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Ocular and orbital echography.

Diagnostic ultrasound is a noninvasive, well tolerated, safe procedure with no toxicity which aids the ophthalmologist by demonstrating the integrity of the intraocular contents or the presence of tumors, retinal detachments, vitreous membranes or hemorrhage, foreign bodies or other structural changes in eyes with opaque media which cannot be adequately evaluated optically. Orbital mass lesions and inflammatory changes can be demonstrated and ultrasonic evaluation should be utilized early in the diagnostic evaluation of exophthalmus in combination with other radiographic techniques. The ultrasound examination does require a certain technical skill and experience in interpretation as well as judgment in order for it to be a reliable diagnostic test.

Cataract↗

[Myopia and cataract].

Myopia and cataracts are frequently associated: a cataract may lead to index myopia; high myopia is complicated by the frequent and early development of cataracts. In this case, given the retinal fragility, there are problems in operating techniques and indication. Extra-capsular extraction with in-the-bag implantation is the safest technique. A lens implant of well-calculated strength can best correct a preexistent myopia. The refractive goal varies according to the patient's age and activity. Intra and postoperative complications are rarer than expected with such abnormal eyes. A useful visual acuity can be obtained in 90% of the cases.

Aged↗

[Possible advantages of visual evoked potentials and computerized static perimetry in early diagnosis of glaucoma].

Aim of the studies was to evaluate usefulness of the visual evoked potentials and static perimetry in diagnosis of an early glaucoma. There were 12 examined cases, divided into 3 equal groups: 1) patients suspected for glaucoma basing on the characteristic picture of the optic disc, 2) patients after acute glaucoma in one eye with no history of the disease in the fellow eye, 3) patients with open angle glaucoma diagnosed in one eye. Abnormal results of the examinations were found only in the second group. The authors concluded that visual evoked potentials and static perimetry could be helpful in proper diagnosis of many difficult cases suspected for glaucoma.

Diagnosis, Computer-Assisted↗

[Persistent hyperplastic primary vitreous--diagnosis and differentiation].

PURPOSE: Presentation of the cases with a rare form of persistent hyperplastic primary vitreous. MATERIAL AND METHODS: 5 children, aged 3-14, were referred to the clinic with suspicion of neoplasm changes or congenital anomalies of the eyeball. Unilateral, pathological lesions, in the form of opaque membranes, partially or totally covered the optic disc, were found. In 2 cases persistent fragments of arteria hyaloidea were also observed. Diagnosis was mode basing on three mirror glass examination, ultrasonography and fluorescein angiography. In differential diagnosis, retinoblastoma and other entities determined in the literature as pseudo-retinoblastoma were taken into account. The children remained under clinical follow-up.

Adolescent↗

[Goniodysplasia in the Bouvier des Flandres].

Ophthalmologic examination of 72 Bouvier des Flandres dogs revealed variable degrees of goniodysgenesis in 37.5% of the animals. All dogs examined were normotensive and asymptomatic. Tonography in 4 dogs showed normal c-values for out-flow facility in all of them. Pedigree analysis demonstrated a recessive inheritance. Although the frequency of goniodysgenesis appears to be very high in this breed, the incidence of glaucoma remains low. Genetic counseling and careful selection of breeding stock showed that gonioscopically normal animals can be produced.

Animals↗

The frequency of genetic eye diseases in a genetic counseling center.

The frequency of genetic eye diseases in a genetic counseling center: In this study the incidence of eye diseases of genetic origin in patients attending our genetic counseling center for a period of almost six years is documented. The frequency of retinitis pigmentosa, congenital cataracts, lens dislocation, microphthalmos, retinoblastoma, congenital glaucoma, congenital ptosis, degenerative myopia, strabismus, optic atrophy, various genetic metabolic diseases have been investigated, and the results are presented. Preventive approaches and prenatal diagnostic possibilities are discussed and the importance of genetic counseling is emphasized.

Adolescent↗

Orbit and optic nerve.

