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A case of myotonic dystrophy with abnormality in the macula, ophthalmic artery and the skull Sachiko Madachi 1 , Hiromi Yano 1 , Takahito Shima 2 , Shinji Uchiyama 2 1Dept. of Ophthalmol, Ishikawa Prefect Cent Hosp 2Dept of Neurol, Ishikawa Prefect Cent Hosp pp.949-953
Published Date 1986/8/15
DOI https://doi.org/10.11477/mf.1410209836
  • Abstract
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A 35-year-old male had been diagnosed as amblyopia in the left eye at the age of 8 years. He noticed loss of vision in the left when waking up and sought medical advice immediately. The visual acuity was 1.5 RE and 0 LE. Besides bilateral cataract and ocular hypotony, various abnormalities were detected in the left fundus.

In the left eye, band-shaped yellowish lesion was located along the papillomacular bundle strewn with white streaks. This band appeared as window defect and blocked fluorescence on fluorescein angiogram. Additionally, there were an aberrant retinal artery crossing the macula and an atypical choroidal coloboma. White dots in the perifoveal area were the chief pathological finding in the right eye.

A striking attenuation, or hypoplasia, of the left ophthalmic artery was detected by carotid angiography. The transient visual loss in the left eye seemed to be due to ischemia of ophthalmic artery.

Consecutive examinations confirmed the diagnosis of myotonic dystrophy with various associated features with abnormalities of the skull including cranial hyper. ostosis, small sella turcica, narrow and high-arched palate and occult spina bifida.

Rinsho Ganka (Jpn J Clin Ophthalmol) 40(8) : 949-953. 1986


Copyright © 1986, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1308 印刷版ISSN 0370-5579 医学書院

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