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At least 865 records · Page 48Linked to original sources
PubMedDate not supplied

[Top of the basilar artery syndrome: clinico-radiological aspects of 25 patients].

UNLABELLED: INTRODUCTION, PATIENTS AND METHODS: We studied 25 patients, 16 women and 9 men (average age 64.8 years; SD 8.6; range from 36 to 79 years) admitted to our hospital with clinical findings compatible with obstruction at the level of the bifurcation of the basilar artery. The patients were selected on clinical and neuroradiological criteria. RESULTS AND CONCLUSIONS: All patients included in the study had presented with two or more infarcts in the vertebro-basilar territory, related to pathology of the rostral region of the basilar artery. Diagnosis was confirmed on CT and MRI. Infarcts were found in the thalamus, brain-stem, cerebellum and parieto-occipital lobe. Infarct of the thalamus associated with another infarct in a different region was the most frequent lesion. The CT-MRI findings in the 25 cases were: 14 patients had unilateral thalamic infarcts associated with another infarct. There were 4 patients with bilateral thalamic infarcts and 5 with bilateral occipital infarcts. In 11 patients the occipital infarct was associated with another infarct at a different level, and 6 patients each had a cerebellar infarct together with a brain-stem infarct. In 12 patients the lesions were localized to three or more areas. The commonest clinical manifestations were: motor deficit (68%), abnormal eye movements (48%), cerebellar dysfunction (40%), alteration of the level of consciousness (32%), visual field defects (20%), pupil anomalies (16%). The risk factors most frequently associated were: arterial hypertension (64%), a history of cerebrovascular accident (28%), smoking (28%), diabetes mellitus (24%) and atrial fibrillation (20%). Mortality was 8%. Unlike the classical descriptions, motor deficit was the most frequent symptom in our series.

Adult↗
PubMed1999

[Degenerative diseases affecting vision].

Degenerative-dystrophic disorders threatening the vision may occur in the cornea, in the lens, in the vitreous body, in the retina and in the optic nerve. Corneal degenerative disorders are rare and mainly of hereditary origin. Keratoplasty is a successful therapeutical method. The surgical treatment of cataract is one of the most fruitful field of ophthalmic surgeons even if the development of cataracts is not in every relations understood. Degenerative disorders of the retina are not subjects of successful treatment, though some new therapeutical modalities are promising. Retinal pigmentepithelium-, and neuroepithelium-transplantation and other surgical procedures in age-related macular degeneration are new approaches in previously applied medical treatment modalities. From clinical point of view degenerative disorders of the retina have the most significance as they occur in elderly and have severe social consequences as well. Excessive degenerative myopia may be reduced in selected cases by surgical approaches. Through encouraging results of experimental and clinical research new therapeutical methods were introduced in the everyday praxis, knowledge some of these new methods are worthy for physicians outside the field of ophthalmology as well.

Cataract Extraction↗
PubMed1999

Persistent hyperplastic primary vitreous with myopia: a case study.

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) is a congenital disorder that manifests a range of ocular anomalies, including leukocoria, microphthalmia, cataract, and a retrolental fibrovascular membrane. In general, the prognosis for visual acuity with PHPV has been poor. A recent report on six patients who have myopia associated with PHPV showed that these patients were not microphthalmic, did not manifest leukocoria, and showed a mean visual acuity at final followup of 20/160. CASE REPORT: The case of a 3-year-old boy with myopic PHPV of the left eye is presented. Full-time wear of the cycloplegic refraction, combined with patching of the non-amblyopic eye, resulted in an improvement in visual acuity. RESULTS: PHPV with myopia may constitute a distinct subtype of PHPV. Patients with myopic PHPV tend to seek treatment later than patients with anterior or posterior PHPV as a result of the lack of microphthalmia and leukocoria. These individuals do not show the long-term complications, such as secondary glaucoma and intraocular hemorrhages, associated with PHPV. CONCLUSION: The acuity improvement in this case of myopic PHPV suggests that a more conservative approach to its surgical management may be appropriate while pursuing refractive correction and amblyopia therapy to attain the best acuity possible.

Child, Preschool↗
PubMed1999

Congenital ocular anomalies and ventricular septal defect in a dromedary camel (Camelus dromedarius).

A 5-wk-old female dromedary camel (Camelus dromedarius) was clinically diagnosed with bilateral corneal dermoids, incomplete congenital cataracts, a left persistent hyaloid artery (PHA), and a ventricular septal defect (VSD). The corneal dermoids were removed by lamellar keratectomy, and vision improved in the left eye. Thirteen months after dermoid surgery, the calf was presented for enlargement of the right eye. Glaucoma was confirmed in the right eye, and corneal fibrosis and cataract were noted in the left eye. Persistence of the VSD was confirmed by cardiac ultrasonography. The calf was euthanized, and necropsy findings confirmed VSD. Histopathologic examination revealed bilateral corneal thinning and fibrosis, cataracts with retrolental fibroplasia, and retinal dysplasia. Additional changes in the right globe were anterior segment dysgenesis, ruptured lens capsule, chronic phacoclastic uveitis, and retinal separation. The PHA was confirmed in the left eye.

