結節硬化症之眼底及臟器變化

Fundus and Systemic Changes in Tuberous Sclerosis

陳立仁(Lee-Jen Chen) ； 林景盛(Jiing-Sheng Lin) ； 謝瑞玟(Jui-Wen Hsieh)

《中華民國眼科醫學會雜誌》 32卷3期 (1993/09) Pp. 319-322

https://doi.org/10.30048/ACTASOS.199309.0008

摘要

本篇收集二十三例結節硬化症病患，觀察其眼底變化及臓器變化。其中十四例在眼底有星細胞瘤，部分病灶伴隨有網膜下出血及色素之變化，顯示星細胞瘤雖起自網膜表層但可影響至網膜下的組識。星細胞瘤多數呈現為平半透明少數呈桑椹狀白色病灶，而後者見於年長之病例，可能為前者之持續性變化而成。
二十三例中有十八例有腦部之鈣化病灶，五例有心臟之肌瘤，二例有腎臟之囊腫，一例死於腎臟之惡性腫瘤，顯示結節硬化症為一侵犯多系統之先天性疾病，對於病患應做頭部、心臟及腎臟之系統檢查。

並列摘要

Tuberous sclerosis is one of the phacomatoses, which may involve multiple organ systems. We collected 23 cases for fundus and systemic examination. 14 cases were with astrocytoma on fundus and most of them are translucent, flat lesions. The mulberry, white lesions were found in two elder patients, which may be the transform of the translucent, flat lesions. There are subretinal hemorrhage and pigmentary change around the lesions, suggesting that astrocytoma is originated from the superficial layer of the retina but may also involved the whole layer of the retina.
There are 18 cases with calcified lesions in the brain, 5 with myoma of the heart, 2 with renal cyst and 1 with renal cell carcinoma. Systemic work up is necessary for the tuberous sclerosis patients.

並列關鍵字

tuberous sclerosis ； astrocytoma