Ocular involvement in epidermolysis bullosa acquisita.

A 23-year-old man had epidermolysis bullosa acquisita that was diagnosed at the age of 20 years. The eye examination showed bilateral, small subepithelial vesicles in the cornea. The direct immunofluorescence microscopic examination of the conjunctiva revealed homogeneous, linear IgG and fibrinogen deposits in the basement membrane area. The same pattern appeared in the skin biopsy specimen. No systemic disease common to epidermolysis bullosa acquisita was found in the patient.

Complete occlusion of the anterior capsular opening after intact capsulorhexis: clinicopathologic correlation.

PURPOSE: To report histopathologic findings of capsule contraction syndrome with complete occlusion of the capsulorhexis opening. METHODS: Case report. In an 81-year-old woman, a complete occlusion of the anterior capsulorhexis opening developed 2 months after phacoemulsification and intraocular lens implantation. We surgically removed the contracted anterior capsule and analyzed the membrane by standard light microscopy and actin immunohistology. RESULTS: Light microscopic analysis of the membrane showed fibrous tissue subcapsularly with metaplastic lens epithelial cells. The contracted capsulorhexis opening was filled completely with proliferated actin-positive lens epithelial cells. CONCLUSIONS: Complete occlusion of the capsulorhexis opening can be attributed to excessive shrinkage of the capsule, probably caused by actin filaments found in the residual lens epithelial cells together with weak zonular support, and to the occlusion of the remaining central defect by massive proliferation of metaplastic lens epithelial cells.

A new, band-shaped and whorled microcystic dystrophy of the corneal epithelium.

Five family members and three unrelated patients (four women, four men, 23 to 71 years old) had a dystrophy of the corneal epithelium. Direct slit-lamp examination showed bilateral or unilateral, gray, band-shaped, and feathery opacities that sometimes appeared in whorled patterns. Retroillumination showed intraepithelial, densely crowded, clear microcysts. Light and electron microscopy disclosed diffuse vacuolization of the cytoplasm of epithelial cells in the affected area. Visual acuity was so reduced in three patients that abrasion of the corneal epithelium was performed. The corneal abnormalities recurred within months, with the same reduction in visual acuity as before. The corneal opacities were progressive in two patients but diminished noticeably in another after he began using a hard contact lens. We found no other ophthalmic irregularities or associated systemic abnormalities and no indication of drug-induced keratopathy.

Retrospective long-term follow-up of the triple procedure (combined keratoplasty and cataract surgery).

PURPOSE: Combined keratoplasty and cataract surgery has been performed at the University Eye Clinic in Tübingen since the nineteen-seventies. The present study was carried out to evaluate the long-term efficacy of this procedure. MATERIAL AND METHODS: Only grafts that had been followed for at least 4 years (6.5 +/- 2.3) were included. We studied the clinical outcome and final visual acuity of 19 patients (mean age 70 +/- 8.4 years) who had undergone "triple procedure surgery" on 23 eyes at the University Eye Clinic in Tübingen between 1983 and 1991. RESULTS: The keratoplasty was the first for 20 eyes and a regraft procedure in three. The mean diameter of the donor buttons was 7.31 +/- 0.4 mm, that of the host transplants 7.17 +/- 0.4 mm. Intra-operative complications included four vis a tergo reactions and one instance of bleeding in the anterior chamber. The most frequent postoperative complications were astigmatism (61%) and secondary glaucoma (4.3%). Postoperatively, six of the affected eyes required additional surgery. Preoperative visual acuity (0.06 +/- 0.08) improved postoperatively to 0.4 +/- 0.26, which was statistically significant. The postoperative retinal vision was 0.5 +/- 0.2. CONCLUSION: The results demonstrate the long-term success of the triple procedure. It can be especially recommended for patients in reduced general health who require rapid visual rehabilitation.

["Loose eye"]. / "Loses Auge". Diagnose: Akzidentelle Selbstenukleation (Avulsio bulbi) mit typischen Dissektionsebenen.

An extremely rare type of injury is presented: unintentional traumatic self-enucleation.

Visualization of Berger's space in the living eye.

The presence of the capsulo-hyaloidal interspace (Berger's space) can be demonstrated in the living human eye when this space is filled with Healon during extracapsular lens surgery combined with primary capsulotomy. This facilitates discission and excision procedures of the posterior capsule and protects the anterior vitreous face from injury.

[Clinical and ultrastructural classification of Reis-Bückler corneal dystrophy]. / Klinische und feingewebliche Abgrenzung der Reis-Bücklersschen Hornhautdystrophie.

