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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Synonyms
- Vascular malformation, lymphatic type; lymphatic anomaly
- Avoid incorrect terms: Cystic hygroma & lymphangioma
Definitions
- Lymphatic malformation (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
- Venolymphatic malformation (VLM): Combined elements of venous malformation & LM
- Lymphatic malformation (LM): Subtype of slow-/low-flow congenital vascular malformation composed of embryonic lymphatic sacs; not neoplastic
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Adams DM et al: Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 137(2):1-10, 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
- ISSVA: http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
- 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
- Maclellan RA et al: Expression of follicle-stimulating hormone receptor in vascular anomalies. Plast Reconstr Surg. 133(3):344e-51e, 2014
- Reem RE et al: Periocular hemangiomas and lymphangiomas. Pediatr Clin North Am. 61(3):541-53, 2014
- Alexander MD et al: Percutaneous sclerotherapy with ethanolamine oleate for lymphatic malformations of the head and neck. J Neurointerv Surg. 6(9):691-4, 2014
- Kollipara R et al: Vascular anomalies in pediatric patients: updated classification, imaging, and therapy. Radiol Clin North Am. 51(4):659-72, 2013
- Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
- Hassanein AH et al: Lymphatic malformation: risk of progression during childhood and adolescence. J Craniofac Surg. 23(1):149-52, 2012
- Kim SW et al: Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg. 137(12):1247-50, 2011
- 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
- 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
- Shiels WE 2nd et al: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009
- Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
- Grimmer JF et al: Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 132(11):1251-6, 2006
- 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
- 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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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Synonyms
- Vascular malformation, lymphatic type; lymphatic anomaly
- Avoid incorrect terms: Cystic hygroma & lymphangioma
Definitions
- Lymphatic malformation (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
- Venolymphatic malformation (VLM): Combined elements of venous malformation & LM
- Lymphatic malformation (LM): Subtype of slow-/low-flow congenital vascular malformation composed of embryonic lymphatic sacs; not neoplastic
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Adams DM et al: Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 137(2):1-10, 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
- ISSVA: http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
- 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
- Maclellan RA et al: Expression of follicle-stimulating hormone receptor in vascular anomalies. Plast Reconstr Surg. 133(3):344e-51e, 2014
- Reem RE et al: Periocular hemangiomas and lymphangiomas. Pediatr Clin North Am. 61(3):541-53, 2014
- Alexander MD et al: Percutaneous sclerotherapy with ethanolamine oleate for lymphatic malformations of the head and neck. J Neurointerv Surg. 6(9):691-4, 2014
- Kollipara R et al: Vascular anomalies in pediatric patients: updated classification, imaging, and therapy. Radiol Clin North Am. 51(4):659-72, 2013
- Lala S et al: Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol. 42(7):917-24, 2013
- Hassanein AH et al: Lymphatic malformation: risk of progression during childhood and adolescence. J Craniofac Surg. 23(1):149-52, 2012
- Kim SW et al: Long-term outcome of radiofrequency ablation for intraoral microcystic lymphatic malformation. Arch Otolaryngol Head Neck Surg. 137(12):1247-50, 2011
- 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
- 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
- Shiels WE 2nd et al: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009
- Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
- Grimmer JF et al: Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity. Arch Otolaryngol Head Neck Surg. 132(11):1251-6, 2006
- 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
- 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
