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AUGENHEILKUNDE 214/1999 – ISSUE 3 Abstracts:
S. 125-135 (Art. 428) 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. Key words Corneal dystrophy - corneal dystrophy of Bowman's layer - honeycomb-shaped corneal dystrophy - Thiel-Behnke dystrophy - geographical corneal dystrophy - Reis-Bücklers dystrophy - ring-like dystrophy [ back ] S. 136-141 (Art. 427) Impairment of mesopic vision by
photorefractive keratectomy of myopia Purpose Halos, glare disability and problems during night driving are significant side effects of photorefractive keratectomy, caused by increasing optical aberrations under reduced conditions of luminiscence. In a previously performed retrospective study an impairment of mesopic vision in most of the investigated eyes was found. To prove these results, a prospective study was conducted. Methods and patients In a prospective, consecutive study mesopic function was investigated in 41 myopic patients, who were interested in PRK. 26 patients underwent surgery (Summit ExciMed UV200LA, optical zone 5mm), which was performed in 30 eyes. Mesopic function (contrast vision, glare sensitivity) was measured by using the Mesoptometer II test in all 30 eyes preoperatively and 6 to 9 months postoperatively. Additionally, 21 eyes were examined after 12 to 15 months of follow up. Results There was a statistically significant reduction of contrast vision and increase of glare sensitivity after 6 to 9 and 12 to 15 months. The Mesoptometer II contrast level of 1 : 5 (with and without glare) can be used as a critical parameter for the descision, whether mesopic function is markedly reduced or not. Before surgery, contrast level of 1 : 5 without glare was not recognized in 30%, with glare in 40%. After 6 to 9 months, this contrast level was not recognized in 73% of the 30 eyes without glare, with glare in 80%. Out of these 30 eyes, 21 eyes were followed over 12 to 15 months. Whereas 38% of these eyes (with glare 48%) did not recognized the critical contrast level before sugery, this rate increased to 67% (with glare 81%) 12 to 15 months after surgery. Conclusions Impairment of mesopic function has to be considered as a possible side effect of PRK (optical zone 5 mm). Although further investigations are needed, the indication for PRK in special professions (pilots, professional drivers) should be very critical discussed. Key words photorefractive keratectomy - PRK - side effects - mesopic vision [ back ] S. 142-146 (Art. 409) Allogen heterotopic
cartilage transplanation for primary corneal replacement in rabbit model Background Aim of the present study was to investigate woundhealing after allogen heterotopic cartilage transplantation in a rabbit model. Material and methods Cartilage discs were transplanted by central lamellar keratoplasty (diameter 5.0 mm) in eleven New Zealand White Rabbits. Cartilage was harvested of the ears of rabbits at the day of transplantation. The cartilage discs 0.5-0.6 mm thick were prepared with 5.2 mm diameter and transplanted within 12 hours. Fixation was performed by eight 10-0 nylon sutures. Postoperatively, dexamethasone ointment containing gentamicin was applied three times a day for three weeks. Clinical course was documented by photographs. For histological examinations the transplanted eyes were enucleated after one day or three days, and after 3, 6 or 12 months. Results Allogen cartilage healed firmly in the cornea within two months. During the course of observation the cartilage grafts thinned and were replaced by normal appearing collagen fibrills. In one case neovascularisation of the cornea occurred and reached the cartilage graft, but did not invate. Histologically, in all cases no leukocytes could be seen in the cartilage or its neighbourhood. The cartilage grafts did not proliferate and remained avascular. The intraocular structures showed no inflammatory reaction. Conclusions Allogen cartilage grafts to a clear corneal pounch do not induce an inflammatory reaction. Therefore, autologous cartilage prepared of ears seems to be useful biological material in ophthalmologic surgical procedures, for example to fill up corneal defects. To investigate if cartilage grafts will be helpful to stop neovascularisation in keratoplasty further studies with vascularisized corneas are necessary. Key words biological corneal replacement - cartilage graft - keratoplasty - corneal neovascularization [ back ] S. 147-155 (Art. 398) Wound healing after glaucoma
filtering surgery - a review Wound healing after glaucoma filtering surgery is characterized by a complex sequence of molecular and cellular events. Results of recent investigations have shown that the activation, migration, proliferation and differentiation of Tenon's capsule fibroblasts represents the central element of the wound healing response. An exaggerated wound healing will result in the closure of the artificial fistula between the anterior chamber and the subconjunctival space, leading to an increase in intraocular pressure. The present review summarizes the current knowledge regarding the most important cellular and molecular mechanisms, which regulate the postsurgical wound healing response in the subconjunctival space. Special emphasis will be put on the specific role of cytokines during the wound healing response. An extensive understanding of these mechanisms appears pivotal for the development of specific therapeutic strategies to inhibit the events which lead to fibrogenesis and scarring in the wound region after glaucoma filtration surgery. The databank was Medline. Key words glaucoma - wound healing - glaucoma filtration surgery [ back ] S. 156-159 (Art. 400) Prognostic value of anterior
