1,533 results match your criteria: "Klippel-Trenaunay-Weber Syndrome"
Pearls and Pitfalls in the Management of Vascular Anomalies.
Semin Intervent Radiol
August 2024
The AVM Center of New York at Lenox Hill, Lenox Hill Hospital, Northwell Health, New York, New York.
Recent advancements in endovascular techniques have transformed the management of vascular malformations, shifting away from traditional surgical resections to less invasive methods like embolization and sclerotherapy. Vascular malformations are congenital anomalies resulting from embryonic angiogenesis failures, often misdiagnosed but manageable through a multidisciplinary approach. These malformations range from high-flow arteriovenous connections to low-flow venous, lymphatic, or mixed lesions, each requiring specific diagnostic and treatment strategies.
View Article and Find Full Text PDFKlippel-Trénaunay-Weber Syndrome: Prenatal Diagnosis and Review of the Literature.
J Clin Ultrasound
October 2024
Gynecology and Obstetrics Unit, Department of Public Health, School of Medicine, University of Naples Federico II, Naples, Italy.
Klippel-Trénaunay-Weber syndrome (KTW) is a rare congenital disease, representing a challenge in prenatal diagnosis due to overlapping characteristics with other syndromes and no specific genetic markers known to date. We have collected all the cases present in the literature on the prenatal diagnosis of KTW, emphasizing common ultrasound findings that can guide the clinician and genetics to the prenatal counseling. Thus, we collected all the information about the postnatal prognosis and the necessity for treatment.
View Article and Find Full Text PDFManagement of penile varicosity in Klippel-Trenaunay-Weber syndrome: a case report.
Int J Impot Res
October 2024
Department of Urology, AOUI Borgo Trento Hospital, University of Verona, Verona, Italy.
Extensile Anterior Approach for Total Hip Arthroplasty in a Patient with Crowe IV Hip Dysplasia and Klippel-Trénaunay Syndrome: A Case Report.
JBJS Case Connect
July 2024
Department of Orthopaedic Surgery, Emory University School of Medicine, Atlanta, Georgia.
Article Synopsis
- A 17-year-old boy with Klippel-Trénaunay syndrome underwent total hip arthroplasty (THA) and a subtrochanteric shortening osteotomy using a direct anterior approach (DAA) due to severe Crowe IV hip dysplasia.
- He began transitioning from limited weight-bearing to a more progressive protocol six weeks after surgery and was able to walk without pain by three months.
- The case highlights the importance of a multidisciplinary approach in caring for patients with KTS undergoing orthopedic procedures, demonstrating that the surgical technique used can be safe and effective for this condition.
Pulmonary Embolism in Klippel-Trenaunay-Weber Syndrome With Slipped Capital Femoral Epiphysis.
J Am Acad Orthop Surg Glob Res Rev
July 2024
From the Department of Biomedical Engineering, National Taiwan University, Taipei, Taiwan (Dr. Chen and Dr. Shih); the Department of Orthopaedics, Cathay General Hospital, Taipei, Taiwan (Dr. Chen); the Department of Orthopaedic Surgery, National Taiwan University Hospital, Taipei, Taiwan (Dr. Kuo and Dr. Wu); the Cochrane Taiwan, Taipei Medical University, Taipei, Taiwan (Dr. Kuo); and the Department of Integrated Diagnostics & Therapeutics, National Taiwan University Hospital, Taipei, Taiwan (Dr. Yeh).
Article Synopsis
- A 12-year-old boy with Klippel-Trenaunay-Weber syndrome had surgery for a condition called unstable slipped capital femoral epiphysis and later developed a pulmonary embolism.
- The text highlights the rarity but severity of pulmonary embolism in children, especially when they have risk factors.
- Emphasizes the need for early intervention for slipped capital femoral epiphysis to reduce immobility and the importance of monitoring for potential complications like femoral head osteonecrosis.
Pediatric Limb Asymmetry: A Unique Presentation of Angioosteohypertrophic Syndrome.
