1,363 results match your criteria Klippel-Trenaunay-Weber Syndrome


Major limb amputations in patients with congenital vascular malformations.

Ann Vasc Surg 2020 May 18. Epub 2020 May 18.

Department of Surgery, Lenox Hill Hospital, Northwell Health System.

Objective: The need for major amputations in patients with vascular malformations is rare. This study reviews our contemporary experience with major amputations in patients with vascular malformations.

Methods: A retrospective review from April 2014 to November 2018 identified 993 patients undergoing management of a vascular malformation involving the upper or lower extremity at a tertiary center. Read More

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http://dx.doi.org/10.1016/j.avsg.2020.04.058DOI Listing

Successful management of Klippel-Trenaunay syndrome in a pregnant Asian woman: A case report.

Medicine (Baltimore) 2020 May;99(19):e19932

Department of Obstetrics and Gynecology, West China Second University Hospital of Sichuan University.

Rationale: Klippel-Trenaunay Syndrome (KTS) is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy. Although extremely rare in pregnant women, the present vascular alterations may be aggravated, consequent to postural and hormonal changes inherent to the pregnancy. Pregnancy is not advised in KTS women due to increased obstetrical risk. Read More

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http://dx.doi.org/10.1097/MD.0000000000019932DOI Listing

Incidence of Sturge-Weber syndrome and associated ocular involvement in Olmsted County, Minnesota, United States.

Ophthalmic Genet 2020 Apr 31;41(2):108-124. Epub 2020 Mar 31.

Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.

: Sturge-Weber syndrome (SWS) is a rare sporadic syndrome characterized by nevus flammeus (port-wine stain, PWS) in the trigeminal nerve distribution, diffuse choroidal hemangioma, and brain leptomeningeal hemangioma. We are unaware of previous reports of SWS incidence in the United States. This study investigated SWS incidence and associated ocular involvement in Olmsted County, Minnesota. Read More

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http://dx.doi.org/10.1080/13816810.2020.1731834DOI Listing

Congenital Triangular Alopecia Associated with Phakomatosis Pigmentovascularis Type II along with Klippel Trenaunay Syndrome.

Indian Dermatol Online J 2020 Jan-Feb;11(1):91-93. Epub 2019 Sep 26.

Department of Dermatology, PGIMER Dr Ram Manohar Lohia Hospital, New Delhi, India.

Phakomatosis pigmentovascularis (PPV) is characterized by the association of a vascular nevus with a pigmentary nevus and is divided into five subtypes. PPV type II or Happle's phakomatosis cesioflammea is the most common subtype comprising of nevus flammeus along with pigmentary nevus in the form of aberrant Mongolian spots, nevus of Ota or less frequently nevus of Ito. It is estimated that around 50% of patients with PPV have systemic involvement, most frequently involving the central nervous system and eye. Read More

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http://dx.doi.org/10.4103/idoj.IDOJ_112_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7001386PMC
September 2019

[Klippel-Trenaunay-Weber syndrome with vesical and uterine involvement treated by endoscopic and endovascular routes].

Medicina (B Aires) 2020 ;80(1):84-86

Servicio de Ginecología, Hospital Militar Central, Buenos Aires, Argentina.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare venous malformation that generally affects the lower limbs and, more infrequently, the upper limbs. It is characterized by cutaneous angiomatous formations, varicose veins and hypertrophy of the affected limb. The involvement of the genitourinary tract is extremely infrequent. Read More

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Klippel-Trenaunay Syndrome.

Authors:
Philip R John

Tech Vasc Interv Radiol 2019 Dec 23;22(4):100634. Epub 2019 Sep 23.

Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Canada. Electronic address:

Klippel-Trenaunay syndrome or KTS is a complex vascular syndrome associated with overgrowth occurring as a result of somatic mutations in the PIK3CA gene. Patients are diagnosed on the basis of physical findings, sometimes with supportive imaging, of commonly a segmental anomaly with a cutaneous port-wine stain, lymphatic and venous malformations and overgrowth. The severity of the component vascular malformations and the degree of overgrowth varies from patient to patient which demands care given by a multi-professional team with regular follow-up in a specialist clinic. Read More

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http://dx.doi.org/10.1016/j.tvir.2019.100634DOI Listing
December 2019

Diagnostic and clinical utility of whole genome sequencing in a cohort of undiagnosed Chinese families with rare diseases.

Sci Rep 2019 12 18;9(1):19365. Epub 2019 Dec 18.

Women's Reproductive Health Key Laboratory of Zhejiang Province, Department of Gynecologic Oncology, Women's Hospital and Institute of Translational Medicine, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, 310006, China.

Rare diseases are usually chronically debilitating or even life-threatening with diagnostic and therapeutic challenges in current clinical practice. It has been estimated that 80% of rare diseases are genetic in origin, and thus genome sequencing-based diagnosis offers a promising alternative for rare-disease management. In this study, 79 individuals from 16 independent families were performed for whole-genome sequencing (WGS) in an effort to identify the causative mutations for 16 distinct rare diseases that are largely clinically intractable. Read More

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http://dx.doi.org/10.1038/s41598-019-55832-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6920370PMC
December 2019

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

Medicine (Baltimore) 2019 Dec;98(50):e18378

Department of Gynecology and Obstetrics, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital.

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. Read More

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http://dx.doi.org/10.1097/MD.0000000000018378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6922477PMC
December 2019
5.723 Impact Factor

Wound-Healing Problems Associated with Combined Vascular Malformations in Klippel-Trenaunay Syndrome.

Adv Wound Care (New Rochelle) 2019 Jun 6;8(6):246-255. Epub 2019 Jun 6.

Department of Plastic and Reconstructive Surgery, Center for Vascular Anomalies, Tonan Hospital, Sapporo, Japan.

In Klippel-Trenaunay syndrome (KTS), management of a wound in the affected limb can be difficult because of the underlying vascular malformations present. This review describes the characteristics of KTS with wound complications in light of the genetic and molecular mechanisms of the disease. KTS is a slow-flow combined vascular malformation characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. Read More

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http://dx.doi.org/10.1089/wound.2018.0835DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6906760PMC

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

Rural Remote Health 2019 11 14;19(4):5348. Epub 2019 Nov 14.

Department of Surgery and Endoscopy, Charles S. Curtis Memorial Hospital, 178-200 West Street, St. Anthony, NL A0K 4S0, Canada

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. Read More

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http://dx.doi.org/10.22605/RRH5348DOI Listing
November 2019

Imaging Findings of Klippel-Trenaunay Syndrome.

J Comput Assist Tomogr 2019 Sep/Oct;43(5):786-792

From the Department of Diagnostic Radiology, Mansoura Faculty of Medicine, Mansoura University, Mansoura, Egypt.

We aim to review the imaging findings of Klippel-Trenaunay syndrome. This disorder characterized clinically by a triad of capillary malformations manifesting as a port-wine stain, venous varicosities typically along the lateral aspect of the lower extremities, and bone and/or soft tissue hypertrophy. Imaging of extremities shows unilateral venous varicosities, persistent embryonic veins, anomalous of the superficial and deep venous system, low-flow venolymphatic malformations, and bony and soft tissue hypertrophy. Read More

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http://dx.doi.org/10.1097/RCT.0000000000000895DOI Listing
October 2019
1 Read
1.602 Impact Factor

A rare case of phakomatosis pigmentovascularis type IIb associated with inverse Klippel-Trenaunay syndrome and Sturge-Weber syndrome.

Indian J Dermatol Venereol Leprol 2019 Nov-Dec;85(6):618-620

Department of Dermatovenereology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

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http://dx.doi.org/10.4103/ijdvl.IJDVL_55_19DOI Listing

Klippel-Trenaunay syndrome as a rare cause of chronic thromboemboembolic pulmonary hypertension.

