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Biomedical subjects

K W Ruprecht

Publications and source records attributed to K W Ruprecht.

At least 19 recordsLinked to original sources
PubMed1999

Incontinentia pigmenti (Bloch-Sulzberger-syndrome): case report and differential diagnosisto related dermato-ocular syndromes.

BACKGROUND: Incontinentia pigmenti (IP; Bloch-Sulzberger syndrome) is an inherited disorder of skin pigmentation that is associated with skin (100%), dental (90%), skeletal (40%), central nervous (40%) and ocular (35%) abnormalities. The pathogenesis is not yet known. The disease is usually seen in females, as it is an X-linked dominantly inherited disease which is lethal in males. PATIENT PRESENTATION: We present a 9-year-old girl with the classical general and ocular signs of IP. She presented in early childhood with inflammatory vesicular skin changes which changed into pigmented skin alterations especially on the trunk. Ocular findings were microphthalmia and retrolental mass formation in one eye and retinal pigmentary changes in the other. In our patient, the spontaneous mutation may have been caused by the family's close neighbourhood to Semipalatinsk, Kasachstan, where regular nuclear tests took place very shortly before the pregnancy with our patient began. DISCUSSION: Ocular involvement is described in about a third of persons affected with IP. A nearly consistent and pathognomonic finding is a pigment retinopathy (mottled diffuse hypopigmentations). A further consistent finding are abnormalities of peripheral retinal vessels with areas of non-perfusion in the outer retina. The retinal pigment epitheliopathy and the abnormalities of retinal vessels are thought to be the underlying pathognomonic findings, with all other ocular signs being secondary (cataract, leucocoria, optic atrophy, strabismus, nystagmus and microphthalmus). Exudative retinal detachment occurs only in a minority, usually in very early childhood, when the skin lesions are exudative as well. IP patients should, however, be clinically observed regularly because of their retinal pigmentary changes.

Abnormalities, Multiple↗
PubMed1998

[Neurophysiologic follow-up in patients with hypophyseal adenoma operations].

BACKGROUND: Pituitary tumors are adenomas of a region of the sella turcica which can produce compression of the anterior visual pathway. PATIENTS AND METHODS: Besides clinical signs of visual improvement such as enhanced visual acuity and visual field, the use of electroophthalmological methods can help monitor patients after neurosurgery. RESULTS: The N75 P100 amplitude of the p-VEP has proven to be the most sensitive marker for measuring-improvements after surgery. This amplitude significantly increased, whereas latency time, visual acuity and visual field showed no statistically significant changes after surgery. CONCLUSIONS: The p-VEP is an important tool in treating patients with compression of the visual pathway.

Adenoma↗
PubMed1998

[Eye and general illnesses in the public school for blind and visually handicapped students in Saarland. Developments in the last 20 years].

BACKGROUND: The aim of the study was the evaluation of how ophthalmological diagnoses and the proportion of multiply handicapped children has changed within the last 20 years at a state school for visually handicapped and blind children. PATIENTS AND METHODS: A profile investigation was conducted on all 105 children at the Landesschule für Blinde und Sehbehinderte des Saarlandes and compared to the results of an examination from 1975. RESULTS: The predominant ophthalmological diagnoses were: optic atrophy (17.5%), ocular albinism (11.9%), scar-stage IV and V of retinopathy of prematurity (11.1%), as well as tapetoretinal dystrophies with related syndromes (8.7%) and myopia magna (7.9%). Blind: 10.3% (1975: 36.4%); visually handicapped: 47.1% (1975: 49.2%); multiply handicapped: 42.5% (1975: 14.4%). CONCLUSIONS: (1) The diseases that dominated in earlier years in schools for the visually handicapped have become rare (cataract, aphakia, buphthalmia, macular dystrophy--all less than 5%); (2) the proportion of completely blind pupils has become much smaller; (3) there is an increasing tendency to educate visually handicapped pupils in regular schools with integrative aids; (4) there is also an increasing proportion of multiply handicapped children (school and kindergarten: 42%, early patronage 74%).