Animals↗
PubMedDate not supplied

Incising the thick retrolental fibrovascular tissue with a hooked sclerotome in persistent hyperplastic primary vitreous.

A technique for incising thick retrolental fibrovascular tissue and extensive cyclitic membrane is reported in a case of anterior persistent hyperplastic primary vitreous. A membranectomy was performed in a 1-month-old post-lensectomy baby via a limbal approach. A sclerotome tip was hooked to cut through an extremely thick fibrovascular tissue by rotating the sclerotome by its grip. Sutherland microscissors (Grieshaber, Switzerland) and a vitrectomy cutter were used for further membranectomy. The baby was followed-up until age 18 months. A total of 3 membranectomy sessions were required because of rapid cyclitic membrane formation, severe centripetal retraction of the membrane on the ciliary processes, and posterior synechia. Thorough membranectomy and cutting the iris edge maintained a clear pupillary area during the 13-month postoperative period. Extremely thick retrolental fibrovascular tissue is a challenging condition that can be dealt with by delicate instrumentation.

Anterior Eye Segment↗
PubMed1999

A three generations family with blepharo-naso-facial malformations suggestive of Pashayan syndrome.

Blepharo-naso-facial syndrome, described by Pashayan et al. (10), is characterized by telecanthus, lateral displacement and stenosis of lacrimal puncta, bulky nose, mask-like facies, trapezoïdal upper lip, torsion dystonia and mental retardation. We report on a family with this rare malformation syndrome, confirming the existence of this syndrome and its dominant inheritance. The proband had a fleshy nose, a prominant nose bridge, an hypoplastic midface, telecanthus with temporal displacement of puncta, lacrimal excretory obstruction. CNS torsion dystonia, increased deep tendon reflexes, Babinski reflexes, poor coordination and joint laxity. The proband's mother, brother and maternal grandfather also showed manifestations of the syndrome. The proband and his brother had delayed developmental milestones. Hearing impairment was present in the proband, his mother and his grandfather but was absent in the proband's brother. The blepharonasofacial syndrome was described by Pashayan et al. (10) in four members of one family, two male and one female sib and their mother. Two other sibs were unaffected. Many of the features of the blepharo-facio-nasal syndrome also occur in other well known syndromes i.e. Waardenburg syndrome. The pedigrees of the family of Pashayan et al. (10) and of our family are compatible with Mendelian dominant inheritance, either autosomal or X-linked. X-linked dominant inheritance cannot be ruled out except by male-to-male transmission, which does not occur in these families. Pashayan et al. (10) suggested that an autosomal gene with variable expressivity appears more likely. More families are needed for defining the transmission of the condition and for mapping the gene involved in the blepharo-naso-facial syndrome.

Abnormalities, Multiple↗
PubMed2000

Imaging of the pediatric orbit.

This article addresses the embryology of the eye, the imaging of common congenital malformations involving the globe, and imaging features of common retro-ocular masses. Clinical entities resulting in alterations in the size and contour, and those producing leukokoria, also are discussed.

Child↗
PubMed2000

[Alternating hemiplegia of childhoood and oculomotor anomalies].

BACKGROUND: Alternating hemiplegia of childhood is a syndrome which begins in the first year of life. It is characterized by repeated attacks of uni-or bilateral hemiplegia or hemiparesia. In most cases paroxysmal manifestations are observed: movements or dystonia++ attacks, episodic nystagmus, abnormal eye movements and disturbance of the neurovegetative system, predominantly in the first year of life. ANALYSIS: In half of the cases, neurological anomalies begin during the neonatal period with a non characteristic aspect. Typical attacks take place after one year of life, sometimes associated with partial epilepsy. In a quarter of cases, the oculomotor anomalies have been known since early life. The diagnosis is made prior to one year on the basis of associated oculomotor anomalies and other symptoms without EEG arguments for epilepsy. Paroxysmal nystagmus is always found. One eye is affect in most cases, generally with horizontal and seldom with vertical movements of large variable pendular amplitude. One eye with nystagmus and the other with mydriasis is sometimes reported. Most attacks last from 30 sec to 3 min. Paroxysmal strabismus described in half of the cases seems to be generally unilateral internuclear transitory ophthalmoplegia. Finally, ocular deviations on the hemiparetic side are described. They are generally unique or sometimes associated with head deviation. Spontaneous blinking is reduced. CONCLUSION: Alternating hemiplegia of childhood is a non-epileptic sporadic, paroxysmal manifestation of unknown pathogenesis. Prognosis is poor. The presence of oculomotor signs suggests the diagnosis.

Diagnosis, Differential↗
PubMed1979

[The use of computerized axial x-ray-tomography (EMI-scan) in ophthalmology (author's transl)].

