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Lymphatic Malformation, Cervical
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Vascular malformation, lymphatic type; lymphatic anomaly
            • Avoid incorrect terms: Cystic hygroma & lymphangioma
          • Definitions

            • Lymphatic malformation (LM): Subtype of slow/low flow congenital vascular malformation composed of embryonic lymphatic sacs; not neoplastic
              • No communication with normal lymphatics
              • Composed of macrocysts > 1 cm &/or microcysts < 1 cm
            • Venolymphatic malformation (VLM): Combined elements of venous malformation & LM

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Adams DM et al: Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 137(2):1-10, 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. Maclellan RA et al: Expression of follicle-stimulating hormone receptor in vascular anomalies. Plast Reconstr Surg. 133(3):344e-351e, 2014
                                        5. Reem RE et al: Periocular hemangiomas and lymphangiomas. Pediatr Clin North Am. 61(3):541-53, 2014
                                        6. Alexander MD et al: Percutaneous sclerotherapy with ethanolamine oleate for lymphatic malformations of the head and neck. J Neurointerv Surg. 6(9):691-4, 2014
                                        7. Kollipara R et al: Vascular anomalies in pediatric patients: updated classification, imaging, and therapy. Radiol Clin North Am. 51(4):659-72, 2013
                                        8. Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
                                        9. Hassanein AH et al: Lymphatic malformation: risk of progression during childhood and adolescence. J Craniofac Surg. 23(1):149-52, 2012
                                        10. Kim SW et al: Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg. 137(12):1247-50, 2011
                                        11. Bloom DC et al: Management of lymphatic malformations and macroglossia: results of a national treatment survey. Int J Pediatr Otorhinolaryngol. 73(8):1114-8, 2009
                                        12. Castro EC et al: Prox-1 and VEGFR3 antibodies are superior to D2-40 in identifying endothelial cells of lymphatic malformations--a proposal of a new immunohistochemical panel to differentiate lymphatic from other vascular malformations. Pediatr Dev Pathol. 12(3):187-94, 2009
                                        13. Shiels WE 2nd et al: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009
                                        14. Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
                                        15. Grimmer JF et al: Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 132(11):1251-6, 2006
                                        16. Katz SE et al: Combined venous lymphatic malformations of the orbit (so-called lymphangiomas). association with noncontiguous intracranial vascular anomalies. Ophthalmology. 105(1):176-84, 1998
                                        17. 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

                                                • Synonyms

                                                  • Vascular malformation, lymphatic type; lymphatic anomaly
                                                  • Avoid incorrect terms: Cystic hygroma & lymphangioma
                                                • Definitions

                                                  • Lymphatic malformation (LM): Subtype of slow/low flow congenital vascular malformation composed of embryonic lymphatic sacs; not neoplastic
                                                    • No communication with normal lymphatics
                                                    • Composed of macrocysts > 1 cm &/or microcysts < 1 cm
                                                  • Venolymphatic malformation (VLM): Combined elements of venous malformation & LM

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Staging, Grading, & Classification

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Adams DM et al: Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 137(2):1-10, 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. Maclellan RA et al: Expression of follicle-stimulating hormone receptor in vascular anomalies. Plast Reconstr Surg. 133(3):344e-351e, 2014
                                                                              5. Reem RE et al: Periocular hemangiomas and lymphangiomas. Pediatr Clin North Am. 61(3):541-53, 2014
                                                                              6. Alexander MD et al: Percutaneous sclerotherapy with ethanolamine oleate for lymphatic malformations of the head and neck. J Neurointerv Surg. 6(9):691-4, 2014
                                                                              7. Kollipara R et al: Vascular anomalies in pediatric patients: updated classification, imaging, and therapy. Radiol Clin North Am. 51(4):659-72, 2013
                                                                              8. Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
                                                                              9. Hassanein AH et al: Lymphatic malformation: risk of progression during childhood and adolescence. J Craniofac Surg. 23(1):149-52, 2012
                                                                              10. Kim SW et al: Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg. 137(12):1247-50, 2011
                                                                              11. Bloom DC et al: Management of lymphatic malformations and macroglossia: results of a national treatment survey. Int J Pediatr Otorhinolaryngol. 73(8):1114-8, 2009
                                                                              12. Castro EC et al: Prox-1 and VEGFR3 antibodies are superior to D2-40 in identifying endothelial cells of lymphatic malformations--a proposal of a new immunohistochemical panel to differentiate lymphatic from other vascular malformations. Pediatr Dev Pathol. 12(3):187-94, 2009
                                                                              13. Shiels WE 2nd et al: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009
                                                                              14. Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
                                                                              15. Grimmer JF et al: Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 132(11):1251-6, 2006
                                                                              16. Katz SE et al: Combined venous lymphatic malformations of the orbit (so-called lymphangiomas). association with noncontiguous intracranial vascular anomalies. Ophthalmology. 105(1):176-84, 1998
                                                                              17. 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