Blindness↗
PubMed1998

[Indocyanine green angiography in acute posterior multifocal placoid pigment epithelial disease].

BACKGROUND: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an idiopathic posterior segment inflammatory disorder of young adults. The pathogenesis remains unsettled. The placoid lesions and characteristic findings on fluorescein angiography have been interpreted as representing a primary pigment epithelial disorder or a choroidal vascular disease. PATIENT: Using Fluorescein and Indocyanine green angiography we examined one patient with APMPPE. RESULTS: In the acute phase, the fluorescein angiogram showed early hypofluorescence of the ophthalmoscopically visible lesions followed by late hyperfluorescence with centripetal staining of fluorescein at the level of the pigment epithelium. Indocyanine green angiograms showed in the acute stage of this disease areas of hypofluorescence in both the early and late pictures that nearly correlated with the placoid lesions. Three weeks later we saw apart from involution of the initial lesions, new angiographic hypofluorescent lesions at the posterior pole of the left eye, which were ophthalmoscopically not visible. During the next four weeks the older and newer lesions went smaller and left scars. Also the choroidal blood flow was restored partially. CONCLUSIONS: Indocyanine green choroidal videoangiography has shown hypofluorescence of the placoid lesions. This may be explained by choroidal hypoperfusion as the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy.

Adult↗
PubMed1998

[A familial anterior corneal degeneration: clinical aspects, histopathology and differential diagnosis].

BACKGROUND: We report on three cases of a progressive anterior corneal degeneration with clinical characteristics of limbal insufficiency occurring in three persons in three consecutive generations of one family. The disease has not yet been described in literature. PATIENTS: A woman aged 68, her 51 years old son and his 19 years old son are affected by the disease, which suggests autosomal dominant inheritance. None of the affected family members suffers from any form of collagenosis, endocrine insufficiency, chronic dermatosis, alteration of fat metabolism or other relevant systemic diseases. There is no accompanying disease of lids and conjunctiva except of a keratoconjunctivitis sicca with alteration of the mucin phase. The youngest affected member has a binocular anterior polar cataract. In the eldest affected member we performed penetrating keratoplasty. HISTOPATHOLOGY: Revealed thinning of corneal epithelium, moderate intra- and subepithelial granulocytary infiltration, a marked hyaline thickening of Bowman's membrane. The adjacent stroma showed accumulation of granulocytes and an increased number of capillaries and fibroblasts. Posterior stroma, Descemet's membrane and endothelium were normal. There were no goblet cells in the central epithelium. CONCLUSIONS: The appearance of the disease in three consecutive generations (strongly suggesting autosomal dominant inheritance) and the progressivity are characteristics of a dystrophical process. Development of a circular pannus and infiltration of epithelium and stroma with granulocytes however classify the disease as a degeneration, clinically and pathologically similar to Salzmann's nodular degeneration and to autosomal dominantly inherited keratitis.

Adult↗
PubMed1998

[Screening for amblyopia, strabismus and refractive abnormalities in 1,030 kindergarten children].

BACKGROUND: There are controversies concerning the necessity of pre-school vision screening. AIM OF THE STUDY: evaluation of the prevalence of pathologic ophthalmologic findings in kindergarten children. MATERIALS AND METHODS: 1030 families were offered a vision screening. Of these, a total of 948 children, aged 3 to 6 years, voluntarily underwent a screening for strabismus, amblyopia and refractive anomalies. The examination was performed in the kindergarten in the absence of the parents. METHODS OF EXAMINATION: A questionnaire concerning general and ophthalmologic history of the child and of the family was evaluated. Visual acuity, cover-uncover-test, Lang-stereotest, retinoscopy, ophthalmoscopy (undilated pupils) were performed and the glasses were evaluated. RESULTS: The screening was highly accepted by the parents and 92% of the families (n = 948) took part. The compliance of the children was very good. A total of 38.7% (n = 381) of the children showed one or more abnormal parameters. 21.4% (n = 229) showed a reduced visual acuity. Strabismus was found in 3.7%. Half of the children with abnormal findings already had had a vision screening, but only 25% had received ophthalmologic treatment. Of those who possessed glasses, 25% came without them, and another 25% had a reduced visual acuity even with their glasses. The main problems were many false-positive results and high costs. CONCLUSIONS: Ophthalmologic and orthoptic screening in kindergarten is technically easy and conclusive in experienced hands. Ideas to reduce costs and to avoid overreferrals are an age-related lowering of the visual acuity limit and a rescreening of suspected children in a screening-setting a second time before sending them to an ophthalmologist. Another possibility to reduce costs would be to perform examinations not by ophthalmologists but by "screening-orthoptists" who should be trained in retinoscopy and ophthalmoscopy.

