Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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February 6, 2020
Venolymphatic Facial Malformation
- Background:
- Venolymphatic malformations arise from congenital rests of embryonic lymphatic and venous systems.
- Late-adult presentation is unusual; most lesions present and are diagnosed in early life.
- Clinical Presentation:
- Usually asymptomatic, but may present with purple skin discoloration, pain, swelling, and size fluctuation (increases in Valsalva)
- Key Diagnostic Features:
- Lymphatic malformations are typically transspatial, multicystic neck masses with fluid-fluid levels.
- Venolymphatic malformations should be considered when areas of enhancement and phleboliths are present.
- Hypertrophy of adjacent fat and remodeling of adjacent bone may be present.
- These are low-flow vascular lesions without evidence for enlarged feeding arteries or arteriovenous shunting on angiographic imaging.
- Imaging of the brain should be performed in large or periorbital lesions to rule out associated intracranial vascular abnormalities.
- Differential Diagnoses:
- Plexiform neurofibroma: T2-weighted MR imaging shows hyperintense and hypointense central focus→target sign, which is characteristic of this neoplasm.
- Infantile hemangioma: Presents in children, with gradual involution; T2-weighted MR imaging shows mild hyperintensity relative to muscle without hypertrophy of regional fat, and contrast-enhanced T1WI shows intense enhancement.
- Masticator space sarcoma: Poorly marginated mass with bone destruction and invasion of adjacent fascial planes/spaces
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Arteriovenous malformation: T1-weighted and T2-weighted MR images show enlarged serpiginous fluid voids.
- Treatment:
- Malformations with severe symptoms and potential complications should be treated.
- Percutaneous sclerotherapy is the most common therapy.
- Surgical resection may be required in conjunction with sclerotherapy.
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