Cureus
June 2024
Department of Pediatrics, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Article Synopsis
- * PWS is distinct from Klippel-Trenaunay syndrome, as it features more severe arterial and venous connections in the affected limb.
- * A recent case report from CHU Med VI Oujda describes a 7-year-old patient with PWS, showing marked asymmetry and other symptoms in the right upper limb, confirmed by arteriography.
Dermato-Radiological Evaluation of Congenital Limb Overgrowth Vascular Syndromes.
Indian Dermatol Online J
April 2024
Department of Radiodiagnosis, ABVIMS and Dr. RML Hospital, New Delhi, Delhi, India.
International Society for the Study of Vascular Anomalies classification defines Congenital Limb Overgrowth Vascular Syndromes (CLOS) as a subset of vascular syndromes with other abnormalities that present with unilateral limb overgrowth. It includes Klippel-Trenaunay Syndrome, Parkes-Weber Syndrome, CLOVES (Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Spinal/Skeletal Anomalies/Scoliosis) Syndrome, Proteus Syndrome, PTEN Hamartomatous Syndrome, and Fibroadipose Vascular Anomaly. Due to their rare and complex nature, a multidisciplinary approach to diagnosis and treatment is required.
View Article and Find Full Text PDFSuccessful interventional treatment of proximal deep vein thrombosis in klippel-trenaunay syndrome.
Cardiovasc Interv Ther
October 2024
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 6-1, Kishibe-Shimmachi, Suita, Osaka, 564-8565, Japan.
Moving Beyond Hemangioma: Interactive, Multidisciplinary, Case-Based Teaching in Vascular Anomalies for Pediatric Residents.
MedEdPORTAL
May 2024
Associate Professor of Pediatrics, Pediatric Hematology/Oncology, Pediatric Residency Program Director, and Assistant Dean of Education, Baylor College of Medicine and CHRISTUS Children's.
Article Synopsis
- Vascular anomalies, including tumors and malformations, are complex conditions that require a collaborative approach from multiple specialists, yet there is a gap in education for healthcare providers on this topic.
- A 2-hour workshop using the POGIL framework was developed for pediatric trainees, involving both didactic learning and interactive discussions to enhance understanding and awareness.
- Participant feedback was positive, highlighting the workshop's effectiveness, although there was no statistically significant improvement in knowledge based on pre- and post-assessment results, indicating that while learners found value, further educational enhancements may be needed.
Rare Presentation of the Vein of Servelle in a Case of Klippel-Trenaunay Syndrome.
Cureus
April 2024
Department of General Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education And Research, Wardha, IND.
Article Synopsis
- * The classic features of KTS include port wine stains (capillary malformations), enlarged limbs or bones, and the presence of varicose veins.
- * A case study highlights a 24-year-old male with KTS who experienced varicose veins since age five and had macrodactyly, also showing a connection to the vein of Servelle.
Upper-Extremity Klippel-Trenaunay Syndrome.
R I Med J (2013)
May 2024
Department of Cardiovascular Medicine, Section of Vascular Medicine, Cleveland Clinic, Cleveland, Ohio.
24-Year-Old Patient with Klippel-Trenaunay Syndrome Underwent Cementless Robotic Cruciate-Retaining TKA: A Case Report.
JBJS Case Connect
April 2024
Cleveland Clinic, Department of Orthopedic Surgery, Cleveland, Ohio.
Article Synopsis
- A 24-year-old man with Klippel-Trenaunay syndrome underwent surgery for severe knee osteoarthritis that didn't improve with non-surgical treatments.
- The treatment plan included advanced imaging, sclerotherapy, and robotic-assisted total knee arthroplasty to address both his joint issues and vascular malformations.
- The study suggests that with a careful, multidisciplinary approach, robotic-assisted knee surgery can be effective for young patients with KTS and severe arthritis.
A Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review.
Adv Biomed Res
February 2024
Department of Laboratory Sciences, Faculty of Medicine, Khomein University of Medical Sciences, Khomein, Iran.
