Posterior optic nerve infarction after lower lid blepharoplasty.

We describe a case of acute and total loss of vision after lower lid blepharoplasty. This major complication followed minor cosmetic surgery. Magnetic resonance imaging (MRI) showed posterior segmental infarction of the optic nerve, a finding not previously demonstrated.

Choroidal folds and papilloedema.

AIMS: To assess the clinical and fluorescein angiographic features of choroidal folds seen in association with papilloedema. METHODS: In a retrospective study, the clinical data from a database on patients with choroidal folds (1963-97), including fundus photography and fluorescein angiography, from 32 patients (64 eyes) with choroidal folds in association with papilloedema were reviewed. The clinical and fluorescein angiographic features and the clinical course of choroidal folds in these patients are described. RESULTS: 32 patients had choroidal folds associated with papilloedema. Folds of two distinct categories were observed, either coarse folds or wrinkles. The folds persisted in all cases, even after resolution of papilloedema. Follow up ranged from 1 month to 20 years. Only one patient suffered permanent visual impairment as a result of a choroidal fold. CONCLUSIONS: Choroidal folds exist in two forms, coarse folds and wrinkles. They persist even after papilloedema has resolved. Final visual acuity did not appear to be affected by the presence of choroidal folds in the majority of patients.

Relationship of the distal optic nerve sheath to the circle of Zinn.

BACKGROUND: This study was designed to determine the relationship of the vascular circle of Zinn (CZ) and its branches to the termination of the subarachnoid space surrounding the optic nerve sheath, with particular reference to optic disc size. METHODS: Serial sections of 29 normal human optic nerves were performed at 6-microm intervals. The position of the CZ and subarachnoid space were measured with WILD Heerbrugg objective graticules. The results were analysed with reference to the optic disc size. RESULTS: The position of the CZ was variable but two major types were recognised. In type 1 the circle was located anterior to the distal sheath, and in type 2 posterior to the sheath. There were more small optic discs observed in type 2. CONCLUSION: In this study the CZ was more posteriorly located in small optic discs. This location may induce risk factors contributing to the development of ischaemic optic neuropathy. The combination of small discs, posterior placement of the CZ, and anatomical variations in the vascular pattern may predispose to ischaemic events.

Self-injurious behavior and Prader-Willi syndrome: behavioral forms and body locations.

With few exceptions (e.g., Lesch-Nyhan syndrome), the specific nature of self-injury in relation to identified genetic syndromes associated with mental retardation is poorly understood. In the present study we surveyed the families of 62 persons with Prader-Willi syndrome to determine the prevalence, topographies, and specific body locations of self-injurious behavior. Self-injury was reported for 81% of the participants. Skin-picking was the most prevalent form, with the front of the legs and head being disproportionately targeted as preferred self-injury body sites. Individuals with the 15q11-q13 deletion injured significantly more body sites than did individuals with maternal disomy 15. Results are discussed in relation to previous self-injury body site findings and implications for the relevance of syndrome-specific behavioral phenotypes.

The MRI appearance of the optic nerve sheath following fenestration for benign intracranial hypertension.

Optic nerve fenestration is carried out in cases of severe benign intracranial hypertension. This study aimed to monitor the optic nerve sheath appearances and orbital changes that occur following this procedure. The eight patients were all female with an average age of 37.3 years and a range of 20-58 years. The duration of symptoms was 2-6 years. Symptoms included headaches, diplopia and visual obscurations. Examination revealed severe papilledema. All investigations, including MRI, biochemical and immunological tests, were negative. Patients had fenestration of a 2 mm x 3 mm segment of the medial aspect of the optic nerve sheath. Imaging was obtained with a 1 T MRI machine using a head coil. Coronal, axial and sagittal 3 mm contiguous sections using STIR sequences with TR 4900 ms, IT 150 ms and TE 60 ms were obtained. Five patients showed clinical improvement. The post-operative MRI findings in four of these included a decreased volume of cerebrospinal fluid (CSF) around the optic nerve sheaths and a localized collection of fluid within the orbit. There were no MRI changes in the three patients with no clinical improvement. Decreased CSF volume around the optic nerve and a fluid collection within the orbit may indicate a favorable outcome in optic nerve fenestration.

