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Lymphatic Malformation
A. Carlson Merrow, Jr., MD, FAAP; H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Lymphatic malformation (LM)
          • Synonyms

            • Common (cystic) LM, lymphatic anomaly
            • Avoid incorrect and confusing terminology
              • Cystic hygroma reserved for abnormal midline posterior nuchal translucency on early fetal ultrasound
                • Different than anterolateral neck LM, which does not implicate chromosomal anomalies
              • Lymphangioma implies neoplasm
                • LM not neoplastic
          • Definitions

            • Subtype of congenital, slow-/low-flow vascular malformation due to error in lymphatic vessel formation
              • Results in well-defined, cyst-like (macrocystic), ± infiltrative, solid-appearing (microcystic) mass of abnormal lymphatic channels
                • Individual cyst size: Macrocyst > 1 cm, microcyst < 1 cm
              • Mass lacks communication with normal lymphatics

          IMAGING

          • General Features

            • Radiographic Findings

              • Fluoroscopic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Microscopic Features

                                • Immunohistochemical Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Koo HJ et al: Ethanol and/or radiofrequency ablation to treat venolymphatic malformations that manifest as a bulging mass in the head and neck. Clin Radiol. pii: S0009-9260(16)00131-8, 2016
                                            2. Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
                                            3. Elluru RG et al: Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 23(4):178-85, 2014
                                            4. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                                            5. Uller W et al: Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 23(4):208-15, 2014
                                            6. Mulliken JB et al: Mulliken and Young's Vascular Anomalies: Hemangiomas and Malformations. Oxford: Oxford University Press, 2013
                                            7. Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
                                            8. Cahill AM et al: Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology. Cardiovasc Intervent Radiol. 34(4):691-704, 2011
                                            9. Flors L et al: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 31(5):1321-40; discussion 1340-1, 2011
                                            10. Dubois J et al: Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 40(6):895-905, 2010
                                            11. Lobo-Mueller E et al: Extremity vascular anomalies in children: introduction, classification, and imaging. Semin Musculoskelet Radiol. 13(3):210-35, 2009
                                            12. Lobo-Mueller E et al: Complex combined vascular malformations and vascular malformation syndromes affecting the extremities in children. Semin Musculoskelet Radiol. 13(3):255-76, 2009
                                            13. Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
                                            14. Mulliken JB et al: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 69(3):412-22, 1982
                                            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

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Lymphatic malformation (LM)
                                                    • Synonyms

                                                      • Common (cystic) LM, lymphatic anomaly
                                                      • Avoid incorrect and confusing terminology
                                                        • Cystic hygroma reserved for abnormal midline posterior nuchal translucency on early fetal ultrasound
                                                          • Different than anterolateral neck LM, which does not implicate chromosomal anomalies
                                                        • Lymphangioma implies neoplasm
                                                          • LM not neoplastic
                                                    • Definitions

                                                      • Subtype of congenital, slow-/low-flow vascular malformation due to error in lymphatic vessel formation
                                                        • Results in well-defined, cyst-like (macrocystic), ± infiltrative, solid-appearing (microcystic) mass of abnormal lymphatic channels
                                                          • Individual cyst size: Macrocyst > 1 cm, microcyst < 1 cm
                                                        • Mass lacks communication with normal lymphatics

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • Fluoroscopic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Microscopic Features

                                                                          • Immunohistochemical Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Koo HJ et al: Ethanol and/or radiofrequency ablation to treat venolymphatic malformations that manifest as a bulging mass in the head and neck. Clin Radiol. pii: S0009-9260(16)00131-8, 2016
                                                                                      2. Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
                                                                                      3. Elluru RG et al: Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 23(4):178-85, 2014
                                                                                      4. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                                                                                      5. Uller W et al: Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 23(4):208-15, 2014
                                                                                      6. Mulliken JB et al: Mulliken and Young's Vascular Anomalies: Hemangiomas and Malformations. Oxford: Oxford University Press, 2013
                                                                                      7. Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
                                                                                      8. Cahill AM et al: Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology. Cardiovasc Intervent Radiol. 34(4):691-704, 2011
                                                                                      9. Flors L et al: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 31(5):1321-40; discussion 1340-1, 2011
                                                                                      10. Dubois J et al: Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 40(6):895-905, 2010
                                                                                      11. Lobo-Mueller E et al: Extremity vascular anomalies in children: introduction, classification, and imaging. Semin Musculoskelet Radiol. 13(3):210-35, 2009
                                                                                      12. Lobo-Mueller E et al: Complex combined vascular malformations and vascular malformation syndromes affecting the extremities in children. Semin Musculoskelet Radiol. 13(3):255-76, 2009
                                                                                      13. Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
                                                                                      14. Mulliken JB et al: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 69(3):412-22, 1982