Ei. Traboulsi et al., Posterior scleral choristoma in the organoid nevus syndrome (Linear nevus sebaceus of Jadassohn), OPHTHALMOL, 106(11), 1999, pp. 2126-2130
Purpose: To highlight the association of posterior osseous and/or cartilagi
nous ocular choristomas with epibulbar choristomas and the nevus sebaceus o
f Jadassohn,
Design: Small case series.
Participants: Four patients with the organoid nevus syndrome. Methods: Clin
ical and histopathologic studies in four patients with epibulbar lesions an
d nevus sebaceus of Jadassohn,
Main Outcome Measures: Ophthalmoscopic findings of peripapillary lesions. C
omputed tomographic and ultrasonographic characteristic of posterior sclera
l lesions, Ocular histopathologic findings in one globe from one of the stu
dy subjects.
Results: Three patients had the triad of posterior osseous/cartilaginous oc
ular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jad
assohn and one patient had anterior epibulbar choristomas and posterior oss
eous/cartilaginous ocular choristomas, Ultrasonography and computed tomogra
phy were valuable in detecting scleral ossification or epibulbar cartilage
or both. The ophthalmoscopic findings were similar to those of a choroidal
osteoma,
Conclusions: The presence of posterior osseous/cartilaginous ocular chorist
omas in a patient with epilepsy or epibulbar lesions or both suggests the d
iagnosis of nevus sebaceus of Jadassohn, Osseous/cartilaginous ocular chori
stomas should be suspected in patients with nevus sebaceus of Jadassohn and
peripapillary hypopigmented fundus lesions.