Pilling G P
J Pediatr Surg. 1975 Feb;10(1):87-96. doi: 10.1016/s0022-3468(75)80015-7.
This report reviews our experience with 26 infants and children who demonstrate the syndrome of congenital aniridia. Twenty patients exhibited congenital sporadic aniridia, and unilateral Wilms' tumor has developed in seven of them. Six of the total of 26 patients had familial aniridia and none has shown evidence of the development of a Wilms' tumor or any other malignant tumor to date. Fraumeni studied 15 patients with congenital sporadic aniridia. Five developed Wilms' tumors. In the combined series of 35 patients with congenital sporadic aniridia, 12 patients have developed Wilms' tumor for an incidence of 34%. A brief review of our experience and a suggested protocol for the evaluation of patients with congenital aniridia is presented.
本报告回顾了我们对26例患有先天性无虹膜综合征的婴幼儿及儿童的诊治经验。20例为先天性散发性无虹膜，其中7例发生了单侧肾母细胞瘤。26例患者中有6例为家族性无虹膜，迄今为止，无一例有肾母细胞瘤或任何其他恶性肿瘤发生的迹象。弗劳梅尼研究了15例先天性散发性无虹膜患者，其中5例发生了肾母细胞瘤。在这35例先天性散发性无虹膜患者的综合病例组中，有12例发生了肾母细胞瘤，发病率为34%。本文简要回顾了我们的经验，并提出了一个评估先天性无虹膜患者的建议方案。
