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Encephalocraniocutaneous Lipomatosis: A Case With Long Term Follow-up

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome identifiable by the presence of psiloliparus naevi, ocular abnormalities such as choristomas and colobomas, and central nervous system abnormalities.1 Since the first case was reported in 1970 by Haberland and Peru, there have been 60 documented cases worldwide.2 The genetic origins of the disease appear to be mosaic and not traceable to one gene or mutation. The disorder is one of mesenchymal origin affecting mostly neural crest cell derivatives.2 The hallmark lesion of ECCL is the central nervous system (CNS) lipoma.2 Central nervous system lipomas were identified in 33 of the 54 cases reviewed by Moog in 2009. Intracranial lipomas were found frequently at the cerebello-pontine angle and spinal lipomas were identified in 12 of 14 patients who had spinal imaging. Arachnoid cysts were another common CNS finding along with asymmetric changes of the hemispheres and ventricles.1 There were also abnormalities noted in the cerebral vessels, including lepto-meningeal angiomatosis, detected only when vascular studies had been performed.1 Although there is much known about the clinical features of this syndrome, there is less known about the long term prognosis associated with this diagnosis. We report a case with initial presentation and 12 years of follow-up.