0
August 1997, Vol 115, No. 8 >
ARTICLE | August 1997

Central Cloudy Corneal Dystrophy of Francois: Title and subTitle BreakA Clinicopathologic Study FREE

Carol L. Karp, MD; Ingrid U. Scott, MD, MPH; W. Richard Green, MD; Tom S. Chang, MD; William W. Culbertson, MD
Arch Ophthalmol. 1997;115(8):1058-1062. doi:10.1001/archopht.1997.01100160228013
Text Size: A A A
Published online
Article
References
Comments

Central cloudy corneal dystrophy of Francois was first described in 1955 by J. Francois; its pathophysiology remains unknown. An 80-year-old woman with bilateral central cloudy corneal dystrophy of Francois was examined after having undergone a combined penetrating keratoplasty and cataract extraction. The corneal button was obtained. Light microscopy revealed stromal staining for acid mucopolysaccharide. Transmission electron microscopy revealed extracellular vacuoles, some of which had fibrillogranular material and electron-dense deposits. Fibrillogranular material was present in and around some keratocytes. Numerous endothelial vacuoles contained light-staining fibrillogranular material and round electron-dense granules. Our findings suggest that the opacities in patients with central cloudy corneal dystrophy of Francois are due to the extracellular accumulation of mucopolysaccharide and lipidlike material. Further studies are needed to elucidate the nature of these deposits.

REFERENCES

1 +
Francois J.  Une nouvelle dystrophie heredofamiliale de la cornee . Bull Soc Belge Ophthalmol . 1955(published in 1956;);111:391-399.
2 +
Francois J, Neetens A.  Nouvelle dystrophie heredofamiliale du parenchyme corneen (heredo-dystrophie mouchetee) . Bull Soc Belge Ophthalmol . 1956(published in 1957;);114:641-646.
3 +
Collier M.  Dystrophie mouchetee du parenchyme corneen avec dystorphie nuageuse central . Bull Soc Ophthalmol Fr . 1964;;64:608-611.
4 +
Collier M.  Elastorrhexie systematisee et dystrophies corneennes chez deux soeurs . Bull Soc Ophtalmol Fr . 1965;;65:301-310.
5 +
Bietti GB.  Contribution a la connaissance des degenerescences corneenes seniles . Arch Ophthalmol . 1965;;25:37-42.
6 +
Collier M.  Dystrophie nuageuse central et dystrophie ponctiforme predescemetique dans une meme famille . Bull Soc Ophthalmol Fr . 1966;;66:575-579.
7 +
Strachan IM.  Cloudy central corneal dystrophy of Francois: five cases in the same family . Br J Ophthalmol . 1969;;53:192-194.
8 +
Bramsen T, Ehlers N, Baggesen LH.  Central cloudy corneal dystrophy of Francois . Acta Ophthalmol . 1976;;54:221-226.
9 +
Ansons AM, Atkinson PL.  Corneal mosaic patterns: morphology and epidemiology . Eye . 1989;;3:811-815.
10 +
Krachmer JH, Dubord PJ, Rodriguez MM, Mannis MJ.  Corneal posterior crocodile shagreen and polymorphic amyloid degeneration . Arch Ophthalmol . 1983;;101:54-59.
11 +
Meyer JC, Quantock AJ, Thonar EJ, Kincaid MC, Hageman GS, Assil KK.  Characterization of a central corneal cloudiness sharing features of posterior crocodile shagreen and central cloudy dystrophy of Francois . Cornea . 1996;;15:347-354.
12 +
Starck T, Hersh PS, Kenyon KR.  Corneal dysgeneses, dystrophies, and degenerations . In: Albert DA, Jakobiec FA, eds. Principles and Practice of Ophthalmology . Philadelphia, Pa: WB Saunders Co; 1994;:chap 3.
13 +
Kempster RC, Hirst LW, de la Cruz Z, Green WR.  Clinicopathological study of the cornea in x-linked ichthyosis . Arch Ophthalmol . 1997;;115:409-415.
14 +
Teng CC.  Macular dystrophy of the cornea: a histochemical and electron microscopic study . Am J Ophthalmol . 1966;;62:436-454.
15 +
Bethell W, McCulloch C, Ghosh M.  Lecithin cholesterol acetyltransferase deficiency: light and electron microscopic findings from two corneas . Can J Ophthalmol . 1975;;10:494-501.
16 +
Nicholson DH, Green WR, Cross HE, Kenyon KR, Massof D.  A clinical and histopathological study of Francois-Neetens speckled corneal dystrophy . Am J Ophthalmol . 1977;;83:554-560.

First Page Preview

First page PDF preview

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Francois J.  Une nouvelle dystrophie heredofamiliale de la cornee . Bull Soc Belge Ophthalmol . 1955(published in 1956;);111:391-399.
2 +
Francois J, Neetens A.  Nouvelle dystrophie heredofamiliale du parenchyme corneen (heredo-dystrophie mouchetee) . Bull Soc Belge Ophthalmol . 1956(published in 1957;);114:641-646.
3 +
Collier M.  Dystrophie mouchetee du parenchyme corneen avec dystorphie nuageuse central . Bull Soc Ophthalmol Fr . 1964;;64:608-611.
4 +
Collier M.  Elastorrhexie systematisee et dystrophies corneennes chez deux soeurs . Bull Soc Ophtalmol Fr . 1965;;65:301-310.
5 +
Bietti GB.  Contribution a la connaissance des degenerescences corneenes seniles . Arch Ophthalmol . 1965;;25:37-42.
6 +
Collier M.  Dystrophie nuageuse central et dystrophie ponctiforme predescemetique dans une meme famille . Bull Soc Ophthalmol Fr . 1966;;66:575-579.
7 +
Strachan IM.  Cloudy central corneal dystrophy of Francois: five cases in the same family . Br J Ophthalmol . 1969;;53:192-194.
8 +
Bramsen T, Ehlers N, Baggesen LH.  Central cloudy corneal dystrophy of Francois . Acta Ophthalmol . 1976;;54:221-226.
9 +
Ansons AM, Atkinson PL.  Corneal mosaic patterns: morphology and epidemiology . Eye . 1989;;3:811-815.
10 +
Krachmer JH, Dubord PJ, Rodriguez MM, Mannis MJ.  Corneal posterior crocodile shagreen and polymorphic amyloid degeneration . Arch Ophthalmol . 1983;;101:54-59.
11 +
Meyer JC, Quantock AJ, Thonar EJ, Kincaid MC, Hageman GS, Assil KK.  Characterization of a central corneal cloudiness sharing features of posterior crocodile shagreen and central cloudy dystrophy of Francois . Cornea . 1996;;15:347-354.
12 +
Starck T, Hersh PS, Kenyon KR.  Corneal dysgeneses, dystrophies, and degenerations . In: Albert DA, Jakobiec FA, eds. Principles and Practice of Ophthalmology . Philadelphia, Pa: WB Saunders Co; 1994;:chap 3.
13 +
Kempster RC, Hirst LW, de la Cruz Z, Green WR.  Clinicopathological study of the cornea in x-linked ichthyosis . Arch Ophthalmol . 1997;;115:409-415.
14 +
Teng CC.  Macular dystrophy of the cornea: a histochemical and electron microscopic study . Am J Ophthalmol . 1966;;62:436-454.
15 +
Bethell W, McCulloch C, Ghosh M.  Lecithin cholesterol acetyltransferase deficiency: light and electron microscopic findings from two corneas . Can J Ophthalmol . 1975;;10:494-501.
16 +
Nicholson DH, Green WR, Cross HE, Kenyon KR, Massof D.  A clinical and histopathological study of Francois-Neetens speckled corneal dystrophy . Am J Ophthalmol . 1977;;83:554-560.

Correspondence

Submit a Letter
CME Course for:


You need to register in order to view this quiz.


To understand the clinical management of acute heart failure syndromes.
Accreditation Information The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
To view and print your certificate and access a summary of your CME courses go to My CME.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Compression-Only CPR: Pushing the Science Forward
Cone
AAM 2010;304:1493-1495.
All you need to read in the other general journals
BMJ 2010;341:c5893-c1494.
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

© 2012 American Medical Association. All Rights Reserved.
Powered bySilverchair Information Systems