Respir Med Res 2019 Nov 9;76:48-53. Epub 2019 Jul 9.

Faculté de Médecine, Université Paris-Sud, Université Paris-Saclay, 94270 Le Kremlin-Bicêtre, France; Inserm UMR_S 999, Hôpital Marie Lannelongue, 92350 Le Plessis-Robinson, France; Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Assistance publique-hôpitaux de Paris (AP-HP), 94270 Le Kremlin-Bicêtre, France. Electronic address:

Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous capillary malformations, soft tissue and bone hypertrophy, and multiple capillary, venous or lymphatic malformations. KTS is associated with recurrent thromboembolic events. We reported herein five cases of chronic thromboembolic pulmonary hypertension (CTEPH) associated with KTS (age minimum-maximum 26-50 years old, 3 males/2 females). Read More

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http://dx.doi.org/10.1016/j.resmer.2019.06.002DOI Listing
November 2019
2 Reads

Massive Coil Nest Migration: Endovascular Retrieval.

J Vasc Interv Radiol 2019 Oct;30(10):1610-1611

Department of Radiology, Division of Vascular and Interventional Radiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts, 02115.

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http://dx.doi.org/10.1016/j.jvir.2019.03.011DOI Listing
October 2019

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

BMJ Case Rep 2019 Aug 20;12(8). Epub 2019 Aug 20.

Radiology, University of the Philippines Manila, Manila, Philippines.

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. Read More

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http://dx.doi.org/10.1136/bcr-2018-228257DOI Listing
August 2019
1 Read

Port-wine stain, limb hypertrophy, dilated veins and blue sclera: Klippel-Trenaunay syndrome.

BMJ Case Rep 2019 Aug 15;12(8). Epub 2019 Aug 15.

Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1136/bcr-2019-230146DOI Listing

Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report.

Medicine (Baltimore) 2019 Aug;98(32):e16741

Rationale: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous port wine capillary malformations, varicose veins with hemihypertrophy of soft tissue and bone.Pelvic and retroperitoneal vascular malformations have been described up to the 30% of patients with KTS while hemangiomas of the urinary tract have been reported in 6% PATIENT CONCERNS:: A 30-year-old man with KTS was referred to our center for primary erectile dysfunction (ED) associated with varicosities of unusual distribution and asymmetry of the lower limbs. Furthermore, he suffered from hypertension and autosomal dominant polycystic kidney disease. Read More

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http://dx.doi.org/10.1097/MD.0000000000016741DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6708871PMC
August 2019
7 Reads

Squamous cell carcinoma arising in a chronic leg ulcer in Klippel-Trenaunay syndrome after the Charles procedure: A case with 40 years of follow up.

J Dermatol 2019 Nov 8;46(11):e403-e405. Epub 2019 Jul 8.

Department of Plastic and Reconstructive Surgery, Center for Vascular Anomalies, Tonan Hospital, Sapporo, Japan.

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http://dx.doi.org/10.1111/1346-8138.15001DOI Listing
November 2019

Lymphaticovenular anastomosis for Klippel-Trenaunay-Weber syndrome.

Int J Surg Case Rep 2019 16;58:67-69. Epub 2019 Apr 16.

Department of Plastic and Reconstructive Surgery, Okayama Saiseikai Genaral Hospital, Okayama, Japan.

Introduction: Since we performed lymphaticovenular anastomosis (LVA) for Klippel-Trenaunay-Weber syndrome and obtained relatively good results, we report the adaptation, effect of treatment, and mechanism of LVA.

Presentation Of Case: Case is a 28-year-old man with an increase of the circumference and pigment changes on the whole right leg and gluteal region from the time he was born. He exhibited signs of right leg cellulitis with a fever about 40° at frequency of the degree once a month. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22102612193021
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http://dx.doi.org/10.1016/j.ijscr.2019.04.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6479102PMC
April 2019
31 Reads

An Engorged Vein over the Lower Abdominal Wall.

J Pediatr 2019 Jul 19;210:233-233.e1. Epub 2019 Apr 19.

Center for Rare Disease and Hemophilia, Taichung Veterans General Hospital, Taichung, Taiwan; Tunghai University, Taichung, Taiwan.

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http://dx.doi.org/10.1016/j.jpeds.2019.03.034DOI Listing
July 2019
4 Reads

Venous thromboembolic and bleeding complications among pregnant women with Klippel-Trenaunay syndrome.

J Am Acad Dermatol 2019 Dec 13;81(6):1277-1282. Epub 2019 Apr 13.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Klippel-Trenaunay syndrome (KTS) is a vascular malformation overgrowth syndrome characterized by capillary malformation, venous malformation, and limb overgrowth, with or without lymphatic malformation. Patients are at an increased risk of hemorrhage and venous thromboembolism (VTE). Consequently, women with this condition often are counseled to avoid pregnancy, but minimal data are available on the relationship between pregnancy, VTE, and bleeding risk. Read More

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http://dx.doi.org/10.1016/j.jaad.2019.04.018DOI Listing
December 2019
10 Reads

An atypical case of Klippel-Trénaunay syndrome presenting with crossed-bilateral limb hypertrophy and postaxial polydactyly: a case report.

BMC Pediatr 2019 04 6;19(1):95. Epub 2019 Apr 6.

Division of Neonatology, Department of Pediatrics, University of Texas Medical Branch at Galveston, Galveston, Texas, USA.

Background: Klippel-Trénaunay syndrome (KTS) is a rare congenital condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft tissue hypertrophy.

Case Presentation: Here we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly.

Conclusion: This case serves to highlight the variable presentation and multiple problems faced by patients with KTS and why multidisciplinary management is mandatory. Read More

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https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887
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http://dx.doi.org/10.1186/s12887-019-1480-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451243PMC
April 2019
8 Reads

Klippel-Trenaunay-Weber Syndrome with Atypical Presentation of Cerebral Cavernous Angioma: A Case Report and Literature Review.

World Neurosurg 2019 Jun 21;126:354-358. Epub 2019 Mar 21.

Department of Radiology, Mayo Clinic, Jacksonville, Florida, USA; Department of Neurologic Surgery, Mayo Clinic, Jacksonville, Florida, USA.

Background: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome characterized by the triad of cutaneous hemangiomas, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. Clinical manifestations, genetic testing, and radiologic imaging are the key steps in diagnosing this syndrome.

Case Description: An 18-month-old boy was brought for follow-up brain magnetic resonance imaging (MRI) with a history of right lower limb hypertrophy, cutaneous varicosities, and hemangiomas diagnosed at birth. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.03.132DOI Listing
June 2019
15 Reads

Congenital Limb Overgrowth Syndromes Associated with Vascular Anomalies.

Radiographics 2019 Mar-Apr;39(2):491-515

From the Divisions of Pediatric Radiology (F.B., K.A.B., C.M.H., A.E.G., S.S.M.) and Interventional Radiology and Image Guided Medicine (F.B., C.M.H., A.E.G.), Department of Radiology and Imaging Sciences; and Department of Hematology and Medical Oncology (M.A.B.), Emory University School of Medicine, 1364 Clifton Rd NE, Suite D112, Atlanta, GA 30322; and Vascular Anomalies Clinic (F.B., K.A.B., C.M.H., A.E.G., M.A.B., R.S., S.S.M.) and Aflac Cancer and Blood Disorders Center (M.A.B.), Children's Healthcare of Atlanta, Atlanta, Ga.

Congenital limb length discrepancy disorders are frequently associated with a variety of vascular anomalies and have unique genetic and phenotypic features. Many of these syndromes have been linked to sporadic somatic mosaicism involving mutations of the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, which has an important role in tissue growth and angiogenesis. Radiologists who are aware of congenital limb length discrepancies can make specific diagnoses based on imaging findings. Read More

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http://dx.doi.org/10.1148/rg.2019180136DOI Listing
March 2020
37 Reads
2.602 Impact Factor

Laser ablation of embryonic veins in children.

