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Case Reports and Small Case Series
October 1998

Nevus Sebaceus of Jadassohn Associated With Macro Optic Discs and Conjunctival Choristoma

Arch Ophthalmol. 1998;116(10):1379-1381. doi:

Following Jadassohn's1 report in 1895, congenital ocular findings such as epibulbar choristomas (lipodermoids, aberrant lacrimal glands, cartilage), corneal pannus, colobomas (eyelid, iris, retina or choroid, optic nerve), and optic nerve hypoplasia2 but not macro optic discs have been described in combination with a facial nevus sebaceus.

A 42-year-old man was first seen with a dark lesion on the left side of the forehead (4×4 cm, Figure 1, A). Best-corrected visual acuity was 0.7 OD and 0.4 OS with a refractive error of−0.5 diopter OU. The right eye showed a superior corneal pannus and an elevated yellowish subconjunctival mass parallel to the superior limbus. The left eye showed an incomplete coloboma of the upper eyelid and an elevated mass adjacent to the nasal limbus. A scleral staphyloma was located at the 5-o'clock position and a corneal pannus in the inferotemporal quadrant (Figure 1, B). In addition, there was a large yellowish mass extending from the lacrimal gland (Figure 1, C). Both optic discs were very large, with the size of the right exceeding that of the left disc. The margin of the right disc was clearly defined except for the inferotemporal quadrant that showed a rather indistinct margin (Figure 2, A). The margin of the left disc was less clearly defined and showed an irregular indistinct contour in both the nasal and the temporal inferior quadrant (Figure 2, B). The branching pattern of the vessels of the right disc was nearly normal except for a slight nasal dislocation. However, the branching pattern of the left eye appeared to be abnormal (Figure 2, B). Both discs appeared relatively pale with a cup-disc ratio of about 0.9 and a vital, irregular neuroretinal rim (Figure 2, A and B). Measurement with laser scanning tomography showed areas of 6.4 and 12 mm2 (right and left, respectively) while the cup volume was 3.5 and 10.8 mm3 (right and left, respectively) (normal, 2.17±0.55 mm2 and 0.37±0.31 mm3, respectively)3 (Figure 2, C). Results of function tests (eg, visual fields, electroretinography) were normal. Histologic findings from the epibulbar mass of the left eye showed ectopic lacrimal gland, fat, and cartilage (Figure 1, D). Histologic examination of the irregular, dark skin lesion on the left side of the forehead showed a nevus of Jadassohn. Findings from all of the neurological and neuroradiological examinations (computed tomography, magnetic resonance imaging) were unremarkable.