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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
- Cystic hygroma reserved for abnormal midline posterior nuchal translucency on early fetal ultrasound
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
- Results in well-defined cyst-like (macrocystic) &/or infiltrative, solid-appearing (microcystic) mass of abnormal lymphatic channels
- Subtype of congenital slow/low flow vascular malformation due to error in lymphatic vessel formation
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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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
- Cystic hygroma reserved for abnormal midline posterior nuchal translucency on early fetal ultrasound
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
- Results in well-defined cyst-like (macrocystic) &/or infiltrative, solid-appearing (microcystic) mass of abnormal lymphatic channels
- Subtype of congenital slow/low flow vascular malformation due to error in lymphatic vessel formation
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