Pediatr Int 2019 Apr 11;61(4):358-363. Epub 2019 Apr 11.

Division of Plastic and Reconstructive Surgery, Johns Hopkins All Children's Hospital, St Petersburg, Florida, USA.

Background: Klippel-Trenaunay syndrome (KTS) and related overgrowth syndromes are rare conditions that often present to academic pediatric units and multidisciplinary vascular anomaly clinics. These conditions are commonly associated with abnormalities of the superficial and deep venous structures and carry an increased risk of venous thromboembolism.

Methods: A retrospective chart review was completed of all patients treated at Johns Hopkins All Children's Hospital with endovenous laser ablation therapy (EVLT) for management of dilated embryonic veins in the setting of limb overgrowth. Read More

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http://dx.doi.org/10.1111/ped.13804DOI Listing
April 2019
8 Reads

Vesical Hemangioma in a Patient with Klippel-Trenaunay-Weber Syndrome.

J Pediatr 2019 May 6;208:293-293.e2. Epub 2019 Feb 6.

Department of Pediatric Nephrology, Ghent University Hospital, Gent, Belgium.

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http://dx.doi.org/10.1016/j.jpeds.2019.01.011DOI Listing
May 2019
3 Reads

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

BMC Womens Health 2019 02 7;19(1):31. Epub 2019 Feb 7.

The International Peace Maternity and Child Health Hospital, School of Medicine, Shanghai Jiaotong University, 145, Guang-yuan Road, Xuhui District, Shanghai, 200030, China.

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. Read More

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https://bmcwomenshealth.biomedcentral.com/articles/10.1186/s
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http://dx.doi.org/10.1186/s12905-019-0731-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367833PMC
February 2019
11 Reads
1.657 Impact Factor

Total Knee Arthroplasty Is Safe and Successful in Patients With Klippel-Trénaunay Syndrome.

J Arthroplasty 2019 04 22;34(4):682-685. Epub 2018 Dec 22.

Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.

Background: Klippel-Trénaunay syndrome (KTS) is a severe vascular malformation that can lead to hypertrophic osteoarthritis. Total knee arthroplasty (TKA) performed in extremities affected with KTS is challenging given the high-risk vascular considerations and occasionally poor bone quality.

Methods: We identified 12 patients with KTS who underwent TKA between 1998 and 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08835403183122
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http://dx.doi.org/10.1016/j.arth.2018.12.028DOI Listing
April 2019
11 Reads

Overgrowth syndrome in neonates: a rare case series with a review of the literature.

BMJ Case Rep 2019 Jan 17;12(1). Epub 2019 Jan 17.

Neonatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

We present here two-term neonates presenting with right lower limb hypertrophy, a port-wine stain, acral abnormalities and clubfeet. These neonates had overlapping features of Klippel Trenaunay syndrome and congenital lipomatous overgrowth, vascular malformation, epidermal nevi and scoliosis/skeletal abnormalities. Such overgrowth syndrome has not been previously described in the literature. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22564
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http://dx.doi.org/10.1136/bcr-2018-225640DOI Listing
January 2019
17 Reads

Kyphoscoliosis with Klippel-Trenaunay syndrome: a case report and literature review.

BMC Musculoskelet Disord 2019 Jan 5;20(1):10. Epub 2019 Jan 5.

Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China.

Background: Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome characterized by the triad of venous varicosities, capillary malformations and limb hypertrophy. However, KTS may rarely occur in combination with kyphoscoliosis.

Case Presentation: We presented an 18-year-old female with KTS and kyphoscoliosis. Read More

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https://bmcmusculoskeletdisord.biomedcentral.com/articles/10
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http://dx.doi.org/10.1186/s12891-018-2393-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320630PMC
January 2019
41 Reads

Superficial Femoral Venous Aneurysm in a Patient with Klippel-Trenaunay Syndrome: The Femoral Hernia Mimic.

