Reis-Bücklers dystrophy.
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Bowman Membrane↗
Biomedical subjects
E G Weidle
Publications and source records attributed to E G Weidle.
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.
Actins↗
[Honeycomb-shaped corneal dystrophy of Thiel and Behnke. Reclassification and distinction from reis-Bücklers' corneal dystrophy].
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.
Adolescent↗
[Rifabutin-induced hypopyon iritis in HIV infection].
BACKGROUND: Rifabutin is a new semisynthetic rifamycin which is approved in Germany for clinical application since 1/15/95. The drug is used for prophylaxis and treatment of Mycobacterium avium intracellulare (MAI) infection in patients with AIDS. In 1994 it was reported that rifabutin can cause anterior uveitis. This side effect is dose-dependent and aggravated, when the drug is combined with clarithromycin and/or fluconazol. PATIENT: A 32-year-old woman suffered from acquired immunodeficiency syndrome and systemic MAI. She was treated with a combination of rifabutin (450 mg/day), clarithromycin (750 mg/day) and ethambutol (800 mg/day). 78 days later she developed a unilateral hypopyon iritis. RESULTS: Rifabutin was discontinued and topical steroids and mydriatics were given. The uveitis disappeared within two weeks. CONCLUSIONS: The ophthalmologist should be aware of this new potential etiology of anterior uveitis. Rifabutin should be withdrawn immediately. Anti-inflammatory eye drops might be helpful.
AIDS-Related Opportunistic Infections↗
Traumatic wound dehiscence after penetrating keratoplasty.
BACKGROUND: Traumatic wound dehiscence after penetrating keratoplasty is probably underestimated. PATIENTS: From the files of the University Eye Hospital Tübingen (1981-1993), 15 patients with a traumatic wound dehiscence after penetrating keratoplasty were investigated. RESULTS: Latency between corneal grafting and wound rupture ranged from 1 month to 25 years (mean 6.2 years). Wound dehiscence was quite evenly distributed over the circle. Intraocular tissue was lost to a variable extent. Three eyes were primarily or secondarily enucleated. Seven eyes (47%) with an originally reduced but useful vision became blind or were removed. Most (67%) of the resutured grafts lost transparency. CONCLUSIONS: Traumatic wound dehiscence is a serious and not very rare complication after penetrating keratoplasty. Functional results are poor.
Adult↗
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.
Aged↗
[Epithelial and stroma corneal dystrophies].
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Cornea↗
[Congenital familial cornea plana with ptosis, peripheral sclerocornea and conjunctival xerosis].
BACKGROUND: Cornea plana is an extremely rare, congenital hereditary malformation of the corneo-scleral shape. The curvatures of cornea and sclera are nearly equal with an indistinct limbus. In addition to the flatness, there is a peripheral sclerocornea that produces a pseudomicrocornea. The low corneal refraction and the short anterior segment often result in hyperopia. Myopia is also described. Usually the posterior segment is not involved. MATERIALS AND METHODS: A young man of 22 years, his three- and five-year-old sons, and his newborn daughter showed this hereditary abnormality of the cornea. In this uncommon anomaly we measured corneal curvature, refraction, diameter and in three of the four patients echographical length of the bulbi. RESULTS: The family showed an autosomal dominant inheritance of the cornea plana. The corneal refraction was less than 32 diopters. The scleral encroachment caused an oval cornea measuring horizontally between 5 and 6.5 mm, vertically 4 to 5 mm. Additionally a pseudoblepharoptosis and a conjunctival xerosis of the father and his sons was observed, which is not regularly found. A-scan measuring of the bulbi revealed age-related normal values. CONCLUSIONS: There is no evidence for progression of this anomaly during life. No therapeutical consequences are necessary. To preserve a satisfactory function a conscientious orthoptical maintainance should be guaranteed.
Adult↗
[Corneal manifestations in Vitamin A deficiency].
BACKGROUND: Vitamin A, the fat soluble vitamin, must be supplied orally before resorption in the mucosa of the small intestine and storage in the liver. Vitamin A deficiency can cause alterations in the anterior segment of the eye, from Bitot spots, which are reversible, to irreversible keratomalacia. PATIENTS AND METHODS: 5 patients suffering from manifestations at the cornea underwent ophthalmological, general and dermatological investigation and measurements of the vitamin A, retinol-binding protein and, in one patient, zinc were performed. 4 patients suffered from cornea manifestations, reaching from almost a reactive ulceration to spontaneous perforation. One patient had relapsing episcleritis. All patients were alcoholics, had hepatopathies and dermatological diseases. Vitamin A and retinol-binding protein were decreased and in one patient a highly decreased zinc was measured. In one patient a systemical and local substitution of vitamin A increased the clinical findings significantly. Two eyes had to undergo a keratoplasty à chaud, one evisceration had to be done. CONCLUSION: Vitamin A deficiency can be the reason for bilateral painless cornea manifestations. An interdisciplinary cooperation is essential for the elaboration of the diagnosis and the treatment.
Adult↗
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.
Adult↗
[Surgical treatment of hyperplastic persistent pupillary membrane].
Clinical findings of two patients with persisting pupillary membranes are described and therapy is discussed. The indication for excision of the pupillary membrane was constituted in one patient (age 6 weeks) by complete occlusion of the optic axis, in the second (age 4 months) by absence of any recordable fixation. In both cases the pupillary membrane could be separated from the anterior lens capsule without lens damage. No strabismus or lens opacification were detected postoperatively. Surgery was undertaken with the intention of avoiding primary lentectomy, since pupillary membranes can usually be separated easily from the anterior lens capsule with viscoelastic substances.
