link
Bookmarks
Klippel-Trenaunay Syndrome
Brian L. Shaffer, MD; Roya Sohaey, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe
0
0
0
0

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

                                        1. Naganathan S et al: Klippel Trenaunay Weber syndrome. StatPearls Publishing, 2020
                                        2. Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
                                        3. Calvo-Garcia MA et al: Imaging evaluation of fetal vascular anomalies. Pediatr Radiol. 45(8):1218-29, 2014
                                        4. Cakiroglu Y et al: Sonographic identification of klippel-trenaunay-weber syndrome. Case Rep Obstet Gynecol. 2013:595476, 2013
                                        5. Nozaki T et al: Syndromes associated with vascular tumors and malformations: a pictorial review. Radiographics. 33(1):175-95, 2013
                                        6. Chen CP et al: Prenatal sonographic findings of Klippel-Trénaunay-Weber syndrome. J Clin Ultrasound. 35(7):409-12, 2007
                                        7. Peng HH et al: Klippel-Trenaunay-Weber syndrome involving fetal thigh: prenatal presentations and outcomes. Prenat Diagn. 26(9):825-30, 2006
                                        8. Rebarber A et al: Obstetric management of Klippel-Trenaunay syndrome. Obstet Gynecol. 104(5 Pt 2):1205-8, 2004
                                        9. 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
                                        10. 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
                                        Loading...
                                        Related Differential Diagnoses
                                        Loading...
                                        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

                                                                              1. Naganathan S et al: Klippel Trenaunay Weber syndrome. StatPearls Publishing, 2020
                                                                              2. Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
                                                                              3. Calvo-Garcia MA et al: Imaging evaluation of fetal vascular anomalies. Pediatr Radiol. 45(8):1218-29, 2014
                                                                              4. Cakiroglu Y et al: Sonographic identification of klippel-trenaunay-weber syndrome. Case Rep Obstet Gynecol. 2013:595476, 2013
                                                                              5. Nozaki T et al: Syndromes associated with vascular tumors and malformations: a pictorial review. Radiographics. 33(1):175-95, 2013
                                                                              6. Chen CP et al: Prenatal sonographic findings of Klippel-Trénaunay-Weber syndrome. J Clin Ultrasound. 35(7):409-12, 2007
                                                                              7. Peng HH et al: Klippel-Trenaunay-Weber syndrome involving fetal thigh: prenatal presentations and outcomes. Prenat Diagn. 26(9):825-30, 2006
                                                                              8. Rebarber A et al: Obstetric management of Klippel-Trenaunay syndrome. Obstet Gynecol. 104(5 Pt 2):1205-8, 2004
                                                                              9. 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
                                                                              10. 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