Venolymphatic malformation is a descriptive term for vascular malformation. It is comprised of dysplastic venous and lymphatic channels. Anatomy can be better categorized under the ISSVA classification of vascular anomalies if it is better known. They were previously described under the spectrum of low-flow or slow-flow vascular malformations.
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Epidemiology
50% of venolymphatic malformations occur at the head and neck region, particularly within the posterior triangle or submandibular regions.
Clinical presentation
painless non-pulsatile mass with overlying normal skin
recurrent pain can occur if there is recurrent phlebothrombosis
Pathology
Genetics
Venous malformations are likely related to a mutation affecting TEK (Ch9p) gene, encoding endothelial cell tyrosine kinase E2 receptor (TIE2). Lymphatic malformations are mostly attributed to the separation failure of the embryonic lymphatic systems due to mutations affecting TIE2/TEK and vascular endothelial growth receptor 3.
Radiographic features
Plain radiograph
phleboliths may be present
Ultrasound
hypoechoic areas with internal loculations
due to a slow flow nature, color Doppler is usually absent
spectral waveforms - monophasic
phleboliths with posterior shadowing
CT
multiple tiny calcified phleboliths "shining pearl sign"
assessment of bone involvement (i.e. secondary remodeling)
MRI
delineation of anatomy and tributaries
T1 hypointense, T2 hyperintense with low signal voids representing the phleboliths
whilst the malformation does not enhance, thin septations within the malformation may show some enhancement
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