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Head and Neck
Diagnosis
Temporal Bone
External Auditory Canal
Congenital Lesions
Periauricular Lymphatic Malformation
Periauricular Lymphatic Malformation
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Abbreviations

          • Lymphatic malformation (LM)

        • Synonyms

          • Vascular malformation, lymphatic type; lymphangioma & cystic hygroma (old terms)

        • Definitions

          • LM: Congenital vascular malformation composed of embryonic lymphatic sacs
          • Venolymphatic malformation (VLM): Combined elements of venous malformation & lymphatic malformation in same lesion

        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. Sodhi KS et al: Role of high resolution ultrasound in parotid lesions in children. Int J Pediatr Otorhinolaryngol. 75(11):1353-8, 2011
                                        2. Wiegand S et al: Lymphatic malformations involving the parotid gland. Eur J Pediatr Surg. 21(4):242-5, 2011
                                        3. Boardman SJ et al: Multimodality treatment of pediatric lymphatic malformations of the head and neck using surgery and sclerotherapy. Arch Otolaryngol Head Neck Surg. 136(3):270-6, 2010
                                        4. 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
                                        5. 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
                                        6. Chen WL et al: Surgical excision of cervicofacial giant macrocystic lymphatic malformations in infants and children. Int J Pediatr Otorhinolaryngol. 73(6):833-7, 2009
                                        7. Duyka LJ et al: Progesterone receptors identified in vascular malformations of the head and neck. Otolaryngol Head Neck Surg. 141(4):491-5, 2009
                                        8. Poldervaart MT et al: Treatment of lymphatic malformations with OK-432 (Picibanil): review of the literature. J Craniofac Surg. 20(4):1159-62, 2009
                                        9. Shiels WE 2nd et al: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009
                                        10. Nehra D et al: Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. J Pediatr Surg. 43(3):451-60, 2008
                                        11. Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
                                        12. Cordes BM et al: Doxycycline sclerotherapy as the primary treatment for head and neck lymphatic malformations. Otolaryngol Head Neck Surg. 137(6):962-4, 2007
                                        13. Grimmer JF et al: Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 132(11):1251-6, 2006
                                        14. Makariou E et al: Cystic hygroma of the neck: association with a growing venous aneurysm. AJNR Am J Neuroradiol. 24(10):2102-4, 2003
                                        15. Giguere CM et al: New treatment options for lymphangioma in infants and children. Ann Otol Rhinol Laryngol. 111(12 Pt 1):1066-75, 2002
                                        16. Elden LM et al: Accuracy and usefulness of radiographic assessment of cervical neck infections in children. J Otolaryngol. 30(2):82-9, 2001
                                        17. Fordham LA et al: Imaging of congenital vascular and lymphatic anomalies of the head and neck. Neuroimaging Clin N Am. 10(1):117-36, viii, 2000
                                        18. Lev S et al: Imaging of cystic lesions. Radiol Clin North Am. 38(5):1013-27, 2000
                                        19. Castellote A et al: Cervicothoracic lesions in infants and children. Radiographics. 19(3):583-600, 1999
                                        20. Koeller KK et al: Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 19(1):121-46; quiz 152-3, 1999
                                        21. Fung K et al: Impact of magnetic resonance imaging on the surgical management of cystic hygromas. J Pediatr Surg. 33(6):839-41, 1998
                                        22. 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
                                        23. Quinn TM et al: Prenatal magnetic resonance imaging enhances fetal diagnosis. J Pediatr Surg. 33(4):553-8, 1998
                                        24. Fageeh N et al: Management of head and neck lymphatic malformations in children. J Otolaryngol. 26(4):253-8, 1997
                                        25. Borecky N et al: Imaging of cervico-thoracic lymphangiomas in children. Pediatr Radiol. 25(2):127-30, 1995
                                        26. Molitch HI et al: Percutaneous sclerotherapy of lymphangiomas. Radiology. 194(2):343-7, 1995
                                        27. Zadvinskis DP et al: Congenital malformations of the cervicothoracic lymphatic system: embryology and pathogenesis. Radiographics. 12(6):1175-89, 1992
                                        28. Caro PA et al: Computed tomography in the diagnosis of lymphangiomas in infants and children. Clin Imaging. 15(1):41-6, 1991
                                        29. Gelbert F et al: MR imaging of head and neck vascular malformations. J Magn Reson Imaging. 1(5):579-84, 1991
                                        30. Yuh WT et al: Magnetic resonance imaging of pediatric head and neck cystic hygromas. Ann Otol Rhinol Laryngol. 100(9 Pt 1):737-42, 1991
                                        31. Som PM et al: Hemorrhaged cystic hygroma and facial nerve paralysis: CT and MR findings. J Comput Assist Tomogr. 14(4):668-71, 1990
                                        32. 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
                                        33. Bill AH Jr et al: A unified concept of lymphangioma and cystic hygroma. Surg Gynecol Obstet. 120:79-86, 1965
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Lymphatic malformation (LM)

