Prevalence and inventory of venous anatomical abnormalities in the arms of patients with combined capillary, venous and lymphatic malformations (Klippel-Trénaunay syndrome).

OBJECTIVES: To investigate the prevalence of arm-involvement in Klippel-Trénaunay syndrome (KTS)-patients and to describe the venous anatomy and/or venous aberrations present in the arm, and if possible, their relationship to complaints (pain, congestion and thromboembolic events). METHODS: A retrospective cohort-study was performed with data from medical records of a large KTS-cohort (n = 173) from a tertiary referral center. Within this cohort, a descriptive study (n = 12) was performed on the KTS-patients with arm involvement and who had been examined with Colour Duplex Ultrasonography (CDU). RESULTS: Our KTS-cohort (n = 173) comprised 43 patients (24.9%) with arm-involvement; in nineteen patients (11.0%) the arm was the only affected limb. Of those KTS patients investigated with CDU, 9 out of 12 (75%) had an aberrant venous anatomy. CONCLUSION: Future research needs to clarity whether the complaints of KTS-patients in general are caused by an aberrant venous anatomy, coagulation alterations and/or other factors. Herein, the KTS-arms may play an important role.

Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study.

BACKGROUND: A number of studies have demonstrated spinal anomalies associated with Klippel-Trenaunay syndrome (KTS). To date, there are no large consecutive series examining the prevalence and subtype distribution of spinal neurovascular malformations in patients with KTS. OBJECTIVE: To report the spectrum and incidence of spinal neurovascular manifestations in the KTS population. METHODS: This was a cross-sectional study. Consecutive patients with definite KTS as defined by International Society for the Study of Vascular Anomalies criteria who underwent spinal neuroimaging at our institution were included. All studies were evaluated by a staff neuroradiologist and a senior radiology resident for the presence of developmental venous anomalies, cavernous malformations (CMs), and arteriovenous shunts (AVS). RESULTS: A total of 116 patients with definite KTS who underwent spinal neuroimaging were included. A total of 23 neurovascular anomalies were found in 19 patients (16.4%), including 4 patients with multiple anomalies. These included 5 patients with spinal cord CMs (4.3%), 14 patients with a paraspinal or epidural venous malformation (12.1%), and 4 patients with an AVS (3.4%). Of the AVS, 3 were epidural arteriovenous fistulas, 1 of which likely formed de novo in an epidural venous malformation. One was a conus medullaris arteriovenous malformation. CONCLUSION: Our study cohort of 116 KTS patients demonstrated a wide spectrum of spinal neurovascular anomalies with a relatively high prevalence. Potential phenotypic descriptions of KTS should include the possibility for spinal neurovascular anomalies.

Skin-Related complications of Klippel-Trenaunay Syndrome: a retrospective review of 410 patients.

BACKGROUND: Little is known about skin-related complications in Klippel-Trenaunay syndrome (KTS), a complex vascular anomaly defined by capillary malformation (CM), venous malformation (VM) ± lymphatic malformation (LM) and limb overgrowth. Reported skin-related complications of KTS include ulceration, vascular ectasias (blebs), bleeding and infection. OBJECTIVE: To determine the spectrum, prevalence and predictors of skin-related complications in KTS. METHODS: A retrospective review of 410 patients fulfilling KTS criteria was performed to assess for the presence of skin-related complications. RESULTS: Skin-related complications were present in 45% of patients. Most prevalent were CM-related complications including blebs, bleeding, thickening (25%), cellulitis (22%) and ulceration (21%). Features positively associated with skin-related complications were presence of LM (OR 17.17; P < 0.001), VM on the buttocks/perineum/genitalia (OR 1.92; P = 0.009), CM on the feet (OR 1.77; P = 0.039) and male sex (OR 1.63; P = 0.034). Features negatively associated with skin-related complications were CM on the trunk (OR 0.59; P = 0.029) and tissue hypertrophy of the hands (OR 0.27; P = 0.025). CONCLUSION: Skin-related complications affect nearly half of patients with KTS. Those with lymphatic involvement or malformation presence in the undergarment area or feet are most at risk.

