Richardson Cross lecture 1989 amblyopia--an historical perspective.
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Biomedical subjects
Publications and source records attributed to P Fells.
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Visual loss following strabismus is rare and usually follows inadvertent perforation of the globe at the time of surgery. Previous studies have reported that the incidence of this complication occurs in 8% to 12.1% of patients undergoing conventional strabismus surgery, and higher incidences have been reported for posterior fixation sutures. We conducted a prospective study to determine the incidence of this complication in our patients. We identified one case of globe perforation in 67 patients (100 eyes). Twenty-two patients (44 eyes) had undergone previous strabismus surgery, and there was no evidence of previous scleral perforation in this group. We discuss the recent advances in strabismus surgery which may account for this difference in the incidence of scleral perforation.
Following reports of a high incidence of chorioretinal scarring related to the Faden Procedure, a retrospective study was carried out at Moorfields Eye Hospital. One hundred unselected Faden procedures were followed up with orthoptic and fundoscopic examination. Our indications and technique for performing this operation are described. A 7% incidence of chorioretinal scarring was noted; this compares well with previously published figures for conventional squint surgery and this operation should therefore be considered safe.
We have sought human eye muscle membrane binding antibodies in patients with Graves' ophthalmopathy using an enzyme-linked immunoassay. Antibodies were found in patients with thyroid autoimmunity irrespective of eye signs, and binding correlated closely (r = 0.94) with binding to skeletal muscle, showing that these antibodies are not site-specific. T cells from patients with thyroid autoimmunity proliferated in response to eye muscle, but again this was not specific for eye muscle or the presence of ophthalmopathy. No single antigen was responsible for inducing proliferation. These results fail to confirm a recent report of eye muscle membrane binding antibodies in a high proportion of patients with ophthalmopathy, and suggest instead that T and B cell autoreactivity to striated muscle antigens is a frequent feature of autoimmune thyroid disease, unlikely to be directly related to eye disease.
We have characterized the mononuclear cell infiltrate in the extraocular muscle of three patients with Graves' disease, using antibodies which permit staining of paraffin-embedded tissue. The majority of lymphocytes, occurring in foci or interstitially, were T cells, most of which stain for CD3 or with UCHL1. T cells few, if any, stained with SN130, directed against the CD45R determinant. This suggests that these lymphocytes comprise a recently activated population within which memory cells may reside. B cells were also found but were predominantly confined to focal aggregates, and in one patient lymphoid follicles were seen. The orbital fat and connective tissue from a further two patients contained very few infiltrating cells which were mainly UCHL1-positive. Eye muscle cells did not express Ia antigens but the interstitial cells between them were Ia-positive and the vascular endothelium in four of the five specimens also stained with Ia. These results indicate that Graves' ophthalmopathy is associated with T cell, and to a lesser extent B cell, responses against the retrobulbar tissues; the extraocular muscle interstitial cells, probably including fibroblasts, may be targets of activation resulting from this infiltration.
The best systemic treatment of dysthyroid ophthalmopathy remains high dosage Prednisolone. Other methods of immunosuppression such as Azathioprine and Cyclosporin A have proved disappointing, as has plasmapheresis. Other localised means may be used and these are radiotherapy to the retrobulbar tissues or orbital decompression. These methods may be used in addition to or instead of high dose Prednisolone but some surgeons prefer to try Prednisolone as a first measure.
We have performed an immunogenetic analysis of 53 patients with severe Graves' ophthalmopathy, 51 patients with Graves' disease but little or no clinically apparent eye disease, and 90 controls. The distribution of restriction fragment length polymorphisms was analysed in the three groups, using probes for the HLA-DQ alpha and DR beta regions, the T-cell receptor C alpha, V alpha, C beta and J gamma genes and the immunoglobulin gene switch regions, S alpha and S mu. There was no abnormal distribution of these polymorphisms in either group of Graves' patients, or differences between the Graves' patients with or without eye disease. It was possible to assign HLA-DR types in most patients using the polymorphisms found after probing with DQ alpha and DR beta; there was no abnormal distribution of DR types (including HLA-DR3) assigned by restriction fragment polymorphisms in the two Graves' groups. These results fail to confirm the reported associations between ophthalmopathy and HLA-DR3 and between Graves' disease and the T-cell receptor C beta polymorphism; they also argue against a strong influence of Gm allotypes in Graves' disease since these genes are in linkage disequilibrium with the S alpha polymorphisms. The association of Graves' disease with HLA-DR3, defined hitherto using serological reagents, may be less strong than previously described.
