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PURPOSE Patients with beta-thalassemia (beta-tha) represent a group with lifelong transfusion-dependent anemias. This study aimed to describe the conjunctival changes and tear film parameters in these patients. METHODS A total of 52 patients (104 eyes) with beta-tha major and 22 normal control subjects (44 eyes) were studied during 1999 through 2000. Tear film break-up time (BUT), Schirmer test, rose Bengal staining, and cytologic evaluation of the conjunctival epithelium were performed in all subjects. The Papanicolaou and May-Grumwald-Giemsa staining procedures were performed on all smears. Patients and control subjects were compared for tear function parameters and conjunctival changes. RESULTS The BUT, Schirmer test, and rose Bengal staining values were significantly lower (P < 0.001) in beta-tha patients than in control subjects. Keratinized cells were observed in conjunctival samples in 41% of patients, with a decrease in the number of goblet cells per slide in 64% of patients. In 9% of beta-tha patients, there were a slightly greater number of inflammatory cells than in control eyes. CONCLUSION Ocular surface disorder of these patients was characterized by goblet cell loss and conjunctival squamous metaplasia. Our findings were correlated positively with the variable age. Epithelial damage by toxic reaction and disorder of tear quality and quantity are implicated as important factors in the pathogenesis of the ocular surface disease in beta-tha patients.

We have correlated the clinical and histopathologic features of the eyes and central nervous system in a patient with Hallervorden-Spatz syndrome who died at age 11 years. The main ocular findings included degeneration of photoreceptors, marked thinning of the outer nuclear and outer plexiform layers, retinal gliosis, narrowing and obliteration of blood vessels with a perivascular cuffing of pigment cells, and degenerative changes in the retinal pigment epithelial cells with accumulation of melanolipofuscin. The positive findings in the brain included a symmetrical, partially destructive lesion of the globus pallidus, especially in its internal fibers and neurons; in addition, we noted gliosis, widely disseminated axonal spheroidal bodies, which were most numerous in the globus pallidus and pars reticulata, as well as deposits of iron. Our histopathologic findings implicate three possible mechanisms, namely, lipid peroxidation, a deficiency of fatty acid membrane components, and increased cGMP which, either singly or in combination, are responsible for a pathogenesis that is common to the eye and brain in Hallervorden-Spatz syndrome.

To evaluate the efficacy of intracameral recombinant tissue plasminogen activator (r-TPA) in prevention of fibrinous effusion after lensectomy, anterior vitrectomy, and posterior chamber intraocular lens (PCIOL) implantation in patients with congenital cataract.The study was done as a double masked randomised clinical trial between April 2002 and November 2003 in Farabi Eye Hospital. 34 eyes of 26 patients with congenital cataract were included in the study and randomised into two groups (18 cases and 16 controls). Mean age was 8.1 years (3--14 years). Each eye underwent lensectomy and anterior vitrectomy with PCIOL implantation. At the end of surgery 20 microg r-TPA was injected intra-camerally in the case group. The control group received only balanced salt solution. All patients received periocular, systemic, and topical steroids after surgery. Patients underwent follow up examinations for 3 months.The incidence of intraocular fibrin membrane formation was significantly lower in the case group on days 1, 3, 7, 14 (p=0.02, p=0.01, p=0.01, and p=0.01, respectively, chi(2) test), but there was no significant difference on days 30 and 90. The frequency of pigmented intraocular lens precipitates was significantly lower in the case group at the end of the third month (p0.001, chi(2) test). No gross ocular side effects were noted after r-TPA injection.It seems that prophylactic intracameral r-TPA is effective in prevention of fibrinous effusion at least in the first 2 weeks after cataract extraction in the paediatric age group and decreases the incidence of pigmented IOL precipitates.

