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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Klippel-Trenaunay syndrome (KTS)
Synonyms
- Klippel-Trenaunay-Weber syndrome
- Capillary-lymphatic-venous malformation
- Angioosteohypertrophy syndrome
- Hemangiectatic hypertrophy
Definitions
- Capillary-lymphatic-venous malformation syndrome with bone/soft tissue overgrowth
- Requires 2 of 3 classic findings
- Large cutaneous capillary malformations (90-100%), port-wine stain
- Bone and soft tissue limb hypertrophy
- Low-flow vascular malformations (70-100%)
- Does not involve high-flow vascular malformations
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Naganathan S et al: Klippel Trenaunay Weber syndrome. StatPearls Publishing, 2020
- Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
- Calvo-Garcia MA et al: Imaging evaluation of fetal vascular anomalies. Pediatr Radiol. 45(8):1218-29, 2014
- Cakiroglu Y et al: Sonographic identification of klippel-trenaunay-weber syndrome. Case Rep Obstet Gynecol. 2013:595476, 2013
- Nozaki T et al: Syndromes associated with vascular tumors and malformations: a pictorial review. Radiographics. 33(1):175-95, 2013
- Chen CP et al: Prenatal sonographic findings of Klippel-Trénaunay-Weber syndrome. J Clin Ultrasound. 35(7):409-12, 2007
- Peng HH et al: Klippel-Trenaunay-Weber syndrome involving fetal thigh: prenatal presentations and outcomes. Prenat Diagn. 26(9):825-30, 2006
- Rebarber A et al: Obstetric management of Klippel-Trenaunay syndrome. Obstet Gynecol. 104(5 Pt 2):1205-8, 2004
- Assimakopoulos E et al: Klippel-Trenaunay-Weber syndrome with abdominal hemangiomata appearing on ultrasound examination as intestinal obstruction. Ultrasound Obstet Gynecol. 22(5):549-50, 2003
- Martin WL et al: Klippel-Trenaunay-Weber (KTW) syndrome: the use of in utero magnetic resonance imaging (MRI) in a prospective diagnosis. Prenat Diagn. 21(4):311-3, 2001
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Klippel-Trenaunay syndrome (KTS)
Synonyms
- Klippel-Trenaunay-Weber syndrome
- Capillary-lymphatic-venous malformation
- Angioosteohypertrophy syndrome
- Hemangiectatic hypertrophy
Definitions
- Capillary-lymphatic-venous malformation syndrome with bone/soft tissue overgrowth
- Requires 2 of 3 classic findings
- Large cutaneous capillary malformations (90-100%), port-wine stain
- Bone and soft tissue limb hypertrophy
- Low-flow vascular malformations (70-100%)
- Does not involve high-flow vascular malformations
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Naganathan S et al: Klippel Trenaunay Weber syndrome. StatPearls Publishing, 2020
- Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
- Calvo-Garcia MA et al: Imaging evaluation of fetal vascular anomalies. Pediatr Radiol. 45(8):1218-29, 2014
- Cakiroglu Y et al: Sonographic identification of klippel-trenaunay-weber syndrome. Case Rep Obstet Gynecol. 2013:595476, 2013
- Nozaki T et al: Syndromes associated with vascular tumors and malformations: a pictorial review. Radiographics. 33(1):175-95, 2013
- Chen CP et al: Prenatal sonographic findings of Klippel-Trénaunay-Weber syndrome. J Clin Ultrasound. 35(7):409-12, 2007
- Peng HH et al: Klippel-Trenaunay-Weber syndrome involving fetal thigh: prenatal presentations and outcomes. Prenat Diagn. 26(9):825-30, 2006
- Rebarber A et al: Obstetric management of Klippel-Trenaunay syndrome. Obstet Gynecol. 104(5 Pt 2):1205-8, 2004
- Assimakopoulos E et al: Klippel-Trenaunay-Weber syndrome with abdominal hemangiomata appearing on ultrasound examination as intestinal obstruction. Ultrasound Obstet Gynecol. 22(5):549-50, 2003
- Martin WL et al: Klippel-Trenaunay-Weber (KTW) syndrome: the use of in utero magnetic resonance imaging (MRI) in a prospective diagnosis. Prenat Diagn. 21(4):311-3, 2001
