Transplantation of endothelium and descemet membrane for aphakic corneal edema in 1980.

PURPOSE: To describe a corneal endothelial layer and Descemet membrane transplantation into the anterior chamber for the treatment of bullous keratopathy in 1980. METHODS: A retrospective report of an intervention case study is presented. A patient with aphakic bullous keratopathy in one eye was interested in undergoing a previously untested corneal transplantation procedure. Snellen visual acuity testing and slit-lamp examination were performed. The main outcome measures were visual acuity and degree of corneal clarity. RESULTS: Visual acuity and corneal clarity improved in the months following the endothelial/Descemet membrane transplant but gradually returned to preoperative levels 1 year later. A subsequent successful penetrating keratoplasty resulted in excellent visual acuity (-20/25). CONCLUSIONS: An attempt to perform endothelial and Descemet membrane transplantation for aphakic corneal edema in 1980 resulted in early improvement in corneal clarity and visual acuity but required full penetrating keratoplasty at 2 years postoperatively. [J Refract Surg. 2010;26(7):525-528.] doi:10.3928/1081597X-20090910-01

Keratoconus associated with corneal stromal amyloid deposition containing TGFBIp.

PURPOSE: To report the identification and characterization of stromal amyloid deposits in patients with keratoconus. METHODS: The excised corneal buttons from 2 patients diagnosed clinically with keratoconus underwent histochemical analysis with Masson trichrome, Congo red, Alcian blue, and periodic acid-Schiff stains, and immunohistochemical analysis for the transforming growth factor beta-induced gene (TGFBI) protein product (TGFBIp), prealbumin, lysozyme, and kappa and lambda light chain expression. After the collection of DNA from both patients, exons 4, 11, 12, 13 and 14 of TGFBI were amplified and sequenced to search for mutations previously associated with dystrophic corneal stromal amyloid deposition. RESULTS: Light microscopic examination of the corneal buttons revealed stromal thinning, epithelial basement membrane abnormalities, and focal disruption of Bowman layer. Multiple stromal deposits were identified that stained red with Masson trichrome, pink with periodic acid-Schiff, and red with Congo red; the Congo red-stained deposits demonstrated birefringence and dichroism with crossed polarizing lenses. Immunohistochemical staining demonstrated reactivity of the stromal deposits with antibodies to TGFBIp but no reactivity with antibodies against prealbumin, lysozyme, or kappa and lambda light chains. Screening of TGFBI exons 4, 11, 12, 13, and 14 revealed 2 previously identified single nucleotide polymorphisms present in the heterozygous state in both individuals but no other coding region variants. CONCLUSIONS: Two cases of keratoconus with clinically unsuspected, presumed secondary stromal amyloid deposition are described. Although TGFBIp is identified in the stromal deposits, no previously reported amyloidogenic mutations are identified in TGFBI in either affected individual, indicating a previously undescribed mechanism of stromal amyloid deposition.