결절경화증환자에서 발생한 성상세포 과오종과 연관된 시신경섬유 결손

• 영문명: Retinal Nerve Fiber Layer Defect Associated with Astrocytic Hamartoma in a Patient with Tuberous Sclerosis
• 발행기관: 대한안과학회
• 저자명: 이가현 이나은 이창규 홍사민 성공제 김찬윤,Ka Hyun Lee, MD, Naeun Lee, MD, Chang Kyu Lee, MD, Sa Min Hong, MD,Gong Je Seong, MD, PhD, Chan Yun Kim, MD, PhD
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume54,number8, 1282~1286쪽, 전체 5쪽
• 주제분류: 의약학 > 기타의약학
• 파일형태: PDF
• 발행일자: 2013.08.15

영문 초록

Purpose: To report the progression of an astrocytic hamartoma of the right optic nerve head as well as the retina, and the progression of retinal nerve fiber defect associated with astrocytic hamartoma in a patient with tuberous sclerosis. Case summary: A 6-year-old boy with tuberous sclerosis and an astrocytic hamartoma of the right optic nerve head, which was found at the time of ophthalmologie examinations, was referred from the pediatric neurologist for evaluation of the vigabatrin- associated visual field changes. Fundus examination revealed 1/2 disc diameter (DD)-sized astrocytic hamartoma located at the margin of the superior part of the optic nerve. The retina of the left eye was normal. Eighteen months after the first visit, enlarged optic disc hamartoma of the right eye and newly onset retinal astrocytic hamartoma located approximately 1.5 DD inferior to the fovea of the left eye were found. Three years later, an increase in the size of the astrocytic hamartoma of the right optic nerve and development of retinal nerve fiber defects were observed. Conclusions: Astrocytic hamartoma in patients with tuberous sclerosis is usually stable without progression. However, in our patient, astrocytic hamartoma showed progression, and development of retinal nerve fiber defects occurred. Regular follow-up is necessary for astrocytic hamartoma in patients with tuberous sclerosis.

키워드

Astrocytic hamartoma, Retinal nerve fiber layer defect, Tuberous sclerosis