Amblyopia↗
PubMed1998

[Two contact lens holders for vitreoretinal surgery].

Two new lens holders for contact lens use in vitreoretinal surgery were developed: (1) a flexible hand-fixed lens holder and (2) a lid-retractor-fixed lens holder. Advantages are: better stabilization and centering of the lens, reduction of air bubbles and blood in the interface between cornea and contact lens.

Contact Lenses↗
PubMed1997

Ocular manifestations in a father and son with EEC syndrome.

BACKGROUND: The ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is a rare disease which follows an autosomal-dominant pattern of inheritance. Due to the ectodermal dysplasia there is atresia of the lacrimal duct system and aplasia of the meibomian glands with a defective tear film. Therefore, vascularized corneal scars often form during early adult life. PATIENTS: Father aged 41 years, and son aged 23 months. Both patients: stenosis/atresia of lacrimal duct systems (the father had twice undergone dacryocystorhinostomy externally) with epiphora, lip-palate clefting, syndactylies of fingers and toes, lobster deformities of hands. Additional ophthalmological findings in the father: bilaterally extracted juvenile cataracts with implantation of intraocular lenses, bilateral extensive vascularized corneal scars. Additional dermatological findings in the father: malignant melanoma of the calf, now in complete remission following several operations on the melanoma and several cycles of chemotherapy for the metastases. DISCUSSION AND THERAPEUTIC CONCLUSIONS: Father and son show the full clinical picture of the EEC syndrome with clefting, lobster-like deformities of the hands and ectodermal dysplasia with tear duct atresia and aplasia of the meibomian glands with defective tear film. During childhood, the main handicapping features are the clefting and the hand deformities with their respective multiple operative revisions. During early adulthood, however, the ocular problems become the predominantly handicapping aspects of the EEC syndrome; due to the ectodermal dysplasia, vascularized corneal scarring develops. Tearing and secondary inflammation due to lacrimal duct atresia has to be treated by early dacryocystorhinostomy. As secondary infections promote the development of corneal scars, one should not postpone the operation too long. Infections have to be treated promptly by local antibiotics. Because of the aplasia of the meibomian glands, artificial tear substitution should be given on a regular basis to support the defective tear film. Thus, the development of vascularized corneal scars can perhaps be delayed. Once corneal scarring has developed, perforating keratoplasty has a poor prognosis due to the ectodermal dysplasia, the absence of the meibomian glands and the defective tear film. Three factors lead to the formation of vascularized corneal scars: recurrent infections of lid margins and conjunctiva due to obstructed tear ducts; defective tear film with insufficient lipid phase due to the aplasia of the meibomian glands; and primary corneal epithelial defects in the course of the generalized ectodermal dysplasia.

Adult↗
PubMed1997

[Transcutaneous or transconjunctival peribulbar anesthesia?].

