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The hallmark of linear nevus sebaceous syndrome in the neonatal period is the presence of linear nevus sebaceous in the craniofacial region. Linear nevus sebaceous have raised borders and yellowish color (Figure 302.1). Linear nevus sebaceous is a type of epidermal nevi. Other types of epidermal nevi are nevus unius lateralis, ichthyosis hystrix, and localized pigmented papilloma.


Figure 302.1. Linear nevus sebaceous syndrome. [A] Coloboma of the left eye and nevus sebaceous of the jaw; [B] linear nevus sebaceous.

Seizures and ocular abnormalities are usually present in the neonatal period. Magnetic resonance imaging of the brain may show ventricular dilatation, cerebral and cerebellar hypoplasia, and hamartomatous brain changes. Ocular calcification may be visualized by CT of the orbits (Figure 302.2). Linear nevus sebaceous syndrome is a sporadic disorder.


Figure 302.2. Linear nevus sebaceous syndrome. [A] Fundus calcification; [B] bilateral retinal calcification.


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calcification optic disk calcification calcification linear nevus sebaceous coloboma Click on figure for animated labels.  Pause pointer on different areas of the figure for labels. Figure must be centered. Gomez, 1987 Spitz, 1996 Alfonso, 1987