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The purpose of this case series is to show photographically the varying clinical appearance of granular corneal dystrophy in three generations of one family and to review the genetic basis of this and related conditions.We present cases for four affected individuals along with slitlamp biomicroscopic photographs.A review of the photographs and the literature suggests that the abnormal keratoepithelin first appears in the superficial cornea as faint subepithelial opacities. With time, these become arranged in the curved lines of a vortex pattern, after which the deposits become scattered in no particular pattern and at all levels of the cornea. In this family, corneal erosions are a regular feature. Mutations of the gene coding for keratoepithelin (beta ig-h3) may give rise to variable clinical manifestations.

A case is reported in which large colobomas of the retina and choroid are found bilaterally in a young female who also has a pituitary tumor and mental retardation. Visual field data are presented and correlated with the retinal appearances. Relevant embryology is reviewed, and a table of systemic abnormalities associated with retinal/choroidal colobomas is presented.

Examination of the ocular fundus via indirect ophthalmoscopy gives the clinician an impression of foveal position relative to the nerve head. In some patients, the fovea appears to be in an unusual position (i.e., ectopic): it may appear to be higher or lower than expected, or closer to or farther from the nerve head. There is little published quantitative information on this subject. The purpose of this study was to examine foveal position in a group of normal adult eyes, so that clinicians and other researchers will be able to determine on a more objective basis whether or not a given patient shows foveal ectopia. Using ocular fundus photographs for 446 normal adult eyes, we found the foveal center to be, on average, 6.11 degrees +/- 3.32 degrees below a horizontal line bisecting the nerve head. For a smaller sample of 66 eyes, we found the average distance between the nerve head and foveal centers to be 4.93 +/- 0.33 mm (right eye) and 4.88 +/- 0.36 mm (left eye). Correlations of these data for right and left eyes are also examined. Nerve head data for the group of 66 right eyes were also analyzed to yield dimensions of a best-fitting ellipse: the mean minor axis was 1.75 +/- 0.2 mm; the mean major axis 1.95 +/- 0.2 mm. Ectopia (heterotopia) of the fovea has been found in association with chorioretinitis, fibrous traction bands, and/or colobomas of the choroid and optic nerve (including anomalous insertion of the optic nerve), microcephalus, and microphthalmia. A number of separate cases with anomalous nerve heads and/or foveal positions are discussed in this paper.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of bilateral inferior conus is reported; relative scotomas were found to be congruent with the location of the conus. Existing theories, proposed to account for scotomas associated with inferior conus, are discussed. A new theory is proposed.

The ophthalmic literature dealing with diseases of the optic nerve, the optic tracts, and the visual cortex was reviewed for the period November 1975 through November 1976. Twenty-nine papers on topics of interest to optometrists were abstracted. The main areas of interest include: papilledema and optic atrophy (with ophthalmoscopic signs of both optic atrophy and papilledema); giant-cell arteritis; papillitis; interesting malformations of the face, palate, and orbital position that occur in conjunction with microphthalmus, situs inversus, and hypoplasia and aplasia of the optic nerve; the proposed association of myopia with unusual eyebrows; myelinated nerve fibers at the nerve head; pigment anomalies; the continuing discussion of nerve-head blood supply; an unexpected cause for nerve-head neovascularization; the importance of the swinging-flashlight test in the diagnosis of glaucoma; an unusual type of glaucomatous cupping; doubts about the peripapillary "halo" as a sign of glaucoma; new uses for old field tests; and new methods of ocular photography.

The ophthalmic literature dealing with diseases of the optic nerve, tracts, and visual cortex was reviewed for the period November 1977 to December 1978. Fifty-nine papers of interest to optometrists have been abstracted. Main areas of interest include: role of axoplasmic transport in production of disc edema and cotton-wool spots; ocular effects of local and systemic vascular disturbances; congenital abnormalities of the eye alone and in association with other bodily malformation; field testing, including an ingenious ophthalmoscopic method which can lead to detection of very small scotomas; a new quantitative method for assessing integrity of the optic nerve in multiple sclerosis and related conditions; stimulating questions on the development of optic atrophy and on its relation to retinal vascularity; considerations of acquired and congenital abnormalities of the cavernous sinus and their ocular manifestations; swinging-flashlight test in hemianoptic patients; a case of subacute sclerosing panecephalitis; methods to enhance magnification during opthalmoscopy; methods enabling a closer investigation of the retinal nerve fiber layer.