The optic nerve is a fiber tract of the brain, and is not a true cranial nerve. Primary pathologic processes seen in the central nervous system and its linings can therefore affect the optic nerve. Along their course the fibers of the visual pathways are also susceptible to secondary extrinsic processes. The MR appearance of many of the primary and secondary processes that affect the optic nerve are characteristic. Where the imaging features of a process are not characteristic, a fairly narrow differential diagnosis can be suggested depending on the imaging appearance and anatomic location of the pathologic entity. This article is a categorical overview of the disease entities that may affect the optic nerve and tracts. Pathologic processes are organized according to the typical sites of involvement along the visual pathways, from the retina to the occipital cortex. Medical imaging, pathologic, clinical, and epidemiological highlights are presented.

Brain Diseases↗

The eye.

In recent years, the growing application of computed tomography (CT) and magnetic resonance (MR) imaging evaluation of the orbit and globe have dramatically increased the role of imaging and, hence, that of the radiologist in assessing ophthalmologic disorders. This article discusses magnetic resonance imaging features of the most common and less common ocular pathologies, with particular emphasis on the potential of magnetic resonance imaging in the field of ophthalmology.

Diagnosis, Differential↗

Cyclopia: craniofacial appearance on MR and three-dimensional CT.

In a case of alobar holoprosencephaly, a neonate who died several minutes after birth was found to have multiple facial and intracranial malformations, including cyclopia. Postmortem MR and CT findings included a single midline orbit, with two globes that contained separate lenses supplied by a single optic nerve. There were two separate superior orbital fissures and two separate lateral rectus muscles.

Abnormalities, Multiple↗

Subacute necrotizing encephalomyelopathy (Leigh's disease): a clinicopathologic study of ten cases.

Archival material and clinical data of 10 autopsy cases of Leigh's disease (LS), aged from 44 days to 9 years at death, were reviewed. Development delay, irregular respiration, feeding difficulty, and abnormal eye signs were the most common symptoms. Seizures (five of ten cases) were also frequent. In most patients, the diagnosis of LS was established postmortemly by the presence of symmetrical spongiform lesions affecting several brain centers at autopsy. The histologic examination disclosed associated hypertrophic cardiomyopathy in six cases, while fatty infiltration of the hepatocytes was observed in four cases. Microvesicular degeneration of the renal tubular epithelial cells was also seen in four cases. Our observations suggest that liver and kidney involvement is a component of LS and that this rare entity has to be considered as a polysystematic disorder, able to affect other organs besides the nervous system and the heart, a fact which has not been emphasized enough in the existing literature.

Autopsy↗

New autosomal-recessive syndrome of Leber congenital amaurosis, short stature, growth hormone insufficiency, mental retardation, hepatic dysfunction, and metabolic acidosis.

We report on a new autosomal-recessive syndrome in 4 Japanese children in 2 families. The key manifestations are Leber congenital amaurosis, short stature, growth hormone insufficiency, mental retardation, hepatic dysfunction, metabolic acidosis, and autosomal-recessive inheritance. There were no consanguineous marriages. Abnormal eye movements were noticed neonatally, and ophthalmological examinations showed no visual acuity, pigmentary retinal degeneration, and nonrecordable electroretinograms in all cases. Inadequate weight gain and short stature gradually became apparent after birth, and at present the height range is -4.6 - -7.2 SD (standard deviations). Developmental delay was noted at age 4 months, and the developmental quotient is 50-70 at present. Deterioration of development and convulsions were not recognized. Elevated serum aminotransferase levels and metabolic acidosis were also found at age 4 months. Proximal renal tubular acidosis was clarified by bicarbonate tolerance tests in 1 case, and may have caused metabolic acidosis. Growth hormone secretion was insufficient by insulin tolerance test in 3 cases. One year of growth hormone therapy in 2 cases did not affect growth velocity. Hepatic dysfunction and metabolic acidosis ameliorated later. No renal cysts were found. A cranial computed tomographic scan and magnetic resonance imaging showed normal findings. Amino acids, organic acids, and very long chain fatty acid levels in plasma were all normal in the 3 cases examined. Histopathological and mitochondrial DNA analyses showed no evidence of mitochondrial disorders.

Acidosis↗