Reis-Bücklers' corneal dystrophy. Immunofluorescent and electron microscopic studies.

The anterior stroma, epithelium, and Bowman's layer have been proposed as the site of primary pathology in Reis-Bücklers' corneal dystrophy (RBCD). Immunofluorescent localization of laminin and bullous pemphigoid antigen (BPA) was compared with the ultrastructure of RBCD. As previously reported, patchy deposition of characteristic "peculiar curly" filaments was found in the supra-Bowman's, subepithelial fibrous tissue. We also recognized areas of early involvement with deposition of this "peculiar curly" material between a distorted epithelial basal lamina and a normal undisturbed Bowman's layer. In normal cornea, laminin and BPA localized to the epithelial basal lamina. In RBCD, laminin and BPA were in a piebald mosaic distribution throughout the aberrant subepithelial fibrous tissue between the basal lamina and the buried Bowman's layer. This indicates that RBCD is an epithelial disease, with the "peculiar curly" material paralleling the distribution of the attachment proteins.

Circinate-pattern interstitial keratopathy in daily wear soft contact lens wearers.

Three male and two female patients (aged 16 to 22 years) developed bilateral deep corneal stromal vascularization and circinate-pattern lipid exudation resembling interstitial keratitis after wearing adequately fitting hydrogel daily wear soft contact lenses comfortably for periods of ten to 24 months. The circinate pattern consisted of lipid deposition in circular distribution either parallel to the limbus or around a deep stromal blood vessel. All had negative serologic test results for syphilis. The cause of these changes is uncertain.

Distribution of phosphatic metabolites in the porcine cornea using phosphorus-31 nuclear magnetic resonance.

This study describes the phosphatic metabolite contribution of the porcine corneal stroma [by phosphorus-31 nuclear magnetic resonance (P-31 NMR)] and the corneal epithelium (by difference calculation) to the total phosphatic metabolic profile of the intact (i.e. whole, undissected) cornea. In order to provide such a regional analysis, the epithelium and endothelium were surgically removed from three sets of 12 corneas. Three additional sets of 12 intact corneas were used as controls. Histological examination of two randomly selected stromata from each set of corneas were analyzed to verify that epithelium and endothelium were removed. Perchloric acid extracts were prepared from the 10 remaining stromata for P-31 NMR analysis. Twenty phosphatic metabolites were measured including six unidentified (unknown) signals. Components with diminished relative concentrations in the stromal profile compared to that of the intact cornea profile are: the triose and hexose phosphates associated with glycolysis (including alpha-glycerophosphate), choline phosphate, ATP, uridine diphosphohexoses and the unknown compound resonating at 0.93 delta. The relative level of inorganic phosphate in the stroma is nearly doubled. The remaining measured phosphates are not significantly changed relative to the total extractable phosphorus content. Consistent with previous biochemical findings, phosphates associated with energy metabolism are found primarily in the epithelial fraction. The metabolic status of the endothelial monolayer cannot be assessed because of low tissue mass and the relatively low sensitivity of P-31 NMR spectroscopy. This study serves as baseline data for the interpretation of experimental and clinical phosphorus NMR data from intact cornea by providing the contribution to the whole corneal spectrum of metabolites of the individual corneal layers.

Management of loose Choyce-style anterior chamber lenses.

Loose Choyce-style anterior chamber intraocular lens implants were removed from four patients. We offer general and specific guidelines for the management of patients with mobile Choyce-style anterior chamber lens, with discussion of corneal decompensation, cystoid macular edema, and iritis in these patients, and for prevention of the problem.

Corneal allograft rejection. The role of the major histocompatibility complex.

The greater success of corneal transplantation compared to other organ transplants has led to the concept that the cornea is a site of "immunological privilege." Corneal cells possess the antigens of the major histocompatibility complex responsible for allograft rejection in other tissues (i.e., HLA antigens). The avascularity of the cornea accounts for the relative protection of the donor cornea from the immunological surveillance of the recipient. As the roles and functions of the major histocompatibility complex are unravelled, the mechanisms responsible for host sensitization, lymphocyte activation and allograft rejection are becoming better understood. In particular, the HLA-DR antigen in humans is believed to play an integral part in allograft rejection. Langerhans cells in human corneal epithelium have been shown to bear this antigen. Evidence suggests that these cells or similar HLA-DR-bearing cells in the cornea play a major role in corneal allograft rejection. In light of these advances in transplantation immunobiology, new methods of suppressing and possibly preventing allograft rejection in corneal transplantation are presented.

Vitreous fluorophotometry in optic nerve disease.

Posterior vitreous fluorophotometry measurements have been shown to be increased in patients with acute retrobulbar neuritis. We studied 12 patients who had optic neuropathy by posterior vitreous fluorophotometry, with and without correction for blood fluorescein concentration. Both uncorrected and corrected posterior vitreous measurements were increased in patients with inflammatory, obstructive, and infiltrative optic neuropathies. Concurrent values from anterior chamber fluorophotometry were not increased in any of the 8 patients studied.

Computed tomography in the management of orbital infections associated with dental disease.

Two patients developed orbital infection secondary to dental infections. In one patient the infection spread from maxillary premolar and molar teeth to the infratemporal and pterygopalatine fossa and then through the inferior orbital fissure to the subperiosteal space. A subperiosteal abscess in the posterior orbital wall developed, which subsequently spread within the muscle cone. In the second patient infection of an anterior maxillary tooth caused a pansinusitis and unilateral orbital cellulitis. In both patients computed tomographic scanning of the orbit proved valuable in localising the infection and, in one case, planning a surgical approach to the orbit. The infection in both patients responded to treatment, with no permanent visual impairment. Appropriate antibiotics and prompt identification and surgical drainage of orbital abscesses are essential for the preservation of vision in cases of orbital infection.

Diagnosing acute retrobulbar neuritis by vitreous fluorophotometry.

We studied six patients with acute retrobulbar neuritis (including two with definite multiple sclerosis) during acute episodes and in convalescence, clinically and with fluorescein angiography and vitreous fluorophotometry. We compared vitreous fluorophotometric readings with visual-evoked responses. In seven of ten eyes with abnormal visual-evoked responses, there was an acute increase in posterior vitreous fluorophotometric readings. Vitreous fluorophotometry may be useful as objective evidence of (1) acute retrobulbar neuritis, (2) involvement of the asymptomatic eye in acute retrobulbar neuritis, (3) the duration of neuritic inflammation in acute retrobulbar neuritis, (4) a recurrence of acute retrobulbar neuritis, (5) alterations in the blood-ocular barrier in patients with acute retrobulbar neuritis in multiple sclerosis, and (6) the effects of corticosteroids and other therapeutic agents in the treatment of acute retrobulbar neuritis.

Ocular abnormalities in the amniogenic band syndrome.

Craniofacial and limb abnormalities are characteristic of amniogenic band syndrome. We present the ocular findings of a patient with this syndrome and compare these abnormalities with those in 20 previously reported cases.