Cystic hygroma is reserved for abnormal midline posterior nuchal translucency on early fetal US
Different than anterolateral neck LM, which does not necessarily implicate chromosomal anomalies
Lymphangioma implies neoplasm
LM is 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) &/or 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
Ultrasonographic Findings
MR Findings
CT 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
Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
Kronfli AP et al: Lymphatic malformations: a 20-year single institution experience. Pediatr Surg Int. 37(6):783-90, 2021
Ramirez-Suarez KI et al: Dynamic contrast-enhanced magnetic resonance lymphangiography. Pediatr Radiol. ePub, 2021
Zobel MJ et al: Management of cervicofacial lymphatic malformations requires a multidisciplinary approach. J Pediatr Surg. 56(5):1062-7, 2021
Kulungowski AM et al: Lymphatic malformations. Semin Pediatr Surg. 29(5):150971, 2020
Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
Clement C et al: An acute presentation of pediatric mesenteric lymphangioma: a case report and literature overview. Acta Chir Belg. 118(5):331-5, 2018
ISSVA Classification of Vascular Anomalies. Published April 2014. Updated May 2018. Accessed April 29, 2020. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
Johnson CM et al: Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations. Pediatr Radiol. 47(9):1196-208, 2017
Kim SH et al: Clinical features of mesenteric lymphatic malformation in children. J Pediatr Surg. 51(4):582-7, 2016
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
Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
Elluru RG et al: Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 23(4):178-85, 2014
Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
Uller W et al: Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 23(4):208-15, 2014
Mulliken JB et al: Mulliken & Young's vascular anomalies : hemangiomas & malformations. Oxford: Oxford University Press, 2013
Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
Cahill AM et al: Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology. Cardiovasc Intervent Radiol. 34(4):691-704, 2011
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
Dubois J et al: Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 40(6):895-905, 2010
Lobo-Mueller E et al: Extremity vascular anomalies in children: introduction, classification, and imaging. Semin Musculoskelet Radiol. 13(3):210-35, 2009
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
Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
Traubici J et al: Mesenteric lymphatic malformation associated with small-bowel volvulus - two cases and a review of the literature. Pediatr Radiol. 32(5):362-5, 2002
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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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 & confusing terminology
Cystic hygroma is reserved for abnormal midline posterior nuchal translucency on early fetal US
Different than anterolateral neck LM, which does not necessarily implicate chromosomal anomalies
Lymphangioma implies neoplasm
LM is 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) &/or 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
Ultrasonographic Findings
MR Findings
CT 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
Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
Kronfli AP et al: Lymphatic malformations: a 20-year single institution experience. Pediatr Surg Int. 37(6):783-90, 2021
Ramirez-Suarez KI et al: Dynamic contrast-enhanced magnetic resonance lymphangiography. Pediatr Radiol. ePub, 2021
Zobel MJ et al: Management of cervicofacial lymphatic malformations requires a multidisciplinary approach. J Pediatr Surg. 56(5):1062-7, 2021
Kulungowski AM et al: Lymphatic malformations. Semin Pediatr Surg. 29(5):150971, 2020
Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
Clement C et al: An acute presentation of pediatric mesenteric lymphangioma: a case report and literature overview. Acta Chir Belg. 118(5):331-5, 2018
ISSVA Classification of Vascular Anomalies. Published April 2014. Updated May 2018. Accessed April 29, 2020. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
Johnson CM et al: Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations. Pediatr Radiol. 47(9):1196-208, 2017
Kim SH et al: Clinical features of mesenteric lymphatic malformation in children. J Pediatr Surg. 51(4):582-7, 2016
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
Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
Elluru RG et al: Lymphatic malformations: diagnosis and management. Semin Pediatr Surg. 23(4):178-85, 2014
Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
Uller W et al: Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 23(4):208-15, 2014
Mulliken JB et al: Mulliken & Young's vascular anomalies : hemangiomas & malformations. Oxford: Oxford University Press, 2013
Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
Cahill AM et al: Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology. Cardiovasc Intervent Radiol. 34(4):691-704, 2011
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
Dubois J et al: Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 40(6):895-905, 2010
Lobo-Mueller E et al: Extremity vascular anomalies in children: introduction, classification, and imaging. Semin Musculoskelet Radiol. 13(3):210-35, 2009
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
Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
Traubici J et al: Mesenteric lymphatic malformation associated with small-bowel volvulus - two cases and a review of the literature. Pediatr Radiol. 32(5):362-5, 2002
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
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