EXCERPT
The organoid nevus syndrome is an oculoneurocutaneous
disorder that usually presents at birth
with unilateral or bilateral epibulbar choristomas
and organoid nevus of the face or scalp and within
the first decade of life with neurologic abnormalities.
1 There is no consistent familial pattern of
inheritance, or sexual or racial predilection. The
syndrome may present with some or all of the features
described in this article. The most common
findings are cutaneous, neurologic, and ophthalmologic.
As with other phakomatoses, other organs may be involved. We report a case of
organoid nevus syndrome that demonstrates the
ability to diagnose this condition on clinical
grounds and initiate an appropriate systemic workup;
treatment considerations for patients with this
syndrome; and a previously unreported feature of the syndrome.
AUTHORS
Dr. Wagner is from the Institute of Ophthalmology and Visual Science,
New Jersey Medical School; and Dr. Facciani is from the Institute of Ophthalmology and Visual Science, New Jersey Medical School, Newark, NJ.
Originally submitted October 31, 2002.
Accepted for publication December 13, 2002.
Address reprint requests to Rudolph S. Wagner, MD, Institute of
Ophthalmology and Visual Science, New Jersey Medical School, 90 Bergen
Street, Doctors Office Center 6th Floor, Newark, NJ 07107.
Supported by an unrestricted grant from Research to Prevent Blindness,
Inc., and the Lion’s Eye Research Foundation of New Jersey.
The authors have no industry relationships to disclose.
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