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Rhegmatogenous Retinal Detachment and Bilateral Optic Disc Coloboma in Organoid Nevus Syndrome

J. Fernando Arevalo, MD; Andres F. Lasave, MD; Fernando A. Arevalo, BS; Jerry A. Shields, MD
JAMA Ophthalmol. 2013;131(1):111-113. doi:10.1001/jamaophthalmol.2013.573.
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Organoid nevus syndrome is characterized by cutaneous sebaceous nevus (nevus sebaceous of Jadassohn), seizures, mental retardation, epibulbar choristomas, and occasionally other systemic abnormalities.1,2 The lesion occurs most often on the scalp and retroauricular area and occasionally on the neck and trunk.3 Organoid nevus syndrome shares similarities with oculoneurocutaneous syndromes, or phakomatoses, and has been also called the linear nevus sebaceous syndrome.1,2 Ocular manifestations are present in up to 50% of patients and include epibulbar choristoma, posterior scleral cartilage, and coloboma of the eyelid, iris, ciliary body, retina, and optic disc.1,2

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Figure 1. Clinical findings in the patient. A, Multiple round, yellowish, elevated lesions involved the scalp in the occipital region. B, Linear nevus sebaceous was seen on her face. C, Incontinentia pigmenti–like lesions with swirling pigmentation of the skin following the lines of Blaschko were seen. D, She had a coloboma of her right upper eyelid and a choristoma that covered half of the cornea in the right eye. E, A choristoma covered the entire cornea in the left eye. The epibulbar choristomas were removed from both eyes, and the esotropia and eyelid colobomas were corrected at age 1 year.

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Figure 2. Ocular B-scan ultrasonography showed evidence of an inferior retinal detachment and coloboma of both optic nerves. A and B, The right eye showed optic disc coloboma and retinal detachment. C, The left eye showed optic disc coloboma. D, Computed tomography showed calcification of the wall of both eyes.




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