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November 1978

Corneal Endothelial DystrophyA Study of 64 Families

Author Affiliations

From the Department of Ophthalmology, Iowa Lions Cornea Center, University Hospitals, Iowa City (Drs Krachmer, Purcell, and Young), and the Department of Preventive Medicine, University of Iowa, Iowa City (Dr Bucher). Dr Purcell is now with St Louis University School of Medicine and Dr Young is in private practice in Presque Isle, Me.

Arch Ophthalmol. 1978;96(11):2036-2039. doi:10.1001/archopht.1978.03910060424004

• A prospective study was undertaken during an 18-month period with 64 families who had endothelial dystrophy. Two hundred twenty-eight relatives were examined. Of those older than the age of 40, 38% were affected. Women were affected more severely and 2.5 times more frequently than men. The disease showed a strong familial tendency: there was one family in which three generations were affected and 16 families in which two generations were affected. There were four families that had members with edema in two generations. There was no association between edema in a parent and edema in a child. The proportion of relatives affected and the severity of involvement increased with age. Fiftythree probands and 18 relatives had endothelial dystrophy with edema (Fuchs' dystrophy). Of these 71, one had glaucoma.

Cibis GW, Krachmer JA, Phelps CD, et al:  The clinical spectrum of posterior polymorphous dystrophy . Arch Ophthalmol 95:1529-1537, 1977.Article
Grayson M:  The nature of hereditary deep polymorphous dystrophy of the cornea: Its association with iris and anterior chamber dysgenesis . Trans Am Ophthalmol Soc 72:516-559, 1974.
Maumenee AE:  Congenital hereditary endothelial dystrophy . Am J Ophthalmol 50:1114-1124, 1960.
Mishima S, Hedbys BO:  Measurement of corneal thickness with the Haag-Streit pachometer . Arch Ophthalmol 80:710-713, 1968.Article
Fuchs E:  Dystrophia epithelialis corneae . Albrecht von Graefes Arch Klin Ophthalmol 76:478-508, 1910.Article
Koeppe L:  Klinische beobachtungen mit der nernstspaltlampe und dem hornhautmikroskop . Albrecht von Graefes Arch Klin Ophthalmol 91:363-379, 1916.Article
Stähli J:  Uber eine besondere form von betauung der kornealrückläche . Klin Monatsbl Augenheilkd 65:106-107, 1920.
Vogt A:  Weitere ergebnisse der spaltlampenmikroskopie des vordern bulbusabschnittes . Albrecht von Graefes Arch Klin Ophthalmol 106:63-103, 1921.Article
Kraupa E:  Pigmentierung der hornhauthinterfläche bei 'Dystrophia epithelialis (Fuchs).' Z Augenheilkd 44:247-250, 1920.
Moeschler H:  Untersuchungen ueber pigmentierung der hornhautrueckflaeche bei 395 am spaltlampenmikroskop untersuchten augen gesunder personen . Z Augenheilkd 48:195-202, 1922.
Goar EL:  Dystrophy of the corneal endothelium (cornea guttata) . Am J Ophthalmol 17:215-221, 1934.
Lorenzetti DWC, Uotila MH, Parikh N, et al:  Central cornea guttata . Am J Ophthalmol 64:1155-1158, 1967.
Becker B, Shaffer RN: Diagnosis and Therapy of the Glaucomas , ed 2. St Louis, CV Mosby Co, 1965, p 214.