Ann Vasc Surg 2019 May 27;57:274.e15-274.e18. Epub 2018 Nov 27.

Department of Vascular Surgery, Waikato District Health Board, Hamilton, New Zealand.

Venous aneurysms are a rare vascular anomaly characterized by a solitary area of dilatation containing all three layers of the venous vessel wall. Described throughout the venous system, venous aneurysms can be classified as deep or superficial based on the vein involved. Although generally asymptomatic, superficial venous aneurysms can be associated with venous insufficiency or venous thromboembolism and are often confused for soft tissue masses or hernias. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08905096183090
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http://dx.doi.org/10.1016/j.avsg.2018.09.028DOI Listing
May 2019
40 Reads
1.029 Impact Factor

Apixaban for the Treatment of Chronic Disseminated Intravascular Coagulation: A Report of Two Cases.

Hamostaseologie 2019 Aug 19;39(3):294-297. Epub 2018 Nov 19.

Department of Oncology and Hematology, BMT with Section of Pneumology, Hubertus Wald Tumorzentrum - University Cancer Center Hamburg, University Medical Center Eppendorf, Hamburg, Germany.

Characteristic features of disseminated intravascular coagulation (DIC) are the opposing risks of bleeding (due to consumptive coagulopathy and hyperfibrinolysis) and organ failure (due to widespread microvascular thromboses). The purpose of anticoagulation in DIC is to attenuate excessive thrombin generation and fibrin deposition. While heparins have been shown to be beneficial in this context, the safety and efficacy of direct oral anticoagulants have not yet been sufficiently addressed. Read More

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http://dx.doi.org/10.1055/s-0038-1675386DOI Listing
August 2019
7 Reads

Septic superficial thrombophlebitis in Klippel-Trenaunay syndrome.

Acta Chir Belg 2019 Apr 2;119(2):137-138. Epub 2018 Nov 2.

a Department of Vascular Surgery , KAT General Hospital , Athens , Greece.

This is a rare case of a young patient with Klippel-Trenaunay syndrome that presented with extensive septic superficial thrombophlebitis of the lower extremity. Treatment included intravenous antibiotics based on cultures, anticoagulant therapy as well as surgical removal of thrombi. Read More

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http://dx.doi.org/10.1080/00015458.2018.1534394DOI Listing
April 2019
12 Reads

Soft tissue angiosarcoma of the breast in a patient with Klippel-Trenaunay-Weber syndrome.

Cir Cir 2018 ;86(6):566-569

Department of Surgery Division of Surgical Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. Read More

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http://www.cirugiaycirujanos.com/files/p4214bx176-circir-6--
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http://dx.doi.org/10.24875/CIRU.18000306DOI Listing
April 2019
29 Reads
0.322 Impact Factor

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

Curr Med Sci 2018 Oct 20;38(5):925-931. Epub 2018 Oct 20.

Department of Interventional Radiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.

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. Read More

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http://link.springer.com/10.1007/s11596-018-1964-4
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http://dx.doi.org/10.1007/s11596-018-1964-4DOI Listing
October 2018
17 Reads

Soft-tissue vascular malformations and tumors. Part 2: low-flow lesions.

Radiologia 2019 Mar - Apr;61(2):124-133. Epub 2018 Oct 3.

University of Virginia Health System, Department of Radiology and Medical Imaging, 1215 Lee Street, Charlottesville, Virginia, USA; University of Missouri Health System, Department of Radiology, One Hospital Dr, Columbia, Missouri, USA.