Once the diagnosis of thyroid eye disease has been made the doctor's problems begin because the management is long term with a condition that has many ramifications over a period of years. Short-term heroics to save vision by use of high-dose systemic steroids, orbital decompression or radiotherapy may have to be followed by control of diplopia by muscle surgery and prisms. Lid retraction, both upper and lower, may merit surgery, and even those patients who have come to terms with their facial appearance may still complain of lacrimation, irritation and grittiness. The advantages of a thyroid clinic, run jointly by a physician and an ophthalmologist, are presented and the patient is helped to live with this distressing condition.
Thirty-four patients with surgically treated bilateral superior oblique palsy are presented. The patients are divided into three groups: Symmetrical palsies, Asymmetrical palsies, A group in whom the bilaterality was initially masked. Bilaterality should be suspected in all cases of traumatic IVth nerve paresis, and particularly in cases with a large 'V' pattern, excyclo deviation of more than 10 degrees on down-gaze and when right hypertropia switches to left hypertropia on lateral down-gaze. Bilateral Harada-Ito procedures alone 'cured' 11 of 17 patients (65 per cent) in groups 1 and 2, and is the operation of choice in acute bilateral superior oblique palsy. Cyclo deviation was reduced by a mean of 5.5 degrees in the primary position and by 6-10 degrees in down-gaze. Patients initially managed with other surgery had a more complicated surgical course and required more operations. Seven patients who initially demonstrated only gross fusion recovered good fusion after Harada-Ito surgery.
The 'rediscovery' of adjustable sutures some 10 years ago has given the ophthalmic surgeon much more confidence in his ability to correct strabismus. Three methods of use are described: during surgery under general anaesthesia with adjustment during the operation using the 'springback' test to centralise the eye; during surgery under general anaesthesia and subsequent adjustment under local anaesthesia using the patient's subjective responses to obtain optimal positioning; and performance of the operation and adjustment under topical local anaesthesia in one procedure. Full details are given of each technique and the indications for their application to particular problems are discussed.
Current methods of medical treatment of this difficult condition are briefly reviewed, with comments on the generally unsatisfactory response. Attention is therefore directed to surgical methods and a short history given of the various routes used for orbital decompression. The author's own results of transantral, and more recently ethmoidal, decompression are given, with a detailed description of the technique for the latter approach. An interesting frequent consequence of postoperative A pattern to the ocular movements is discussed in the light of Koornneef's concept of orbital fascial anatomy, and suggestions are made for its avoidance.
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The first 46 patients to undergo surgery for squint with adjustable sutures at Moorfields Eye Hospital, City Road, London, are presented. The indications for surgery were: dysthyroid ophthalmopathy, fourth nerve palsy, monocular aphakia with strabismus and miscellaneous conditions. The technique of surgery is briefly described. The results show a high incidence of alignment, indicating the value of the technique and this is supported by long-term follow-up data where available. Treatment failures are analysed in detail.
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Cataracts may arise in association with various major and minor disorders restricting galactose metabolism, and the risk is broadly associated with the degree of galactose intolerance. A family is described in which a girl presented at the age of 7 3/4 years with cataracts, galactosuria, and partial deficiencies of the enzymes galactokinase and galactose-1-phosphate uridyl transferase. Galactose intolerance as determined by an oral test was impaired and fluctuated with variation in activity of the above galactose enzymes. Minor defects were also present in the parents and a maternal half-brother. The child has a compound disorder of galactose metabolism differing from those previously described. Assessment of galactose tolerance may be useful in the investigation of families with an incidence of cataract.