Reis-Bücklers' corneal dystrophy has been incorrectly defined in the English literature: the corneal opacities described in most reports on it do not correspond to Bücklers' original findings, but are equivalent to Thiel and Behnke's honeycomb corneal dystrophy. Moreover, the synonym "annular dystrophy" is based on a misunderstanding and ought to be replaced by the term "maplike dystrophy". Perhaps due to the misnomer, annular or honeycomblike subepithelial opacities have come to be regarded as Reis-Bücklers' dystrophy. Subsequently, histologic and ultra-structural features were also evolved from such supposed cases, and the curly, electron-dense filaments were regarded as pathognomonic. The erroneous definition in the standard literature has been causing diagnostic confusion ever since. Research in the older literature and studies of the author's own patients have established the following original features of Reis-Bücklers' dystrophy: (1) dominant inheritance, (2) early manifestation and rapid progression, (3) painful attacks during childhood, (4) subepithelial corneal opacities extending almost to the limbus, (5) maplike opacity pattern, (6) sheetlike deposits replacing Bowman's membrane in histologic sections, (7) electron-dense rod-shaped bodies observed by electron microscopy. The original Reis-Bücklers' dystrophy resembles granular corneal dystrophy (Groenouw I) histochemically and ultrastructurally, but differs from it in its clinical symptoms, corneal opacity pattern, histopathological arrangement, and probably gene linkage as well. The condition commonly referred to as Reis-Bücklers' dystrophy in the literature is in fact Thiel and Behnke's honeycomb corneal dystrophy.

[Differential diagnosis of corneal dystrophies of the Groenouw I, Reis-Bückler and Thiel-Behnke type]. / Differentialdiagnose der Hornhautdystrophien vom Typ Groenouw I, Reis-Bücklers und Thiel-Behnke.

In the literature there are different definitions of Reis-Bücklers' corneal dystrophy. The corneal disease corresponding to the original description of Reis and Bücklers is interpreted by some authors as an atypical form of granular corneal dystrophy. On the other hand, the honeycomb-shaped corneal dystrophy of Thiel and Behnke is erroneously considered to be Reis-Bücklers' dystrophy. To establish the criteria to make a proper differential diagnosis, we studied the clinical, genetic, histological and electron-microscopical features of the three types of corneal dystrophy in 177 subjects with granular corneal dystrophy, 25 subjects with Reis-Bücklers' dystrophy, and 19 subjects with Thiel-Behnke's dystrophy. Granular corneal dystrophy shows typical corneal opacity patterns, and in our series these were divided into two family-specific variants. Histologically, there are amorphous granules under the epithelium (and possibly throughout the stroma) that consist of electron-dense rod-shaped bodies. The clinical symptoms in Reis-Bücklers' dystrophy (ocular pain in early childhood) differ from those in granular dystrophy. In addition, the corneal opacity pattern (maplike appearance) and the sheetlike arrangement of the deposits in a histological section are different, but the two types of dystrophy are similar with regard to histochemistry and electron microscopy. Thiel-Behnke's corneal dystrophy resembles Reis-Bücklers' dystrophy clinically, but differs from it in its honeycomb-shaped opacity pattern, the fibrous tissue in histology, and the curly dense filaments found in electron microscopy. Correct classification of the three corneal dystrophies is essential for precise diagnosis and may eliminate the present misunderstandings.

[Honeycomb-shaped corneal dystrophy of Thiel and Behnke. Reclassification and distinction from reis-Bücklers' corneal dystrophy]. / Die wabenförmige Hornhautdystrophie (Thiel-Behnke). Neubewertung und Abgrenzung gegenüber der Reis-Bücklers'schen Hornhautdystrophie.

BACKGROUND: The honeycomb-shaped dystrophy of Thiel and Behnke has been misunderstood for a long time and has erroneously been classified as Reis-Bücklers' dystrophy. The mistake originates in historical misunderstandings concerning the interpretation of the original Reis-Bücklers' dystrophy which results in a nonuniform nomenclature and a permanent confusion in the evaluation of the superficial stroma dystrophies. This paper intends to clarify the original identity of the honeycomb corneal dystrophy and to distinguish it from the Reis-Bücklers' dystrophy. PATIENTS AND METHODS: We examined seven patients of the original family of Thiel and Behnke. Five of them were newly detected, two of them were reexamined. The histological findings were reevaluated, and the diagnosis was proved by electron microscopic study of corneal specimens. RESULTS: The honeycomb dystrophy is characterized by: (1) dominant inheritance, (2) early manifestation, (3) slow progression, (4) painful erosions during childhood, (5) subepithelial corneal opacities with a clear limbal zone, (6) honeycomb-shaped opacity pattern, (7) recurrence in the graft following keratoplasty, (8) subepithelial fibrous tissue in wave-like accumulation in histologic sections, (9) curly filaments observed by electron microscopy. The clinical symptoms hardly differ from those of Reis-Bücklers' dystrophy. The histological appearance, however, is clearly distinct and curly filaments are the pathognomonic ultrastructural features. CONCLUSIONS: The honeycomb dystrophy represents a definite corneal disease. The terms "Reis-Bücklers' dystrophy" and "ring dystrophy" used for it up to now are wrong and should be eliminated in this context. The only correct term of the disease is honeycomb-shaped dystrophy (Thiel-Behnke) according to its first description and major clinical features. The eponym Reis-Bücklers' dystrophy should only be used for the corneal dystrophy described by Reis and Bücklers.

