Hereditary Deep Dystrophy of the Cornea (Polymorphous)

Hogan, Michael J.; Bietti, G. B.

doi:10.1016/0002-9394(69)94569-3

Cited by 32 publications

(7 citation statements)

References 6 publications

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“…various entities-are being classified in a single broad category. For example, the report by Hogan and Bietti (1969) showing warts characteristic of cornea guttata and calcium nodules in the deep stroma differs so strikingly from the other reported cases of PPD that it may represent an unrelated endothelial dystrophy. The remaining cases reporting the ultrastructural findings (Boruchoff and Kuwabara, 1971;Hanselmayer, 1972;Grayson, 1974;Bron and Tripathi, 1974;Rodrigues et al, 1975), including the present study, show similar changes in regard to Descemet's membrane but vary in the findings involving the endothelial layer.…”

Section: Histopathological Findings Light Microscopymentioning

confidence: 60%

“…As the condition is rare, usually non-progressive, and commonly causes minimal visual impairment, few cases have necessitated keratoplasty. Three light microscopic studies (Morgan and Patterson, 1967;Hanselmayer, 1972a;Malbran, 1972) and 6 ultrastructural studies (Hogan and Bietti, 1969;Boruchoff and Kuwabara, 1971;Hanselmayer, 1972b;Grayson, 1974;Bron and Tripathi, 1974;Rodrigues et al, 1975) of these specimens have resulted in a variety of findings, and the term 'polymorphous' seems as applicable microscopically as it is clinically. A recent study (Rodrigues et al, 1975) suggested that the characteristic ultrastructural finding in PPD is multilayers of epithelial cells on the back of the cornea.…”

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confidence: 99%

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Posterior polymorphous dystrophy: a light and electron microscopic study.

Johnson¹,

Brown²

1978

British Journal of Ophthalmology

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SUMMARY Microscopic study of a keratoplasty specimen in a case of posterior polymorphous dystrophy demonstrated via serial sections a multilaminar Descemet's membrane with focal fusiform nodular protrusions of connective tissue. The latter are thought to represent the polymorphous lesions of the posterior limiting layers of the cornea seen clinically. Ultrastructurally, fibroblast-like cells lined the posterior surface of the cornea. It is postulated that those reported cases of PPD in which epithelial-like transformation of the endothelial layer has been described may represent more advanced cases, in which corneal oedema is likely to be a prominent feature clinic,ally. An evolving process of metaplasia rather than a static one might explain the variety of altered endothelial cells, fibroblasts, and epithelial cells which have been reported on the posterior cornea in the few PPD keratoplasty specimens studied to date.Posterior polymorphous dystrophy (PPD) is a bilateral, congenital, usually dominantly inherited disorder of the posterior limiting layers of the cornea which is characterised clinically by discrete geographic nodular or vesicular lesions at the level of Descemet's membrane that protrude towards the anterior chamber. As the condition is rare, usually non-progressive, and commonly causes minimal visual impairment, few cases have necessitated keratoplasty. Three light microscopic studies (Morgan and Patterson, 1967;Hanselmayer, 1972a;Malbran, 1972) and 6 ultrastructural studies (Hogan and Bietti, 1969;Boruchoff and Kuwabara, 1971;Hanselmayer, 1972b;Grayson, 1974;Bron and Tripathi, 1974;Rodrigues et al., 1975) of these specimens have resulted in a variety of findings, and the term 'polymorphous' seems as applicable microscopically as it is clinically. A recent study (Rodrigues et al., 1975) suggested that the characteristic ultrastructural finding in PPD is multilayers of epithelial cells on the back of the cornea. We wish to report the morphological findings in a case of PPD that lacked epithelial cells on the posterior cornea, and compare the pathology in this case with that in previously reported cases.

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Section: Histopathological Findings Light Microscopymentioning

confidence: 60%

mentioning

confidence: 99%

Posterior polymorphous dystrophy: a light and electron microscopic study.

Johnson¹,

Brown²

1978

British Journal of Ophthalmology

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“…We think that inflammation is the cause of the acquired type of posterior polymorphous keratopathy, but a metabolic disorder as suggested by Hogan and Bietti (1969) cannot be excluded. The condition is regarded by many as nonprogressive.…”

Section: Discussionmentioning

confidence: 89%

“…Thus Morgan and Patterson (1967) proposed that the excrescences from the Descemet's membrane are formed by the endothelium as a variant of Hessel-Henle bodies. Hogan and Bietti (1969) detected calcium crystals on a corneal button with polymorphous changes, and regarded the condition as a sequel to a metabolic disorder. Boruchoff and Kuwabara (1971) presenting their case, reported the presence of extensive peripheral synechiae obliterating the trabeculum, and suggested that the condition is a type of mesodermal dysplasia.…”

Section: Discussionmentioning

confidence: 99%

Posterior polymorphous keratopathy.

