSION: The occurrence of an uncommon disorder (spontaneous CSF leak) in the setting of a rare congenital disorder in 2 unrelated patients is intriguing. Whether this represents coincidence or a link is not clear but deserves further observations and investigation.

Isolated bilateral external iliac vein aplasia.

We present a case of 11-year-old girl with a history of prominent superficial veins over abdomen and thorax since birth. A superficial vein extending from either inguinal region joined in umbilical region and extended up to right supraclavicular region. Other features of Klippel-Trenaunay syndrome like nevus, limb edema were absent. On radiological investigations both external iliac veins could not be visualized and venous return from lower limbs was draining into the right subclavian vein via these superficial veins. Both external iliac veins could not be identified during surgery.

Atypical conduction aphasia and the right hemisphere: Cross-hemispheric plasticity of phonology in a developmentally dyslexic and dysgraphic patient with early left frontal damage.

We report the rare case of a patient, JNR, with history of mixed handedness, developmental dyslexia, dysgraphia, and attentional deficits associated with a Klippel-Trenaunay syndrome and a small subcortical frontal lesion involving the left arcuate fasciculus. In adulthood, he suffered a large right perisylvian stroke and developed atypical conduction aphasia with deficits in input and output phonological processing and poor auditory-verbal short-term memory. Lexical-semantic processing for single words was intact, but he was unable to access meaning in sentence comprehension and repetition. Reading and writing deficits worsened after the stroke and he presented a combination of developmental and acquired dysgraphia and dyslexia with mixed lexical and phonological processing deficits. This case suggest that a small lesion sustained prenatally or early in life could induce a selective rightward shift of phonology sparing the standard left hemisphere lateralisation of lexical-semantic functions.

Anesthesia for surgery related to Klippel-Trenaunay syndrome: a review of 136 anesthetics.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by the triad of varicose veins or venous malformations, capillary malformations that may involve neurovascular structures, and bony or soft tissue hypertrophy in affected limbs. Areas such as the trunk, bowel, bladder, and spinal cord may be involved as well. KTS should not be confused with Klippel-Feil syndrome, which involves abnormalities of the cervical vertebrae. Anesthetic management for patients with KTS has only been described in limited case reports that caution about potential airway difficulty but do not report surgical hemorrhage requiring transfusion. METHODS: We performed an electronic search of the Mayo Clinic medical record database to identify patients who had undergone an anesthetic for surgery related to KTS. Review of medical records was performed for type of surgery, anesthetic technique, airway management and difficulty, medications used, intraoperative fluid administration, transfusion requirements, vascular access used, and postoperative complications. RESULTS: Eighty-two unique patients were identified who underwent 134 general anesthetics and 2 lumbar neuraxial anesthetics for surgeries related to KTS. Preoperatively, 27% of patients had a history of recurrent bleeding, 24% recurrent cellulitis, 9% deep vein thrombosis, and 2% pulmonary embolism. The mean age at time of surgery was 21 ± 15 years. The majority of surgical procedures involved laser coagulation or varicose vein sclerotherapy or stripping. All of the 74 direct laryngoscopies and tracheal intubations were performed on the first attempt without difficulty. Mask ventilation was possible in all 131 patients for whom this was attempted, with only 1 requiring an oral airway. Documented estimated blood loss ranged from 20 to 18,000 mL, with a mean of 740 ± 2739 mL. Use of a tourniquet did not obviate the possibility of substantial blood loss. The only significant postoperative complication involved a calf hematoma after vein stripping and avulsion that required return to the operating room for evacuation. CONCLUSIONS: Patients with KTS have multiple associated comorbidities relevant to perioperative management. In contrast to previous reports, difficulty with airway management was not encountered. Surgery related to severe KTS may be associated with massive hemorrhage despite tourniquet use, and the anesthesiologist should anticipate the need for appropriate fluid resuscitation. Neuraxial techniques may be considered only if the possibility of trauma to neurovascular malformations has been excluded with recent spine imaging.

Angiogenic and prothrombotic markers in extensive slow-flow vascular malformations: implications for antiangiogenic/antithrombotic strategies.