                                              • Synonyms

                                                • Vascular malformation, lymphatic type; lymphangioma & cystic hygroma (old terms)

                                              • Definitions

                                                • LM: Congenital vascular malformation composed of embryonic lymphatic sacs
                                                • Venolymphatic malformation (VLM): Combined elements of venous malformation & lymphatic malformation in same lesion

                                              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. Sodhi KS et al: Role of high resolution ultrasound in parotid lesions in children. Int J Pediatr Otorhinolaryngol. 75(11):1353-8, 2011
                                                                              2. Wiegand S et al: Lymphatic malformations involving the parotid gland. Eur J Pediatr Surg. 21(4):242-5, 2011
                                                                              3. Boardman SJ et al: Multimodality treatment of pediatric lymphatic malformations of the head and neck using surgery and sclerotherapy. Arch Otolaryngol Head Neck Surg. 136(3):270-6, 2010
                                                                              4. 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
                                                                              5. 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
                                                                              6. Chen WL et al: Surgical excision of cervicofacial giant macrocystic lymphatic malformations in infants and children. Int J Pediatr Otorhinolaryngol. 73(6):833-7, 2009
                                                                              7. Duyka LJ et al: Progesterone receptors identified in vascular malformations of the head and neck. Otolaryngol Head Neck Surg. 141(4):491-5, 2009
                                                                              8. Poldervaart MT et al: Treatment of lymphatic malformations with OK-432 (Picibanil): review of the literature. J Craniofac Surg. 20(4):1159-62, 2009
                                                                              9. Shiels WE 2nd et al: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009
                                                                              10. Nehra D et al: Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. J Pediatr Surg. 43(3):451-60, 2008
                                                                              11. Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
                                                                              12. Cordes BM et al: Doxycycline sclerotherapy as the primary treatment for head and neck lymphatic malformations. Otolaryngol Head Neck Surg. 137(6):962-4, 2007
                                                                              13. Grimmer JF et al: Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 132(11):1251-6, 2006
                                                                              14. Makariou E et al: Cystic hygroma of the neck: association with a growing venous aneurysm. AJNR Am J Neuroradiol. 24(10):2102-4, 2003
                                                                              15. Giguere CM et al: New treatment options for lymphangioma in infants and children. Ann Otol Rhinol Laryngol. 111(12 Pt 1):1066-75, 2002
                                                                              16. Elden LM et al: Accuracy and usefulness of radiographic assessment of cervical neck infections in children. J Otolaryngol. 30(2):82-9, 2001
                                                                              17. Fordham LA et al: Imaging of congenital vascular and lymphatic anomalies of the head and neck. Neuroimaging Clin N Am. 10(1):117-36, viii, 2000
                                                                              18. Lev S et al: Imaging of cystic lesions. Radiol Clin North Am. 38(5):1013-27, 2000
                                                                              19. Castellote A et al: Cervicothoracic lesions in infants and children. Radiographics. 19(3):583-600, 1999
                                                                              20. Koeller KK et al: Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 19(1):121-46; quiz 152-3, 1999
                                                                              21. Fung K et al: Impact of magnetic resonance imaging on the surgical management of cystic hygromas. J Pediatr Surg. 33(6):839-41, 1998
                                                                              22. 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
                                                                              23. Quinn TM et al: Prenatal magnetic resonance imaging enhances fetal diagnosis. J Pediatr Surg. 33(4):553-8, 1998
                                                                              24. Fageeh N et al: Management of head and neck lymphatic malformations in children. J Otolaryngol. 26(4):253-8, 1997
                                                                              25. Borecky N et al: Imaging of cervico-thoracic lymphangiomas in children. Pediatr Radiol. 25(2):127-30, 1995
                                                                              26. Molitch HI et al: Percutaneous sclerotherapy of lymphangiomas. Radiology. 194(2):343-7, 1995
                                                                              27. Zadvinskis DP et al: Congenital malformations of the cervicothoracic lymphatic system: embryology and pathogenesis. Radiographics. 12(6):1175-89, 1992
                                                                              28. Caro PA et al: Computed tomography in the diagnosis of lymphangiomas in infants and children. Clin Imaging. 15(1):41-6, 1991
                                                                              29. Gelbert F et al: MR imaging of head and neck vascular malformations. J Magn Reson Imaging. 1(5):579-84, 1991
                                                                              30. Yuh WT et al: Magnetic resonance imaging of pediatric head and neck cystic hygromas. Ann Otol Rhinol Laryngol. 100(9 Pt 1):737-42, 1991
                                                                              31. Som PM et al: Hemorrhaged cystic hygroma and facial nerve paralysis: CT and MR findings. J Comput Assist Tomogr. 14(4):668-71, 1990
                                                                              32. 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
                                                                              33. Bill AH Jr et al: A unified concept of lymphangioma and cystic hygroma. Surg Gynecol Obstet. 120:79-86, 1965
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