Orbital Imaging Manifestations of Neurocutaneous Syndromes Revisited

Publication date: Available online 30 December 2016
Source:Current Problems in Diagnostic Radiology
Author(s): Abanti Das, Sanjay Sharma
Neurocutaneous syndromes or phakomatosis represent a heterogeneous group of multi-systemic disorders involving structures of ectodermal origin. Characteristic ocular manifestations are described for individual entities which are often the first clues to the underlying diagnosis. However, opaque ocular media or involvement of retrobulbar orbit limits adequate clinical evaluation. This underlines the role of imaging, especially cross-sectional imaging modalities like Computed Tomography (CT) and Magnetic Resonance Imaging (MRI), which offer a comprehensive evaluation of orbit and its contents. This review aims to summarize the cross-sectional imaging features of orbital manifestations of common neurocutaneous syndromes encountered in clinical practice.



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