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Lymphangioma
Roya Sohaey, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Cystic lymphangioma (CL)
              • Nonnuchal cystic hygroma (CH)
                • Nuchal CH is common and considered separate diagnosis
                • 80% of all CLs are CH (different prognosis)
                • Location is posterior-lateral neck
              • Axillary lymphangioma
              • Cutaneous lymphangioma
            • Definitions

              • Congenital lymphovascular malformation
              • Defect in connection between lymphatic channels and venous system leads to progressive infiltrative cystic mass

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Saccone G et al: Prenatal ultrasound diagnosis of fetal chest wall cystic lymphangioma: an Italian case series. Eur J Obstet Gynecol Reprod Biol. 236:139-42, 2019
                                        2. Jiao-Ling L et al: Treatment and prognosis of fetal lymphangioma. Eur J Obstet Gynecol Reprod Biol. 231:274-9, 2018
                                        3. Li JL et al: Fetal lymphangioma: prenatal diagnosis on ultrasound, treatment, and prognosis. Eur J Obstet Gynecol Reprod Biol. 231:268-73, 2018
                                        4. Tongsong T et al: Natural course of fetal axillary lymphangioma based on prenatal ultrasound studies. J Ultrasound Med. 37(5):1273-81, 2018
                                        5. Arisoy R et al: Prenatal diagnosis and outcome of lymphangiomas and its relationship with fetal chromosomal abnormalities. J Matern Fetal Neonatal Med. 1-7, 2015
                                        6. Koelblinger C et al: Fetal magnetic resonance imaging of lymphangiomas. J Perinat Med. 41(4):437-43, 2013
                                        7. Surico D et al: Antenatal diagnosis of fetal lymphangioma by ultrasonography. Eur J Obstet Gynecol Reprod Biol. 168(2):236, 2013
                                        8. Chiappinelli A et al: Congenital abdominal cystic lymphangiomas: what is the correct management? J Matern Fetal Neonatal Med. 25(7):915-9, 2012
                                        9. Wiegand S et al: Sclerotherapy of lymphangiomas of the head and neck. Head Neck. 33(11):1649-55, 2011
                                        10. Cozzi DA et al: Fetal abdominal lymphangioma enhanced by ultrafast MRI. Fetal Diagn Ther. 27(1):46-50, 2010
                                        11. Mikovic Z et al: Intrauterine treatment of large fetal neck lymphangioma with OK-432. Fetal Diagn Ther. 26(2):102-6, 2009
                                        12. Pascoli I et al: EXIT (ex utero intrapartum treatment) technique - management of a giant fetal lymphangioma. J Matern Fetal Neonatal Med. 23(2):190-2, 2009
                                        13. Hachisuga M et al: Prenatal diagnosis of a retroperitoneal lymphangioma: a case and review. Fetal Diagn Ther. 24(3):177-81, 2008
                                        14. Langer JE et al: Extensive fetal abdominopelvic lymphangioma. Ultrasound Q. 24(2):115-9, 2008
                                        15. Goldstein I et al: Prenatal diagnosis of fetal chest lymphangioma. J Ultrasound Med. 25(11):1437-40, 2006
                                        16. York DG et al: Fetal abdomino-perineal lymphangioma: differential diagnosis and management. Prenat Diagn. 26(8):692-5, 2006
                                        17. Chiaverini C et al: Prenatal ultrasonographic detection of an axillo-thoracic lymphangioma: an ethical dilemma. Prenat Diagn. 23(11):946-8, 2003
                                        18. Ruano R et al: Prenatal diagnosis of a large axillary cystic lymphangioma by three-dimensional ultrasonography and magnetic resonance imaging. J Ultrasound Med. 22(4):419-23, 2003
                                        19. Ho M et al: Prenatal diagnosis of abdominal lymphangioma. Ultrasound Obstet Gynecol. 20(2):205-6, 2002
                                        20. Deshpande P et al: Prenatal diagnosis of fetal abdominal lymphangioma by ultrasonography. Ultrasound Obstet Gynecol. 17(5):445-8, 2001
                                        21. Zanotti SD et al: Prenatal sonographic diagnosis of axillary cystic lymphangiomas. J Clin Ultrasound. 29(2):112-5, 2001
                                        22. McCoy MC et al: Prenatal diagnosis and management of massive bilateral axillary cystic lymphangioma. Obstet Gynecol. 85(5 Pt 2):853-6, 1995
                                        23. Reichler A et al: Early prenatal diagnosis of axillary cystic hygroma. J Ultrasound Med. 14(8):581-4, 1995
                                        24. Fonkalsrud EW: Congenital malformations of the lymphatic system. Semin Pediatr Surg. 3(2):62-9, 1994
                                        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

