Optic Nerve Hypoplasia and the Syndrome of Nevus Sebaceous of Jadassohn: A New Association

Barrett Katz, Clayton A. Wiley, Vincent W. Lee

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


The nevus sebaceous of Jadassohn (NSJ) syndrome is a not uncommon pediatric dermatosis, with malignant potential. It is the cutaneous manifestation of another phakomatosis, characterized by neurologic, ophthalmic, cardiovascular, skeletal, and urogenital involvement. The features of this syndrome overlap those of the oculo-auriculo-vertebral dysplasia of Goldenhar and tuberous sclerosis. The extent of system involvement suggests a developmental insult during the first few weeks of gestation. A clearly genetic basis has not been established. An infant with NSJ syndrome is described who had associated optic nerve hypoplasia. His clinical, pathologic, and radiologic findings, including computed tomography (CT) and magnetic resonance imaging, are presented.

Original languageEnglish (US)
Pages (from-to)1570-1576
Number of pages7
Issue number12
StatePublished - 1987


  • linear nevus sebaceous syndrome of Jadassohn
  • linear nevus syndrome
  • optic nerve hypoplasia
  • phakomatosis

ASJC Scopus subject areas

  • Ophthalmology


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