[Orogenital and conjunctival involvement in hereditary and autoimmune blistering diseases]. / Schleimhautbeteiligung bei blasenbildenden Erkrankungen.

Chronic involvement of orogenital and conjunctival mucosa in the course of either genetically based (epidermolysis bullosa hereditaria) or auto-immunologically mediated (as for example pemphigus vulgaris, mucous membrane pemphigoid or epidermolysis bullosa acquisita) blistering diseases can cause significant morbidity. To provide accurate care, recognition of clinical, pathogenic and diagnostic features as well as awareness of recent advances in the development of new therapeutic modalities are mandatory and thus will be discussed in this review.

Ocular involvement in anti-epiligrin cicatricial pemphigoid.

PURPOSE: To report an anti-epiligrin cicatricial pemphigoid (AECP) patient with severe ocular involvement and to provide a practical approach to distinguishing AECP patients from those with other subepidermal blistering diseases. METHODS: Techniques included direct and indirect immunofluorescence microscopy, Western blot and immunoprecipitation studies, as well as interdisciplinary examinations of mucous membranes and skin. RESULTS: This study describes a patient with clinical features of cicatricial pemphigoid, circulating anti-basement membrane zone IgG antibodies, and subepidermal blisters. Histopathology and immunofluorescence analysis suggested the diagnosis of a cicatricial pemphigoid-like type of epidermolysis bullosa acquisita. However, Western blot and immunoprecipitation studies demonstrated that the patient's serum contained autoantibodies against laminin 5 alpha3 subunit, leading to the diagnosis of an AECP. CONCLUSION: Since patients with AECP have an increased relative risk for malignant tumors, it is important to distinguish this entity within the spectrum of cicatricial pemphigoid patients by additional studies such as Western blot or immunoprecipitation.

[Clinical management of severe ocular surface disease]. / Visusrehabilitation bei schwersten Veränderungen der Augenoberfläche.

Severe ocular surface diseases, such as Stevens-Johnson syndrome, ocular cicatricial pemphigoid or severe ocular burns may result in a significant loss of corneal stem cells, eventually leading to vision impairment or even corneal blindness. In case of unilateral involvement, limbal autografting, by means of transplanting limbal stem cells from the healthy fellow eye, has proved to be an effective procedure for restoring the integrity of the ocular surface. Limbal allografts may be performed in patients with bilateral disease, however, systemic immunosuppression is mandatory in these cases, with a long-term outcome that is frequently reduced compared to limbal autografts due to acute or chronic graft rejection. In recent years, amniotic membrane transplantation has been successfully employed as an additional tool in ocular surface reconstruction. The AlphaCor synthetic cornea, which is made of flexible acrylic may be considered as an alternative in patients with repeated corneal graft failures. Both limbal transplantation and the AlphaCor have been shown to be effective in eyes with an adequate tear film, but are most likely to fail in severe dry eyes or in patients with cicatrising diseases. Such conditions are the domain of keratoprostheses (KPros) with rigid optics, which certainly can be considered as the 'last resort' to restore vision in patients with profound corneal blindness not amenable to conventional corneal and limbal grafting. The osteo-odonto-keratoprosthesis according to Strampelli and modified by Falcinelli makes use of a "biological" support consisting of a longitudinal section of one of the patient's teeth that is also supported by the surrounding alveolar bone tissue. Compared to other devices favourable long-term results have been reported. In patients lacking any usable teeth, implantation of a keratoprosthesis with haptics made of Dacron (Pintucci-KPro) or tibial bone (Temprano-KPro) might be considered.

[Amniotic membrane transplantation with limbal stem cell transplantation as a combined procedure for corneal surface reconstruction after severe thermal or chemical burns]. / Amnion-Limbus-Transplantation zur Oberflächenrekonstruktion nach schwerer Verätzung und Verbrennung.

BACKGROUND: Severe thermal and chemical burns may result in limbal deficiency leading to persistent epithelial defects, complete conjunctival epithelial ingrowth and vascularisation of the cornea. If sufficiently severe, these burns may lead to very significant visual impairment. Amniotic membrane transplantation with limbal transplantation has recently been proposed as a new method for corneal surface reconstruction. PATIENTS AND METHODS: A total of 14 patients (age 18-62 years, mean age 42 years) with limbal deficiency resulting from thermal ( n=1) or chemical burns ( n=13) underwent surgery. The corneal pannus was completely removed and the amniotic membrane was grafted onto the cornea. Limbal transplantation using autografts obtained from contralateral eyes was performed simultaneously in seven cases. Allografts from a donor were transplanted in seven cases with bilateral involvement. These patients received oral cyclosporin A postoperatively. The mean follow-up time was 18 months. RESULTS: In all cases of limbal autografts the corneal surface showed a complete and stable epithelialisation within a few weeks. Out of seven patients with limbal allografts three displayed recurrent epithelial defects in the long term. The initially semitransparent amniotic membrane became more translucent and biomicroscopically invisible within several months after surgery. There was an increase in visual acuity in most cases, limited mostly by irregular astigmatism due to the initial stromal loss. CONCLUSIONS: Amniotic membrane transplantation with limbal transplantation allows reconstruction ocular surfaces severely damaged by chemical or thermal burns. In most cases, however, additional surgical procedures such as lamellar or penetrating keratoplasty are required for adequate visual rehabilitation.

