Cervicofacial venous malformations. MRI features and interventional strategies

Abstract

We retrospectively evaluated 53 consecutive patients with cervicofacial venous malformation who had sclerotherapy. This review included a demographic analysis, MRI reexamination and tabulation of interventional therapeutic strategies. All patients whose MRI studies were included in this review demonstrated characteristic findings: space occupying lesion with hyperintense T2 signal abnormality, patchy contrast enhancement, and no flow signal on the gradient echo images.We concluded that a complete MRI work-up of these patients requires post-contrast scanning and gradient-echo imaging in addition to the standard T1 and T2 weighted spin echo imaging. The majority of patients had sporadic (non-familial) venous anomalies. Sinus pericranii (SP) was identified in six patients (11%) and blue rubber bleb nevus syndrome (BRBNS) was found in two patients (4%). MRI findings of sinus pericranii are discussed in detail. Although sodium tetradecyl and/or absolute ethanol are the most commonly used sclerosants, a wide variety of therapeutic strategies (depending on the nature of the abnormality) are also needed for these patients.

Figure 1

Small VM.The lesion is triangular shaped and located adjacent to the right maxilla, anterior to the masticator muscles. The lesion is hyperintense on the T2-weighted image (A), isointense to the musculature on the Tl-weighted image (arrow) (B) and demonstrates significant contrast enhancement, particularly within the central portion of the lesion, on the post-contrast fatsaturated T-1-weighted image (C). There is mild soft tissue prominence in the corresponding region, associated with a mild degree of signal abnormality, which is most consistent with mild oedema. VMs are characteristically conspicuous on the T2-weighted images and demonstrate obvious contrast enhancement. T1-weighted images are typically insensitive for these lesions. Lymphatic malformations (LMs), a main differential for these lesions, are expected to demonstrate no contrast enhancement or minimal peripheral enhancement (“rings and arcs”). No flow-related signal is seen in or around the lesion on the gradient-echo image (D).

Figure 2

Medium-sized VM. The lesion is somewhat hyperintense on the T2-weighted image (A) and involves several adjacent compartments including the left face, parapharyngeal/paralaryngeal, as well as, the oral cavity (curved arrow). The airway at this level appears to be significantly distorted (small arrows). The lesion demonstrates mild diffuse contrast enhancement. The degree of intensity of the lesion on the T2-weighted image and the degree of contrast enhancement of the lesion are somewhat less conspicuous than expected for a VM. Airway involvement is particularly important at the time of sclerotherapy and may require protection during and after the procedure.

Figure 3

Large VM. The lesion is very large involving several compartments of the head and neck, and extends into the anterior mediastinum as seen on this sagittal T2-weighted image. There are several rounded areas of signal void, consistent with phleboliths, which are typically seen in VMs.

Figure 4

Large VM. The lesion is very extensive involving several compartments in the head and neck bilaterally and demonstrates significant contrast enhancement as seen on this post-contrast fat saturated T1-weighted image (A). The face is significantly distorted bilaterally and the airway is narrowed at the level of the oropharynx (small arrow). The pinna on the left is also involved (large arrow). A direct intralesional contrast injection (B) performed during sclerotherapy / coil embolization shows characteristic contrast filling within the malformation with contrast extending through the diploic space (the socalled “intraosseous dural sinuses”) (arrows) and draining into the intracranial dural sinuses (sinus pericranii).

Figure 5

Large VM following NBCA embolization / sclerotherapy. An axial CT image of the upper neck showing abnormal lobulated soft tissue masses, particularly on the left side, which is an expected CT appearance of a large VM. Scattered hyperdensities are representing NBCA embolic material. NBCA is the embolic material of choice preoperatively because it causes only minimal soft tissue oedema (other sclerosant agents, particularly alcohol, cause significant oedema).