There now exist protocols for specific scanning which make it possible to obtain tranverse sections of a few millimetres in thickness of the orbits and their contents, with minimum sweep and with a very low dose of radiation for the lens. Normal scanner anatomical findings in the orbit may thus be defined for the eyeball (outer coats, vitreous, lens) as well as retrobulbar structures (optic nerve, oculomotor muscles in particular). Intraocular tumours (e.g. retinoblastoma) are visible on enlarged scan films. The value of scannings lies above all in the determination of the site, size and shape of orbital lesions and, where applicable, their relations with neighbouring extra- and intra-cerebral structures. In addition, certain conditions such as exophthalmos during hyperthyroidism have characteristic scan findings. It nevertheless remains difficult to attempt to predict a histological diagnosis on the basis of scan findings, the risk of a false positive being particularly significant. Particular emphasis must be placed upon the contribution of techniques of treatment of the films obtained (enlargement, contrast media, histograms, lateral views) as well as the complementary use of other methods of radiological investigation (in particular arteriography and phlebography) which scanning cannot replace but which it should usually precede since it is atraumatic and non-invase.

Eye↗
PubMed2000

[Strabismus and binocular vision disorders in premature infants with retinopathy treated with cryotherapy].

PURPOSE: To assess frequency of refraction errors, abnormal eye position and binocular vision disorders in children with retinopathy treated with cryotherapy. MATERIAL AND METHODS: Subjects of the study were 67 children, aged 2-7, treated with cryotherapy for active phase retinopathy. RESULTS: The largest group consisted of children with good visual acuity (48%). Refraction error was found in 94% of the examined eyes. Hypermetropia was more common (54%) than myopia (40%). Majority of the examined children had squint, and esotropia was much more frequent (52%). The deviation in 37% of children was over 10 degrees. In the group with birth weight under 1000 grams as well as in those with birth weight 1000-1500 grams the squint occurred in 48%, and in children weighing over 1500 grams in 4.0%. Only 54% of the studied children had normal binocular vision. CONCLUSIONS: Retinopathy of prematurity is a risk factor for development of refraction errors, strabismus and amblyopia. In infants with retinopathy of prematurity treated with cryotherapy it is necessary to have vision check-ups during their early childhood years.

Child↗
PubMedDate not supplied

Ocular findings in glutaric aciduria type 1.

PURPOSE: To determine the nature and course of ocular abnormalities in glutaric aciduria (acidemia) type 1 (GA1). METHODS: Fifteen children with GA1 have been studied in the Republic of Ireland. A retrospective review of the records of the 6 children who died during their illness and prospective clinical examination of 9 survivors were performed. RESULTS: Seven of the 15 children had abnormal eye findings. Ocular complications included intraretinal hemorrhages, cataract, gaze palsy, strabismus, ametropia, and pigmentary retinopathy. CONCLUSION: Ocular involvement is common in glutaric aciduria. Complete ophthalmologic evaluation is recommended in all patients suspected to have this rare disease. Intraretinal hemorrhages due to GA1 could be misinterpreted as resulting from child abuse, and it is important to include this disorder with the differential diagnosis of child abuse.

Amino Acid Metabolism, Inborn Errors↗
PubMed2000

[Fabry's disease; towards a treatment].

Fabry's disease, deficiency of the enzyme alpha-galactosidase A, is an X-linked lysosomal storage disorder. Clinical symptoms are caused by continuous deposition of specific glycolipids in endothelial cells, neural cells, skin and cornea. These depositions give rise to skin (angiokeratoma) and eye abnormalities (cornea verticillata), acroparaesthesias and anhidrosis and later in life cause renal insufficiency and cardiovascular complications. Hemizygous males suffer from Fabry's disease, whereas female carriers (heterozygotes) are usually asymptomatic. Recently, an atypical presentation of Fabry's disease was described in males who only presented with cardiac involvement. Therefore, the actual number of Fabry patients in the Netherlands could be higher than the predicted 300. Diagnosis in males can be established by measuring alpha-galactosidase enzyme activity in plasma, leukocytes or fibroblasts. Apart from kidney transplantation only symptomatic therapy is available today. Enzyme supplementation therapy (as shown in Gaucher's disease) and substrate deprivation are possible ways of treatment in the future.

Cardiomyopathy, Hypertrophic↗
PubMed2001

Ophthalmologic disease in children.

MR imaging has become essential in the evaluation of a spectrum of ophthalmologic diseases in children. Orbit, globe, optic nerve, and optic tract disease processes that are frequently evaluated by MR imaging include congenital malformations, and inflammatory, neoplastic, and traumatic lesions. MR imaging evaluation aids in differentiating these lesions for diagnosis, the extent of disease, treatment planning, follow-up, and prognosis.

Child↗
PubMed2001

Fraser syndrome with bladder pseudoexstrophy.

We report a one year old girl with Fraser Syndrome (the association of craniofacial abnormalities, syndactaly and cryptophlamos) and multiple urogenital abnormalities including clitorimegaly, left renal agenesis and a unique urinary bladder exstrophy variant (psuedoexstrophy) with intact bladder which herniates through the lower abdominal wall defect.

Abnormalities, Multiple↗