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Encephalocraniocutaneous Lipomatosis
Gilbert V├ęzina, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Encephalocraniocutaneous lipomatosis (ECCL)
            • Synonyms

              • Haberland syndrome, Fishman syndrome
            • Definitions

              • Rare congenital neurocutaneous syndrome characterized by ipsilateral scalp, eye, and brain abnormalities
              • 1st described in 1970 by Catherine Haberland

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Bieser S et al: Grade II pilocytic astrocytoma in a 3-month-old patient with encephalocraniocutaneous lipomatosis (ECCL): Case report and literature review of low grade gliomas in ECCL. Am J Med Genet A. 167(4):878-81, 2015
                                          2. Lin AW et al: Characteristic imaging findings in encephalocraniocutaneous lipomatosis. Neurology. 84(13):1384-5, 2015
                                          3. Bauld H et al: Encephalocraniocutaneous Lipomatosis: A Case With Long Term Follow-up. Can J Neurol Sci. 41(1):106-8, 2014
                                          4. Chandravanshi SL: Encephalocraniocutaneous lipomatosis: a case report and review of the literature. Indian J Ophthalmol. 62(5):622-7, 2014
                                          5. Chiang CC et al: Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis--a case report and review of the literature. Childs Nerv Syst. 30(1):13-7, 2014
                                          6. Jain P et al: Encephalocraniocutaneous lipomatosis with neurocutaneous melanosis. J Child Neurol. 29(6):846-849, 2013
                                          7. Lee RK et al: Encephalocraniocutaneous lipomatosis: a rare case with development of diffuse leptomeningeal lipomatosis during childhood. Pediatr Radiol. 42(1):129-33, 2012
                                          8. Prontera P et al: Encephalocraniocutaneous lipomatosis (ECCL) in a patient with history of familial multiple lipomatosis (FML). Am J Med Genet A. 149A(3):543-5, 2009
                                          9. Gokhale NR et al: Encephalocraniocutaneous lipomatosis: a rare neurocutaneous syndrome. Indian J Dermatol Venereol Leprol. 73(1):40-2, 2007
                                          10. Moog U et al: Brain anomalies in encephalocraniocutaneous lipomatosis. Am J Med Genet A. 143A(24):2963-72, 2007
                                          11. Hunter AG: Oculocerebrocutaneous and encephalocraniocutaneous lipomatosis syndromes: blind men and an elephant or separate syndromes? Am J Med Genet A. 140(7):709-26, 2006
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Encephalocraniocutaneous lipomatosis (ECCL)
                                                    • Synonyms

                                                      • Haberland syndrome, Fishman syndrome
                                                    • Definitions

                                                      • Rare congenital neurocutaneous syndrome characterized by ipsilateral scalp, eye, and brain abnormalities
                                                      • 1st described in 1970 by Catherine Haberland

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Angiographic Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Bieser S et al: Grade II pilocytic astrocytoma in a 3-month-old patient with encephalocraniocutaneous lipomatosis (ECCL): Case report and literature review of low grade gliomas in ECCL. Am J Med Genet A. 167(4):878-81, 2015
                                                                                  2. Lin AW et al: Characteristic imaging findings in encephalocraniocutaneous lipomatosis. Neurology. 84(13):1384-5, 2015
                                                                                  3. Bauld H et al: Encephalocraniocutaneous Lipomatosis: A Case With Long Term Follow-up. Can J Neurol Sci. 41(1):106-8, 2014
                                                                                  4. Chandravanshi SL: Encephalocraniocutaneous lipomatosis: a case report and review of the literature. Indian J Ophthalmol. 62(5):622-7, 2014
                                                                                  5. Chiang CC et al: Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis--a case report and review of the literature. Childs Nerv Syst. 30(1):13-7, 2014
                                                                                  6. Jain P et al: Encephalocraniocutaneous lipomatosis with neurocutaneous melanosis. J Child Neurol. 29(6):846-849, 2013
                                                                                  7. Lee RK et al: Encephalocraniocutaneous lipomatosis: a rare case with development of diffuse leptomeningeal lipomatosis during childhood. Pediatr Radiol. 42(1):129-33, 2012
                                                                                  8. Prontera P et al: Encephalocraniocutaneous lipomatosis (ECCL) in a patient with history of familial multiple lipomatosis (FML). Am J Med Genet A. 149A(3):543-5, 2009
                                                                                  9. Gokhale NR et al: Encephalocraniocutaneous lipomatosis: a rare neurocutaneous syndrome. Indian J Dermatol Venereol Leprol. 73(1):40-2, 2007
                                                                                  10. Moog U et al: Brain anomalies in encephalocraniocutaneous lipomatosis. Am J Med Genet A. 143A(24):2963-72, 2007
                                                                                  11. Hunter AG: Oculocerebrocutaneous and encephalocraniocutaneous lipomatosis syndromes: blind men and an elephant or separate syndromes? Am J Med Genet A. 140(7):709-26, 2006