The anatomic distribution of isolated and syndrome-associated port-wine stain.

BACKGROUND/OBJECTIVES: To determine the role of sex in port-wine stain (PWS) distribution and describe the epidemiologic and anatomic differences between syndrome-associated and non-syndrome-associated PWS using modern criteria. METHODS: A retrospective review of PWS patients aged 18 years and younger from 1995 to 2018 seen in the Department of Dermatology at an academic tertiary referral center. Cases were reviewed for sex, anatomic location, and presence of associated syndrome. 4,527 records were reviewed on the basis of ICD billing codes for congenital vascular malformations, with 516 meeting inclusion criteria. RESULTS: 516 patients were included in the analysis: 234 (45.4%) men and 282 (54.6%) women. A female preponderance of Sturge-Weber syndrome (18 of 23, 78%, P = .03) and a trend toward more female-isolated PWS (149 of 269, 55%, P = .72) were found. No lateral predominance observed for isolated PWS was found: 112(41.6%) limited left-side lesions and 113(42%) limited right-side lesions (P = .41). A trend toward Klippel-Trenaunay syndrome (KTS)-associated PWS occurring more commonly isolated to the left side (76 (45.5%) vs 59 (35.12%) P = .29) was found. Nine percent of SWS patients had a PWS on the body. Five percent of KTS patients had a facial PWS. The lower limb was the most common location overall of body PWS with 33.8% of isolated PWS and 81.5% of KTS patients having a lower limb lesion. CONCLUSIONS: Female children were more likely to be diagnosed with SWS, and a trend toward more isolated PWS in women was found. No lateral predominance of isolated PWS was found, but KTS-associated PWS was more common on the left. A considerable proportion of lesions do not appear in anatomic locations traditionally considered typical in the setting of associated syndromes, which underscores the importance of conducting a complete physical examination and adhering to diagnostic criteria for those syndromes.

Cancer Risk in Klippel-Trenaunay Syndrome.

Background: Klippel-Trenaunay syndrome (KTS) is an overgrowth syndrome defined by capillary/venous/lymphatic malformations (CVLM) with soft tissue and/or bone hypertrophy. Whether KTS predisposes to cancer is not clear. Methods and Results: We surveyed members of the K-T Support Group (KTSG) and reviewed PubMed for "Klippel Trenaunay Syndrome" or "CVLM" and "cancer." Individuals with cancer were reviewed for confirmation of KTS, tumor type, location, and age at presentation. Of 223 KTSG respondents, 24 (10.8%) reported 26 malignancies or benign brain tumors (diagnosed from 6 months to 68 years of age, median 41 years), including 3 who were younger than 18 years (2 with Wilms tumor). Nine of twenty-six cancers were basal cell carcinomas (4% of respondents). From 475 articles, we identified 11 cancers or brain tumors in 10 individuals with KTS. Four of these were in children (Wilms tumor n = 2; rhabdomyosarcoma n = 1; serous borderline tumor n = 1). Tumors in adults included basal cell carcinoma (n = 1), squamous cell carcinoma of skin (n = 2), and angiosarcoma, Hodgkin disease, glioblastoma, malignant hemangiopericytoma in one patient each. Ulceration or lymphedema associated with VLM or capillary malformations were associated with some basal cell or squamous cell carcinomas and angiosarcomas. Conclusions: The risk of embryonal cancer other than Wilms tumor in children with KTS does not appear to be higher than in the general population. Wilms tumor incidence is under 5%, and routine surveillance is not indicated. In adults, particular attention should be paid to skin in the area of malformations. These conclusions may not apply to all overgrowth syndromes with vascular malformations.