We conducted complete eye examinations and retinal function tests on nine patients who had undergone jejunoileal bypass (JIB) surgery and subsequently had low serum vitamin A levels. All patients had manifested clinically demonstrable keratoconjunctival xerosis; in addition, two had abnormally high dark-adaptation final thresholds, and two had concomitant implicit prolongation in the electroretinogram. The age-matched control groups that consisted of 14 patients with various gastrointestinal disorders and four asymptomatic volunteers showed fewer or no ocular abnormalities. Our findings suggest that, even though patients who underwent JIB surgery may manifest keratoconjunctival xerosis and have low serum vitamin A levels for several months to years, retinal function will remain normal until the tissue stores of vitamin A are depleted. A long-term ophthalmologic follow-up of JIB patients, therefore, is recommended.

We performed ocular examinations on 58 corticosteroid-treated pediatric patients with inflammatory bowel disease (IBD) and on 58 age-matched controls. Posterior subcapsular cataracts (PSC) were detected in 12 of the 58 treated patients (20.7%) and in none of the controls. The difference in mean intraocular pressure (IOP) between the treated patients (15.89 +/- 4.11 mm Hg) and control subjects (13.63 +/- 2.35 mm Hg) was significant statistically (P0.001). Twenty-one patients (36.2%) were characterized as "IOP responders" (IOPor = 20 mm Hg, change in IOPor = 6 mm Hg between visits, or a difference in IOPor = 6 mm Hg between the two eyes). Formation of PSC was not correlated significantly (P0.05) with the total dose of prednisone, duration of treatment, average daily dose, or number of days on high doses (or = 25 mg). Raised IOP was correlated (P = 0.005) only with average daily dose (12.4 +/- 10.9 mg/day; range, 0-47 mg/day) 30 days before examination. When the dose of corticosteroid was reduced to10 mg/day, 2 patients manifested regression of PSC, and 12 IOP responders showed a decrease in IOP to within 2 SD of the mean control IOP. Only 3 of the 58 treated patients (5.2%) manifested both PSC and raised IOP. A significant inverse correlation (P = 0.02) was established between IOP at first examination and formation of PSC. We propose that the mechanisms for steroid-induced lens opacities and raised IOP do not share the same genetic basis. Because 52% of these children developed either PSC or raised IOP with prednisone therapy, we advocate careful ophthalmologic monitoring of pediatric patients receiving corticosteroids for IBD or any other condition.

We report on a patient who developed necrotizing contact dermatitis after a single topical application of tincture of benzoin and a pressure bandage following enucleation of an eye. The toxicity manifested in this case was more severe than any documented previously in the ophthalmic literature.

The clincopathological features and pathogenesis of secondary mosaic degeneration of the cornea (anterior crocodile shagreen of Vogt) are described. The structural basis for the normal anterior corneal mosaic pattern seems to lie in the particular arrangement of many prominent collagen lamellae of the anterior stroma that thake an oblique course to gain insertion into Bowman's layer. Since, at normal intraocular pressure, Bowman's layer is under tension, when viewed from the anterior surface the cornea appears smooth. By releasing the tension, however, a reproducible polygonal ridge pattern becomes manifest. It is suggested that a prolonged phthisical state of the eye is one condition wherein the mosaic pattern may become permanent and that, as a secondary event, this is followed by irregular calcification of Bowman's layer which particularly involves the ridges projecting into the epithelium. Biomicroscopically these ridges corresponded to the branching reticular arrangement of the mosaic opacities.

A series of 75 spoilt soft lenses with opacities (mostly manifesting as discrete spots or as large areas of cloudiness, chalk-white in appearance) were subjected to histochemical, electron microscopical, electron probe x-ray microanalytical, x-ray diffraction, atomic absorption spectro-photometric, and biochemical analyses. The results showed that in many cases calcium was implicated in the deposit formation and that a broad correlation existed between the amount of calcium present and the degree of opacification. The possible mode of calcium deposition and resulting implications in lens intolerance are discussed. It is advocated that a search should be made for a modified or new soft lens material which does not permit calcium deposition, and that the medical practitioner should strive towards improved assessment of the systemic and ocular conditions which may help in identifying patients at risk of early lens failure.