Evaluation of donor tissue with a new videokeratoscope: the Keratron Scout.

PURPOSE: To evaluate the percentage of corneas with an epithelial surface quality sufficient for a reliable screening of donor eyes with keratoconus and after photorefractive keratectomy (PRK) or laser in situ keratomileusis (LASIK) treatment for refractive errors. The usefulness of the computer-assisted, video-keratography system Keratron Scout in making this determination was evaluated. METHODS: Forty eyes from 20 donors were screened within 30 minutes after enucleation and within 24 hours postmortem. At first a meticulous slit-lamp evaluation of the corneal surface and stroma was performed; thereafter eight pictures per eye were taken with the Keratron Scout, and an ultrasound pachymetry reading of the corneal center was taken. "True curvature," algorithm-based color maps were used for analysis. Epithelium was not removed, because all corneas were suitable for transplantation. Balanced salt solution was used to keep the corneas moist. The low intraocular pressure regularly found in postmortem eyes was raised to within normal ranges by intravitreal injection. Corneas were classified according to the integrity of their epithelial surface, which influences the regularity of the topography maps. RESULTS: The quality of the images acquired varied, depending mostly on the quality of the epithelium. Regular topography patterns were found in 15 eyes (37.5%); in 14 eyes (35%), minimal epithelial roughness or sloughing in just one quadrant of the cornea was detected. Moderate to severe epithelial irregularities or defects were found in nine eyes (22.5%). Screening for abnormal shape was possible in 90% of the tested eyes, where no changes caused by prior refractive surgery or keratoconus were apparent. CONCLUSION: The postmortem quality of the epithelial surface allows screening for keratoconus or refractive procedures in the majority of donor eyes. Video-keratography with the Keratron Scout seems to offer a sensitive and quick method for this purpose, thereby adding an additional safety feature to eye banking in view of the rapid development of corneal refractive surgery.

Eurosol versus fetal bovine serum-containing corneal storage medium.

PURPOSE: To evaluate the usability of Eurosol, a new medium-term corneal storage medium without components of bovine origin. METHODS: Ten pairs of human donor corneas were placed in tissue culture at 31 degrees C for 7, 14, 21, 28, or 35 days. One cornea of each pair was cultivated in conventional storage medium on Earls' minimum essential medium base containing 2% fetal bovine serum; the other one was stored in Eurosol. Corneas were examined with inverse light microscopy; corneal thickness was measured; and scanning electron microscopy was performed. RESULTS: No significant difference in corneal thickness and endothelial cell count was found at any time. Scanning electron microscopy showed a complete endothelial cell layer on all corneas. CONCLUSION. The findings indicate a potential clinical applicability of the tested serum-free medium-term storage medium, offering a safer alternative to conventional media containing fetal bovine serum.

One year results of European Multicenter Study of intrastromal corneal ring segments. Part 1: refractive outcomes.

PURPOSE: To assess the efficacy, predictability, stability, and safety of correcting myopia with intrastromal corneal ring segments (ICRS, KeraVision, Inc.). SETTING: Twelve European investigational sites. METHODS: Patients with myopia of -1.0 to -6.0 diopters (D) and best spectacle-corrected visual acuity (BSCVA) of 20/20 or better (except in 3 eyes) were assigned to receive 1 of 5 ICRS thicknesses (0.25, 0.30, 0.35, 0.40, or 0.45 mm). Efficacy was assessed by postoperative uncorrected visual acuity (UCVA), predictability of refractive outcome (deviation from predicted cycloplegic refraction spherical equivalent), and stability of refractive effect (manifest refraction spherical equivalent [MRSE]). Safety was assessed by maintenance or loss of preoperative BSCVA and induced manifest refraction cylinder. Measurements were made preoperatively and 1 and 7 days and 1, 2, 3, 6, and 12 months postoperatively. RESULTS: One hundred fifty-nine ICRSs were implanted in the eyes of 107 patients (52 patients had bilateral implantation). Preoperatively, UCVA was worse than 20/40 in 98% of eyes (155/159); 12 months postoperatively, it was 20/20 or better in 63% of eyes (83/132) and 20/40 or better in 96% (127/132). Predicted refractive corrections for each ICRS thickness generally correlated with achieved corrections. At 12 months postoperatively, 90% of eyes (124/138) were within +/-1.00 D of plano (MRSE). Two or more lines of BSCVA were lost in 6% of eyes (8/135); all 8 eyes had BSCVAs of 20/25 or better. CONCLUSION: The ICRS safely, effectively, and predictably reduced or eliminated low to moderate myopia. Refractive correction was stable through the 12 month follow-up.