Pain, psychiatric comorbidities, and psychosocial stressors associated with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. Patients with KTS have lower scores in general in mental health, physical function, and quality of life than the general population. OBJECTIVE: To determine the prevalence of pain and psychiatric comorbidity in patients with KTS. METHODS: A retrospective review of 410 patients with KTS evaluated during 1976-2012 was conducted to identify the presence of pain, psychiatric comorbidities, and psychosocial stressors. RESULTS: Pain was reported by 260 patients (63.4%) and was associated with any KTS complication (P < .0001) and venous malformations of the lower extremities (P = .0008) and feet (P = .0007). Ninety-five patients had a diagnosed psychiatric condition (23.2%), most commonly depression (15.1%) and anxiety (5.1%). Pain (P = .0016), superficial thrombosis (P = .0269), deep embolic/thrombotic events (P = .0005), gastrointestinal complications (P = .0085), genitourinary complications (P = .0163), and capillary malformation of the hands (P = .0040) were associated with having a psychiatric diagnosis. LIMITATIONS: This is a retrospective study that relied on physician detection and reporting of variables. CONCLUSION: Pain and psychiatric conditions, particularly depression and anxiety, are common in patients with KTS. Awareness of the psychosocial impact of KTS and appropriate screening are important.

Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome.

OBJECTIVE: Patients with Klippel-Trénaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities (CLOVES) syndrome have central phlebectasia and enlarged persistent embryonic veins that are often incompetent and prone to thromboembolism. The purpose of the study was to determine the presence of phlebectasia and the incidence of symptomatic pulmonary embolism (PE). METHODS: A retrospective review was conducted of patients referred to the Vascular Anomalies Center at our institution during a 21-year period who were diagnosed with KTS and CLOVES syndrome. Of these, the patients who had PE were screened for thromboembolic risk factors in addition to phlebectasia and the presence of persistent embryonic veins. Treatment outcomes following subsequent endovascular and medical therapies were reported. RESULTS: A total of 12 KTS patients of 96 (12.5%) and 10 CLOVES syndrome patients of 110 (9%) suffered PE. Fourteen patients (64%) developed PE after surgery or sclerotherapy. All of the patients had abnormally dilated central or persistent embryonic veins; 20 patients were treated with anticoagulation (1 died at the time of presentation, and no information was available for 1) after PE, and 14 (66%) patients underwent subsequent endovascular treatment. Five patients developed recurrent PE despite anticoagulation. Two of the patients died of PE. No patients treated with endovascular closure of dilated veins had subsequent evidence of PE. CONCLUSIONS: Patients with KTS and CLOVES syndrome are at high risk for PE, particularly in the postoperative period.

[Vascular Cutaneous Abnormalities in Togo: a 120-Case Study]. / Anomalies vasculaires cutanées au Togo : étude de 120 cas.

The purpose of this study was to describe the epidemio-clinical profile, and treatment of vascular cutaneous abnormalities in Togo. It was a retrospective study of patients recorded in dermatology for vascular cutaneous abnormality between 1998 and 2017. During the study period, 120 (0.1%) of 88,869 patients received in dermatology have consulted for vascular cutaneous abnormalities. Their mean age was 25.3 months and the sex-ratio (M/F) 0.4. The most recurring vascular cutaneous abnormalities were vascular tumors (97 cases; 80.8%), mainly infantile hemangioma (IH) (93 cases; 77.5%). The mean time of onset of IH after birth was 2.9 months. The IH (54 cases; 50.5%) and port wine stains (8 cases, 53.3%) were predominantly localized at the cephalic region and Klippel-Trenaunay syndrome cases on lower limbs. We opted for a therapeutic abstention in the majority of the patients (50 cases of IH, 16 cases of simple vascular abnormalities, all Klippel-Trenaunay syndrome cases and telegiectasia cases). Of the 43 IH cases treated, the main drugs used were corticosteroids (23 cases of which 17 between 1998 and 2011 and 6 from 2012) followed by propranolol (11 cases from 2012). We had a favorable response in 9 of the 15 patients seen again. Cutaneous vascular abnormalities are very rare in dermatology in Togo and are dominated by IH with female predominance. In IH treatments, propranolol use, started in 2012 in Togo, is increasing when corticotherapy has declined.

