Congenital vascular malformation with variable lymphatic ± venous vascular elements
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
VLM: Combined elements of venous malformation and LM
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
Consider
Image Interpretation Pearls
Selected References
Shoji MK et al: The use of sirolimus for treatment of orbital lymphatic malformations: a systematic review. Ophthalmic Plast Reconstr Surg. 36(3):215-21, 2020
Colletti G et al: Vascular malformations of the orbit (lymphatic, venous, arteriovenous): diagnosis, management and results. J Craniomaxillofac Surg. 47(5):726-40, 2019
Barnacle AM et al: Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience. Br J Ophthalmol. 100(2):204-8, 2016
Lally SE: Update on orbital lymphatic malformations. Curr Opin Ophthalmol. 27(5):413-5, 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
Balakrishnan K et al: Standardized outcome and reporting measures in pediatric head and neck lymphatic malformations. Otolaryngol Head Neck Surg. 152(5):948-53, 2015
Kanagalingam S et al: Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation. BMJ Case Rep, 2015
Danial C et al: An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol. 70(6):1050-7, 2014
ISSVA 2018 Classification for Vascular Anomalies. Published April 2014. Revised May 2018. Accessed July 2021. http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
Hill RH 3rd et al: Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg. 28(2):119-25, 2012
Chadha V et al: Orbital venous-lymphatic malformation. Eye (Lond). 23(12):2265-6, 2009
Gündüz K et al: Well-circumscribed orbital venous-lymphatic malformations with atypical features in children. Br J Ophthalmol. 93(5):656-9, 2009
Mavrikakis I et al: The role of thrombosis as a mechanism of exacerbation in venous and combined venous lymphatic vascular malformations of the orbit. Ophthalmology. 116(6):1216-24, 2009
Poon CS et al: Orbital lesions: differentiating vascular and nonvascular etiologic factors. AJR Am J Roentgenol. 190(4):956-65, 2008
Smoker WR et al: Vascular lesions of the orbit: more than meets the eye. Radiographics. 28(1):185-204; quiz 325, 2008
Liu H et al: Bilateral orbital lymphangiomas in two patients with generalized lymphangiomatosis. AJNR Am J Neuroradiol. 28(3):491-2, 2007
Ernemann U et al: Navigation-assisted sclerotherapy of orbital venolymphatic malformation: a new guidance technique for percutaneous treatment of low-flow vascular malformations. AJNR Am J Neuroradiol. 25(10):1792-5, 2004
Lewin JS: Low-flow vascular malformations of the orbit: a new approach to a therapeutic dilemma. AJNR Am J Neuroradiol. 25(10):1633-4, 2004
Suzuki Y et al: Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol. 84(6):614-7, 2000
Tunc M et al: Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol. 83(1):76-80, 1999
Kazim M et al: Orbital lymphangioma. Correlation of magnetic resonance images and intraoperative findings. Ophthalmology. 99(10):1588-94, 1992
Graeb DA et al: Orbital lymphangiomas: clinical, radiologic, and pathologic characteristics. Radiology. 175(2):417-21, 1990
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Orbital lymphatic malformation (LM or OLM)
Orbital venolymphatic malformation (VLM or OVLM)
Synonyms
Avoid incorrect terms: Cystic hygroma and lymphangioma
Congenital vascular malformation with variable lymphatic ± venous vascular elements
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
VLM: Combined elements of venous malformation and LM
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
Consider
Image Interpretation Pearls
Selected References
Shoji MK et al: The use of sirolimus for treatment of orbital lymphatic malformations: a systematic review. Ophthalmic Plast Reconstr Surg. 36(3):215-21, 2020
Colletti G et al: Vascular malformations of the orbit (lymphatic, venous, arteriovenous): diagnosis, management and results. J Craniomaxillofac Surg. 47(5):726-40, 2019
Barnacle AM et al: Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience. Br J Ophthalmol. 100(2):204-8, 2016
Lally SE: Update on orbital lymphatic malformations. Curr Opin Ophthalmol. 27(5):413-5, 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
Balakrishnan K et al: Standardized outcome and reporting measures in pediatric head and neck lymphatic malformations. Otolaryngol Head Neck Surg. 152(5):948-53, 2015
Kanagalingam S et al: Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation. BMJ Case Rep, 2015
Danial C et al: An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol. 70(6):1050-7, 2014
ISSVA 2018 Classification for Vascular Anomalies. Published April 2014. Revised May 2018. Accessed July 2021. http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
Hill RH 3rd et al: Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg. 28(2):119-25, 2012
Chadha V et al: Orbital venous-lymphatic malformation. Eye (Lond). 23(12):2265-6, 2009
Gündüz K et al: Well-circumscribed orbital venous-lymphatic malformations with atypical features in children. Br J Ophthalmol. 93(5):656-9, 2009
Mavrikakis I et al: The role of thrombosis as a mechanism of exacerbation in venous and combined venous lymphatic vascular malformations of the orbit. Ophthalmology. 116(6):1216-24, 2009
Poon CS et al: Orbital lesions: differentiating vascular and nonvascular etiologic factors. AJR Am J Roentgenol. 190(4):956-65, 2008
Smoker WR et al: Vascular lesions of the orbit: more than meets the eye. Radiographics. 28(1):185-204; quiz 325, 2008
Liu H et al: Bilateral orbital lymphangiomas in two patients with generalized lymphangiomatosis. AJNR Am J Neuroradiol. 28(3):491-2, 2007
Ernemann U et al: Navigation-assisted sclerotherapy of orbital venolymphatic malformation: a new guidance technique for percutaneous treatment of low-flow vascular malformations. AJNR Am J Neuroradiol. 25(10):1792-5, 2004
Lewin JS: Low-flow vascular malformations of the orbit: a new approach to a therapeutic dilemma. AJNR Am J Neuroradiol. 25(10):1633-4, 2004
Suzuki Y et al: Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol. 84(6):614-7, 2000
Tunc M et al: Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol. 83(1):76-80, 1999
Kazim M et al: Orbital lymphangioma. Correlation of magnetic resonance images and intraoperative findings. Ophthalmology. 99(10):1588-94, 1992
Graeb DA et al: Orbital lymphangiomas: clinical, radiologic, and pathologic characteristics. Radiology. 175(2):417-21, 1990
STATdx includes over 200,000 searchable images, including x-ray, CT, MR, and ultrasound images. To access all images, please log in or subscribe.