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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Lymphatic malformation (LM)
Synonyms
- Lymphangioma, cystic hygroma
Definitions
- Congenital malformation with lymphatic features
- May be part of venolymphatic (mixed) malformation
- Distinguishing LM from venous or mixed malformation has treatment implications
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
Image Interpretation Pearls
Selected References
- Aluffi Valletti P et al: A single-center experience in the management of head and neck lymphangiomas. Oral Maxillofac Surg. 24(1):109-15, 2020
- Chen WL et al: Comprehensive treatment of massive macroglossia due to venous and lymphatic malformations. Int J Oral Maxillofac Surg. 49(7):874-81, 2020
- Cronan J et al: The role of interventional radiologists in the treatment of congenital lymphatic malformations. Semin Intervent Radiol. 37(3):285-94, 2020
- Hussein A et al: Imaging of vascular malformations. Radiol Clin North Am. 58(4):815-30, 2020
- International Society for the Study of Vascular Anomalies: ISSVA Classification of Vascular Anomalies. Published May 2018. Accessed August 20, 2020. https://www.issva.org/classification
- Jin D et al: Diagnosis of lymphangiomatosis: a study based on CT lymphangiography. Acad Radiol. 27(2):219-26, 2020
- Nelson KJ et al: Effect of long-term phosphodiesterase-5 inhibitor use on refractory lymphatic malformations in adult and teen patients. J Vasc Surg Venous Lymphat Disord. 9(1):258-61, 2020
- Pang C et al: Emerging importance of molecular pathogenesis of vascular malformations in clinical practice and classifications. Vasc Med. 25(4):364-77, 2020
- Iacobas I et al: Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus. Pediatr Blood Cancer. e28036, 2019
- Nelson BL et al: Lymphangioma of the dorsal tongue. Head Neck Pathol. 14(2):512-5, 2019
- Ozeki M et al: The impact of sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies. Orphanet J Rare Dis. 14(1):141, 2019
- Triana P et al: Oral sirolimus: an option in the management of neonates with life-threatening upper airway lymphatic malformations. Lymphat Res Biol. 17(5):504-11, 2019
- Kolay SK et al: Oral lymphangiomas - clinical and histopathological relations: An immunohistochemically analyzed case series of varied clinical presentations. J Oral Maxillofac Pathol. 22(Suppl 1):S108-11, 2018
- Sadick M et al: Interdisciplinary management of head and neck vascular anomalies: clinical presentation, Diagnostic Findings and Minimalinvasive Therapies. Eur J Radiol Open. 4:63-8, 2017
- Mahady K et al: Vascular anomalies of the head and neck in children. Quant Imaging Med Surg. 5(6):886-97, 2015
- Renton JP et al: Current treatment paradigms in the management of lymphatic malformations. Laryngoscope. 121(1):56-9, 2011
- Gloviczki P et al: Vascular malformations: an update. Perspect Vasc Surg Endovasc Ther. 21(2):133-48, 2009
- Hong JP et al: Giant lymphangioma of the tongue. J Craniofac Surg. 20(1):252-4, 2009
- Wiegand S et al: Microcystic lymphatic malformations of the tongue: diagnosis, classification, and treatment. Arch Otolaryngol Head Neck Surg. 135(10):976-83, 2009
- Edwards PD et al: Lymphatic malformation of the lingual base and oral floor. Plast Reconstr Surg. 115(7):1906-15, 2005
- Macdonald AJ et al: Giant ranula of the neck: differentiation from cystic hygroma. AJNR Am J Neuroradiol. 24(4):757-61, 2003
- Rowley H et al: Management of a giant lymphatic malformation of the tongue. Arch Otolaryngol Head Neck Surg. 128(2):190-4, 2002
- Davies D et al: Localized microcystic lymphatic malformations--ultrasound diagnosis. Pediatr Dermatol. 16(6):423-9, 1999
- Koeller KK et al: Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 19(1):121-46; quiz 152-3, 1999
- Brennan TD et al: Lymphangiomas of the oral cavity: a clinicopathologic, immunohistochemical, and electron-microscopic study. J Oral Maxillofac Surg. 55(9):932-5, 1997