BACKGROUND: Transconjunctival peribulbar anesthesia is reported to permit better identification of the bulbar limits because of a better view into the fornix; it is also said to be less painful compared to transcutaneous peribulbar anesthesia. The aim of our study was to compare the two injection techniques. PATIENTS AND METHODS: This study comprised 46 patients undergoing eye operations under local anesthesia. They were allocated randomly in a prospective and simple masked study. Twenty-three patients received transconjunctival injections and 23 patients transcutaneous peribulbar injections. Before injection, all patients received Oxybuprocain eyedrops for anesthesia of the conjunctiva. In all cases no separate injections for lid akinesia were performed. Before and 20 min after the injection, the following parameters were assessed: pain score (visual analog scale), frequency of conjunctival chemosis and the necessity for supplemental anesthesia. For identification of the bulbar limits we checked if it was possible to see the caudal and the cranial fornix. RESULTS: The transconjunctival injection was significantly (P = 0.05) more painful (5.6 +/- 2.4) than the transcutaneous injection (4.2 +/- 2.4). Among the patients with transconjunctival injection, in 66.6% the cranial fornix could not or was only poorly seen. CONCLUSIONS: Transconjunctival peribulbar anesthesia appears not to be more advantageous than transcutaneous peribulbar anesthesia.

Anesthesia, Local↗
PubMed1997

Does warming of anesthetic solutions improve analgesia and akinesia in retrobulbar anesthesia?

PURPOSE: The authors assess the effect of warming local anesthetic solutions on pain of injection and on bulbar akinesia and analgesia of retrobulbar anesthesia (RBA). METHODS: Seventy patients undergoing RBA for cataract surgery were enrolled in a prospective, double-blind trial. They were allocated randomly to receive 5 ml either warm (37 degrees C) or cold (20 degrees C) anesthetic solution for RBA. Additionally, O'Brien's method was used to create an akinesia of the orbicularis oculi muscle. The following data were collected before and 20 minutes after retrobulbar injection: pain of injection, eye motility (Kestenbaum test), and corneal sensitivity (0: no sensitivity; 1: sensitivity remaining) at four different sites. The pain of injection was registered using an ordinal analogous scale before and immediately after the injection. Furthermore, data acquisition included any possible side effects and the bulbar length, measured with ultrasound. RESULTS: The score for injection pain (4.5 +/- 2.3 points), horizontal eye motility (0.2 +/- 0.8 mm), vertical eye motility (0.9 +/- 2.1 mm) all were lower for the warm group in comparison to the cold group (average pain score: 5.2 +/- 2.6 points; horizontal eye motility: 0.7 +/- 1.6 mm; vertical eye motility: 1.2 +/- 2.0 mm). Two patients in the warm group and four patients in the cold group had remaining corneal sensitivity. None of the differences were significant. CONCLUSIONS: Data indicate no significant difference in bulbar analgesia and akinesia after RBA between injections of warm and cold anesthetic solutions.

Adult↗
PubMed1996

[Perioperative physiological and cognitive functions following oral premedication with 3.75 mg midazolam in operations with retrobulbar anesthesia].

UNLABELLED: The number of surgical procedures performed as day surgery has significantly increased in recent years. Therefore, a safe and short postoperative recovery period has become increasingly important. The aim of the present study was to investigate perioperative cognitive and physiological function after oral premedication with low-dose midazolam (3.75 mg), especially during the postoperative period. METHODS: Forty-seven men (age > 69 years, weight 50-90 kg) scheduled for elective cataract surgery under retrobulbar anaesthesia (RBA) were included in the study. The patients were randomly assigned to either group 1 (n = 28), receiving 3.75 mg midazolam p.o. (Dormicum), or group 2 (n = 19), receiving a placebo orally 30 min before RBA. We measured the following parameters: sedation (modified Glasgow coma scale); anxiety (visual analogue scale); numerical and verbal memory (digit span and reproduction of previously presented words); concentration (Revisions test of Stender/Marschner). To identify depression of ventilation, pulse oximetry and nasal end-tidal PCO2 were monitored intraoperatively. RESULTS: After premedication with 3.75 mg midazolam, patients were significantly more sedated (P < 0.01) and systolic blood pressures were significantly reduced (P < 0.05); 30 min after midazolam premedication only concentration was significantly (P < 0.05) decreased. The results of the other cognitive functions did not differ. No differences in cognitive and physiological functions between and groups could be found 2 h after the operation (293 +/- min after premedication). Intraoperatively, there were no significant differences in end-tidal PCO2 and oxygenation between the groups. In both groups anxiety and blood pressure were significantly higher pre- than postoperatively. CONCLUSION: Oral administration of low-dose midazolam (0.049 +/- mg/kg) seems to be appropriate for premedication before ambulatory surgical procedures in elderly patients. In the interest of patient safety, standardised oral premedication with 3.75 mg midazolam may not be sufficient for some of the patients.

