Abstract:
BACKGROUND:Bilateral stromal corneal opacifications are a differential diagnostical challenge to identify associated systemic diseases. CASE-REPORT:A 47-year old civil engineer (height 167 cm) with bilateral stromal corneal clouding presented with visual loss for the last 27 years: VA 20/100 OD and 20/50 OS (following penetrating keratoplasty OS). The cornea showed milky-whitish, cloudy, diffuse stromal deposits without a separate lipoid arc. The posterior segment showed tapetoretinal degeneration. Scotopic ERG was decreased. A suspicious stiffness of interphalangeal joints on both hands was observed. There was an aortic and mitral insufficiency grade I. Serum levels of LDL, HDL and triglycerides were normal. The biomicroscopical diagnosis of Scheie's syndrome (mucopolysaccharidosis I-S) was confirmed by a deficiency of alpha-L-iduronidase in leukocytes (0.02 nmol/min/mg protein, normal range: 0.3 - 1.5). CONCLUSION:The differential diagnosis of bilateral corneal stromal opacification includes in addition to the mucopolysaccharidoses HDL-deficiency diseases (LCAT deficiency, Tangier disease, Fish eye disease), Schnyder's crystalline stromal dystrophy, cystinosis, gout and mucolipidoses. MPS I-S may easily be detected by alpha-L-iduronidase deficiency in leukocytes and increased mucopolysaccharides in the urine. Furthermore, patients with MPS I-S need general medical care because of cardiovascular abnormalities, joint stiffness and myopathies.
journal_name
Klin Monbl Augenheilkdjournal_title
Klinische Monatsblatter fur Augenheilkundeauthors
Viestenz A,Shin YS,Viestenz A,Naumann GOdoi
10.1055/s-2002-35688subject
Has Abstractpub_date
2002-10-01 00:00:00pages
745-8issue
10eissn
0023-2165issn
1439-3999journal_volume
219pub_type
杂志文章abstract:BACKGROUND:A meticulous fundoscopy is an essential examination before administering an orthoptic occlusion therapy (patching), even in cases with a "clear indication" for patching. In this case of an 8-year-old hyperopic boy the subsequent fundoscopy revealed a bilateral maculopathy that explained the stagnation of the...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1027270
更新日期:2008-05-01 00:00:00
abstract::In a preceding paper with the same title the result was represented by curves which enable factors used in calculating the true diameter to be determined. Instead of the curves a quadratic equation is used to produce the same factors. The data for the equation are given in two tables. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050076
更新日期:1988-01-01 00:00:00
abstract::The panel of therapeutic options available for the treatment of retroviral disorders has expanded explosively in recent years with the result that it has now become possible to reconstitute the immune function of individuals with advanced HIV-disease to a remarkable degree. The ophthalmologist is thus confronted with ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2000-10520
更新日期:2000-02-01 00:00:00
abstract:PURPOSE:To determine the role of Indocyanin Green (ICG) angiography in localizing occult new vessels associated with age-related macular degeneration (ARMD) and assess the possibilities of ICG guided laser photocoagulations. PATIENTS AND METHODS:Fluorescein and ICG angiographies (IMAGEnet system) of 62 patients with o...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035228
更新日期:1996-05-01 00:00:00
abstract:BACKGROUND:We describe a case of central serous chorioretinopathy associated with a large adrenal myelolipoma. HISTORY AND SIGNS:A 55-year old man was admitted to our clinic complaining for metamorphopsia and blurred vision in his left eye. Standard ophthalmologic examination and fluorescein angiography established th...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2003-38187
更新日期:2003-03-01 00:00:00
abstract::The histological and ultrastructural findings in the ciliary ganglion of glaucomatous patients show proliferation of the satellite cells, vacuolate degeneration of the cytoplasma with loss of the mitochondria and ergastoplasma, and necrosis of the nucleus with decrease of chromatin. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050838
更新日期:1986-11-01 00:00:00
abstract::Elevated protein concentrations in the aqueous humor, without Tyndall phenomenon, were found in a case of Coats' Disease. In particular, proteins of smaller size were involved. The permeability of the retinal vessels was presumed to be impaired. The resulting increase in protein concentrations in the aqueous humor cou...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057404
更新日期:1980-01-01 00:00:00