[Corneal opacity as the leading symptom of hereditary lecithin-cholesterol acyltransferase (LCAT) deficiency. Case report and a review of the literature]. / Hornhauttrübung als Leitsymptom des hereditären Lecithin-Cholesterin-Acyltransferase-(LCAT-) Mangels.

Familial lecithin: cholesterol acyltransferase (LCAT) deficiency is an inborn error of lipid metabolism clinically characterized by anemia, proteinuria, and corneal opacification. The authors review the literature dealing with 34 biochemically proven and 2 probable cases of LCAT deficiency, and describe the first case from a German-speaking country. Ocular findings were bilateral diffuse nebulous corneal opacification composed of innumerable minute grayish dots throughout the stroma. At the periphery of the cornea these dots increased, forming a ringlike band with indistinct margins. A small lipid deposit was also seen in the retina of the right eye. Vision was 20/20, but glare disability was significantly increased. Corneal opacification was also noticed in all cases of the literature. Anemia was detected at the time of diagnosis in 92% and proteinuria in 76% of the reviewed cases. Corneal opacification is the one absolutely obligatory clinical feature; moreover it is uniform and pathognomonic: a true indicating sign of LCAT deficiency.

[Keratoplasty à chaud as a therapeutic measure in acute corneal infections]. / Keratoplastik à chaud als therapeutische Massnahme bei akuten Hornhautinfektionen.

Over a period of 2 1/2 years penetrating keratoplasty was performed on 11 patients suffering from fulminating bacterial infection of the cornea at the florid stage, in 4 cases with perforation. Therapeutic keratoplasty is indicated for hypopyon ulcer and corneal abscess in 3 situations: (1) when the infected cornea perforates spontaneously; (2) when perforation threatens, even though the symptoms of the infection may be regressive; (3) when medication fails to change the course. Early operative intervention is to be recommended in each case. The aim of therapeutic keratoplasty is to eliminate the dangerous pathologic process in the cornea and to shorten the duration of the condition. This can be achieved with impressive speed. In some cases a primary result is attainable which is visually satisfactory and permanent, as was the case with 2 patients in the group described in this paper; as is to be expected, however, follow-up operations are often necessary and offer a second opportunity of restoring vision as well.

[Monitoring of vision development following early surgery of congenital cataracts using the Acuity-card method]. / Uberwachung der Sehentwicklung nach Frühoperation kongenitaler Katarakte mit der Acuity-card-Methode.

The recently developed acuity card procedure provides rapid assessment of visual acuity in preverbal infants and thus offers the possibility of monitoring the visual development of infants after early surgery for congenital cataract. This report describes our experience with these acuity cards in seven patients with bilateral and seven patients with unilateral congenital cataract. The age at operation was between 1 week and 17 months; visual acuity was assessed up to an age of maximally 2.5 years. In agreement with previous reports, the development of acuity in infants with unilateral aphakia was better if surgery had occurred within the first 6 months of life than at a later time. The results of acuity assessment were generally in good agreement with the clinical signs. The advantages of the acuity cards in clinical use are that: (1) they provide an additional criterion for surgery, including cases of secondary cataract formation; (2) they allow early assessment of the success of the operation; (3) they can be used to judge occlusion therapy; (4) they have proved to be highly motivating for parents to comply conscientiously with the therapeutic measures. Thus, the acuity card procedure has proved to be extremely useful for the assessment of visual development in infants operated on for congenital cataract.

[Posterior crystalline corneal dystrophy]. / Die posteriore kristalline Hornhautdystrophie.

A mother and daughter are described who have symmetrically arranged crystalline opacities in the area anterior to Descemet's membrane. These appear in the slit-lamp as symmetrically arranged, multicolored scintillating dots in the central part of the cornea. The daughter has, in addition, crystalline vitreal opacities. Microscopic examination of the endothelium revealed irregularly situated white spots in a circumscribed area anterior to the endothelium. None of the eyes showed any signs of inflammation. Metabolic tests yielded no signs of systematic metabolic disease.