Abstract
A comparative study of clinical pathology and retinal vascular structure is described as studied by vascular casting in an eye of a patient with the Bardet-Biedl syndrome. At the time of examination the eye had been almost blind for at least 4 years. The histopathological examination showed a largely uniform loss of the outer retinal layers. The gross pathological examination of the cast ocular fundus showed three distinct zones, an inner zone inside the temporal vascular arcades where retinal vessels had been cast, a mid peripheral zone with bone spicules, and a peripheral zone with neither cast vessels nor bone spicules. The findings are discussed in relation to possible pathophysiological mechanisms involved in the development of retinal dystrophy in the Bardet-Biedl syndrome.
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- BISLAND T. The Laurence-Moon-Biedl syndrome. Report of a typical case with complete necropsy. Am J Ophthalmol. 1951 Jun;34(6):874–884. [PubMed] [Google Scholar]
- Bek T., Jensen P. K. Three-dimensional structure of human retinal vessels studied by vascular casting. Acta Ophthalmol (Copenh) 1993 Aug;71(4):506–513. doi: 10.1111/j.1755-3768.1993.tb04627.x. [DOI] [PubMed] [Google Scholar]
- Campo R. V., Aaberg T. M. Ocular and systemic manifestations of the Bardet-Biedl syndrome. Am J Ophthalmol. 1982 Dec;94(6):750–756. doi: 10.1016/0002-9394(82)90299-9. [DOI] [PubMed] [Google Scholar]
- Haim M. Prevalence of retinitis pigmentosa and allied disorders in Denmark. II. Systemic involvement and age at onset. Acta Ophthalmol (Copenh) 1992 Aug;70(4):417–426. doi: 10.1111/j.1755-3768.1992.tb02109.x. [DOI] [PubMed] [Google Scholar]
- Harrison J. M., Van Heuven W. A. Rod-cone interactions in the ERG of a patient with Bardet-Biedl syndrome. Doc Ophthalmol. 1985 Aug 30;60(2):203–209. doi: 10.1007/BF00158036. [DOI] [PubMed] [Google Scholar]
- Katsumi O., Tanino T., Hirose T., Larson E. W., Skladzien C. J. Laurence-Moon-Bardet-Biedl syndrome: electrophysical and psychophysical findings. Jpn J Ophthalmol. 1985;29(3):282–289. [PubMed] [Google Scholar]
- Leys M. J., Schreiner L. A., Hansen R. M., Mayer D. L., Fulton A. B. Visual acuities and dark-adapted thresholds of children with Bardet-Biedl syndrome. Am J Ophthalmol. 1988 Nov 15;106(5):561–569. doi: 10.1016/0002-9394(88)90586-7. [DOI] [PubMed] [Google Scholar]
- McLoughlin T. G., Shanklin D. R. Pathology of Laurence-Moon-Bardet-Biedl syndrome. J Pathol Bacteriol. 1967 Jan;93(1):65–79. doi: 10.1002/path.1700930106. [DOI] [PubMed] [Google Scholar]
- Riise R. Visual function in Laurence-Moon-Bardet-Biedl syndrome. A survey of 26 cases. Acta Ophthalmol Suppl. 1987;182:128–131. doi: 10.1111/j.1755-3768.1987.tb02610.x. [DOI] [PubMed] [Google Scholar]
- Rizzo J. F., 3rd, Berson E. L., Lessell S. Retinal and neurologic findings in the Laurence-Moon-Bardet-Biedl phenotype. Ophthalmology. 1986 Nov;93(11):1452–1456. doi: 10.1016/s0161-6420(86)33546-2. [DOI] [PubMed] [Google Scholar]
- Runge P., Calver D., Marshall J., Taylor D. Histopathology of mitochondrial cytopathy and the Laurence-Moon-Biedl syndrome. Br J Ophthalmol. 1986 Oct;70(10):782–796. doi: 10.1136/bjo.70.10.782. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Schachat A. P., Maumenee I. H. Bardet-Biedl syndrome and related disorders. Arch Ophthalmol. 1982 Feb;100(2):285–288. doi: 10.1001/archopht.1982.01030030287011. [DOI] [PubMed] [Google Scholar]
- Stănescu B., Nereanţu F. Laurence-Moon-Bardet-Biedl syndrome with Stargardt's juvenile macular degeneration. Clinical and pathological study. Ophthalmologica. 1971;162(2):76–81. doi: 10.1159/000306248. [DOI] [PubMed] [Google Scholar]