Cibis G W, Tripathi R C, Tripathi B J
Section of Ophthalmology, Children's Mercy Hospital, Kansas City, Mo.
Ophthalmic Surg. 1994 Sep-Oct;25(9):580-3.
A pupillary membrane in a case of congenital pupillary-iris-lens membrane with goniodysgenesis was surgically peeled from the lens without causing cataract formation. Histopathology revealed ectopic iris. The ectopic iris found in this condition differentiates congenital pupillary-iris-lens membrane with goniodysgenesis as an entity from persistent pupillary membrane, hereditary goniodysgenesis, and Rieger's anomaly. We suggest that congenital pupillary-iris-lens membrane with goniodysgenesis is a neurocristopathy. The finding of ectopic iris muscle is consistent with avian chimera experiments that have suggested that iris sphincter muscle is derived from the neural crest, not neural ectoderm. Membranes in this condition can be successfully removed when they cause vision loss and amblyopia.
在一例伴有前房角发育异常的先天性瞳孔-虹膜-晶状体膜病例中,手术剥除了与晶状体相连的瞳孔膜,未导致白内障形成。组织病理学检查显示存在异位虹膜。在此病例中发现的异位虹膜将伴有前房角发育异常的先天性瞳孔-虹膜-晶状体膜与持续性瞳孔膜、遗传性前房角发育异常及里格尔异常区分开来。我们认为伴有前房角发育异常的先天性瞳孔-虹膜-晶状体膜是一种神经嵴病。异位虹膜肌的发现与鸟类嵌合体实验结果一致,该实验表明虹膜括约肌起源于神经嵴,而非神经外胚层。当这种情况下的膜导致视力丧失和弱视时,可以成功将其切除。