A theory is proposed to explain the direction of the optic nerve on the basis of differing embryonic growth of axons on opposite sides of the nerve head. Several ophthalmoscopic indicators of the direction of the optic nerve are discussed. Some clinical implications are presented.

Ellipses were fitted to 129 nerve heads and corresponding blind spots in an attempt to determine how faithfully the elliptical nature of the nerve head is reflected in the blind spot. The blind spots are found to be 2 to 3 times more elliptical (based on the ratio of the long axis to the short axis) than the nerve heads. The correlation between the ratios mentioned above is found to be negligible (Pearson r = 0.07 for Goldmann 1-2 blind spots and 0.18 for Goldmann 1-4 blind spots).

A 36-year-old male had been constantly wearing a patch to avoid diplopia since an operation on the right parietal area of the brain. Postsurgical visual field evaluation indicated a complete bilateral left hemianopia with macular splitting. The patient had a constant alternating comitant exotropia with no suppression. By terminating the patching, establishing the appropriate fixing eye, and applying relieving prisms, diplopia was removed. Once binocularity was thus established, it was found (over the course of 7 months) that binocular function was possible with diminishing amounts of prism power. During this 7-month period, measurements of the patient's heterophoria, motor fusion, and sensory fusion revealed that he adapted from his occlusion-induced exotropia toward an habitual heterophoria. Such vergence adaptation has been defined as orthophorization. The occurrence of vergence adaptation after sensory fusion indicates that sensory fusion plays an important role in the etiology of prism adaptation.

Ophthalmoscopic appearances of congenital and acquired presumed ocular toxoplasmosis are described. Four illustrative cases of presumed ocular toxoplasmosis are discussed, with some emphasis on related general health history. Some speculations are offered to account for the varied ophthalmoscopic appearances of these lesions.

Ocular fundus photographs of the optic nerve head of 396 normal eyes were obtained. An ellipse was fitted to points digitized around the circumference of each nerve head. The length of the major and minor axes, and the orientation of the major axes were determined. This paper gives frequency distributions for the degree of ellipticalness of 396 nerve heads. The long axis is on average 11.5% longer than the short axis and the top of the long axis tends on average to be tilted toward the midline of the head by 10 degrees. In addition, it appears that the population of Elschnig type III nerve heads includes higher elliptical values than that for the Elschnig type II nerve heads.

Visual function and ocular structures were evaluated in a family with retinitis pigmentosa (RP) by dark adaptometry, electroretinography (ERG), perimetry, and fundus photography. All members examined showed gross rod dysfunction by dark adaptometry and ERG. The inferior and nasal segments of each patient's retina showed denser accumulations of pigment than the other segments. All patients showed superior field losses, the extent of which was correlated with the age and sex of the individual. The clinical impression formed from the assessment of visual fields in this sample was that males are more severely affected than females.

L'auteur presente 2 cas d'anomalie congenitale du nerf crânien associee a une perte au niveau du champ visuel autre qu'une constriction concentrique, qu'une restriction peripherique, qu'une perte ceontro-cœcale temporale ou qu'un scotome

Ocular fundus photographs of 100 eyes were obtained from an institutionalized population of patients with Down's Syndrome (66 males, 34 females). The vascularity of the optic nerve head was assessed in each photograph by counting the number of arterioles, venules, and fine vessels crossing the disc margin. The results of this analysis were compared to similar data obtained from 100 ocular fundus photographs of normal patients and show that there are more large vessels crossing the disc margin in those with Down's Syndrome.

The ophthalmic literature dealing with diseases of the optic nerve, tracts, and visual cortex was reviewed for the period November 1976 to November 1977. Thirty-three papers of interest to optometrists have been abstracted. Main areas of interest include response of posterior segment to anterior-segment disturbances; structural anomalies in nerve-head region, including a new potential cause for hypoplasia of optic nerve; speculation on possible common origins of RPE/Bruch's membrane disturbances and endothelium/Descemet's membrane disturbances; a vascular hypothesis for pathogenesis of optic-nerve-head drusen; an explanation for reversibility of infantile glaucomatous cupping; glaucomatocyclitic crisis; the role of axoplasmic flow in papilledema; new developments in field testing for early glaucomatous loss; effects of cerebral vascular accidents on color vision; and two interesting instances of systemic disease with ocular manifestations.