Aged↗
PubMed1996

Bacterial anterior chamber contamination with foldable silicone lens implantation using a forceps and an injector.

PURPOSE: To compare the rate of anterior chamber (AC) contamination during implantation of a foldable three-piece silicone lens using a forceps and an injector. SETTING: Department of Ophthalmology, University of Saarland, Homburg (Saar), Germany. METHODS: In a prospective, randomized study, we cultured AC aspirates of patients who had phacoemulsification with silicone lens implantation through a 3.5 mm temporal clear corneal incision. In Group A, the lens was implanted with a forceps and in Group B, with an injector. Immediately after lens implantation, the AC aspirate (0.05 to 0.10 ml) was collected and sent to the microbiology department. All cultures were incubated for 3 days. As a topical antibiotic, gentamicin was given preoperatively and intraoperatively. RESULTS: One hundred patients (50 in each group) were examined. In each group the bacterial contamination of the AC was 2%. CONCLUSION: Bacterial contamination of the AC was not significantly different after silicone lens implantation with a forceps and with an injector.

Aged↗
PubMed1996

[Drug side-effects of the eye--a contribution to quality assurance in ophthalmology].

BACKGROUND: The aim of this research is to catalogue the increasing number of side effects of medications on the eyes and, by putting this catalogue into a data bank, to make it available for use in hospitals. Here a deliberate search is made for medications, which could possibly cause certain side effects as well as a list of possible undesired side effects caused by a certain group of medications analogous to the main groups in the so-called "Rote Liste". METHODS: With the use of the medical literature data bank MEDLINE (CD-ROM) and a search strategy suitable to the topic, German or English publications are included in the data bank, when dose, duration and method of application has been stated, and there had to be a definite or at least possible connection between the side effects observed and the substance causing the side effects. The relevant datas in each article, for example, details about the substance, its application, the publication reference, the individual side effects, categorized according to anatomical or functional aspects, and, if necessary, details about reversibility and therapy were registrated on the data bank. RESULTS: The most common side effects in systemic therapy relate to conjunctiva, cornea/sclera, retina as well as a large number of changes in vision. In external application the undesired side effects were also mainly on conjunctiva, cornea/sclera, as well as on eyelids, uvea, and changes in visual acuity. In medications taken externally ophthalmological side effects were most frequently caused by cytostatics, dermatics, corticoids and antibiotics. In the case of external application on the eye, by far the most undesired side effects were caused by glaucoma medications, followed by antibiotics, mydriatics and corticoids. CONCLUSION: In future, with the use of the data bank, it should be possible to quickly observe a relation between undesired side effects and medications which could possibly cause these side effects.

Adverse Drug Reaction Reporting Systems↗
PubMed1996

[0.15 mg clonidine as oral premedication in retrobulbar anesthesia].

BACKGROUND: In the present clinical study, oral premedication of 0.15 mg clonidine has been explored with view to anxiolytic effects and physiological stress parameters. METHODS: The study involved 44 patients of ASA-class I-III, all scheduled to undergo ophthalmological surgery under retrobulbar anesthesia (RBA). One hour before RBA 22 patients received 0.15 mg clonidine and 22 patients placebo p.o. The following parameters were assessed at different times perioperatively: oxygenation (SpO2), heart rate, blood pressure, intraocular pressure and anxiety. Anxiolysis was determined using the Erlanger Anxiety Scale. RESULTS: There were no significant differences among the groups in SpO2 and heart rate. Significantly decreased blood pressures were measured after clonidine premedication (p < 0.01). In contrast to clonidine after placebo the preoperative anxiety increased. Right before operation anxiety was decreased significantly (p < 0.01) by clonidine compared to placebo. CONCLUSIONS: In our opinion, oral clonidine (0.15 mg) is useful for premedication of surgery under RBA.

Administration, Oral↗