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Orbital Lymphatic Malformation
Joshua E. Lantos, MD; H. Christian Davidson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Orbital lymphatic malformation (LM or OLM)
                • Orbital venolymphatic malformation (VLM or OVLM)
              • Synonyms

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

                • Congenital vascular malformation with variable lymphatic ± venous vascular elements
                • 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
                • VLM: Combined elements of venous malformation and LM

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Shoji MK et al: The use of sirolimus for treatment of orbital lymphatic malformations: a systematic review. Ophthalmic Plast Reconstr Surg. 36(3):215-21, 2020
                                              2. Colletti G et al: Vascular malformations of the orbit (lymphatic, venous, arteriovenous): diagnosis, management and results. J Craniomaxillofac Surg. 47(5):726-40, 2019
                                              3. Barnacle AM et al: Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience. Br J Ophthalmol. 100(2):204-8, 2016
                                              4. Lally SE: Update on orbital lymphatic malformations. Curr Opin Ophthalmol. 27(5):413-5, 2016
                                              5. Merrow AC et al: 2014 revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics. 36(5):1494-516, 2016
                                              6. Balakrishnan K et al: Standardized outcome and reporting measures in pediatric head and neck lymphatic malformations. Otolaryngol Head Neck Surg. 152(5):948-53, 2015
                                              7. Kanagalingam S et al: Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation. BMJ Case Rep, 2015
                                              8. Danial C et al: An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol. 70(6):1050-7, 2014
                                              9. ISSVA 2018 Classification for Vascular Anomalies. Published April 2014. Revised May 2018. Accessed July 2021. http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
                                              10. Hill RH 3rd et al: Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg. 28(2):119-25, 2012
                                              11. Chadha V et al: Orbital venous-lymphatic malformation. Eye (Lond). 23(12):2265-6, 2009
                                              12. Gündüz K et al: Well-circumscribed orbital venous-lymphatic malformations with atypical features in children. Br J Ophthalmol. 93(5):656-9, 2009
                                              13. Mavrikakis I et al: The role of thrombosis as a mechanism of exacerbation in venous and combined venous lymphatic vascular malformations of the orbit. Ophthalmology. 116(6):1216-24, 2009
                                              14. Poon CS et al: Orbital lesions: differentiating vascular and nonvascular etiologic factors. AJR Am J Roentgenol. 190(4):956-65, 2008
                                              15. Smoker WR et al: Vascular lesions of the orbit: more than meets the eye. Radiographics. 28(1):185-204; quiz 325, 2008
                                              16. Liu H et al: Bilateral orbital lymphangiomas in two patients with generalized lymphangiomatosis. AJNR Am J Neuroradiol. 28(3):491-2, 2007
                                              17. Ernemann U et al: Navigation-assisted sclerotherapy of orbital venolymphatic malformation: a new guidance technique for percutaneous treatment of low-flow vascular malformations. AJNR Am J Neuroradiol. 25(10):1792-5, 2004
                                              18. Lewin JS: Low-flow vascular malformations of the orbit: a new approach to a therapeutic dilemma. AJNR Am J Neuroradiol. 25(10):1633-4, 2004
                                              19. Suzuki Y et al: Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol. 84(6):614-7, 2000
                                              20. Tunc M et al: Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol. 83(1):76-80, 1999
                                              21. Kazim M et al: Orbital lymphangioma. Correlation of magnetic resonance images and intraoperative findings. Ophthalmology. 99(10):1588-94, 1992
                                              22. Graeb DA et al: Orbital lymphangiomas: clinical, radiologic, and pathologic characteristics. Radiology. 175(2):417-21, 1990
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Orbital lymphatic malformation (LM or OLM)
                                                            • Orbital venolymphatic malformation (VLM or OVLM)
                                                          • Synonyms

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

                                                            • Congenital vascular malformation with variable lymphatic ± venous vascular elements
                                                            • 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
                                                            • VLM: Combined elements of venous malformation and LM

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Shoji MK et al: The use of sirolimus for treatment of orbital lymphatic malformations: a systematic review. Ophthalmic Plast Reconstr Surg. 36(3):215-21, 2020
                                                                                          2. Colletti G et al: Vascular malformations of the orbit (lymphatic, venous, arteriovenous): diagnosis, management and results. J Craniomaxillofac Surg. 47(5):726-40, 2019
                                                                                          3. Barnacle AM et al: Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience. Br J Ophthalmol. 100(2):204-8, 2016
                                                                                          4. Lally SE: Update on orbital lymphatic malformations. Curr Opin Ophthalmol. 27(5):413-5, 2016
                                                                                          5. Merrow AC et al: 2014 revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics. 36(5):1494-516, 2016
                                                                                          6. Balakrishnan K et al: Standardized outcome and reporting measures in pediatric head and neck lymphatic malformations. Otolaryngol Head Neck Surg. 152(5):948-53, 2015
                                                                                          7. Kanagalingam S et al: Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation. BMJ Case Rep, 2015
                                                                                          8. Danial C et al: An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol. 70(6):1050-7, 2014
                                                                                          9. ISSVA 2018 Classification for Vascular Anomalies. Published April 2014. Revised May 2018. Accessed July 2021. http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
                                                                                          10. Hill RH 3rd et al: Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg. 28(2):119-25, 2012
                                                                                          11. Chadha V et al: Orbital venous-lymphatic malformation. Eye (Lond). 23(12):2265-6, 2009
                                                                                          12. Gündüz K et al: Well-circumscribed orbital venous-lymphatic malformations with atypical features in children. Br J Ophthalmol. 93(5):656-9, 2009
                                                                                          13. Mavrikakis I et al: The role of thrombosis as a mechanism of exacerbation in venous and combined venous lymphatic vascular malformations of the orbit. Ophthalmology. 116(6):1216-24, 2009
                                                                                          14. Poon CS et al: Orbital lesions: differentiating vascular and nonvascular etiologic factors. AJR Am J Roentgenol. 190(4):956-65, 2008
                                                                                          15. Smoker WR et al: Vascular lesions of the orbit: more than meets the eye. Radiographics. 28(1):185-204; quiz 325, 2008
                                                                                          16. Liu H et al: Bilateral orbital lymphangiomas in two patients with generalized lymphangiomatosis. AJNR Am J Neuroradiol. 28(3):491-2, 2007
                                                                                          17. Ernemann U et al: Navigation-assisted sclerotherapy of orbital venolymphatic malformation: a new guidance technique for percutaneous treatment of low-flow vascular malformations. AJNR Am J Neuroradiol. 25(10):1792-5, 2004
                                                                                          18. Lewin JS: Low-flow vascular malformations of the orbit: a new approach to a therapeutic dilemma. AJNR Am J Neuroradiol. 25(10):1633-4, 2004
                                                                                          19. Suzuki Y et al: Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol. 84(6):614-7, 2000
                                                                                          20. Tunc M et al: Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol. 83(1):76-80, 1999
                                                                                          21. Kazim M et al: Orbital lymphangioma. Correlation of magnetic resonance images and intraoperative findings. Ophthalmology. 99(10):1588-94, 1992
                                                                                          22. Graeb DA et al: Orbital lymphangiomas: clinical, radiologic, and pathologic characteristics. Radiology. 175(2):417-21, 1990