abstract::Anastomoses between vessels of the fundus may occur as congenital malformations or secondary to vascular diseases affecting the eye. Congenital anastomoses can be of the racemose hemangioma type, with severe malformation of the retinal vessels, ocular complications, and neurologic manifestations, or as simple A-V shun...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1979-10-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to evaluate the functional outcome of Brilliant Blue G (BBG) and the staining properties in macular surgery. METHODS:BBG was applied during vitrectomy for macular holes (n = 21) or epiretinal membranes (n = 18) in a prospective, non-comparative consecutive series of patients (Brilli...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0031-1273351
更新日期:2011-08-01 00:00:00
abstract::Timolol lowered intraocular pressure in six patients with severe neovascular glaucoma only from about 45 mmHg to about 38 mmHg. The intense pain suffered by five patients was not alleviated. Hence, Timolol treatment does not seem very effective in neovascular glaucoma, although it may be of some value as a supplement ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057639
更新日期:1980-01-01 00:00:00
abstract:BACKGROUND:Development of a reflectance oximeter to measure non-invasively the changes in oxygen saturation (SO 2 ) in the vessels of the human eye fundus, with the goal of obtaining a better understanding of the retinal circulation in health and disease. MATERIAL AND METHODS:For retinal oximetry, an instrument must i...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2002-30648
更新日期:2002-04-01 00:00:00
abstract::In the presence of leukokoria the diagnosis of retinoblastoma is usually easy when the mediae are transparent. If they are not, however, one is frequently obliged to enucleate a painful or useless eye in order to rule out this diagnosis. In a series of eight cases having undergone enucleation for this reason at the La...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1057514
更新日期:1980-04-01 00:00:00
abstract::Background To evaluate the technique of inverted internal limiting membrane (ILM) flaps for the management of large macular holes and autologous ILM free flaps for non-closing macular holes. Patients and methods All macular holes were treated with pars plana vitrectomy and dual blue assisted ILM flap technique. The in...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0042-119694
更新日期:2017-04-01 00:00:00
abstract::Mycotic Septicaemia (especially with the Candida species) is not an uncommon hazard of hospitalized patients, especially those on intravenous hyperalimentation. Two such patients with endogenous mycotic bilateral endophthalmitis are presented. In spite of typical ocular symptoms diagnosis was delayed. Two further unil...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-03-01 00:00:00
abstract::This review considers diseases of the visual pathways at different levels, their consequences for relevant everyday functions and their rehabilitation. Infrachiasmatic lesions are mostly associated with central or ring scotomas causing reading problems. The use of an eccentric fixation locus in combination with text m...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-0028-1109874
更新日期:2009-11-01 00:00:00
abstract::Neurofibromatosis 2 is an autosomal-dominant disease, which is characterized by vestibular schwannomas, cataract, retinal hamartomas as well as tumors of the peripheral and central nerve system, demonstrating a variety of expression. The ophthalmologist plays an important role in making the diagnosis, as several ocula...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2005-857956
更新日期:2005-04-01 00:00:00
abstract:PURPOSE:The aim of the present article is to review the methodical and technological development of pars plana vitrectomy. Special attention is drawn to safety, efficiency and functionality of the innovative 25-gauge und 23-gauge vitrectomy systems which are compared to the standard 20-gauge vitrectomy system. This was...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2007-963518
更新日期:2007-09-01 00:00:00
abstract::A 55-year-old man was referred to our clinic with a mature, swollen cataractous lens and a secondary phakolytic glaucoma. However, the patient refused to undergo surgery. Twelve years later he returned. Meanwhile the lens had been almost completely absorbed; visual acuity was 20/32 and there had been no severe damage...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1055071
更新日期:1982-04-01 00:00:00
abstract:BACKGROUND:In facial palsies with lagophthalmic keratopathy an hour-glass dressing is indicated, which has the disadvantage of a moisture chamber with visual impairment by steaming up. After weeks of placement dermal irritations are possible. An alternative is the gravity dependent lidloading, using gold implantation i...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035353