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Lymphatic malformation (LM)
Synonyms
- Lymphangioma, cystic hygroma
Definitions
- Congenital malformation with lymphatic features
- May be part of venolymphatic (mixed) malformation
- Distinguishing LM from venous or mixed malformation has treatment implications
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
Image Interpretation Pearls
Selected References
- Aluffi Valletti P et al: A single-center experience in the management of head and neck lymphangiomas. Oral Maxillofac Surg. 24(1):109-15, 2020
- Chen WL et al: Comprehensive treatment of massive macroglossia due to venous and lymphatic malformations. Int J Oral Maxillofac Surg. 49(7):874-81, 2020
- Cronan J et al: The role of interventional radiologists in the treatment of congenital lymphatic malformations. Semin Intervent Radiol. 37(3):285-94, 2020
- Hussein A et al: Imaging of vascular malformations. Radiol Clin North Am. 58(4):815-30, 2020
- International Society for the Study of Vascular Anomalies: ISSVA Classification of Vascular Anomalies. Published May 2018. Accessed August 20, 2020. https://www.issva.org/classification
- Jin D et al: Diagnosis of lymphangiomatosis: a study based on CT lymphangiography. Acad Radiol. 27(2):219-26, 2020
- Nelson KJ et al: Effect of long-term phosphodiesterase-5 inhibitor use on refractory lymphatic malformations in adult and teen patients. J Vasc Surg Venous Lymphat Disord. 9(1):258-61, 2020
- Pang C et al: Emerging importance of molecular pathogenesis of vascular malformations in clinical practice and classifications. Vasc Med. 25(4):364-77, 2020
- Iacobas I et al: Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus. Pediatr Blood Cancer. e28036, 2019
- Nelson BL et al: Lymphangioma of the dorsal tongue. Head Neck Pathol. 14(2):512-5, 2019
- Ozeki M et al: The impact of sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies. Orphanet J Rare Dis. 14(1):141, 2019
- Triana P et al: Oral sirolimus: an option in the management of neonates with life-threatening upper airway lymphatic malformations. Lymphat Res Biol. 17(5):504-11, 2019
- Kolay SK et al: Oral lymphangiomas - clinical and histopathological relations: An immunohistochemically analyzed case series of varied clinical presentations. J Oral Maxillofac Pathol. 22(Suppl 1):S108-11, 2018
- Sadick M et al: Interdisciplinary management of head and neck vascular anomalies: clinical presentation, Diagnostic Findings and Minimalinvasive Therapies. Eur J Radiol Open. 4:63-8, 2017
- Mahady K et al: Vascular anomalies of the head and neck in children. Quant Imaging Med Surg. 5(6):886-97, 2015
- Renton JP et al: Current treatment paradigms in the management of lymphatic malformations. Laryngoscope. 121(1):56-9, 2011
- Gloviczki P et al: Vascular malformations: an update. Perspect Vasc Surg Endovasc Ther. 21(2):133-48, 2009
- Hong JP et al: Giant lymphangioma of the tongue. J Craniofac Surg. 20(1):252-4, 2009
- Wiegand S et al: Microcystic lymphatic malformations of the tongue: diagnosis, classification, and treatment. Arch Otolaryngol Head Neck Surg. 135(10):976-83, 2009
- Edwards PD et al: Lymphatic malformation of the lingual base and oral floor. Plast Reconstr Surg. 115(7):1906-15, 2005
- Macdonald AJ et al: Giant ranula of the neck: differentiation from cystic hygroma. AJNR Am J Neuroradiol. 24(4):757-61, 2003
- Rowley H et al: Management of a giant lymphatic malformation of the tongue. Arch Otolaryngol Head Neck Surg. 128(2):190-4, 2002
- Davies D et al: Localized microcystic lymphatic malformations--ultrasound diagnosis. Pediatr Dermatol. 16(6):423-9, 1999
- Koeller KK et al: Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 19(1):121-46; quiz 152-3, 1999
- Brennan TD et al: Lymphangiomas of the oral cavity: a clinicopathologic, immunohistochemical, and electron-microscopic study. J Oral Maxillofac Surg. 55(9):932-5, 1997
