Presentation
Bilateral neck swelling.
Patient Data
Multiple, bilateral, large confluent homogeneous non-enhancing masses of low attenuation are seen within both deep cervical planes. The masses also extend to the superior mediastinum below and the right retropharyngeal region above.
Intraspinal mass opposite C2 compresses the cervical cord which extends through and widens bilateral neural exit foramina.
Case Discussion
The typical homogeneous low attenuation density of the mass lesions was highly suggestive of plexiform neurofibromatosis. It's important to differentiate this from similar cystic masses like cystic lymphangiomas.
On clinical examination, the patient had a large cafe au lait patch on her nape and bilateral axillary freckling. A diagnosis of neurofibromatosis type 1 was made.