title: Cicatricial upper eyelid retraction in encephalocraniocutaneous lipomatosis: a report of two cases and review of literature.
abstract: OBJECTIVE
  To describe two patients with encephalocraniocutaneous lipomatosis (ECCL) and to review the literature on this disorder.
  
  
  METHODS
  Brain and orbit CT scans were performed on two patients with ECCL. Both patients were examined by the same ophthalmologist and neurologist during at least a 2-year follow-up period.
  
  
  RESULTS
  Unilateral skull hamartomas, intracranial abnormalities, epibulbar choristomas, and ocular adnexal changes including a specific form of cicatricial upper eyelid retraction were present in both patients.
  
  
  CONCLUSIONS
  ECCL is a special form of oculocerebrocutaneous disease that has significant adnexal findings that are essential for the diagnosis of this rare disorder.
authors.affiliation: Department of Ophthalmology, School of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil. aavecruz@fmrp.usp.br
authors.forename: Antonio A V
authors.initials: AA
authors.lastname: Cruz
authors.forename: Tarciso
authors.initials: T
authors.lastname: Schirmbeck
authors.forename: João Monteiro
authors.initials: JM
authors.lastname: Pina-Neto
authors.forename: Carolina A R
authors.initials: CA
authors.lastname: Funayama
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging; D002675:Child, Preschool; D002921:Cicatrix / Q000000981:diagnostic imaging / Q000209:etiology*; D005141:Eyelid Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D005260:Female; D006801:Humans; D007223:Infant; D008068:Lipomatosis / Q000150:complications* / Q000000981:diagnostic imaging; D008297:Male; D020752:Neurocutaneous Syndromes / Q000150:complications* / Q000000981:diagnostic imaging; D009915:Orbit / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed