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Interdisciplinary treatment and ophthalmological findings in Parry-Romberg syndrome.

Parry-Romberg syndrome is a rare pathologic process, characterized by progressive hemifacial atrophy. A case of Parry-Romberg syndrome with ocular involvement is reported. A 27-year-old male patient with Parry-Romberg syndrome was interdisciplinary investigated and treated. For reconstruction of hemifacial soft tissues a free vascular parascapular graft was performed. Opthalmological findings included an evident enophthalmos and an eyelid lag with keratopathy and epiphora. Furthermore pupillary disturbances and endothelial precipitates were detectable. Complete fundus examination showed a unilateral optic disc swelling, central vitreous opacities and peripheral pigmentary disturbances. In the presented case of Parry-Romberg syndrome a rare association to ophthalmological involvement could be found. Beside the enophthalmos and eyelid alterations, also a panuveitis with papillitis should be treated by steroid therapy.

Adult↗

Enhancing effects of fluorescein on beta-lactam rash. I: High incidence of cefclidin rashes in an ophthalmological volunteer trial.

Disseminated maculopapular eruptions were frequently observed in a volunteer trial of cefclidin's use in ophthalmological and neurological examinations (8/12; 67%) It appeared at 8-12 days (mean +/- SD, 9.6 +/- 1.1 days) from the initiation of the trial and subsided within 1-2 days (mean +/- SD, 1.8 +/- 0.4 days). Patch testing with cefclidin produced a +/- reaction in 1 of 8 cases, and the drug-induced lymphocyte stimulation test (DLST) elicited a positive response (SI: 2.8) in 1 of 8 and a weakly positive response (1.8 < or = SI < 2) in 2 of 8. From these findings, it seems likely that the eruptions may be partially mediated by delayed type hypersensitivity (DTH) reactions to cefclidin. No such eruption was observed in the phase II trial of cefclidin where only 2.8% of 1.122 volunteers developed the eruption. The volunteers were given both fluorescein and oxybuprocain in their eyes to measure ocular tension on days -1, 0, 1, 3, 5, 7, 9, 11, 13 and weeks 3, 5, 7 after the initiation of cefclidin. Fluorescein and/or oxybuprocain may affect cefclidin to induce these abnormal reactions in the volunteers.

Adult↗

Lipoid proteinosis: A case with ophthalmological and psychiatric findings.

Lipoid proteinosis (LP) is an uncommon, recessively inherited disorder. The disease usually has its onset in the newborn period and is manifested by hoarseness. The skin and mucous membrane involvement arises between the first and second year of age. A 14-year-old male presented with the complaint of blistering on various sites of his skin, from the age of 12 months, resulting in scarring. Ophthalmological and psychiatric findings also appeared during the clinical course. The histological findings of skin biopsy included extensive deposits of amorphous eosinophilic material in the papillary dermis. No known therapy exists for LP.

Adolescent↗

Midazolam and flumazenil in ophthalmology.

Midazolam's pharmacodynamic properties are used in ophthalmology in several indications: 1. to relieve anxiety and unwanted reactions during radial keratotomy in ambulatory patients; 2. to provide sedation and amnesia during cataract surgery using local anaesthesia, in combination with low doses of phenoperidine; 3. to induce general anaesthesia, combined with fentanyl and vecuronium, for intraocular surgery in the elderly; and 4. to decrease intraocular pressure. Flumazenil is not in routine use but is usually administered to antagonise the deleterious effects of midazolam on upper airway resistances and the respiration system and may be used to reverse "paradoxical", i.e. anxiogenic reactions to benzodiazepines in the elderly.

Aged↗

Smith-Lemli-Opitz syndrome: neuropathological and ophthalmological observations.

The case of a three-year-old boy with the Smith-Lemli-Opitz syndrome is reported. In addition to the constellation of skeletal and genital anomalies classically described in this syndrome, this patient had spontaneous opsoclonus-like eye movements, strabismus, lack of visual following responses and of opticokinetic reflexes. At autopsy the cerebellar vermis was found to be absent. There were retinal hemangiomas. Microscopical examinations showed loss of Purkinje cells and extensive neuronal degeneration within dentate nuclei, associated with patchy demyelination of cerebellar peduncles and central white matter. These findings may contribute to the explanation of the pathophysiology of opsoclonus and some of the neuro-ophthalmological findings.

Abnormalities, Multiple↗

Infantile spasms: a neuro-ophthalmological study.

A complete neuro-ophthalmological examination was made of 80 children affected by infantile spasms. Ocular features were inconstant and aspecific in the idiopathic, perinatal and postnatal groups. However, infantile spasms in the prenatal symptomatic group frequently were associated with ocular findings related to an ectodermic defect, as well as with brain defects. It is suggested that ocular findings are helpful in the investigation of infantile spasms, both in detecting associated brain malformations, and as an indication of whether further examination by computerized tomography is necessary.

Diagnosis, Differential↗

Neuro-ophthalmological evaluation of patients with myelomeningocele and Chiari malformations.