Article Synopsis
- * Von Willebrand Disease is the most common inherited bleeding disorder, caused by a deficiency in von Willebrand Factor, which is crucial for blood clotting.
- * A case is presented of a 13-year-old boy with both KTS and von Willebrand Disease type 3, a unique combination not previously reported, suggesting a possible shared genetic mutation.
Article Synopsis
- * A case study of a 12-year-old girl with KTS is discussed, highlighting her experience of recurrent profuse vaginal bleeding, alongside a review of 7 similar cases in older individuals who experienced uncontrollable bleeding and anemia.
- * Endovascular procedures were the main treatment approach for KTS cases with genital issues, while recent studies point to a link between KTS and the PIK3CA gene mutation, suggesting potential pharmacological treatment options.
Klippel-Trenaunay syndrome or not? An exploration of atypical presentations.
BMJ Case Rep
March 2024
Vascular Surgery, Northern Health, Epping, Victoria, Australia.
Article Synopsis
- - Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder usually seen in infants, marked by a combination of a port-wine stain, early varicose veins, and limb overgrowth.
- - An unusual variant of KTS, known as 'inverse KTS,' presents with limb shortening instead of the typical lengthening, while still showing port-wine stains and varicosities.
- - This report discusses two cases of 'inverse KTS,' raising questions about whether they are simply variants of KTS or represent a distinct clinical syndrome, contributing to our understanding of this complex condition.
Hemangiomatosis of the spleen in a patient with Klippel-Trenaunay syndrome: A case report.
Indian J Pathol Microbiol
October 2024
Gujarat Adani Institute of Medical Sciences, Bhuj, Gujarat, India.
Hemangiomatosis of the spleen is a benign vascular condition occurring as a manifestation of systemic angiomatosis. It is usually associated with Klippel-Trenaunay syndrome (KTS), which is a rare congenital malformation characterized by a triad of varicose veins, bony and soft tissue hypertrophy, and cutaneous and visceral hemangiomas and/or venous malformations. The association of splenic hemangiomatosis with KTS is less documented in the literature.
View Article and Find Full Text PDFKTWS (Klippel-Trenaunay-Weber syndrome): A systematic presentation of a rare disease.
J Cosmet Dermatol
June 2024
General Physician, Islamic Azad University of Medical Sciences, Sari, Iran.
Article Synopsis
- Klippel-Trenaunay-Weber syndrome (KTWS) is a rare condition with symptoms like varicosities, vascular malformations, and soft tissue hypertrophy, diagnosed through clinical assessment and imaging techniques.
- A case study of a 47-year-old male revealed that severe gastrointestinal symptoms, including rectorrhagia, can occur in undiagnosed KTWS patients, necessitating urgent treatment for life-threatening complications.
- The patient underwent successful treatment for a ruptured aneurysm and experienced significant improvement in symptoms after follow-up care, highlighting the importance of continuous monitoring and management in KTWS cases.
Recurrent hematuria involving urinary system with Klippel-Trenaunay syndrome: A case report.
Medicine (Baltimore)
February 2024
Department of Urology, Affiliated Hospital of Shaoxing University, Shaoxing, Zhejiang, China.
Article Synopsis
- - Klippel-Trenaunay syndrome (KTS) is a rare condition related to mutations in the PIK3CA gene, characterized by skin pigmentation, varicose veins, and malformations in the lower limbs, potentially involving the urinary system and causing painless hematuria.
- - The case study discusses a hospitalized woman experiencing massive painless hematuria along with significant lower limb abnormalities, where she was diagnosed with KTS through various examinations like CT urography and cystoscopy.
- - Treatment for the patient included placement of D-J tubes and anti-inflammatory medication, resulting in significant improvement, with conservative management being effective for many KTS cases, while more invasive options are reserved for severe complications.
Kidney transplant and Klippel-Trenaunay-Weber syndrome: an unusual association.