Vascular malformations and tumors, also known as "vascular anomalies", comprise an extensive variety of lesions involving all parts of the body. Due to a lack of a complete understanding of the origin and histopathology of such lesions, this field has been traditionally obscured by the use of an unclear nomenclature. Knowledge of the classification and clinical and imaging characteristics of this group of lesions is paramount when managing these patients. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00338338183014
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http://dx.doi.org/10.1016/j.rx.2018.02.012DOI Listing
November 2019
35 Reads

Anesthetic challenges in a case of Klippel-Trenaunay Syndrome with severe anaemia.

J Clin Anesth 2019 Mar 2;53:27-28. Epub 2018 Oct 2.

Department of Anesthesiology, AIIMS, Rishikesh 249203, India. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S09528180183113
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http://dx.doi.org/10.1016/j.jclinane.2018.09.028DOI Listing
March 2019
35 Reads

Rectal Venous Malformation Treated by Superior Rectal Artery Embolization.

Cardiovasc Intervent Radiol 2019 Jan 30;42(1):154-157. Epub 2018 Aug 30.

Interventional Radiology Unit, Radiology Department, John Radcliffe Hospital, 2nd Floor, Oxford, OX3 9DU, UK.

A 25-year-old female was referred to the Interventional Radiology Department for investigation and treatment of a rectal venous malformation (RVM) causing large recurrent episodes of rectal bleeding and chronic anaemia. Magnetic resonance imaging (MRI) demonstrated a large venous malformation affecting the rectum, lower pelvis and left thigh. After three failed attempts at injection foam sclerotherapy using fluoroscopic colonoscopy, a multidisciplinary team proposed an embolization procedure of the arterial inflow to the venous malformation. Read More

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http://dx.doi.org/10.1007/s00270-018-2067-0DOI Listing
January 2019
1 Read

Marked acanthocytosis in the setting of Klippel-Trenaunay syndrome: A case report.

Int J Lab Hematol 2019 02 16;41(1):e10-e12. Epub 2018 Aug 16.

Laboratory Haematology Service, Department of Pathology, Centro Hospitalar Universitário do Porto, Porto, Portugal.

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http://dx.doi.org/10.1111/ijlh.12913DOI Listing
February 2019
27 Reads

Management of Pregnancy with Klippel-Trenaunay-Weber Syndrome: A Case Report and Review.

Authors:
Rati Chadha

Case Rep Obstet Gynecol 2018 12;2018:6583562. Epub 2018 Jul 12.

Department of Obstetrics and Gynecology; Division of Maternal Fetal Medicine, Foothills Medical Center, University of Calgary, Calgary, AB, Canada.

Background: Klippel-Trenaunay-Weber syndrome is a rare neurocutaneous syndrome with vascular involvement. Given the rarity of the syndrome, its management in pregnancy is based on the outcome of a few case reports and expert opinion.

Case Summary: The management of a complicated case with its antepartum, intrapartum, and postpartum concerns has been addressed in this review. Read More

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https://www.hindawi.com/journals/criog/2018/6583562/
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http://dx.doi.org/10.1155/2018/6583562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6077650PMC
July 2018
19 Reads

Klippel-Trenaunay Syndrome: A Dramatic Presentation.

Eur J Vasc Endovasc Surg 2018 08 13;56(2):299. Epub 2018 Jul 13.

Vascular Surgery Unit, University of Pisa, Via Paradisa 2, Pisa 56124, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.ejvs.2018.06.012DOI Listing
August 2018
7 Reads

Port-wine stain as a clue for two rare coexisting entities.

BMJ Case Rep 2018 Jul 13;2018. Epub 2018 Jul 13.

Department of Dermatovenereology, Hospital de Braga, Braga, Portugal.

Phakomatosis pigmentovascularis (PPV) is an uncommon dermatosis characterised by the presence of both pigmentary and vascular abnormalities. Its pathogenesis is not elucidated, and the prognosis is mainly determined by the presence of extracutaneous manifestations, such as Klippel-Trenaunay syndrome (KTS), that is defined by the triad of a port-wine stain (PWS), anomalous veins and progressive overgrowth of the affected extremity. Herein, we report a case of an adult patient, who presented with a large PWS, nevus of Ota, ocular melanosis, and limb hypertrophy and varicosities. Read More

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http://dx.doi.org/10.1136/bcr-2018-225721DOI Listing
July 2018
10 Reads

Walking hyponatremia syndrome of inappropriate antidiuretic hormone secretion secondary to carbamazepine use: a case report.

