Demidenko Alexander, Jakobiec Frederick A, Hanna Eissa, Walton David S
Massachusetts Eye and Ear Infirmary, Boston, MA 02114, USA.
J Pediatr Ophthalmol Strabismus. 2010 May-Jun;47(3):178-82. doi: 10.3928/01913913-20100505-09. Epub 2010 May 20.
A 10-year-old boy with clinically confirmed congenital pupillary-iris-lens membrane with goniodysgenesis underwent light microscopic examination of the enucleated eye. The anterior segment changes consisted of the absence of the iridial pupillary sphincter muscle and dilator muscle processes. Endothelialization and descemetization of the anterior chamber angle and the anterior surface of the iris covered the original eccentric pupillary opening (occlusio pupillae), but grew around the fibrotic edges of the surgically created, patent pseudopupil, probably secondary responses to surgery. The anterior chamber findings in this case establish a localized syndrome that includes absence of the pupillary sphincter and dilator muscle processes. Incomplete development of the iris may be partly attributable to an abnormality of stromal development and inductions by the anterior neuroectodermal layer, together with anomalies of the pupillo-iridial vasculature.
一名10岁男孩，临床确诊为先天性瞳孔-虹膜-晶状体膜合并前房角发育异常，对其摘除的眼球进行了光学显微镜检查。前段改变包括虹膜瞳孔括约肌和瞳孔开大肌突缺失。前房角和虹膜前表面的内皮化和后弹力层化覆盖了原来偏心的瞳孔开口（瞳孔闭锁），但围绕手术形成的开放性假瞳孔的纤维化边缘生长，这可能是手术的继发反应。该病例的前房检查结果确立了一种局部综合征，包括瞳孔括约肌和瞳孔开大肌突缺失。虹膜发育不全可能部分归因于基质发育异常以及前神经外胚层的诱导，同时伴有瞳孔-虹膜血管系统异常。
