Ragge N K, Acheson J, Murphree A L
Division of Ophthalmology, Children's Hospital Los Angeles, USC School of Medicine, California, USA.
Eye (Lond). 1996;10 ( Pt 1):86-91. doi: 10.1038/eye.1996.13.
Iris mammillations are rarely described, distinctive villiform protuberances that can cover the iris. In the majority of reported cases they are unilateral and sporadic, and are seen in association with oculodermal melanosis. In past literature and current clinical practice they are frequently confused with the iris nodules seen in neurofibromatosis type 1. Their clinical significance is not established, although it has been suggested that iris mammillations may be an external sign of ocular hypertension or intraocular malignancy. We report a series of 9 patients between the ages of 3 and 28 years with iris mammillations. The mammillations appear as regularly spaced, deep brown, smooth, conical elevations on the iris, of uniform height or increasing in height as the pupil margin is approached. They often overlie a naevus or an exceptionally deeply pigmented iris, such as that seen in melanosis oculi. One case had an associated ciliary body mass. They tend to occur in more highly pigmented ethnic groups and can be dominantly inherited. Iris mammillations may occur in association with systemic conditions including phakomatosis pigmentovascularis type IIb and neurofibromatosis type 1 when they may even coexist with iris hamartomas.
虹膜乳头状隆起很少被描述，是一种可覆盖虹膜的独特绒毛状突起。在大多数报告病例中，它们是单侧且散在性的，与眼皮肤黑素沉着症相关。在过去的文献和当前的临床实践中，它们常与1型神经纤维瘤病中所见的虹膜结节相混淆。尽管有人提出虹膜乳头状隆起可能是眼压升高或眼内恶性肿瘤的外在表现，但其临床意义尚未明确。我们报告了9例年龄在3至28岁之间患有虹膜乳头状隆起的患者。这些乳头状隆起表现为虹膜上规则分布、深褐色、光滑的圆锥形隆起，高度一致或随着接近瞳孔边缘而增高。它们常覆盖在痣或色素异常深的虹膜上，如眼黑素沉着症中所见。1例伴有睫状体肿物。它们倾向于在色素沉着较高的种族中出现，并且可能为显性遗传。虹膜乳头状隆起可能与包括IIb型色素血管性错构瘤病和1型神经纤维瘤病在内的全身性疾病相关，此时它们甚至可能与虹膜错构瘤共存。
