caa a22 4500 465777066 CHVBK 20180323111945.0 cr unu---uuuuu 170327e19900101xx s 000 0 eng 10.1007/BF02764283 doi (NATIONALLICENCE)springer-10.1007/BF02764283 Posterior axial corneal malformation and uveoretinal angiodysgenesis — a neurocristopathy? [Elektronische Daten] [Cornelia Mooy, Brian Clark, William Lee] This clinicopathological report describes an unusual combination of axial corneal malformation and angiodysgenesis in the uvea, retina and optic nerve in three eyes. In each specimen there was hypocellularity in the posterior axial stroma, with corresponding loss of the corneal endothelium. The vascular malformation consisted of numerous telangiectatic endothelium-lined tubes with inconspicuous or absent media. One globe was obtained from a stillborn fetus (36 weeks) in which renal agenesis and a sireniform malformation (mermaid fetus) occurred in conjunction with a Fallot's tetralogy, pulmonary hypoplasia and atresia of the trachea and duodenum. Eyes with almost identical malformations were obtained from a 39-week female neonate who died after 5 h as a consequence of renal agenesis and pulmonary hypoplasia. This combination of ocular tissue malformations can be explained by embryological studies, which have shown that the corneal stroma and endothelium and the ocular periendothelial vascular tissues are derived from the neural crest. Springer-Verlag, 1990 Mooy Cornelia Pathology Department, University of Glasgow, Scotland aut Clark Brian Pathology Department, University of Glasgow, Scotland aut Lee William Pathology Department, University of Glasgow, Scotland aut Graefe's Archive for Clinical and Experimental Ophthalmology Springer-Verlag 228/1(1990-01-01), 9-18 0721-832X 228:1<9 1990 228 417 https://doi.org/10.1007/BF02764283 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF02764283 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Mooy Cornelia Pathology Department, University of Glasgow, Scotland aut NATIONALLICENCE 700 1- Clark Brian Pathology Department, University of Glasgow, Scotland aut NATIONALLICENCE 700 1- Lee William Pathology Department, University of Glasgow, Scotland aut NATIONALLICENCE 773 0- Graefe's Archive for Clinical and Experimental Ophthalmology Springer-Verlag 228/1(1990-01-01), 9-18 0721-832X 228:1<9 1990 228 417 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer