[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
[Skip to Content Landing]
April 1983

Posterior Keratoconus Associated With Systemic Abnormalities

Author Affiliations

From the Departments of Ophthalmology (Drs Streeten and Spitzer) and Pathology (Drs Streeten and Karpik), State University Hospital, Upstate Medical Center, Syracuse, NY. Dr Karpik is now with Rush-Presbyterian-St Luke's Medical Center, Chicago.

Arch Ophthalmol. 1983;101(4):616-622. doi:10.1001/archopht.1983.01040010616019

• A 16-year-old girl with bilateral congenital posterior keratoconus of the localized type had corneal transplantation performed on one eye. Around the scarred central corneal cone there was a ring of Descemet's membrane with large knoblike excrescences. A small anterior subcapsular cataract was also present in this eye. The pattern of corneal scarring, as seen by electron microscopy, suggested an iridocorneal or iridocorneolenticular adhesion present at the 8- to 20-mm embryonic stage. Associated systemic defects, which could be dated to the same period, were median facial clefting and severe genitourinary abnormalities. Several external features, such as short stature, abnormal gait, broad nose, and upward displacement of the lateral canthi, were similar to those in the Haney-Falls syndrome of familial posterior keratoconus. No appreciable chromosomal abnormalities were found.

Butler TH:  Keratoconus posticus . Trans Ophthalmol Soc UK 1930; 50:551-556.
Goldsmith AJB:  Bilateral circumscribed posterior conical cornea . Trans Ophthalmol Soc UK1943 ;63:180-181.
Jacobs HB:  Posterior conical cornea . Br J Ophthalmol 1957;41:31-39.Article
Haney WR, Falls HF:  The occurrence of congenital keratoconus posticus circumscriptas: In two siblings presenting a previously unrecognized syndrome . Am J Ophthalmol 1961;52:53-57.
Karlin DB, Wise GN:  Keratoconus posticus . Am J Ophthalmol 1961;52:119-121.
Collier M:  Le kératocone postérieur . Arch Ophthalmol 1962;22:376-391.
Kratchmer JH, Rodrigues MM:  Posterior keratoconus . Arch Ophthalmol 1978;96:1868-1873.
Wolter JR, Haney WP:  Histopathology of keratoconus posticus circumscriptus . Arch Ophthalmol 1963;69:357-362.Article
Migeon BR:  Short arm deletions in group E and chromosomal 'deletion' syndromes . J Pediatr 1966;69:432-438.Article
Mann I: The Development of the Human Eye , ed 3. London, British Medical Association, 1964, p 37.
Young ID, Macrae WG, Hughes HL, et al: Keratoconus posticus circumscriptus, cleft lip and palate, mental retardation, short stature, and genitourinary abnormalities in siblings. J Med Genet, in press.
Kenyon KR:  Mesenchymal dysgenesis in Peter's anomaly, sclerocornea and congenital endothelial dystrophy . Exp Eye Res 1975;21:125-142.Article
Alexander RA, Grierson I, Garner A:  Oxytalan fibers in Fuchs' endothelial dystrophy . Arch Ophthalmol 1981;99:1622-1627.Article
Greene PB:  Keratoconus posticus circumscriptus: Report of a case . Arch Ophthalmol 1945;34:432-433.Article
Johnston MC, Noden DM, Hazelton RD, et al:  Origins of avian ocular and periocular tissues . Exp Eye Res 1979;29:27-43.Article
Le Lièvre CS, Le Douarin NM:  Mesenchymal derivatives of the neural crest: Analysis of chimaeric quail and chick embryos . J Embryol Exp Morphol 1975;34:125-154.
Kissel P, André JM, Jacquier A: The Neurocristopathies . New York, Masson Publishing USA Inc, 1981, p 189.