Twenty-eight patients (14 females and 14 males aged between four and 34 years) with myelomeningocele and Chiari malformations were examined neuro-ophthalmologically. All patients had been under proper neurosurgical care and no reduction of visual function was found that could be related to optic atrophy or other changes in the visual pathways. However, ocular motility disorders, including strabismus, were common, although no case of down-beat nystagmus was observed. Manifest strabismus was found in 11 patients. Esotropia with or without A-pattern was seen in nine patients. Spontaneous or gaze-related nystagmus and abnormal optokinetic nystagmus represented the most common disturbances of ocular motility. Abnormalities of horizontal eye-movements were more common than those of vertical eye-movements. Strabismus of the manifest type was combined with other ocular motility disturbances in most cases.

Adolescent↗

Benign paroxysmal tonic upgaze of childhood with ataxia. A neuro-ophthalmological syndrome of familial origin?

A new neuro-ophthalmological syndrome has been described recently, consisting of paroxysmal tonic upward deviation of the eyes with ataxia. Episodes occur daily and are always relieved by sleep. Onset is usually under one year of age and the symptoms gradually disappear during childhood. The authors describe three new patients in whom an autosomal dominant mode of inheritance was a constant finding, as well as clumsiness and delayed acquisition of independent gait. Treatment with levodopa was of clear benefit.

Age Factors↗

Children with blindness due to retinopathy of prematurity: a population-based study. Perinatal data, neurological and ophthalmological outcome.

A population-based group of 27 children with total blindness due to retinopathy of prematurity (ROP), born in Sweden from 1980 to 1990, was examined. They constituted all but two of the total of 29 children with total blindness due to ROP known to the national register of visually impaired children when reviewed from 1980 to 1 January 1995. All children had a gestational age of less than 31 weeks and most had had a complicated perinatal period. The retinal disease was discovered late, most often after it had already progressed to bilateral retinal detachment. Repeated vitreoretinal surgery had been performed in most children, but postoperative visual function did not improve. Three-quarters of the group had major neurological impairment (mental retardation, cerebral palsy, or epilepsy) at age 4 to 14 years. There was an impression that extensive ophthalmological efforts delayed neurodevelopmental assessments and examinations as well as adequate habilitation.

Adolescent↗

Ophthalmological findings in children born after intracytoplasmic sperm injection.

PURPOSE: To investigate visual function and ocular morphology in children born after intracytoplasmic sperm injection (ICSI) and compare them with visual function and ocular morphology in matched children born after spontaneous conception. METHODS: A total of 137 children born after ICSI and 159 control children were examined at the age of 5 years in a blinded study. The two groups were matched for age, gender and maternal age. The children underwent a detailed ophthalmological investigation by an ophthalmologist and an orthoptist. RESULTS: A total of 90.4% of the ICSI children had a visual acuity (VA) in the better eye of >or= 0.8, compared with 93.7% of the controls (NS). No significant differences were found between ICSI children and controls with regard to hyperopia, myopia, astigmatism, heterotropia, stereoscopic vision and convergence. One child with Goldenhar syndrome and one with Marcus Gunn syndrome were observed in the ICSI group. One child in the ICSI group was treated for retinopathy of prematurity. CONCLUSION: In this limited study group, no significant differences in visual function and/or ocular morphology were found between children born after ICSI and matched control children at the age of 5 years.

Case-Control Studies↗

Lithium effects on ophthalmological-electrophysiological parameters in young healthy volunteers.

The effects of lithium on the visual system in man have been tested. Ten healthy volunteers followed a 10-day lithium regimen. Lithium plasma levels in the range of 0.6-0.8 mmol/l were obtained. Clinically used ophthalmological-electrophysiological tests were performed. Most of the tests showed no significant change during the lithium treatment. The lithium regimen resulted in a strong reduction of the slow phase. Also the "Arden-ratio", measured by electrooculogram (EOG), was influenced by lithium. The "Arden-ratio" reflects the corneo-fundal potential in retinal illumination and dark adaptation and is influenced by the state of adaptation. All the demonstrated effects were fully reversible at the end of lithium administration. Possible mechanisms of the action of lithium are discussed (for example Ca2+-fluxes, c-AMP, cGMP). These lithium-induced effects on the visual system in man may give some hint of the individual responsiveness to lithium.

Adult↗

Paediatric and ophthalmologic observations in offspring of alcohol abusing mothers.

The offspring of nine women who had abused alcohol and drugs during pregnancy were studied. Of the 30 children, 10 had fetal alcohol syndrome (FAS) or fetal alcohol effects (FAE). Ophthalmological impairments associated with FAS--impaired vision, optic nerve hypoplasia, cataract, increased tortuosity of retinal vessels--were common. The severity of teratogenic lesions varied among the children and was often related to the level of abuse during pregnancy. Most women were unable to take care of their children during periods of substance abuse. All children with FAS/FAE had learning difficulties. Four mothers abstained from alcohol or drugs during some of their pregnancies and gave birth to children without birth defects.

Adult↗

Ophthalmological examination of patients in long-term treatment with tranexamic acid.

