SCALP syndrome: What is it and its ophthalmic manifestations
Published in the American Journal of Ophthalmology Case Reports, the authors describe systemic and ocular anomalies in a rare case of SCALP syndrome.

A 3 month old girl with infantile seizures and global developmental delay was referred for opinion regarding her eyes. This girl was the first child born to non-consanguineous parents. Perinatal history was complicated by prematurity at 35 weeks gestation and intrauterine growth retardation.

On examination, she was fixing and following with her right eye only. The left eye demonstrated no visual response. A large angle esotropia was present (Fig. 1A). She was not able to abduct either eye. There were bilateral large temporal limbal dermoids (Fig. 1B and C). The cornea adjacent to the dermoids showed evidence of exposure keratopathy secondary to poor tear film coverage. Anterior segment was normal. Fundus examination revealed bilateral optic nerve pallor with peri-papillary atrophy and a small optic disc on the left.

Systemic examination revealed numerous cutaneous abnormalities. Giant melanocytic naevi were seen across the left side of her scalp, face, neck, limbs and torso (Fig. 2). A linear sebaceous naevus was present involving her mid-forehead. A palpable subcutaneous soft tissue mass over her left parietal bone surrounded by a large sebaceous naevus across her left scalp was consistent with a region of aplasia cutis congenita.

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