할러만-스트라이프 증후군 1예

• 영문명: A Case of Hallermann-Streiff Syndrome
• 발행기관: 대한안과학회
• 저자명: 박홍재 이수정 김완수,Hong-Jae Park, M.D., Soo Jung Lee, M.D., Ph.D., Wan-Soo Kim, M.D., Ph.D.
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume48,number9, 1285~1290쪽, 전체 6쪽
• 주제분류: 의약학 > 기타의약학
• 파일형태: PDF
• 발행일자: 2007.09.30

영문 초록

Purpose: We report a case of Hallermann-Streiff syndrome with 360° posterior synechiae, small pupils and aphakia. Methods: A five-year-old female presented with decreasing visual acuity of both eyes. Visual acuity was not checkable due to mental retardation. Microcornea, microphthalmia, nystagmus and esotropia were found, and a fundus examination was not available due to 360° posterior synechiae and small pupils. She had developmental delays, bird-like face and hypotrichosis. A pediatric physician was consulted who diagnosed her with Hallermann-Streiff syndrome. Refraction and fundus examinations were impossible due to her small pupils, so synechiolysis was done. Results: After synechiolysis and pupil dilatation in right eye with iris retractors, continuous curvilinear capsulorhexis (CCC) was attempted. However, the anterior capsule was unusually fragile and fibrtic. Therefore, the CCC failed. In addition, the crystalline lens and the zonule were not found. The posterior capsule was fragile similar to the anterior capsule. Complete posterior CCC (PCCC) was impossible. We could not find any formed vitreous in the vitreous cavity during anterior vitrectomy. We diagnosed the condition as aphakia with only two layers of membranes. Two weeks later, synechiolysis in the left eye was done. The left eye was also diagnosed with aphakia, and only synechiolysis was performed. Conclusions: The possibility of aphakia must be always considered in cases of Hallermann-Streiff syndrome.

키워드

Aphakia, Hallermann-Streiff Syndrome, Posterior synechiae, Small pupil