The clinical features of pigmented lens striae were studied biomicroscopically in seven patients. The lenses of three of these, obtained by cataract surgery, were examined by electron microscopy to determine the structure of the striae. We concluded that the striae are not formed by remnants of tunica vasculosa lentis, as earlier workers believed, but consist of fine fibrils of zonular bundles entrapping pigment granules of uveal origin.

We encountered two clinically similar but genetically unrelated cases of a disorder characterized by an unusual white pupillary-iris-lens membrane with extension to a prominent Schwalbe's line, the membranes were vascularized and their appearance changed over time. In both cases the abnormality was unilateral. Anomalous chamber angles were seen on gonioscopy. The clinical appearance is similar to a combination of iridogoniodysgenesis and pupillary membrane. However, our two cases are unique and fit into neither category. We postulate that this is a new entity consisting of an anomalous chamber angle and iris-lens membrane resulting from a localized iris (pupillary membrane), infarct with secondary neovascularization, during formation of the chamber angle.

Three patients who initially presented to the ophthalmologist with a history of gradual deterioration of visual acuity showed, on biomicroscopic examination, a linear meshwork of opacities in the vitreous attached to the posterior surface of the lens in the form of pseudopodia lentis. Amyloidosis of the vitreous associated with systemic amyloid disease was diagnosed because of the characteristic nature of the opacities. This was confirmed initially in two of the cases by light and electron microscopical examination of a biopsy of the clinically normal conjunctiva, subsequently by systemic investigations, and finally, by direct histochemical and elecron microscopical examination of the vitreous which became available after vitrectomy in all three cases. It was not possible to examine other members of the families. The relevant literature on vitreous amyloidosis is reviewed in relation to our own findings and the importance of a diagnostic conjunctival biopsy is emphasized. This paper is the first British report on primary vitreous amyloidosis. The pathogenesis of vitreous amyloid opacities is discussed in the light of current concepts of amyloidosis. Since the vitreous opacities due to amyloid deposits appear to be one of the manifestations of systemic amyloid disease, surgical removal of the opacities by vitrectomy may not produce longlasting results. Moveover, in contemplating surgical treatment the ophthalmologist should bear in mind the poor prognosis of systemic amyloidosis.

Band-like structures, clinically similar to Haab's striae, occur in posterior polymorphous dystrophy (PPMD) and are often confused with them. The difference in clinical appearance based on difference in histopathology allows a clear differentiation. The edge of Haab's striae are thickened, curled, and secondarily proliferate Descemet's membrane, while the area between the edge is thin and smooth. Posterior polymorphous dystrophy bands are the exact opposite. The band is a thickening of Descemet's membrane between the edges with thinner, more normal Descemet's membrane outside of them. PPMD bands may be associated with typical vesicles or appear as the only sign of dystrophy, even in the eyes of patients with a known family history.

Prostaglandins (PGs), or their derivatives, are potent ocular hypotensive agents which may prove useful in glaucoma therapy. PGF2 alpha (250 micrograms in 50 microliter saline) or epinephrine 2% solution (50 microliter) was topically applied twice daily for 2 weeks to one eye of six cynomolgus monkeys for each agent. Contralateral control eyes received their respective vehicles. By light microscopy, there was no evidence of inflammation, corneal changes, retinal pathology (including cystoid macular edema), or other adverse effects. Likewise, by electron microscopy of the peripheral cornea, anterior chamber angle, iris base and ciliary body, no differences were noted between treated and control eyes. Therefore, multiple dosing with PGF2 alpha in subhuman primate eyes did not result in notable histopathological changes that would contraindicate a clinical trial in glaucoma patients.

A case of mucolipidosis I had clinical and histopathologic features that were a combination of changes found in both mucopolysaccharidoses and sphingolipidoses. Corneal clouding, spokelike cataracts, tortuous conjunctival and retinal vessels, and strabismus were clinical findings. Histopathologically, there were inclusion vacuoles similar to those seen in mucopolysaccharidoses; lamellar bodies typical of sphingolipidoses were rare. These vacuoles were seen in conjunctival and corneal epithelium and fibrocytes, conjunctival and retinal vessel endothelium, and all retinal cell layers.