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Purpose: In colobomatous eyes, the risk of retinal detachment increases with age. This study elucidates the anatomic conditions and pathologic process of retinal detachment associated with colobomas., Methods: The records, including histologic slides, of 14 children (1 day to 17 months old) and 7 adults (17 to 78 years old) with colobomas were examined., Results: In children, colobomas were associated with lethal malformations. The extracolobomatous inner retinal layers extended centrally, forming the intercalary membrane. Duplication of the outer retinal layers and a horizontal shift of Müllerian glia created a triangle and a locus minoris resistentiae adjacent to the laterally displaced pigment epithelium. Part of the locus was an incomplete layer of photoreceptors excluding Müllerian glia. In adults, atrophy of the intercalary membrane, manifested as central schisis, thinning of the neuroepithelium, and hole formation, was related to a paucity of blood vessels within and underneath the intercalary membrane and the size of colobomas. The margins featured blood vessels, pigment epithelial hypertrophy, and choroidal and scleral thickening in a compact, intertwined arrangement., Conclusions: Glial atrophy, schisis, and hole formation in the intercalary membrane and separation of the locus minoris resistentiae from the pigment epithelium can disrupt barriers to fluid flow and set the stage for rhegmatogenous retinal detachment. That process is exacerbated by scleral ectasia, increasing vitreous traction at the margin, and retinovascular ischemia within the intercalary membrane. A vascularized, compact margin resembling a laser barrier, found predominantly in adults, may protect against retinal detachment.

Purpose: We describe a 39-year-old Japanese woman with chronic angle closure glaucoma secondary to spherophakia and frail zonular fibres. The patient was 143 cm in height with short fingers and had no family history of eye problems. High intraocular pressure, total optic disc cupping and severe visual field constriction were found in the right eye., Methods: The patient was treated successfully with trabeculectomy in the right eye and laser iridotomy in the left eye., Conclusion: The clinical findings imply that this was a borderline case of Weil-Marchesani syndrome.

We report two cases clinically diagnosed as idiopathic uveal effusion syndrome, where indocyanine green angiogram suggest non-specific choroidal inflammation as the underlying cause. Treatment with non-steroidal anti-inflammatory drugs was beneficial.

Aims: To describe the causes of severe visual impairment and blindness in children in schools for the visually handicapped in the Czech Republic in 1998., Methods: Pupils attending all 10 primary schools for the visually handicapped were examined. A modified WHO/PBL eye examination record for children with blindness and low vision was used., Results: 229 children (146 males and 83 females) aged 6-15 years were included in the study: 47 children had severe visual impairment (20.5%) (visual acuity in their better eye less than 6/60), and 159 were blind (69.5%) (visual acuity in their better eye less than 3/60). Anatomically, the most affected parts of the eye were the retina (124, 54.2%), optic nerve (35, 15.3%), whole globe (25, 10.9%), lens (20, 8.7%), and uvea (12, 5.2%). Aetiologically (timing of insult leading to visual loss), the major cause of visual impairment was retinopathy of prematurity (ROP) (96, 41.9 %), followed by abnormalities of unknown timing of insult (97, 42.4%), and hereditary disease (21, 9.2%). In 90 children (40%), additional disabilities were present: mental disability (36, 16%), physical handicap (16, 7%), and/or a combination of both (19, 8%). It was estimated that 127 children (56%) suffer from visual impairment caused by potentially preventable and/or treatable conditions (for example, ROP, cataract, glaucoma)., Conclusions: Establishing a study group for comprehensive evaluation of causes of visual handicap in children in the Czech Republic, as well as for detailed analysis of present practice of screening for ROP was recommended.

Purpose: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP), a rare paraneoplastic syndrome causing visual loss in patients with systemic carcinoma., Results: A 70-year-old woman developed visual symptoms 13 months after surgery and local irradiation therapy for uterine cancer. Following bilateral external beam irradiation supplemented with subsequent drainage of subretinal fluid in the left eye, the visual acuity improved from 0.01 to 0.15 in this eye only. The visual acuity remained at this level until she died 4 1/4 years after the onset of eye symptoms., Conclusion: This is the fourth case that survived longer than 24 months after the onset of visual symptoms of the 22 previously reported cases with BDUMP. It demonstrates that radiotherapy may have a vision-preserving effect in this group of patients. The patient also developed two different paraneoplastic phenomena--a nephrotic syndrome before and BDUMP after treatment for uterine cancer.

