Case of the Week

Patient photograph (A) demonstrates a fleshy nodular growth over the right upper eyelid. Cutaneous hyperpigmentation (some following Blaschko’s lines) was also noted over the face and neck region on the left side (not shown). MRI CISS images (B and C) show bilateral optic disc colobomas (thin red arrows) and arachnoid cysts (blue arrows with red outlines). T1 imaging also shows lipomas (B, blue arrow; D, blue arrows with red outlines) surrounding the trigeminal nerve (D, thin red arrow). The lipomas involve the CP angles bilaterally and extend along the course of the trigeminal nerves through Meckel’s cave. Coronal SPGR image (E) shows evidence of left occipital polymicrogyria (dashed red arrows). T2 coronal image (F) shows volume loss involving the left cerebral hemisphere (more conspicuous in the frontal lobe), with consequent dilation of the ipsilateral lateral ventricle.