Medical College of Wisconsin
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Ocular features of the organoid nevus syndrome. Retin Cases Brief Rep 2010;4(4):385-6

Date

10/01/2010

Pubmed ID

25390925

DOI

10.1097/ICB.0b013e3181c702ad

Abstract

PURPOSE: The purpose of the study was to demonstrate the ocular features of organoid nevus syndrome.

METHODS: A 10-day-old male infant was referred for management of retinoblastoma as a result of the presence of intraocular calcification. The examination showed a cutaneous lesion in the midline, epibulbar vascular lesions, and a cutaneous nodule at the lateral canthus. Fundus evaluation showed circumpapillary flat discoloration, which appeared hyperdense on ultrasound.

RESULTS: All these features were consistent with organoid nevus syndrome. The cutaneous lesion was consistent with nevus sebaceous, the epibulbar lesion with choristoma, and the fundus lesion with cartilaginous choristoma.

CONCLUSION: The presence of intraocular calcification often leads to a mistaken diagnosis in organoid nevus syndrome. Appropriate identification is important to diagnose this multisystem disorder.

Author List

Kraus NJ, Ramasubramanian A, Shields CL, Shields JA

Author

Aparna Ramasubramanian MBBS Chief, Associate Professor in the Ophthalmology department at Medical College of Wisconsin