Fourteen patients with hereditary angioneurotic oedema (Quincke' oedema) treated for an average period of 6 years (ranging from 15 months to 8 years) with tranexamic acid was given an ophthalmological examination in order to reveal any possible toxic damages caused by the treatment. As large doses of tranexamic acid given to animals have been shown to result in retinal degeneration, the examination was focused on checking the retinal function. A complete ophthalmic examination revealed no toxic effects in the eyes of any of the patients. No retinal damages were found that could have been caused by the tranexamic acid. The central corneal thickness was normal.

Adolescent↗

Ophthalmological findings in nephropathia epidemica in Lapland.

We report ophthalmological findings in 15 cases of nephropathia epidemica. The patients, 13 men and 2 women, were 20 to 62 (mean 30) years of age. The onset of the disease was characterized by high fever, nausea, headache, abdominal pain, backache, somnolence, red throat, proteinuria, and oliguria. The symptoms subsided rapidly during the polyuria stage. Transitory myopia occurred in 8 patients (53%). Conjunctival injection and haemorrhages were seen in 3 patients (20%). One patient had acute glaucoma with oedema in the cornea and shallowing of the anterior chamber, with subsequent anterior uveitis and haemorrhages in the ocular fundus, and another patients had acute glaucoma. Three patients had photophobia which occurred in 2 patients without any glaucoma or anterior uveitis.

Adult↗

The ophthalmological findings in autosomal recessive severe juvenile arteriosclerosis.

Severe juvenile arteriosclerosis (JAS) is a rare, fatal disease with probably autosomal recessive inheritance. Precocious arterial murmurs and calcification of aorta and major arteries are associated with anaemia, growth retardation, prolonged systolic hypertension, gradual renal failure, gastrointestinal perforations, and early death. The ophthalmological findings in 5 boys and 1 girl between 7 and 17 years of age were retinal vascular tortuosity of main vessels, pre-capillary arterioles and post-capillary venules, sausage-like bulging of central main arterioles and microaneurysms of arterioles of the central fundus. Narrowing and local constriction was a typical feature of peripheral arterioles. The combination of findings documented by fluorescein angiography in 4 children was typical for severe juvenile arteriosclerosis.

Adolescent↗

Neuronal intranuclear inclusion disease. Clinical ophthalmological features and ophthalmic pathology.

Monozygotic twin sisters were afflicted by a chronic progressive neurological disease characterized by slurred speech, nystagmus and oculogyral spasms as well as further extrapyramidal and lower motor neuron abnormalities. At autopsy severe loss of nigral and craniospinal motor neurons was noted. In the nuclei of most nerve cell types of the central and peripheral nervous system, roundish inclusion bodies of 3 to 10 microns in diameter were found. Ocular pathology revealed the presence of identical inclusion bodies in the ganglion cells and ganglion cell loss in the posterior retina. Retinal astrocytosis and loss of myelinated axons of the optic nerve were interpreted as reactive features. No inclusions were found in the retinal pigment epithelium. Careful neuro-ophthalmological studies of the first-degree relatives revealed low b-wave of the ERG with other slight aberrations. These were assumed to represent either a carrier or a subclinical state of this presumably inherited disorder.

Adolescent↗

A model for testing the mutagenicity of excimer laser radiation in ophthalmology.

Because excimer laser radiation is applied more and more in therapeutic and refractive corneal surgery, the possibility of mutagenic side effects of this treatment should be considered. A mutagenicity model is presented approximating closely the ophthalmological conditions when mutagenic effects are studied: Heparinized human blood is filled into the anterior chamber of an enucleated cow eye. After irradiation of the cornea with the excimer laser, the blood is removed and whole blood cultures are set up. Lymphocytes are stimulated, fixed during metaphase, and scored for chromatid and chromosome aberrations. First results obtained with this model after 248 nm laser irradiation (500 J, 277 mJ/cm2, 10 or 20 Hz) revealed no mutagenic effects. However, when the blood was exposed under the same physical conditions in a quartz chamber (positive control), statistically highly significant increases of the yields of chromatid aberrations were observed.

Adult↗

Canine Tear Proteomics: A New Frontier in Veterinary Ophthalmology.

Canine tear proteomics is an emerging field with significant potential for advancing both veterinary ophthalmology and comparative biomedical research. Tears are a readily accessible, non-invasive biofluid that contain a mixture of proteins involved in ocular surface protection, immune defense, and intercellular signaling. In dogs, tear proteomics studies have revealed biomarkers associated with various ocular and non-ocular diseases including keratoconjunctivitis sicca (KCS), glaucoma, neoplasia, and diabetes mellitus. This review compiles all previous studies conducted on the comprehensive canine tear proteome and highlights some of the key tear proteomic studies in human biomedical research. Tear film composition, study design, technological advancements, and select tear proteins are discussed along with key protein alterations and their use as potential biomarkers of disease. Fundamental challenges, clinical implications, and future directions of this rapidly growing field are discussed in detail. As proteomic technology and analytical techniques continue to evolve, canine tear proteomics will become a valuable tool for the veterinary ophthalmologist, enabling the early identification and diagnosis of ocular disease as well as providing a means for monitoring treatment outcomes, disease progression, and overall prognosis for the canine patient.

Animals↗