Skip to main content
Journal cover image

Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy?

Publication ,  Journal Article
Mooy, CM; Clark, BJ; Lee, WR
Published in: Graefes Arch Clin Exp Ophthalmol
1990

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.

Duke Scholars

Published In

Graefes Arch Clin Exp Ophthalmol

DOI

ISSN

0721-832X

Publication Date

1990

Volume

228

Issue

1

Start / End Page

9 / 18

Location

Germany

Related Subject Headings

  • Uvea
  • Syndrome
  • Retinal Vessels
  • Optic Nerve
  • Ophthalmology & Optometry
  • Neural Crest
  • Iris
  • Infant, Newborn
  • Humans
  • Female
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Mooy, C. M., Clark, B. J., & Lee, W. R. (1990). Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy? Graefes Arch Clin Exp Ophthalmol, 228(1), 9–18. https://doi.org/10.1007/BF02764283
Mooy, C. M., B. J. Clark, and W. R. Lee. “Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy?Graefes Arch Clin Exp Ophthalmol 228, no. 1 (1990): 9–18. https://doi.org/10.1007/BF02764283.
Mooy CM, Clark BJ, Lee WR. Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy? Graefes Arch Clin Exp Ophthalmol. 1990;228(1):9–18.
Mooy, C. M., et al. “Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy?Graefes Arch Clin Exp Ophthalmol, vol. 228, no. 1, 1990, pp. 9–18. Pubmed, doi:10.1007/BF02764283.
Mooy CM, Clark BJ, Lee WR. Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy? Graefes Arch Clin Exp Ophthalmol. 1990;228(1):9–18.
Journal cover image

Published In

Graefes Arch Clin Exp Ophthalmol

DOI

ISSN

0721-832X

Publication Date

1990

Volume

228

Issue

1

Start / End Page

9 / 18

Location

Germany

Related Subject Headings

  • Uvea
  • Syndrome
  • Retinal Vessels
  • Optic Nerve
  • Ophthalmology & Optometry
  • Neural Crest
  • Iris
  • Infant, Newborn
  • Humans
  • Female