Papilloedema, a complication of progressive diaphyseal dysplasia: a series of three case reports.

BACKGROUND/AIMS: Progressive diaphyseal dysplasia (PDD) is a rare, autosomal dominant, osteosclerotic dysplasia affecting both endochondrally and intramembranously derived bones. Severely affected patients can develop progressive stenosis of the optic canals and compressive optic neuropathy. Although raised intracranial pressure (ICP) has been described in patients with PDD in whom visual loss has occurred, the elevation of ICP in those patients has been thought to be either non-contributory or only partially responsible for the accompanying visual loss. METHODS: Three cases were reviewed and the clinical and radiological characteristics are described here. RESULTS: All three patients had bilateral optic disc swelling with no radiological evidence of either compressive optic neuropathy or thrombosis of the intracranial venous sinuses. The aetiology of the disc swelling was proved to be papilloedema in the first two cases and was probably the dominant cause in the third case. CONCLUSION: The visual loss documented in at least two of the three patients reported appears to be solely attributable to raised ICP. Normalisation of the ICP has led to an improvement and stabilisation of the visual function in all three patients. Patients with PDD probably require periodic ophthalmic assessments.

The venous closing pressure in central retinal vein obstruction.

OBJECTIVE: To assess the rate of change in the central retinal venous closing pressure in central retinal vein obstruction over time, and its relationship to visual acuity improvement and the development of rubeosis iridis. METHODS: Fifty patients presenting with central retinal vein obstruction of less than three months' duration, between the ages of 40 and 80 years, were reviewed prospectively. The central retinal venous closing pressure was measured by digital ocular compression. Patients were discharged from the study after the six-month visit. RESULTS: All patients had elevated venous closing pressure at presentation, whereas at six months only 24 patients had persistent elevation. Of 16 patients with lowering of the venous closing pressure within four months of onset of central retinal vein obstruction, 11 (69%) had two or more lines of visual acuity improvement. Only two of 10 patients (20%) developing lowering of the venous closing pressure thereafter had visual improvement. No patient developed rubeosis iridis after the venous closing pressure lowered. CONCLUSION: The central retinal venous closing pressure is raised in central retinal vein obstruction to about central retinal arterial diastolic pressure, and is its pathognomonic sign. This sign is easily elicited via digital pressure on the eyelid, and has prognostic significance for visual acuity improvement and the development of rubeosis iridis.

[Vascular anomalies and complications of optic nerve drusen]. / Anomalies et complications vasculaires dans les drusen du nerf optique.

BACKGROUND: Drusen of the optic disc are associated with slowly progressive optic neuropathy, characterized by accumulation of acellular laminated concretions in the prelaminar portion of the optic nerve. Papillary hemorrhages and vascular shunts have been reported with disc drusen but their frequency and clinical significance is not well known. METHODS: Retrospective study of fundus photographs of 116 patients with disc drusen referred to the National Hospital for Neurology and Neurosurgery, London, between 1965 and 1991. RESULTS: Hemorrhages were found in 23 eyes from 16/116 (13.8%) patients. Most cases (68.8%, 11/16 cases) occurred in patients with buried drusen, and most hemorrhages were deeply located. Vascular shunts were present in 6.9% (8/116 cases), most frequently in patients with exposed drusen (6/8 cases), most being of the venous type (7/8 cases). DISCUSSION: Vascular anomalies are not rare in disc drusen, as 20.7% (24/116 cases) of our patients presented either disc hemorrhages or shunt vessels. Their presence supports the hypothesis of the slowly progressive nature of disc drusen and the more advanced stage of optic neuropathy in such eyes.

Ocular neuromyotonia.