Nefrologia (Engl Ed)
December 2023
Servicio de Nefrología, Hospital Universitari Germans Trias i Pujol, Badalona, Spain; Grupo REMAR-IGTP, Instituto de Investigación Germans Trias i Pujol (IGTP), Badalona, Spain.
Total knee arthroplasty in patients with Klippel Trenaunay syndrome and knee osteoarthritis: A case report and a literature review.
Medicine (Baltimore)
January 2024
Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Article Synopsis
- - Klippel Trenaunay syndrome (KTS) is a rare congenital disorder that involves symptoms like wine stains on the skin, varicose veins, and abnormal growth of bones and soft tissues, primarily appearing at birth or in early childhood; its incidence is estimated at 2-5 cases per 100,000 people, with a higher prevalence in males.
- - A case study describes a 67-year-old male with chronic knee pain who was found to have significant leg discrepancies and symptoms consistent with KTS; he underwent total knee arthroplasty (TKA) and experienced a good recovery over a one-year follow-up.
- - The conclusion emphasizes that TKA can be an effective treatment for arthritis in KTS patients
Vesical Haemangioma in Klippel-Trenaunay-Weber Syndrome: A Clinical Case Report.
Cureus
December 2023
Urology, Wye Valley NHS Trust, Hereford, GBR.
Klippel-Trenaunay-Weber (KTW) syndrome, a rare vascular disorder, often presents with cutaneous capillary malformations and soft tissue hypertrophy. However, urinary tract involvement in the form of vesical haemangiomas is a seldom-encountered clinical condition. We present a case of a 37-year-old male with KTW syndrome who exhibited recurrent gross haematuria, prompting clinical evaluation.
View Article and Find Full Text PDFMultiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome.
Ann Plast Surg
February 2024
Department of Anatomic Pathology, Pathological Sciences, Kyushu University Hospital, Fukuoka, Japan.
Article Synopsis
- * A case is reported of a 66-year-old woman with KTS who developed severe chyluria and hematuria due to a lymphatic-ureteral fistula, resulting in additional health issues, including lymphedema and cellulitis.
- * The patient underwent two surgeries involving 14 lymphaticovenular anastomoses (LVAs), which successfully resolved her chyluria and hematuria, and she showed improvement in her overall health
Value of the short time inversion recovery sequence of magnetic resonance imaging in the staging of Klippel-Trenaunay syndrome complicated with lymphedema.
J Vasc Surg Venous Lymphat Disord
March 2024
Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, Pr China. Electronic address:
Article Synopsis
- This study aimed to explore the utility of MRI, specifically the short time inversion recovery sequence, in staging Klippel-Trenaunay syndrome complicated limb lymphedema (KTS-LE).
- Researchers analyzed 46 KTS-LE patients, classifying them into three stages based on international clinical standards and comparing various MRI indicators among these stages.
- Results highlighted the "honeycomb sign" as a significant imaging marker for diagnosing stage II KTS-LE, offering valuable insights into the condition's severity that other signs did not.
The Prospective Natural History Study of Patients with Intractable Venous Malformation and Klippel-Trenaunay Syndrome to Guide Designing a Proof-of-Concept Clinical Trial for Novel Therapeutic Intervention.
Lymphat Res Biol
February 2024
ARTham Therapeutics, Inc., Yakohama, Japan.
Article Synopsis
- - This study aimed to evaluate the natural progression of venous malformation (VM) and Klippel-Trenaunay Syndrome (KTS) over a 6-month period by observing changes in lesion volume and other health factors in 34 patients.
- - The results showed no significant overall change in lesion volume or other health metrics such as pain, quality of life, and coagulation markers, but some patients had notable changes linked to local infections.
- - Findings suggest that conducting a Phase 2 study to test new treatments without a placebo is justified, using this data as a standard for comparison.
Images in Vascular Medicine: A case of Klippel-Trenaunay syndrome.
Vasc Med
June 2024
Department of Cardiovascular Medicine, Section of Vascular Medicine, Cleveland Clinic, Cleveland, OH, USA.