선상 지선 모반증후군에 동반한 복합성 결막 분리종 -1예 보고-
Complex Conjunctival Choristoma in Linear Nevus Sebaceous Syndrome -A case report-
유진영, 고재경, 강석진, 김병기,
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유진영 ( Yoo Jin-Young )
가톨릭대학교 의과대학 임상병리학교실
고재경 ( Koh Jae-Kyung )
가톨릭대학교 의과대학 임상병리학교실
강석진 ( Kang Seok-Jin )
가톨릭대학교 의과대학 임상병리학교실
김병기 ( Kim Byung-Kee )
가톨릭대학교 의과대학 임상병리학교실
KMID : 0357920000340090680
Abstract
Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder characterized by nevus sebaceus, seizures, and mental retardation, and is often accompanied by ocular anormalies. We recently experienced a case of conjunctival choristoma associated with linear nevus sebaceous syndrome in a 17-month-old boy. The child was the product of a full-term uncomplicated pregnancy and normal delivery with normal karyotype of 46, XY. He was noted at birth to have a conjunctival mass on the right eye and linear nevi over the midline facial region and right temporoparietal scalp. Ophthalmologic examination revealed optic atrophy, peripapillary staphyloma, macular hypogenesis, and osseous choristoma of the choroid. A computed tomography scan demonstrated widening of the subarachnoid space and moderate atrophy of the cerebral and cerebellar hemispheres. He has been followed by ophthalmology and completely free of seizures since birth. The patient underwent excision of the conjunctival mass and biopsies of the skin lesion at 17 months of age. Histologic examination of the skin biopsies was characteristic for the linear sebaceous nevus of Jadassohn. Histologic findings of the conjunctival lesion showed a complex choristoma that contained lacrimal gland tissue and cartilage.
키워드
Choristoma;Nevus sebaceus
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