encephalocraniocutaneous lipomatosis

Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland syndrome, is a rare congenital neurocutaneous syndrome characterized by unilateral lipomas of the cranium, face, and neck, ipsilateral lipodermoids of the eye, and ipsilateral brain anomalies.

The clinical features of ECCL overlap with other neurocutaneous syndromes.

Radiographic features

  • unilateral cerebral hemispheric atrophy ipsilateral to scalp lipoma
  • diffuse, ipsilateral leptomeningeal enhancement 
  • intracranial lipomas
  • spinal lipomas/lipomatosis (cervico-thoracic>lumbar)
  • polymicrogyria of temporal, parietal and/or occipital lobes
  • enlargement of the lateral ventricle, intracranial cysts

History and etymology

The syndrome was first described by Catherine Haberland and Maurice Perou in 1970 .

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