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Case Reports
. 2018 Apr 21:11:10-12.
doi: 10.1016/j.ajoc.2018.04.018. eCollection 2018 Sep.

SCALP syndrome: What is it and its ophthalmic manifestations

Geoffrey Z P Chan  1 , Louis Stevenson  1 , Swati Sinkar  1 , Geoffrey C Lam  1   2   3
Affiliations
Case Reports

SCALP syndrome: What is it and its ophthalmic manifestations

Geoffrey Z P Chan et al. Am J Ophthalmol Case Rep. .

Abstract

Purpose: To present the ophthalmic manifestations of a 3-month old female with SCALP syndrome.

Observations: The patient presented with multiple ocular anomalies including bilateral limbal dermoids, esotropia and left optic nerve hypoplasia.

Conclusions: We describe systemic and ocular anomalies in a rare case of SCALP syndrome. This report provides additional information on the ocular anomalies not previously described that may be associated with this clinical entity.

Keywords: Aplasia cutis; Limbal dermoid; Melanocytic naevus; Optic nerve hypoplasia; SCALP syndrome; Sebaceous nevus.

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Figures

Fig. 1
Fig. 1
Clinical photograph of face (A) demonstrates linear sebaceous nevi involving the midline of the forehead and large angle esotropia. Bilateral temporal limbal dermoids in right (B) and left (C) eye were confirmed on histopathology.
Fig. 2
Fig. 2
Giant melanocytic naevi distributed across the left scalp, neck and torso.
Fig. 3
Fig. 3
Left scalp demonstrating a region of aplasia cutis congenita. A palpable subcutaneous soft tissue mass over her left parietal bone surrounded by sebaceous naevi.
Fig. 4
Fig. 4
Magnetic resonance imaging of the brain and orbits. Constructive Interference in Steady State (CISS) sequence (A) demonstrates left optic nerve hypoplasia within surrounding dural sheath (arrow). Cranial axial T2 weighted sequence (B) demonstrates polymicrogyria (thin arrow) with increased sulcation of the right posterior parietal lobe and temporo-occipital cortex.
See this image and copyright information in PMC

References

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