Liakos¹,

Casey²

1978

British Journal of Ophthalmology

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SUMMARY Seven cases with posterior polymorphous changes of the cornea are reported. After clinical and pathological examination of the above cases, as well as a short review of the literature, the following points are made: (1) Some cases are congenital, being either familial or sporadic, but others are acquired. (2) The term 'posterior polymorphous keratopathy' covers all the variants of the condition and is preferred to the traditional 'posterior polymorphous dystrophy'. (3) The congenital type is a mild variant of the mesodermal dysplasia, whereas the acquired type follows local disease. (4) The condition can be static, but over 50% of cases are slowly progressive, calling for penetrating keratoplasty.The condition of posterior polymorphous keratopathy is rare, and only a few cases have been reported. General conclusions on the heredity, the histological picture, and the course of the disease cannot be drawn, mainly because the number of cases reported each time is insufficient. Here we present 7 cases of posterior polymorphous dystrophy seen in our clinic over a period of 6 years. The family history, clinical associations, and, where possible, the histology are analysed. Case reports CASE 1A man aged 45 was first diagnosed as having a corneal dystrophy in 1960 when he was 32 years old. This progressed until 1969, when he was referred to our unit. Slit-lamp examination revealed bullous keratopathy in the right eye, with deeper layer changes consisting of whorl-like opacities, vesicles, irregularities, and folds. Owing to corneal oedema the gonioscopic view of the anterior chamber angle was limited; however, in a few places goniosynechiae were evident. In the left eye the anterior corneal layers were normal, but posterior polymorphous changes were easily seen. A band of peripheral anterior synechiae was seen between 4 and 6 o'clock and confirmed by gonioscopy (Fig. 1). Visual acuity was recorded as 6/60 in the right eye and 6/9 in the left. A full-thickness corneal graft restored visual acuity of 6/6 for 2 years but was finally rejected, and 2 subsequent grafts had the same fate.

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“…Posterior polymorphous corneal dystrophy (PPCD) is a very rare, bilateral autosomal dominant disorder affecting primarily the innermost corneal layers, the descemet membrane (DM) and the endothelium, biomicroscopically presenting as geographical lesions, bands and vesicles (Hogan and Bietti 1969;Jirsova et al 2009;Krachmer 1985). Moreover, changes in the posterior stroma and basal membrane of the epithelium were also described in some patients ).…”

Section: Introductionmentioning

confidence: 99%

Recurrence of posterior polymorphous corneal dystrophy is caused by the overgrowth of the original diseased host endothelium

Merjava

Malinova

Lišková

et al. 2011

Histochem Cell Biol

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Posterior polymorphous corneal dystrophy (PPCD) is a rare, bilateral autosomal dominant disorder affecting primarily the corneal endothelium and descemet membrane (DM). The aim of this study was to establish the origin of abnormal endothelium in a patient with PPCD exhibiting cornea graft failure after keratoplasty surgery. A sex-mismatched graft obtained from a patient with PPCD who underwent repeat penetrating keratoplasty and the patient's original cornea were investigated. Combined fluorescent immunohistochemistry for cytokeratin (CK) 19 (a marker of aberrant PPCD endothelium) with fluorescence in situ hybridization (FISH) of the sex chromosomes were used in order to characterize the cells on the posterior graft surface. The pathological endothelium of the failed PPCD cornea revealed strong positivity for CK19 using fluorescent immunohistochemistry. In all the CK19-positive cells, both X and Y chromosomes were simultaneously detected using FISH. The results clearly showed the original cells of the patient (XY), within 3.5 years, almost totally overgrown the posterior corneal surface of the graft (XX). Moreover, an abnormal posterior collagenous layer populated by fibroblast-like cells was observed between DM and the endothelium in the failed graft, but its exact origin could not be established due to the low number of cells. Simultaneous detection of CK19 using fluorescent immunohistochemistry together with the detection of gonosomes using FISH was performed for the first time in the cornea and allowed us to prove that the recurrence of PPCD was caused by pathological abnormal proliferation and migration of recipient cells into donor graft.

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Hereditary Deep Dystrophy of the Cornea (Polymorphous)

Cited by 32 publications

References 6 publications

Posterior polymorphous dystrophy: a light and electron microscopic study.

Posterior polymorphous dystrophy: a light and electron microscopic study.

Posterior polymorphous keratopathy.

Recurrence of posterior polymorphous corneal dystrophy is caused by the overgrowth of the original diseased host endothelium

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