Biomedical subjects
Publications and source records attributed to J Mullaney.
The clinical and pathological presentation of Behçet's disease are discussed. Necrotising arteriolitis and phlebitis with thromboses are demonstrated when a rare enucleation specimen became available for examination in this condition. Immunohistochemical studies show mural IgG, IgA, and C3 deposits in episcleral and some choroidal veins. The value of the clinical use of fibrinolytic agents is discussed.
The pathological findings of pseudophacoanaphylactic endophthalmitis are described in a patient with a combined trabeculectomy, extracapsular cataract extraction and an "in-the-bag" posterior chamber lens implant, and who developed sympathetic ophthalmitis following a perforating eye injury within hours of his operation. Apart from the typical sympathetic histological findings, the eye revealed a pseudophaco-anaphylactic endophthalmitis reaction which appeared to be totally related to the polymethylmethacrylate (P.M.M.A.) optic. The "in-the-bag" polypropylene closed loops revealed no significant histological reaction.
The histological findings in four globes obtained at autopsy from clinically uncomplicated cases with intraocular lens implants, one glass posterior chamber and three anterior chamber lenses consisting of two pupillary fixated and one "in-the-bag" fixated Binkhorst lens, are reported. Minimal changes could be detected in the tissues with which the implants were in contact. Scanning electron microscopy of the cornea in the three anterior chamber implants showed a loss of endothelial cells in the axial region of roughly 30-50%. There was marked vertical disparity in the loss. Scanning microscopy of the implants showed evidence of surface degradation of the nylon loops of all the anterior chamber implants.
A case of bilateral uveal melanoma in a 60-year-old woman in association with primary bilateral ovarian carcinoma is described. This is the first case in which ultrastructural studies have been performed on the ocular tumours. Seven previously described cases are summarised, and the extreme rarity of such reports would suggest that this may indeed be a new syndrome.
An 18-year-old white man presented with a non-painful yellow raised swelling on the inferior limbus of his right eye. Systemic and ocular examination revealed no other abnormalities. The lesion was dealt with by simple excision, but when it recurred fairly soon it was removed in toto and replaced by a lamellar graft, without recurrence. Histological examination revealed a typical xanthogranuloma. The question is, why should a healthy male with no other manifestations develop a lesion like this on his limbus?
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Seven patients, who satisfied the criteria laid down by Mason and Barnes (1969) for inclusion under the heading Behçet's disease, were assessed clinically and investigated with particular reference to immune status and blood fibrinolytic activity. In one patient in whom it was possible to obtain an eye for examination, there was evidence, on microscopic examination, of an immune vasculitis as well as clot formation, particularly in the veins. All the patients were treated with fibrinolytic agents and the favourable results obtained with this treatment suggest that it should be considered as the first line of therapy in what is otherwise a very difficult condition to manage.
Sporadic colobomata may be associated with a variety of secondary changes, and these have been classified and discussed with special reference to unusual findings published during recent years. Clinically some cases may appear to be neoplastic, and pathological examination may be important in demonstrating their true nature. A malformation apparently not previously reported is described.
Juvenile nephronophthisis has been recognized recently as an important cause of chronic renal failure in childhood and adolescence. This report describes clinical and morphological findings in monozygotic twins in whom the triad of juvenile nephronophthisis, congenital hepatic fibrosis and retinal hypoplasia coexisted. The findings are discussed in relation to previous reports of nephronophthisis associated with either retinal abnormalities or congenital hepatic fibrosis. The occurrence of similar ocular and hepatic anomalies with the other cystic kidney diseases, polycystic disease and medullary sponge kidney disease is reviewed. A spectrum of oculo-hepato-renal syndromes is identified with many interrelated features which suggest a shared basic aetiology.
The humble coloboma has been taken as a model in an attempt to illustrate the benefit of continued observation and the application of increasingly sophisticated methods of investigation in the genetic and biochemical areas. The recent literature is reviewed with particular reference to publications dealing with colobomata and systemic malformations. Polycystic renal disease has been produced in animals and the application of the method used to a study of the eye may help to explain some of the unusual systemic clinical combinations which may occur in human cases. A simple factor may yet emerge to account for these anomalies rather than the multifactorial explanations previously postulated.
A tumour presumed to be a juvenile glioma of the optic nerve was removed by a Kronlein procedure from a 4-year old boy in 1925. In 1973 a severe proptosis made it necessary to remove the atrophic eye and a large retro-ocular tumour. Histological examination, supported by electron microscopy, showed the second tumour to be an anaplastic pilocytic astrocytoma. The possible histogenesis and this unusual behaviour pattern are discussed.
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