L'objectif de cette étude était de décrire le profil épidémioclinique et thérapeutique des anomalies vasculaires cutanées au Togo. Il s'agit d'une étude rétrospective portant sur les dossiers des patients reçus en dermatologie pour une anomalie vasculaire cutanée entre 1998 et 2017. Durant la période d'étude, 120 (0,1 %) des 88 869 patients reçus en dermatologie ont consulté pour une anomalie vasculaire cutanée. L'âge moyen des patients était de 25,3 mois et le sex-ratio (H/F) de 0,4. Les anomalies vasculaires les plus recensées étaient les tumeurs vasculaires (97 cas ; 80,8 %), principalement les hémangiomes infantiles (HI) [93 cas ; 77,5 %]. Le délai moyen d'apparition des HI après la naissance était de 2,9 mois. Les HI (54 cas ; 50,5 %) et les angiomes plans (8 cas, 53,3 %) étaient localisés de façon prépondérante à la région céphalique et les cas de syndrome de Klippel-Trenaunay aux membres inférieurs. Nous avons opté pour une abstention thérapeutique chez la plupart des patients (50 cas d'HI, 16 cas de malformations vasculaires simples, tous les cas de syndrome de Klippel-Trenaunay et le cas de télangiectasies). Sur les 43 cas d'HI traités, les principaux médicaments utilisés étaient les corticoïdes (23 cas dont 17 entre 1998­2011 et 6 à partir de 2012) suivis du propranolol (11 cas à partir de 2012). Nous avions noté une réponse favorable chez 9 des 15 patients revus. Les anomalies vasculaires cutanées sont très rares en dermatologie au Togo et sont dominées par les HI, avec une prédominance féminine. Dans le traitement des HI, l'usage du propranolol, débuté en 2012 au Togo, est en augmentation pendant que celui de la corticothérapie baisse.

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.

Copy number variants in a population-based investigation of Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder that is thought to occur sporadically; however, reports of familial occurrence suggest a genetic component. We examined KTS cases to identify novel, potentially causal copy number variants (CNVs). We identified 17 KTS cases from all live-births occurring in New York (1998-2010). Extracted DNA was genotyped using Illumina microarrays and CNVs were called using PennCNV software. CNVs selected for follow-up had ≥10 single nucleotide polymorphisms (SNPs) and minimal overlap with in-house controls or controls from the Database of Genomic Variants. We identified 15 candidate CNVs in seven cases; among them a deletion in two cases within transcripts of HDAC9, a histone deacetylase essential for angiogenic sprouting of endothelial cells. One of them also had a duplication upstream of SALL3, a transcription factor essential for embryonic development that inhibits DNMT3A, a DNA methyltransferase responsible for embryonic de novo DNA methylation. Another case had a duplication spanning ING5, a histone acetylation regulator active during embryogenesis. We identified rare genetic variants related to chromatin modification which may have a key role in regulating vascular development during embryogenesis. Further investigation of their implications in the pathogenesis of KTS is warranted. © 2016 Wiley Periodicals, Inc.

The persistent embryonic vein in Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a congenital malformation syndrome with prominent vascular anomalies. A persistent embryonic vein (PEV) may be located on the affected leg(s) of patients with KTS. Our understanding of PEVs of the legs is limited and their nomenclature is confusing. The objective of this study was to obtain further insight in the prevalence, nomenclature and etiology of PEVs of the legs in KTS and to propose a standardized description of anomalous leg veins in KTS. We investigated 70 KTS patients for the presence of PEVs (lateral marginal vein, LMV) of the legs by duplex ultrasonography. We performed histopathological analysis of a surgically excised PEV (LMV) of a typical KTS patient, and we conducted an extensive literature study. Duplex ultrasonography showed LMVs in 12/70 (17.1%) patients. The terms used to describe PEVs in the leg are quite variable, while indicating only two types: lateral marginal vein (LMV) and persistent sciatic vein (PSV). The histology of the excised LMV showed remarkable similarity with that of varicose veins found in the general population. In conclusion, the prevalence of LMVs in our KTS cohort is 17.1%. Two PEVs can be found in the legs and we propose nomenclature based on anatomical criteria, thereby using only the terms persistent lateral marginal vein and persistent sciatic vein, combined with the patency of the deep venous system. We hypothesize that PEVs are most likely caused by a genetic defect leading to abnormal venous pattern formation, which is further supported by our histopathological findings.

Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis.

There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients.

Diagnosis and management of extensive vascular malformations of the lower limb: part II. Systemic repercussions [corrected], diagnosis, and treatment.

At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. Extensive vascular malformations are often more complex than they appear and require a multidisciplinary therapeutic approach. Vascular malformations may be associated with underlying disease or systemic anomalies. Part II of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limb highlights the systemic repercussions [corrected] (bone, articular, visceral, and hematologic involvement), diagnosis, and treatment of these lesions.

Klippel-Trenaunay syndrome and spinal arteriovenous malformations: an erroneous association.

BACKGROUND AND PURPOSE: KTS is a rare limb overgrowth disorder with slow-flow vascular anomalies. This study examines the presumed association between KTS and spinal AVMs. MATERIALS AND METHODS: We performed a MEDLINE search of articles and reviewed textbooks of spinal diseases to study the association between KTS and spinal AVM. Our goal was to ascertain the basis on which the diagnosis of KTS was established and to evaluate the evidence of its association with spinal AVMs. In addition, the data base of the Vascular Anomalies Center at Children's Hospital Boston was queried for patients with KTS, and the association with spinal AVM was investigated. RESULTS: Twenty-four published reports on spinal AVMs in 31 patients with KTS were reviewed. None of these references provided solid evidence of the diagnosis of KTS in any patient. Clinical data were either incompatible with the diagnosis of KTS or were inadequate to establish the diagnosis. Alternative possible diagnoses (CLOVES syndrome and CM-AVM) were suggested by the first author for 9 of the patients reported in these articles. The medical records of 208 patients with the diagnosis of KTS were analyzed; not a single patient had clinical or radiologic evidence of a spinal AVM. CONCLUSIONS: An association between KTS and spinal AVM, as posited in numerous references, is most likely erroneous. The association has neither been reliably proved in the limited published literature nor encountered in a large cohort.

Vascular malformations. Part II: associated syndromes.

UNLABELLED: Cutaneous vascular malformations are rare disorders representing errors in vascular development. These lesions occur much less commonly but are often confused with the common infantile hemangioma. It is important to properly diagnose vascular malformations because of their distinct differences in morbidity, prognosis and treatment. Vascular malformations may be associated with underlying disease or systemic anomalies. Several of these syndromes are well defined and can often be distinguished on the basis of the flow characteristics of the associated vascular malformation. LEARNING OBJECTIVE: At the completion of this learning activity, participants should be able to better recognize underlying diseases or systemic anomalies that may be associated with vascular malformations. Participants should also better understand the various syndromes and conditions discussed and become more familiar with their management.

Klippel-Trénaunay syndrome: the importance of "geographic stains" in identifying lymphatic disease and risk of complications.

BACKGROUND: Klippel-Trénaunay syndrome (KTS) is a rare congenital anomaly classically defined as the triad of vascular stain, soft tissue and/or bony hypertrophy, and venous varicosities. OBJECTIVE: To determine whether the morphologic characteristics of the associated vascular stains in KTS are predictive of the presence of lymphatic involvement and/or complications. SETTING: Outpatient dermatology practice, tertiary care medical center. METHODS: We retrospectively reviewed all cases of KTS identified between January 1989 and September 2001 at the University of California San Francisco (UCSF) Department of Dermatology. Forty patients were identified. We further classified them by type of cutaneous vascular stain, either "geographic" or "blotchy/segmental." Patients were further classified as having definite, probable, possible, or no evidence of lymphatic disease. We also reviewed the charts for other possibly associated manifestations and complications of KTS. RESULTS: Of those with sharply demarcated geographic stains (n=22), 21 had definite or probable evidence of lymphatic disease. Of those with blotchy port-wine stains (n=17), 16 had possible or no evidence of lymphatic disease (P <.001). Determination of the type of stain had 95% sensitivity and 94% specificity in differentiating the definite or probable presence of definite or probable lymphatic disease from possible or no evidence of lymphatic disease. Complications occurred in 19 (86%) of 22 patients with a geographic stain vs 7 of 17 (41%) with a blotchy/segmental stain (P <.003). CONCLUSION: This study demonstrates that the presence of a geographic vascular stain is a predictor of the risk of both associated lymphatic malformation and complications in patients with KTS. Since these stains are present at birth, this clinical observation can help in identifying individuals with KTS at greatest risk for complications and in need of closer observation.