chamber hemorrhage after glaucoma filtering surgery Purpose Evaluation of the prognostic significance of a postoperative anterior chamber hemorrhage (ACH) after glaucoma filtering surgery. Patients and Methods Records of 332 patients, who had undergone filtering surgery between 1/1993 and 2/1997, were analyzed retrospectively with particular concern on epidemiologic data, pre- and postoperative regulation of intraocular pressure (IOP), complications including ACH, postoperative 5-fluouracil (5-FU) therapy and postoperative IOP lowering therapy. Surgical success was defined as (1) IOP reduction below 21 mm Hg, (2) relative IOP reduction at least 20% and (3) no reoperation to control IOP until the first-year examination. Results 60 out of 332 patients (18.1%) suffered from ACH after glaucoma filtering surgery. 24 patients with ACH were treated with 5-FU, postoperatively. When all patients with ACH were considered in the evaluation, the difference between the success rate of patients with ACH and the success rate of patients without ACH was not statistically significant. In contrast, when ACH was the only complication in patients, who were not treated with 5-FU, the success rate was significantly worse (38%), when compared with the control group (patients, who did not have ACH or who had other complications in addition to ACH) (67%) (p=0.008). When patients with these criteria received 5-FU postoperatively, the difference between success rates of both groups was not any longer statistically significant (p=0.99). Discussion The postoperative occurrence of anterior chamber hemorrhage is associated with a higher failure rate of filtering surgery. Early administration of 5-FU is recommended in these cases. Key words glaucoma - goniotrepanation - anterior chamber hemorrhage - prognostic factors - wound healing [ back ] S. 160-170 (Art. 415) Modulation of immune mediated
diseases of the eye The eye is the target of a variety of immune mediated diseases. Advances
in the field of immunology have permitted a better understanding of
these challenges. This progress has been accompanied by the development
of more specific immunomodulating agents. Corticosteroids are still
a major agent in the management of ocular immunmediated diseases, but
are now supplemented by other potent immunomodulating agents. The exicitement
in pharmacological research has in turn provided more insight in immune
mechanisms and some of the newer agents have been used as "probes" for
a better understanding of basic mechanisms. Key words Immunology - immunmodulation - inflammation - keratoplasty - uveitis [ back ] S. 171-174 (Art. 396) Churg-Strauss syndrome Background The Churg-Strauss syndrome, also known as allergic granulomatosis with angiitis, is a rare necrotizing vasculitis with unknown pathogenesis. The necrotizing granulomatous vasculitis involves small and medium-sized arteries, capillaries and veins, leading to the characteristic changes of intra- and extravascular, eosiniphilic granulomas, accompanied by clinically symptoms: bronchial asthma, hypereosinophilia and fever. Ocular manifestations are rarely reported. Case report A 53-year-old woman suffered from bronchial asthma, relapsing lung infiltrates and sinusites since 1994. In August 1997 she suddenly disclosed vasculitic skin manifestations on both legs and a mononeuritis of the left peroneus nerve. At the same time visual acuity of the right eye decreased, before she had shown some attacks of amaurosis fugax. The funduscopy showed a central retinal artery occlusion. Laboratory findings: blood eosinophilia of 20%, elevated IgE value to 396 kU/l (normal value < 120 kU/l), and negative parameters for antineutrophil cytoplasmatic antibodies (p- and c-ANCA). Conclusion The clinical and laboratory findings are characteristic signs for the Churg-Strauss syndrome. Without such typical manifestations the histologic examination leads to the diagnosis and helps to differentiate this disease from other necrotizing vasculitides, e.g. panarteriitis nodosa or Wegener's granulomatosis. Key words Churg-Strauss syndrome - systemic necrotizing vasculitis - IgE-elevation - hypereosinophilia [ back ] S. 175-177 (Art. 397 DF) Bilateral neuro-retinitis
with zoster infection Background Infections with varicella zoster virus may involve the optic nerve and the retina. Different pathomechanisms have been discussed. We present a case with an autoimmune inflammatory reaction according to the clinical course. Patient A 69-year-old female was referred to our clinic because of suspected bilateral anterior ischemic optic neuropathy. She complained of severe visual loss the day before admission. Her ophthalmological and general history was unremarkable apart from treatment with 5 to 7.5 mg prednisolone alternately because of rheumatoid arthritis. Best corrected visual acuity was 1/15 OD and 0.1 OS. A relative afferent pupillary defect on the right eye was present. Optic disc oedema with multiple hemorrhages of the retina extending into the peripheral fundus, slightly attenuated retinal arteries and macular oedema were seen fundoscopically in both eyes. Therapy and clinical outcome After immediate treatment with steroids (initial dose 250 mg prednisolone per day) visual acuity improved. Because of a clinically suspected and serologically proven active varicella-zoster infection an additional virostatic therapy with valaciclovir was started and steroids were lowered gradually. Within 2 months, visual acuity increased to 0.8 OD and 1.0 OS. Oedema of optic discs and macula resolved and retinal hemorrhages disappeared. Conclusion A severe hemorrhagic neuro-retinitis involving the optic discs was seen in the course of a varicella-zoster infection, possibly reactivated by chronic steroid therapy of a rheumatoid arthritis. Because of the normalization of visual function an ischemic pathogenesis is unlikely. An autoimmune inflammatory reaction seems to be the predominant mechanism, supported by the good effect of steroid and valaciclovir therapy. Key words neuro-retinitis - optic disc oedema - valaciclovir therapy - steroid therapy - varicella-zoster [ back ] S. 178-182 (Art. 445) Chloroquine- and chlorphentermin-induced
