- 期刊
- OpenAccess
網膜色素變性症之臨床觀察
Clinical Observation on Pigmentary Degeneration of Retina
並列摘要
Pigmentary degeneration of retina is a disease of unknown etiology, but mostly with hereditary tendency, characterized by a chronic and progressive course. Most of the patients have their onset in childhood, and get blind late in middle age or older. In the early stage, changes occur as degeneration of neuro-epithelium, especially of rod cells; and later, whole retina, pigment epithelium, choriocapillaris are involved. Clinically, besides the major symptom of night blindness, we can see fundoscopically attenuated arteries, retinal optic nerve atrophy, and demomstrable narrowing of visual field, and in late stage, loss of vision. With recent development of fundus fluorescein angiography, electroretinography and electro-oculography., much about the disease can be studied; yet its etiology and treatment leave more to be further investigated. This report comprises the study on 65 cases among 73214 patients of National Taiwan University Hospital, Ophthalmic O.P.D. from 1961 to 1970. Visual acuity was checked with Landolt's ring, visual field with Forster's perimeter, dark adaptation with Goldmann-Weekers' adaptometer, intraocular pressure with Schi'фtz tonometer, skiascopy and fundoscopy after 2% Homatropine, and VDRL test for syphilis. The number of cases studied is 65 including male 55 cases (85%), female 10 cases (15%), The result of the statistic is summarized briefly 1) Male Patients occupy the majority: 55 cases (85%) While female 10 Patents are cases (15%). 2) Distribution of age of the first visit of cases is listed as following: age 11-20 (34%) 21-30(20%) 31-40(15%) 41-50(15%) 51-60(14%) over 61(2%). 2) Age at onset of the disease is studied and revealed as the following: below 10 year old(50%) 11-20(6%) 21-30(12%) 31-40(6%) 41-50(12%) 51-60(4%). 3) Among 24 cases whose family histories were studied, 14 cases (60%) have shown positive family history and 10 cases (40%) negative. 4) As chief complaint is concerned night blindness occupied the majority (44 cases 68%) and next to this is blurring vision, visual field diminution etc. 5) Fundoscopic changes are listed as below. Bone corpuscle like pigment (92%), attenuated arteries (51%), optic nerve atrophy (48%), and change involving posterior pole (55%). 6) Visual field examination revealed 9 eyes had normal field, 17 eyes mild constriction, 21 eyes moderate constriction, 32 eyes marked constriction and 4 eyes ring scotoma. 7) Visual acuity and visual field deteriorate already in the age group of 11-20 and aggravate rapidly in the age group of 41-50. In a few cases good acuity and field were retained 8) Test of dark adaptation by Goldmann-Weekers' Adaptometer revealed 100% of 30 studied cases demonstrated impairment of dark adaptation. 9) Two cases of Laurence-Moon-Biedl syndrome are included in this report. 10) Other findings involve posterior polar cataract, strabismus and vitreous opacity. 11) VDRL test showed that out of 25 cases examined only 1 case was detected as positve. 12) Follow up study of 12 cases showed progressive deterioration in the fundus condition, visual field and vision according with age.