An unusual type of rhegmatogenous retinal detachment is described and compared with a control group of patients with detached retina. Features of the condition, in addition to retinal detachment, include severe anterior and posterior uveitis, choroidal detachment, hypotony, deepened anterior chamber, posterior synechiae, iridophakodonesis, and a poor surgical and visual prognosis due to massive periretinal proliferation. The disease occurs in a disproportionately high ratio in blacks. These cases may be mistaken for uveitis, Harada's syndrome, the uveal effusion syndrome, dislocated lens, or malignant melanoma. It is postulated that the detached retina initiates a series of exaggerated pathophysiological changes in the eye, with the severe inflammation leading to choroidal detachment and hypotony. Such eyes are inoperable until the inflammation, hypotony, and choroidal detachment are reversed with corticosteroid treatment. The surgical and visual prognosis is very poor.

Three patients had during pregnancy visual disturbances caused by central serous choroidopathy. One of them had a central scotoma in her first and second pregnancy. The 2 other patients had a central scotoma in their first pregnancy. Symptoms disappeared spontaneously after delivery. Except for the ocular abnormalities the pregnancies were without complications. The complaints can be misinterpreted as pregnancy-related optic neuritis or compressive optic neuropathy, but careful biomicroscopy of the ocular fundus should avoid superfluous diagnostic and therapeutic measures.

The clinical and fluorescein angiographic findings in 2 cases of retinal detachment occurring in toxaemia of pregnancy are presented. Both cases showed angiographic evidence of abnormal choroidal leakage of dye unassociated with signs of retinal angiospasm. Both cases also showed angiographic evidence of delayed filling of the choriocapillaris. We believe this finding has not previously been reported. These observations provide further clinical evidence that retinal detachment in toxaemia of pregnancy is secondary to choroidal damage.

Thirty-three patients with central vein occlusion were evaluated for the effects of scatter xenon arc panretinal photocoagulation. Thirty-four eyes (2 eyes of 1 patient) were randomly divided into a treatment (15 eyes) and a nontreatment group (19 eyes). The average follow-up per eye has been 29 months. The visual prognosis was not significantly better in either group. There was no difference in the development of fundus neovascularisation between groups. None of the patients in the treated group developed rubeosis or neovascular glaucoma. Two patients in the nontreatment group initially had rubeosis and 3 developed it after entry. All 5 involved eyes developed neovascular glaucoma. A significantly greater amount of central retinal capillary drop-out was present in the untreated eyes that developed rubeosis than those untreated eyes which did not. The eyes in the treated group had a similar range of capillary drop-out area, but none of these eyes developed rubeosis or neovascular glaucoma. Panretinal photocoagulation prevented the development of rubeosis and neovascular glaucoma in susceptible eyes.

During the period 1980-6 102 patients from 14 European eye clinics were diagnosed as having birdshot chorioretinopathy (BSCR). All were Caucasian, and the series consisted of 47 men and 55 women, with a mean age of 52.5 years. The major findings in this rare disorder concern the ocular fundus. Most marked are the patterned distribution of depigmented spots without hyperpigmentation, radiation from the optic disc in association with vitritis, retinal vasculopathy with frequent cystoid macular oedema, and involvement of the optic nerve head. The distribution and appearance of the lesions suggest that they are related to the major choroidal veins. Complications of the disease were epiretinal membranes, retinal neovascularisation, recurrent vitreous haemorrhage, subretinal neovascular membranes occurring both in the juxtapapillary and macular regions, and optic atrophy. The medical history was not contributary. HLA testing showed very strong disease association with HLA A29 (95.8%). The evidence suggests that it is a single disease entity rather than a group of disorders because of the remarkable similarity in the ophthalmological appearance and the clinical course, combined with the exceptionally high association with HLA A29.

An unusual case of Waardenburg syndrome associated with a detailed and fixed pupil of the lighter eye is described. Pharmacological investigations were performed to localise the site of the pupillary lesion. A lack of cholinergic reactivity was demonstrated, possibly due to congenital agenesis of the sphincter pupillae. Sympathetic activity was not impaired. Spiral ganglion agenesis and midline congenital anomalies are common features in Waardenburg syndrome. These lesions as well as the fixed dilated pupil might be due to an embryonal inductive failure.

A case of bilateral microhaemangiomas of the pupillary borders in a 69-year-old woman is described. When first seen she presented with acute angle closure glaucoma and spontaneous hyphaema. Photographs and iris fluorescein angiography are presented and microhaemangiomas are discussed.