One year results of European Multicenter Study of intrastromal corneal ring segments. Part 2: complications, visual symptoms, and patient satisfaction.

PURPOSE: To assess intraoperative and postoperative complications, visual symptoms, and patient satisfaction after implantation of intrastromal corneal ring segments (ICRS, KeraVision, Inc.) for the correction of myopia and to demonstrate the reversibility and adjustability of refractive corrections with this method. SETTING: Twelve European investigational sites. METHODS: Patients with myopia of -1.0 to -6.0 diopters (D) were assigned to receive 1 of 5 ICRS thicknesses (0.25, 0.30, 0.35, 0.40, or 0.45 mm). Complications and visual symptoms were noted, and patient satisfaction was assessed at each postoperative visit (1 and 7 days and 1, 2, 3, 6, and 12 months). Refractive data were assessed after removal or exchange of the ICRS. RESULTS: Of 163 eyes of 110 patients enrolled, 159 eyes of 107 patients were implanted with an ICRS (52 patients had bilateral implantation). Intraoperative complications occurred in 2% of eyes (4/163 eyes in 3 patients) that were withdrawn from the study: 3 eyes had anterior surface perforations and 1 had a posterior microperforation into the anterior chamber. Most patients had no visual symptoms at 12 months; symptoms usually occurred rarely or sometimes and were mild in severity. At 12 months, good or excellent patient satisfaction was reported for 94% of eyes. Twelve ICRSs (8%) were removed, mainly because of undercorrection and induced astigmatism, and 2 were exchanged. All eyes were within +/-1.00 D of the preoperative manifest refraction spherical equivalent. CONCLUSIONS: The ICRS was safe for correction of low to moderate myopia. Severe postoperative visual symptoms were rare, and patient satisfaction was high. The refractive correction was largely reversible.

Recurrent keratoconus in a patient with Leber congenital amaurosis.

PURPOSE: Clinical history of a 17-year-old patient with Leber congenital amaurosis (LCA) with histologically proven recurrent keratoconus (KC) two years after corneal transplantation in one eye and a recurrence-like appearance with a more global contour on the other eye four years after corneal grafting is reported. The possible mechanisms for this recurrence are discussed in light of the fact that this is, to the best of our knowledge, the first penetrating keratoplasty reported in LCA. METHODS: Computerized videokeratography (CVKG) and specular microscopy were performed preoperatively. The patient underwent regrafting, and the excised corneal button was examined by light microscopy and transmission electron microscopy. RESULTS: Analysis of CVKG showed a keratoconus-like pattern on the right eye, with the left eye demonstrating the aspects usually seen in keratoglobus. Histologic examination revealed the features usually observed in progressed keratoconus. CONCLUSION: Recurrence of keratoconus in a graft has not yet been described after such a short time until now. A "true" recurrence of the disease is postulated; it could be caused by an "aggressive" genetic factor that also leads to the frequent KC in patients with LCA. This mechanism also could explain the high incidence and rapid progress of KC in this disease.

[Intrastromal corneal ring segments (ICRS, KeraVision Ring, Intacs): clinical outcome after 2 years]. / Intrastromale Corneale Ringsegmente (ICRS, KeraVision Ring, Intacs): Klinische Ergebnisse nach 2 Jahren.

BACKGROUND: Since 1996 Intrastromal Corneal Ring Segments (Intacs, KeraVision, Inc., Fremont, CA, USA) have been used for the correction of mild to moderate myopia at the Salzburg Eye Clinic. Aim of this study was to evaluate the stability, reversibility and adjustability of this new method. PATIENTS AND METHODS: Our experiences and final results of 54 surgeries--30 eyes with a minimum follow-up of 2 years--, as well as the potential reversibility (3 explanations, 2 of them followed by PRK) and adjustability (3 exchanges) are reported in detail. RESULTS: None of the eyes lost more than one line in BSCVA (in 14 of 30 eyes the BSCVA improved, "Safety-Index": 1.13). 73% (22/30) reached an UCVA of 1.0 or better, 47% (14/30) of 1.25 or better ("Efficacy-Index": 0.79). After two years 47% (14/30) were within +/- 0.5 D of the attempted correction. We observed no significant intra- or postoperative complications. After ICRS removal the refractive data returned to within +/- 0.75 D (MRSE) and +/- 0.5 D (mean keratometry) of preoperative values, respectively. Patients with an ICRS exchange obtained an improved UCVA between 0.8 and 1.0, gaining between 2 to 4 lines. CONCLUSIONS: Within the follow-up period of 2 years the ICRS seem to provide a very stable correction of low to moderate myopia. The procedure is reversible to a large extent, potentially adjustable (within certain limits) and carries a minimal risk only. After explantation PRK can be performed with good visual results.