BACKGROUND: Venous and combined malformations are slow-flow haemodynamically inactive lesions that are present at birth and worsen slowly with advancing age, showing no tendency towards involution. The pathogenesis of vascular anomalies has not been fully elucidated, but their formation and progression are closely related to angiogenesis. Localized intravascular coagulation associated with venous or combined malformations is characterized by low fibrinogen, high D-dimers, and normal platelet count. OBJECTIVES: To assess the relationship of angiogenic factors with prothrombotic and endothelial damage/dysfunction markers in patients with extensive slow-flow vascular malformations. METHODS: A 2-year study (2005-2007) included 31 consecutive patients with extensive slow-flow vascular malformations from one centre. RESULTS: Serum levels of the endothelial receptor tyrosine kinase TIE-2, matrix metalloproteinase (MMP)-9 and angiopoietin (Ang)-2 and plasma levels of D-dimer, plasminogen activator inhibitor type 1 (PAI-1), tissue-type plasminogen activator and von Willebrand factor (vWf) were significantly increased in patients compared with healthy controls, whereas serum levels of vascular endothelial growth factor (VEGF)-C, VEGF-D, MMP-2, Ang-1, platelet-derived growth factor (PDGF)-AB and PDGF-BB were significantly decreased in patients compared with controls. A strong positive correlation was present between Ang-1 and PDGF-AB levels (r = 0.63, P < 0.001), between PDGF-AB and PDGF-BB levels (r = 0.67, P < 0.001), and between fibrinogen and PAI-1 levels (r = 0.41, P = 0.031). A strong negative correlation was present between Ang-1 and vWf levels (r = -0.48, P = 0.006), between D-dimer and fibrinogen levels (r = -0.71, P < 0.001), and between PDGF-AB and vWf levels (r = -0.42, P = 0.017). CONCLUSIONS: These findings suggest that angiogenic, coagulation and endothelial damage/dysfunction markers are possibly linked in pathogenesis of extensive slow-flow vascular malformations, and might have therapeutic implications.

Management of knee arthropathy in patients with vascular malformations.

OBJECTIVE: To describe our experience with surgical intervention for symptomatic intraarticular vascular malformations of the knee in patients with peripheral vascular malformations including Klippel-Trénaunay syndrome (KTS). METHODS: Eleven patients underwent surgical intervention for symptomatic intraarticular vascular malformations of the knee between 1987 and 2008. Seven patients had KTS, and 4 patients had venous malformations. Surgical indications, imaging studies, clinical course, surgical procedures, complications, and follow-up visits were reviewed and recorded. RESULTS: A total of 11 patients (8 males; 3 females; mean age, 11.7 years; range, 2.5-23 years) underwent 12 surgical procedures. Five patients had an amputation, and 6 patients had knee synovectomies. One patient had bilateral knee synovectomies. Surgical indications included pain, swelling, limited mobility, and/or loss of knee motion. The average time of follow-up was 54 months (range, 7-109 months). Patient-reported pain scores decreased significantly from a mean of 2.9 +/- 1.4 preoperatively to 1.3 +/- 0.9 postoperatively (P = 0.01). CONCLUSIONS: When necessary, surgical intervention for intraarticular vascular malformations of the knee (amputation or synovectomy) may be effective in decreasing pain and improving mobility in patients with peripheral vascular malformations. It is possible that early synovectomy may slow or prevent the rapid destructive arthritis that occurs in these knees. Surgeons and patients should anticipate complications related to bleeding from vascular malformations. We recommend a multidisciplinary approach to the patient with KTS, particularly when surgical intervention is indicated.

Late puerperal hemorrhage of a patient with Klippel-Trenaunay syndrome: A case report.

INTRODUCTION: The Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder. The obstetric course of women with KTS varies. Complications include bleeding, disseminated intravascular coagulation (DIC), thromboembolic events, etc. PATIENT CONCERNS:: Here, we report a case of late puerperal hemorrhage of a Chinese puerpera with KTS. The repeating severe hemorrhage, the DIC, and the Kasabach-Merrit syndrome made the treatment more difficult. DIAGNOSIS: KTS is a mixed malformation with a vascular component that is characterized by abnormal development of veins, capillaries, and lymphatics. Our patient was first diagnosed with KTS at the last trimester of pregnancy. INTERVENTIONS: Massive infusion of blood products, two laparotomies, as well as bilateral internal iliac artery embolization was carried out. OUTCOMES: Although the patient survived from the life-threatening hemorrhage, she lost her uterus forever. CONCLUSION: An interdisciplinary cooperation of obstetrician, anesthesiologist, vascular surgeon, and intensive care physician is highly recommended. Prophylactic anticoagulation is generally advised in the gestational and postpartum period.