J Med Case Rep 2018 Jul 11;12(1):202. Epub 2018 Jul 11.

Facultad de Medicina, Universidad Francisco Marroquín, 6 Avenida 7-55, zona 10, 01010, Guatemala City, Guatemala.

Background: Severe hyponatremia is rare when carbamazepine is used as monotherapy. It is common to encounter this imbalance in the hospital setting, but rare in the ambulatory one. Here, we present a case of hyponatremia secondary to carbamazepine use in an otherwise asymptomatic patient. Read More

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http://dx.doi.org/10.1186/s13256-018-1744-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6040056PMC
July 2018
23 Reads

Klippel-Trenaunay and Parkes-Weber syndromes: two case reports.

J Vasc Bras 2017 Oct-Dec;16(4):320-324

Universidade de São Paulo - USP, School of Medicine, Unit of Interventional Radiology, São Paulo, SP, Brazil.

Parkes-Weber syndrome is a congenital vascular disease that comprises capillary, venous, lymphatic, and arteriovenous malformations. Although Parkes-Weber syndrome is a clinically distinct entity with serious complications, it is still frequently misdiagnosed as Klippel-Trenaunay syndrome, which consists of a triad of malformations involving the capillary, venous, and lymphatic vessels, without arteriovenous fistulas. Both syndromes are generally diagnosed with Doppler ultrasound and confirmed by magnetic resonance angiography. Read More

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http://dx.doi.org/10.1590/1677-5449.005417DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5944310PMC
June 2018
8 Reads

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

J Vasc Surg Venous Lymphat Disord 2018 07;6(4):511-516

Division of Vascular and Interventional Radiology, Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Electronic address:

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. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S2213333X183012
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http://dx.doi.org/10.1016/j.jvsv.2018.01.015DOI Listing
July 2018
21 Reads

Gastrointestinal tract involvement in Klippel-Trénaunay syndrome.

Lancet Gastroenterol Hepatol 2018 07;3(7):518

Department of Gastroenterology, Pontificia Universidad Católica de Chile, Santiago de Chile, Chile. Electronic address:

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http://dx.doi.org/10.1016/S2468-1253(18)30140-7DOI Listing
July 2018
6 Reads

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

J Am Acad Dermatol 2018 Nov 5;79(5):899-903. Epub 2018 Jun 5.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.jaad.2018.05.1245DOI Listing
November 2018
26 Reads

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

Osteoporos Int 2018 Jul 17;29(7):1679-1681. Epub 2018 May 17.

Department of Clinical and Experimental Medicine, University of Messina, Pad. B, 2nd floor, A.O.U. Policlinico "G. Martino" Via C. Valeria, 98125, Messina, Italy.

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. Read More

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http://dx.doi.org/10.1007/s00198-018-4563-0DOI Listing
July 2018
7 Reads

Management of giant embryonic vein in Klippel-Trénaunay syndrome.

J Vasc Surg Venous Lymphat Disord 2018 07;6(4):523-525

Division of Vascular and Interventional Radiology, Mayo Clinic, Rochester, Minn.

Klippel-Trénaunay syndrome is a rare mixed malformation characterized by congenital varicose veins, low-flow venous and lymphatic malformations, hypertrophy of soft tissue and bone, and capillary malformations. A 35-year-old man with a diagnosis of Klippel-Trénaunay syndrome presented to the clinic with significant pain and swelling in the left leg. Initial conservative management with compression therapy failed. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S2213333X183015
Publisher Site
http://dx.doi.org/10.1016/j.jvsv.2018.04.005DOI Listing
July 2018
7 Reads