AIMS/BACKGROUND: Ocular neuromyotonia is characterised by spontaneous spasm of extraocular muscles and has been described in only 14 patients. Three further cases, two with unique features, are described, and the underlying mechanism reviewed in the light of recent experimental evidence implicating extracellular potassium concentration in causing spontaneous firing in normal and demyelinated axons. METHODS: Two patients had third nerve neuromyotonia, one due to compression by an internal carotid artery aneurysm, which has not been reported previously, while the other followed irradiation of a pituitary tumour, a common association in the published reports. Selective activation occurred in both, where neuromyotonic activity was triggered by prolonged voluntary activation of specific extraocular muscles with or without spread of activity to other third nerve muscles. The other patient had fourth nerve involvement, where spasms of the superior oblique muscle were induced only by alcohol, a phenomenon which has not been described. RESULTS: The two patients with third nerve involvement responded to carbamazepine and in one, an improvement in a chronic partial third nerve paresis occurred. The other has not required treatment and remains asymptomatic by refraining from alcohol. CONCLUSIONS: A careful examination, including the effects of prolonged voluntary muscle action is required to initiate episodes and to demonstrate selective activation. Imaging is mandatory to exclude compressive intracranial lesions, particularly where there is no history of pituitary fossa irradiation. A trial of anticonvulsants should be considered in all patients. Extracellular potassium may play a role in spontaneous firing and ephatic transmission in ocular neuromyotonia.

The retina.

Prominent amongst last year's diverse papers on the retina were a study of the clinical manifestations of dominant cerebellar ataxia with pigmentary macular dystrophy, a review of the pathogenesis of carcinoma associated retinopathy, the Ischaemic Optic Neuropathy Decompression Trial, and a review of congenital optic disc anomalies. Ocular complications of several neurosurgical procedures were also reported during this period.

Visual outcome of patients with idiopathic ischaemic and non-ischaemic retinal vasculitis.

This study investigated whether patients with ischaemic retinal vasculitis have a worse visual outcome than those with non-ischaemic disease. A retrospective study was made of 53 patients with idiopathic retinal vasculitis (RV), with minimum 5 year follow-up. Patients were categorised into ischaemic and non-ischaemic groups by fluorescein angiography. Visual outcome was determined by visual acuity at their last attendance. Twenty patients (38 eyes) had ischaemic RV; 33 patients (63 eyes) had non-ischaemic RV. At presentation there was no significant difference between the groups in the proportion of eyes with poor vision (6/60 or less). Ischaemic RV patients had a worse visual outcome than those with non-ischaemic RV: 13 of 38 (34%) eyes in the ischaemic group had a final poor vision compared with 4 of 63 (6%) eyes in the non-ischaemic group (Fisher's exact test, p = 0.0005).

Incidence of acute symptomatic toxoplasma retinochoroiditis in south London according to country of birth.

OBJECTIVE: To determine the incidence of acute symptomatic toxoplasma retinochoroiditis presenting to ophthalmologists for patients born in Britain and elsewhere. DESIGN: Population based, cross sectional study. SETTING: 11 districts in south Greater London. SUBJECTS: All patients presenting to NHS ophthalmologists with symptoms due to acute toxoplasma retinochoroiditis in 1992-3. MAIN OUTCOME MEASURE: Intraocular inflammation in association with a retinochoroidal scar, active adjoining retinitis, and IgG serum antibodies to toxoplasma. RESULTS: The estimated incidence of acute symptomatic retinochoroiditis for all people born in Britain was 0.4/100,000/year. If a mean of two symptomatic episodes per lifetime is assumed, 100 people born in Britain may be affected each year, about a fifth of the estimated 500-600 congenitally infected people born each year. CONCLUSIONS: A substantial proportion of people with acute symptomatic toxoplasma retinochoroiditis were born outside the country, and the number born in Britain was smaller than the number previously estimated to develop retinochoroidal lesions due to congenital toxoplasmosis. These findings suggest that prenatal screening for toxoplasmosis in Britain may be of limited benefit.