Cataract↗
[Detection of phosphoglucomutase in the cornea and corneal cell cultures using isoelectric focusing and enzymatic stain methods].
PGM-1 and its isoenzymes had been studied in the human cornea, in cornea cell cultures, in erythrocytes and lymphocytes. Qualitative and quantitative comparisons between cornea of healthy persons and cornea cell cultures of patients with cornea dystrophies showed differences between certain phenotypes and their signals of activity.
Cells, Cultured↗
Corneal graft rejection: incidence, manifestation, and interaction of clinical subtypes.
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Corneal Transplantation↗
Elevated prolactin levels in human aqueous humor of patients with anterior uveitis.
Evidence is accumulating that prolactin (PRL) may play a physiological role in the regulation of humoral and cell-mediated immune responses. On the basis of these observations, we measured levels in the serum and aqueous humor of 28 patients with cataract or anterior uveitis with concomitant cataract. Intraocular concentrations were measured in a range from 0.1 to 3.4 ng/ml. Whereas serum PRL levels failed to show significant differences between the two groups (P = 0.39), intraocular concentrations were significantly higher in uveitis patients (P less than 0.001). The level in aqueous humor did not reflect the serum concentration in either group. To the best of our knowledge, this is the first study measuring PRL concentrations in human aqueous humor.
Adolescent↗
[Current status of linkage studies for gene localization in corneal dystrophies].
Corneal dystrophies with primarily autosomal dominant inheritance have been clearly identified both histologically and histochemically. No information is available to date on the causal enzymatic protein defect of the individual forms of dystrophy. By using linkage analysis to study families with various corneal dystrophies, an attempt is being made with polymorphous markers to find an indirect indication for localization in a chromosome. Numerous factors which exclude linkage can be named for granular, lattice and Schnyder's corneal dystrophy. Our linkage analysis studies of a second family with Schnyder's dystrophy support our indications of linkage with the sites for adenylate kinase (AK1) and the AB0 blood groups on chromosome 9.
Chromosome Aberrations↗
[Bromocriptine in therapy of chronic recurrent anterior uveitis].
In animal studies, bromocriptine (Pravidel) has demonstrated immunosuppression because of inhibition of prolactin. After publication of positive results in the treatment of uveitis in a few case reports and in an uncontrolled study we conducted a double-blind placebo-controlled study in 13 patients with chronic recurrent anterior uveitis who had experienced three or more recurrences during the previous year. Therapy was started gradually during the symptom-free interval and continued for 1 year with 2.5 mg twice daily. Two recurrences or relevant side effects led to discontinuation of the therapy. In the bromocriptine group, 2 of 7 patients had no recurrences during the study period. Two patients had to stop because of two recurrences but, in contrast to earlier recurrences, they responded to local corticosteriod treatment within a few days. The remaining 3 patients (1 with one recurrence, 2 without) had to stop because of the side effects (arterial hypotension, arthritic complaints). In 5 of 6 patients in the placebo group treatment was stopped because of recurrences and in 1 patients because she believed she was experiencing a side effect from bromocriptine (breast atrophy). In summary, bromocriptine seems to have a prophylactic effect on anterior uveitis. Side effects were frequent but mild compared to other immunosuppressives. In case of arterial hypotension, additional therapy could make this drug more tolerable.
Adolescent↗
[Eye involvement in hyperimmunoglobulin E syndrome].
The Hyperimmunoglobulinemia-E-Syndrome (HIE) is characterized by high levels of serum-IgE, diminished counts of T-suppressor-cells, eosinophilia, and disturbed chemotactic functions of neutrophil granulocytes. An 8 years old female, suffering from hyperimmunoglobulinemia E (HIE), showed marginal corneal ulcera since five years. Positive conjunctival smears included several bacterias and chlamydia, but never local herpes virus. Inspite of treatment by antibiotics, virostatica, and therapeutical contact lens, a spontaneous corneal perforation occurred. A penetrating keratoplasty was successful for a short time, but soon ulceration rose again. At last a second penetrating keratoplasty was necessary. The early status showed tidy results, but some weeks later marginal ulceration occurred again. There was no clinical proof for herpetical genesis of the course, but evidence is conceivable for pathomechanisms similar to conjunctivitis vernalis.
Child↗
[Isolated infiltration of the vitreous body as a sign of blast crisis in chronic myeloid leukemia].
A 29-year-old man with chronic myeloid leukemia and two successfully treated blast crises exhibited ocular symptoms. Eighteen months after the diagnosis of leukemia he presented with unilateral hyperplastic iris stroma, anterior chamber inflammation and vitreous infiltration. Uveitis was diagnosed tentatively, and local therapy was begun with corticosteroids. Because the findings remained unchanged an invasive diagnostic evaluation followed. Although iris biopsy revealed only unspecific mononuclear cell infiltration (mainly T-lymphocytes), selectively aspirated vitreous material contained myeloid cells of different stages of maturation and blast cells. Peripheral blood, liquor and bone marrow showed no signs of acceleration or transition of the primary disease. Thus, an extramedullary blast crises with isolated vitreous infiltration was diagnosed.
Adult↗