1. A subject who has suffered from central serous chorio-retinopathy in his left eye noticed differences in the colour of a given light as perceived by each eye alone. Standard screening tests (colour order and colour matching) indicated a tritan defect in the left eye; the right eye was normal on these tests.2. The subject was dichromatic in his left eye, trichromatic in his right. The left-eye distimulus colour-matching functions, spectral luminosity, and wave-length discrimination functions were indistinguishable from corresponding data for congenital tritanopia. Comparable right-eye data were normal.3. Spectral dichromatic colour matches were invariant under changes of intensity and under addition of a common light to both halves of the field. (Grassmann's laws of linearity are satisfied.)4. Increment threshold versus intensity (t.v.i.) curves for a blue (481.9 nm) test on a yellow background yielded the normal three branches (for Pi(4)(mu), Pi(1)(mu) and Pi(3)(mu) respectively) in the trichromatic eye. In the dichromatic eye a single mechanism was found. It had the field sensitivity of Pi(4)(mu) whether measured with the blue, or with a violet (429.5 nm) test. No trace of Pi(3)(mu) or Pi(1)(mu) was ever discovered in the tritanopic eye. Both are normal in the trichromatic eye.5. The field sensitivities of Pi(4), Pi(5) and Pi(3) of the normal eye are well fitted by linear combinations of the spectral colour-matching functions of the trichromatic eye. Pi(4) and Pi(5) of the dichromatic eye are well fitted by linear combinations of the tritanopic matching functions.6. Colour matches made by the trichromatic eye do not match when viewed by the tritanopic eye, almost certainly because the ocular media of the two eyes have wave-length-dependent differences in absorption. For the largest difference (430 nm) the trichromatic eye transmits about 2.2 times more light than its fellow. When allowance is made for these differences, the field sensitivities of Pi(4) and Pi(5) of the two eyes do not differ. The field sensitivities of Pi(4) and Pi(5) of the normal eye, on the other hand, differ significantly from those of the average spectra obtained on four normal trichromats by Stiles, in a way that cannot be attributed to differences in transmittance of ocular media.7. It is concluded that classical (or acquired) tritanopia is not distinguishable in its manifestations from congenital tritanopia; furthermore, tritanopia can be regarded as a reduced form of normal trichromacy, once allowances are made for absorption of the ocular media and for variations among normal trichromats.8. Despite extensive search no evidence could be uncovered which might exclude the hypothesis that the colour vision in tritanopia depends exclusively upon absorption in only two foveal cone pigments, one long-wave-absorbing and one medium-wave-absorbing.

The light and electron micrscopic findings in an eye enucleated from a 38-year-old woman with iris naevus (Cogan-Reese) syndrome are presented. The clinical manifestations of this disease, namely, iridic stromal matting, iridic nodule formation, and unilateral angle-closure glaucoma, appear to be secondary to an endothelial membrane on the anterior surface of the iris. The iris naevus syndrome is now thought to represent one end of the clinical spectrum of an iridocorneal endothelial syndrome which also includes those clinical entities classified previously as Chandler's syndrome and essential iris atrophy.

We examined 15 Japanese patients who had gyrate atrophy of the choroid and retina with hyperornithinaemia. Their visual acuities fell to 0.2 or worse in the second or third decade of life. Myopia developed late in the first decade, and the refractions decreased to -10 or -15 dioptres at age 20. Tunnel vision developed at approximately age 20. Our results suggested that the visual functions of Japanese patients were worse in the third decade or later than similarly affected Finnish patients.

Experimental and clinical observations have been evaluated in an attempt to gain further information about the effect of ciliary process enlargement on the angle of the anterior chamber. Swelling of the ciliary processes produced by the topical instillation of prostaglandin E2 into the eyes of rabbits has been described and compared to similar changes in a patient with ciliary block glaucoma. There seems little question but that most of what is manifest in the anterior chamber is but a reflection of very significant pathologic change in the posterior chamber.

Eyes from cardiomyopathic hamsters (UM-X7.1 strain) were examined histologically for evidence of ocular defects. Changes observed included microphthalmia, scleral ectasia, scleral rupture, keratoconus, retinal detachment, retinal dysplasia, retinal fragmentation, retinal thinning, fibrosis of iris and ciliary body, ectopia lentis, and cataract formation. Lesions characteristic of cardiomyopathic hamsters were observed in the myocardium and skeletal muscle. This strain may be a suitable animal model to study the pathogenesis of ocular changes seen in certain congenital connective tissue disorders in man.