更新日期:1995-08-01 00:00:00
abstract::Optical coherence tomography (OCT) enables noninvasive high-resolution 3D imaging of the human retina, and thus plays a fundamental role in ophthalmology. Via OCT examination, even subtle retinal changes can be captured, which occur in very early stages of different diseases (e.g., glaucoma, diabetes mellitus, or age-...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0043-121705
更新日期:2017-12-01 00:00:00
abstract::A study is presented of a case of serous detachment of the mucula secondary to congenital pit of the optic disc. We observed during fluorescein angiography a late leakage of dye at the temporal margin of the disc. A lasercoagulation of this lesion gave a favourable result. All these findings support Gass' explanation ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1977-12-01 00:00:00
abstract::A Pakistani wall plate depicting the anatomy of the eye is described and illustrated. The picture is partly Greek (the hollow optic nerve), in part medieval Arab (the optical image on the disk), and in part modern (e.g., the refraction anomalies). A brief account is given of how Greek science passed to the Arabs and t...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 历史文章,杂志文章
doi:10.1055/s-2008-1050281
更新日期:1988-10-01 00:00:00
abstract::We examined 38 patients with disturbed blood supply to the optic nerve. The observations suggest: 1. men are more frequently affected than women. 2. the first attack of apoplexia nervi optici occurs most often in the left eye. 3. diabetes mellitus and most probably arterial hypertension are risk factors. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract::The serum of 63 patients suffering from various clinical types of endogenous uveitis with unknown etiology was investigated for the presence of circulating immune complexes (CIC). None of the patients was suffering from a systemic disease usually associated with CIC. Significantly elevated CIC levels were detected in ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054906
更新日期:1983-09-01 00:00:00
abstract:PURPOSE:Imaging methods of the optic nerve head appear to have an increasing impact in glaucoma diagnosis. The aim of this study is to evaluate the specifity of the Heidelberg Retina Tomograph (software version 1.7 and 3.0) in subjects with physiological cupping and large optic discs. PATIENTS AND METHODS:27 eyes of 2...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0032-1328094
更新日期:2014-02-01 00:00:00
abstract::Radiation Retinopathy is a progressive chronic disease triggered by ionising radiation and is characterised by vascular endothelial damage that can lead to macular edema, optic disc edema and proliferative retinopathy. We discuss a case of a patient with radiation retinopathy and optic disc edema who we treated with a...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/a-0735-9865
更新日期:2020-03-01 00:00:00
abstract:UNLABELLED:A new low vision aid (LVA)-the Low Vision Enhancement System or LVES, which were as developed at the Johns Hopkins University in Baltimore, is now commercially available. This instrument allows a magnification up to 10 times with control of contrast and luminance while the field of view is very large: 60 x 4...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035025
更新日期:1997-02-01 00:00:00
abstract:BACKGROUND:The aim of this study is to report the feasibility of one to two weeks delayed reconstruction after eyelid tumor excision. PATIENTS AND METHODS:A retrospective case-series study was designed. Fourteen consecutive outpatients referred to a tertiary referral eye center for eyelid tumor management were enrolle...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0031-1299208
更新日期:2012-04-01 00:00:00
abstract::Detachment surgery without drainage was introduced in 1953 by Custodis and modified by Lincoff. In this method the retinal hole determines the surgical procedure. Subretinal fluid will disappear by fixating an explant the sclera under tension in the area of the hole. The size of the explant depends upon the size of th...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1978-08-01 00:00:00
abstract::In anophthalmos the forces which stimulate development and growth of the bony orbit are absent. As a result these cases have not only a tiny socket but also an underdeveloped bony orbit, which cannot retain an artificial eye. The small conjunctival sac and bony orbit can be mechanically enlarged by means of Hema socke...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050502
更新日期:1987-09-01 00:00:00