Multifocal venous malformations: two interesting case presentations

Multiple venous abnormalities involving the orbit, head and neck regions have been previously described. We present two similar cases of low-flow orbital venous vascular malformations (VVMs) in association with multiple low-flow VVMs in the head and neck as well as in the peripheral limb. Literature search revealed that this association has not been previously reported.

Retrospective review of two patients with multifocal VVMs was performed.

Case 1: A 46 year-old Chinese female had a history of multiple vascular lesions involving the right orbit, the tongue, right neck region, right arm and one finger. Valsalva manoeuvre resulted in distension of these lesions. CT scan of the orbits demonstrated bilateral low-flow VVMs. CT angiogram also revealed multiple developmental venous anomalies in the brain. Case 2: A 16 year-old Chinese female had a history of multiple venous malformations involving the neck, left forearm and oral region. Dynamic manoeuvres demonstrated the distensible nature of these lesions. MRI scans of the orbits showed bilateral extraconal low-flow VVMs. Similar mass lesions were seen on MRI scans of the neck.

Multiple cervicofacial venous malformations with developmental abnormalities of the intracranial venous system have been described previously. However, their associations with similar VVMs in the peripheral limb regions have not been reported. Our cases illustrate this rare association. VVMs appear to be benign in nature with symptoms mainly due to mass effect. Management can be conservative in the absence of any sight threatening complication.