lipidosis in rat retina Background The amphiphilic drugs chloroquine and chlorphentermine are known to cause lipidosis in the human and rat retina. Methods We treated femal albino Wistar rats orally with chloroquine for 12 weeks, followed by a period of 4 months with normal feed and another group with chlorphentermine for 4-16 weeks. The animals were submitted to electroretinography, and the retinae were prepared for histological investigations. Results Chloroquine caused severe lipidosis in the neuroretina and slight photoreceptor cell degeneration after 12 weeks of treatment. The b-wave was reduced to 30% of initial values. After withdrawal the lipidosis remitted, but the degeneration of the photoreceptor cell layer continued to progress. The a-wave and b-wave amplitudes were reduced to 25% and 16% of initial values, respectively. Chlorphentermine caused pronounced lipidosis in the pigment epithelium and less numerous in the neuroretina after 16 weeks; no photoreceptor cell degeneration was found. The b-wave was reduced to 80% of initial values, the a-wave appeared unaffected. Conclusion Whether lipidosis is the primary cause of changes in the electroretinogram and of receptor cell degeneration is doubtful. Excessive lipid storage may be the cause of secondary changes. It is unlikely that lipidosis in pigment epithelium played a role. Key words amphiphilic drugs - chloroquine - chlorphentermine - lipidosis - rat retina - electroretinogram - photoreceptor cell degeneration [ back ] S. 183-184 (Art. 365 DF) Primary pigment epithelial
cysts of the iris Purpose We want to present a case of bilateral, unusually large pigment epithelial cysts of the iris. Patient A four-year old girl presented with multiple, unusually large, bilateral pigment epithelial iris cysts and tags at the pupillary margin. The right eye additionally presented with two adhesions of the cysts at the corneal endothelium. Otherwise, the anterior and posterior segment as well as the intraocular pressure were normal. The visual acuity was at least 0.8 without glasses, and there was no evidence of strabism or other functional impairments. At normal daylight and with non influenced pupils, the visual axis was not covered by the cysts. Since the lesions anamnestically appeared to be congenital and the clinical course had been benign, no therapy was initiated. The patient has been checked on at regular intervalls without any functional or morphological change. Conclusion Pigment epithelial cysts of the iris usually do not cause any complications due to their small size and stationary clinical course. In rare cases, the lesions can be so large, that they get into contact with the corneal endothelium and sometimes nearly cause visual impairment by covering the visual axis. Key words Iris - pigmentepithelial cyst - congenital cyst [ back ] S. 185-187 (Art. 442 DF) Hyperplasia of sebaceous
glands (Fordyce's disease) in an oral mucocutaneous graft: Follow-up
over 45 years Background Diffuse sebaceous gland hyperplasia in transplanted buccal mucosa is a very rare condition. We report on a further patient with this entity. Patient In 1953, a now 68-year-old man suffered a severe alkali burn. Transplantation of oral mucosa was performed that same day in order to prevent perforation of the eye. Over the following 45 years, the patient developed multiple yellowish nodules within the grafts. Histologically, these nodules proved to be normally differentiated sebaceous glands. Conclusions Diffuse sebaceous gland hyperplasia originating from within oral mucosa and developing over an extended period of time is a clinical entity known as Fordyce's disease. It is this a late complication of mucocutaneous transplantation, and although it constitutes mainly a problem of cosmesis, functionally it can lead to aberrant secretion of sebum. Key words Oral mucocutaneous graft - chemical burn - hyperplasia of sebaceous glands - Fordyce's disease [ back ] S. 188-192 (Art. 346) Synophthalmia in an
early human fetus Background The great majority of reports dealing with human cyclopia and synophthalmia are derived from fetuses at the end of pregnancy. Thus, data concerning early organization of the ocular anlage in this impressive ocular developmental anomaly are almost completely lacking. We therefore present a 12 week-old synophthalmic fetus. Case report After uneventful pregnancy spontaneous abortion occurred. Within the face of the hypotrophic fetus there was a central pigmented ocular anlage with an inferior fissure. A nose (proboscis) was not discernible. Histologically, there were two vesicles caudally which fused to one vesicle cranially. Differentiation was more advanced in the anterior and temporal parts of the anlagen. A coherent inner layer (of the optic cup) was not developed. Trisomy 13 was suspected but could not be proved by FISH-technique. Conclusions The fetus demonstrates that fusion of the ocular anlagen is finished at the end of the third gestational month and that synophthalmia/cyclopia is very likely to be associated with maldevelopment of the optic cup. As far as we know there is evidence for the first time that organization of the anlagen improves not only in the posterior to anterior and in the medial to lateral but also in the cranial to caudal direction. Key words Synophthalmia - Cyclopia - Directions of differentiation - Trisomy 13 [ back ]
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