                                                  • Synonyms

                                                    • Cystic lymphangioma (CL)
                                                    • Nonnuchal cystic hygroma (CH)
                                                      • Nuchal CH is common and considered separate diagnosis
                                                      • 80% of all CLs are CH (different prognosis)
                                                      • Location is posterior-lateral neck
                                                    • Axillary lymphangioma
                                                    • Cutaneous lymphangioma
                                                  • Definitions

                                                    • Congenital lymphovascular malformation
                                                    • Defect in connection between lymphatic channels and venous system leads to progressive infiltrative cystic mass

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Saccone G et al: Prenatal ultrasound diagnosis of fetal chest wall cystic lymphangioma: an Italian case series. Eur J Obstet Gynecol Reprod Biol. 236:139-42, 2019
                                                                              2. Jiao-Ling L et al: Treatment and prognosis of fetal lymphangioma. Eur J Obstet Gynecol Reprod Biol. 231:274-9, 2018
                                                                              3. Li JL et al: Fetal lymphangioma: prenatal diagnosis on ultrasound, treatment, and prognosis. Eur J Obstet Gynecol Reprod Biol. 231:268-73, 2018
                                                                              4. Tongsong T et al: Natural course of fetal axillary lymphangioma based on prenatal ultrasound studies. J Ultrasound Med. 37(5):1273-81, 2018
                                                                              5. Arisoy R et al: Prenatal diagnosis and outcome of lymphangiomas and its relationship with fetal chromosomal abnormalities. J Matern Fetal Neonatal Med. 1-7, 2015
                                                                              6. Koelblinger C et al: Fetal magnetic resonance imaging of lymphangiomas. J Perinat Med. 41(4):437-43, 2013
                                                                              7. Surico D et al: Antenatal diagnosis of fetal lymphangioma by ultrasonography. Eur J Obstet Gynecol Reprod Biol. 168(2):236, 2013
                                                                              8. Chiappinelli A et al: Congenital abdominal cystic lymphangiomas: what is the correct management? J Matern Fetal Neonatal Med. 25(7):915-9, 2012
                                                                              9. Wiegand S et al: Sclerotherapy of lymphangiomas of the head and neck. Head Neck. 33(11):1649-55, 2011
                                                                              10. Cozzi DA et al: Fetal abdominal lymphangioma enhanced by ultrafast MRI. Fetal Diagn Ther. 27(1):46-50, 2010
                                                                              11. Mikovic Z et al: Intrauterine treatment of large fetal neck lymphangioma with OK-432. Fetal Diagn Ther. 26(2):102-6, 2009
                                                                              12. Pascoli I et al: EXIT (ex utero intrapartum treatment) technique - management of a giant fetal lymphangioma. J Matern Fetal Neonatal Med. 23(2):190-2, 2009
                                                                              13. Hachisuga M et al: Prenatal diagnosis of a retroperitoneal lymphangioma: a case and review. Fetal Diagn Ther. 24(3):177-81, 2008
                                                                              14. Langer JE et al: Extensive fetal abdominopelvic lymphangioma. Ultrasound Q. 24(2):115-9, 2008
                                                                              15. Goldstein I et al: Prenatal diagnosis of fetal chest lymphangioma. J Ultrasound Med. 25(11):1437-40, 2006
                                                                              16. York DG et al: Fetal abdomino-perineal lymphangioma: differential diagnosis and management. Prenat Diagn. 26(8):692-5, 2006
                                                                              17. Chiaverini C et al: Prenatal ultrasonographic detection of an axillo-thoracic lymphangioma: an ethical dilemma. Prenat Diagn. 23(11):946-8, 2003
                                                                              18. Ruano R et al: Prenatal diagnosis of a large axillary cystic lymphangioma by three-dimensional ultrasonography and magnetic resonance imaging. J Ultrasound Med. 22(4):419-23, 2003
                                                                              19. Ho M et al: Prenatal diagnosis of abdominal lymphangioma. Ultrasound Obstet Gynecol. 20(2):205-6, 2002
                                                                              20. Deshpande P et al: Prenatal diagnosis of fetal abdominal lymphangioma by ultrasonography. Ultrasound Obstet Gynecol. 17(5):445-8, 2001
                                                                              21. Zanotti SD et al: Prenatal sonographic diagnosis of axillary cystic lymphangiomas. J Clin Ultrasound. 29(2):112-5, 2001
                                                                              22. McCoy MC et al: Prenatal diagnosis and management of massive bilateral axillary cystic lymphangioma. Obstet Gynecol. 85(5 Pt 2):853-6, 1995
                                                                              23. Reichler A et al: Early prenatal diagnosis of axillary cystic hygroma. J Ultrasound Med. 14(8):581-4, 1995
                                                                              24. Fonkalsrud EW: Congenital malformations of the lymphatic system. Semin Pediatr Surg. 3(2):62-9, 1994