Wilms tumor screening is unnecessary in Klippel-Trenaunay syndrome.

BACKGROUND: Children with hemihypertrophy are screened for Wilms tumor, because this condition is a risk factor for developing the neoplasm. Patients with Klippel-Trenaunay syndrome (KTS) are often considered potential candidates for Wilms tumor, because they have unilateral overgrowth of the lower limb. In our experience, however, an association between KTS and Wilms tumor has not been observed. METHODS: To determine whether KTS and Wilms tumor are associated, we reviewed our institutional experience for patients with both diagnoses and searched the Klippel-Trenaunay literature for patients with Wilms tumor. The National Wilms Tumor Study Group database also was studied to identify patients with KTS. Two-sided exact binomial tests were used to evaluate whether patients with 1 condition had an increased risk for the other. Ninety-five percent confidence intervals for these 2 risks were compared with the general population risks of Wilms tumor (1 in 10 000) and KTS (1 in 47 313). RESULTS: None of the 115 patients with KTS followed at our institution developed Wilms tumor. One case of Wilms tumor has been reported in 1363 patients with KTS in the literature, giving a confidence interval of (1/57 377) and (1/267). None of the 8614 patients in the National Wilms Tumor Study Group database had KTS, giving a confidence interval of (0, 1/2336). Because the risks of KTS and Wilms tumor in the population fall within these confidence intervals, one cannot conclude that the risks of KTS among Wilms tumor patients or Wilms tumor among KTS patients are any different from the corresponding risks in the general population. CONCLUSIONS: Patients with KTS are not at increased risk for developing Wilms tumor and thus should not undergo routine ultrasonographic screening.

[Vascular dysplasia in vascular surgery in an African area: 28 cases at the Dakar teaching hospital]. / Anomalies vasculaires congenitales en chirurgie vasculaire en milieu Africain: à propos de 28 cas colligés au CHU de Dakar.

This retrospective study was conducted to analyze the anatomic and clinical data concerning vascular dysplasia in vascular surgery patients seen in an African hospital and to assess its role in treatment. Twenty-eight patients (14 women and 14 men) were treated for vascular dysplasia between 1994 and 1998. There were 8 cases of hemangioma and 20 cases of vascular malformation. Average age of patients with hemangioma was 3.23 years, with a sex ratio of 0.33. There were 4 cases of mixed hemangioma, 3 of cutaneous hemangioma, and 1 of subcutaneous hemangioma. Cervicocephalic (n=4) and limb (n=3) localizations were the most frequent. Duplex Doppler did not reveal any signs of muscle disease. Four patients underwent surgery, for an early indication in 3 and a late indication in 1. Resection suture was performed in 3 cases and resection graft in one. Mean follow-up was 85.90 days. There was one case of early suppuration. There were no cases of recurrence or late complications and the operative wounds healed well. Vascular malformations concerned 20 patients (sex ratio 1.5, average age 15.55 years) with 16 cases of venous malformation including 4 patients with Klippel Trenaunay syndrome, three with arteriovenous malformation and one with capillary angioma. CT and arteriography were obtained in all cases. Limb localizations predominated (14 patients). In-depth extension was found in 6 cases of venous malformation. Sixteen patients underwent surgery for functional indications in 7. Resection suture was performed in 11 cases. Mean follow-up was 85.90 days. There were 4 early and 2 late postoperative complications. There has been no recurrence. Anatomic and clinical polymorphism of vascular dysplasia requires multidisciplinary collaboration in which vascular surgery plays an important role in complex or complicated forms of the disease in patients attending facilities with limited resources.