A 55-year-old white male underwent an uneventful cataract extraction with implantation of a posterior chamber intraocular lens (IOL) in his left eye. Two years after the cataract surgery, his visual acuity decreased due to opacification of the posterior lens capsule. This report addresses the effect of laser capsulotomy on the patient's visual acuity, intraocular pressure (IOP), and visual field. Although the first two have improved nicely, the size of the last remains considerably below the norm for his phakic contemporaries.

Application of computer digitizing methods to conventional kinetic visual field test data permits mathematical analysis of these data. In some cases this provides more information than is available by a simple inspection of the field charts. This is demonstrated by analysis of multiple isopter data for a patient with open-angle glaucoma and a patient with retinitis pigmentosa.

Arterioles of the human retinal circulation are directly visible and exhibit wide variations in their course. Not only is there marked variation in arteriolar tortuosity within a given age group, but also it has been proposed that the degree of arteriolar tortuosity is different in younger and older age groups. This paper reports on the development of a computer-assisted technique for measurement of arteriolar tortuosity and on the frequency distribution of this index in younger (mean age 22 years) and older (mean age 54.3 years) populations without hypertension.

Normal anatomical variations of the eye are a constant source of interest to the clinician. This is particularly true for the optic-nerve head, where neural, vascular, and supportive tissue converge, each with its own variations. Such diverse anomalies as cleft palate, reduced visual acuity, visual-field loss, and distortions of the optic-nerve head may all be related to a single factor affecting embryonic or later development. This paper presents a number of nerve-head malformations in the context of their embryonic development, clinical appearance, and implications for visual function.

Intraocular pressures (IOP's) were measured using the Digilab Pneuma-tonometer with the subject in both the sitting and supine positions. The IOP with the Pneuma-tonometer was greater (17.03 mm Hg) in the supine position than in the sitting position (12.90 mm Hg). The IOP measured with the Pneuma-tonometer, with the subject sitting, was similar to the IOP measured with the Goldmann applanation tonometer (13.42 mm Hg). Inasmuch as IOP tends to be lower with the patient in a sitting position, the clinician should be alert to the possibility that some patients with borderline Goldmann IOP's may have pressures well above the normal range when they are lying down. Due to the continuous recording provided by the Pneuma-tonometer, the ocular pulse pressure can be measured. We found a mean ocular pulse pressure amplitude of 1.77 mm Hg. All values are shown plus or minus 1 SD. Comparison of the ocular pulse pressure amplitude for the two eyes could aid in detecting patients with suspected carotid artery stenosis.

Development of Isolated Female Larvae of the Potato-Root Eelworm (Heterodera rostochiensis Woll.)

Goldmann perimetry was done on 11 pseudophakic subjects and results were compared to those of age-matched phakic subjects and a third group of aphakic individuals who were wearing rigid gas permeable contact lenses. Testing showed that the I-3 and I-4 fields of the subjects who had had cataract surgery were generally constricted when compared to the normal phakic population. Possible explanations for this include media clarity not being as important a factor in determining field size as was previously thought, peripheral retinal changes, and implant material affecting contrast sensitivity.

One predicted cost of female infidelity in socially monogamous\ud species is that cuckolded males should provide less parental\ud care. This relationship is robust across species, but evidence is ambiguous\ud within species. We do not know whether individual males reduce\ud their care when paired with cheating females compared with when\ud paired with faithful females (within-male adjustment) or, alternatively,\ud if the males that pair with cheating females are the same males that\ud provide less parental care in general (between-male effect). Our exceptionally\ud extensive long-term data set of repeated observations of a wild\ud passerine allows us to disentangle paternal care adjustment within\ud males—within pairs and between males—while accounting for environmental\ud variables. We found a within-male adjustment of paternal\ud provisioning, but not incubation effort, relative to the cuckoldry in\ud their nest. This effect was mainly driven by females differing consistently\ud in their fidelity. There was no evidence that this within-male adjustment\ud also took place across broods with the same female, and we\ud found no between-male effect. Interestingly, males that gained more\ud extrapair paternity provided less care. Data from a cross-foster experiment\ud suggested that males did not use kin recognition to assess paternity.\ud Our results provide insight into the role of individual variation in\ud parental care and mating systems.