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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 reserved for abnormal midline posterior nuchal translucency on early fetal ultrasound
Different than anterolateral neck LM, which does not implicate chromosomal anomalies
Lymphangioma implies neoplasm
LM 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
CT Findings
MR Findings
Ultrasonographic 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
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 and Young's vascular anomalies : hemangiomas and 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
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
Abbreviations
Lymphatic malformation (LM)
Synonyms
Common (cystic) LM, lymphatic anomaly
Avoid incorrect & confusing terminology
Cystic hygroma reserved for abnormal midline posterior nuchal translucency on early fetal ultrasound
Different than anterolateral neck LM, which does not implicate chromosomal anomalies
Lymphangioma implies neoplasm
LM 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
CT Findings
MR Findings
Ultrasonographic 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
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 and Young's vascular anomalies : hemangiomas and 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
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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