Multimodality imaging approach in a patient with Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder presenting with asymmetric limb hypertrophy, cutaneous capillary malformations and lower extremity varicosities. We discuss a 27-year-old man born with varicosities on both lower extremities, which progressively enlarged. Physical examination showed a grossly enlarged right hand. There were multiple compressible varicosities, diffuse port-wine stains on the right leg and limb-length discrepancy on the left leg. CT angiogram and Doppler ultrasound revealed several venous varicosities. Ectatic veins in the right leg converge into the lateral marginal vein of Servelle, an embryonic vein, typically seen in KTS patients. KTS is diagnosed clinically and imaging plays a role in differentiating this from other disease entities that present similarly. Doppler ultrasound is the initial imaging of choice to characterise varicosities and to identify thrombosis and reflux. Plain radiographs confirm limb hypertrophy. MRI and CT angiograms are useful to evaluate vascular anomalies and its accompanying soft tissue changes.

A case of Klippel-Trenaunay syndrome involving only upper limbs.

The case of a 14-year-old girl suffering from Klippel-Trenaunay syndrome associated with multiple port-wine stain type vascular anomalies and varicose veins involving upper limbs is described. Finger deformations are common in Klippel-Trenaunay syndrome. In our patient, striking hypertrophy of soft tissues and overgrowth of bones were observed on both forearms and finger deformations were observed particularly on right thumb and index finger. Although lower limb involvement is very common amongst the patients with Klippel-Trenaunay syndrome, in our patient involvement of the lower limbs was not observed.

Radiological Aspect of Klippel-Trénaunay Syndrome: A Case Series With Review of Literature.

Klippel-Trénaunay syndrome (KTS) is a rare angio-osteo-hypertrophic syndrome characterized by vascular malformations, soft tissue and/or bone hypertrophy, and varicose veins. For the purpose of describing the imaging findings and elucidating the role of medical imaging in the diagnosis and assessment of patient with KTS, we have reviewed the imaging data of 14 KTS patients. The imaging features on different imaging modalities were analyzed. Unilateral lower limb involvement was evident in 71% of cases (n=10) and bilateral but asymmetric lower limb involvement in the remaining 29% of cases (n=4). The most commonly depicted imaging features were varicosities in 93% (n=13), muscle hypertrophy in 79% (n=11) and venous anomalies in 64% (n=9). Other less common imaging findings included lymphedema in 29% (n=4), arterial malformations 29% (n=4), soft tissue hemangiomas 21% (n=3), pelvic and thigh phleboliths 21% (n=3), venous aneurysms 21% (n=3), bone abnormalities 14% (n=2) and lymphadenopathy 14% (n=2). A severe unilateral lower limb deformity resulting in contractures and muscle atrophy of the whole limb was depicted in 1 case. The pathognomonic marginal vein of Servelle was identified in 2 cases. AV shunt was highly suspected in 4 cases and was confirmed by DSA in 1 case, making Klippel-Trénaunay-Weber syndrome a more apt diagnosis. Associated ipsilateral duplicated renal artery was found in 1 case. We have concluded that medical imaging is the cornerstone in the diagnosis and assessment of severity and complications, follow-up and differentiation of KTS from other similar conditions. Different imaging modalities play complementary roles in the evaluation of KTS patients.

Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome: a case report.

BACKGROUND: Klippel-Trenaunay-Weber syndrome is a rare syndrome; unfortunately, very few studies of the connection between hypersplenism, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome have been published. CASE PRESENTATION: We report the case of a 40-year-old white man with a typical clinical presentation of Klippel-Trenaunay-Weber syndrome, including "port-wine stains," varicose veins, hypertrophy of lower extremities, and arteriovenous fistula, as well as an unfortunate development of hypersplenism and nephrotic syndrome. CONCLUSIONS: This case report described considerable atypical relevance of Klippel-Trenaunay-Weber syndrome and hypersplenism together with nephrotic syndrome. A multidisciplinary approach was made. Unfortunately, hypersplenism is characterized by pancytopenia that suggests splenectomy, whereas nephrotic syndrome is an indication for renal biopsy; the splenectomy and renal biopsy were delayed due to our patient's severe condition. Deeper analysis including study of other patients with Klippel-Trenaunay-Weber syndrome would help us to understand the connection between elevated spleen and liver sizes, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome.