How long can donor sclera be safely stored?

PURPOSE: To determine whether after prolonged storage of sclera in glycerin, there is any bacteriologic contamination that will reactivate, whether reconstituted sclera retains its tensile strength, and whether sclera retains its microstructural integrity. METHODS: Sixty-six scleral shells stored in glycerin for 9 to 19 years, as well as 11 controls stored for 6 months to 4 years, were studied by cutting a small wedge of tissue from the anterior margin of each and directly inoculating into thioglycolate broth, cutting an equatorial ring and determining its break strength using a tensiometer, and cutting a small piece from the remaining posterior portion and examining by scanning electron microscopy. RESULTS: After such prolonged storage, bacteriologic contamination was not detected, tensile strength generally increased with increasing duration of storage, and ultrastructural integrity was maintained on scanning electron microscopy. CONCLUSIONS: This study suggests that storage of scleral shells can be safely prolonged; we hope this can facilitate an increased supply of donated sclera to patients and surgeons.

Studies on associations of antinuclear antibodies with antibodies to an uveitogenic peptide of retinal S antigen in children with uveitis.

OBJECTIVE: To determine if, in children with uveitis, antinuclear antibodies (ANA) are associated with antibodies to an uveitogenic peptide of a soluble retinal antigen and to the homologous nuclear antigen, histone 3 (H3). ANA occur in most children with juvenile rheumatoid arthritis (JRA) and associated uveitis. An uveitogenic segment of retinal soluble antigen (S antigen peptide) is homologous with a similarly uveitogenic peptide of H3. We investigated a possible association between ANA positivity, antibodies to H3, and antibodies to the uveitogenic S antigen peptide. METHODS: The sera of 31 children with uveitis (20 of whom had associated JRA) were tested for the presence of ANA by indirect immunofluorescence. Antibodies to H3 and to an uveitogenic peptide of S antigen (an 18 mer segment having the amino acid sequence DTNLASSTIIKEGIDKTV) were measured by enzyme immunoassay. RESULTS: 19 of 20 children (95%) with JRA and associated uveitis and none of 11 with uveitis not associated with JRA had positive tests for ANA (X2 = 14.97; p < 0.00001). 16 of 19 ANA positive sera from subjects with JRA (84%) displayed reactivity with the chromosomal regions of metaphase cells. 9 of 20 patients with JRA with uveitis (45%) and 2 of 11 patients (18%) with uveitis not associated with JRA had antibodies to H3. Two uveitic patients with JRA (10%) and 2 non-JRA patients with uveitis (18%) reacted with S antigen peptide. Antibodies to H3 occurred significantly more frequently in children with uveitis than in all adult control subjects (X2 = 12.98; p = 0.003) and in adults with uveitis (X2 = 5.62; p = 0.022). CONCLUSION: Humoral immune responses to the uveitogenic peptide of S antigen and the homologous H3 antigen appear not to be uniquely important in the immunopathology of uveitis associated with JRA. Antibodies to isolated H3 do not exclusively account for ANA positivity in the uveitic patient with JRA. A unique immunopathogenic mechanism for the development of uveitis associated with JRA is suggested by the observations that (1) children with uveitis associated with JRA are more likely to be ANA positive than children with uveitis not associated with JRA, and (2) children with uveitis associated with JRA are significantly more likely to be ANA positive and to have antibodies to H3 than adults with uveitis.

Corneal melting after pterygium removal followed by topical mitomycin C therapy.

Right corneal melting and scleral necrosis developed in a 77-year-old man 5 months after pterygium excision followed by topical administration of mitomycin C drops (0.4 mg/mL) for 4 weeks. We believe that these were delayed complications of the mitomycin C therapy, and we caution against prolonged use of the drug postoperatively.

Antinuclear antibodies in arthritic and nonarthritic children with uveitis.

We determined whether a positive test for antinuclear antibodies correlated with uveitis only in children with juvenile rheumatoid arthritis (JRA) or whether they also represented a serologic marker for isolated idiopathic chronic uveitis in children. We conclude that the immunopathogenesis of uveitis associated with JRA is different from that of idiopathic chronic uveitis.

Terrien's marginal degeneration with corneal cyst.

Spontaneous ruptures in Descemet's membrane in Terrien's degeneration can result in a corneal intralamellar pocket of fluid. We present a case and discuss its management.

Recurrence of orbital pseudotumour after 10 years.

We describe a patient in whom left orbital pseudotumour developed on two occasions 10 years apart. Treatment with oral corticosteroids produced dramatic recovery on both occasions.

Conjunctivitis and pterygium associated with the American Indian type of polymorphous light eruption.

We describe three patients with the American Indian type of polymorphous light eruption (actinic prurigo), two Cree Indian sisters and a Cree Indian boy, who had eye symptoms similar to those seen in limbal-type vernal catarrh.

Pituitary apoplexy presenting with light-near dissociation of the pupils.

We describe a patient with pituitary apoplexy causing sudden visual loss and light-near dissociation of the pupils but minimal ophthalmoplegia. Good visual recovery occurred despite an 8-day delay in neurosurgical treatment.

Orbital involvement in chronic lymphocytic leukemia.

In a 68-year-old man with chronic lymphocytic leukemia diagnosed on the basis of peripheral lymphocytosis, marked bilateral exophthalmos developed owing to massive orbital involvement by the disease. At the time there was no lymphadenopathy or evidence of organ infiltration. The response to radiotherapy was excellent. Orbital involvement is rare as an early clinical feature of chronic lymphocytic leukemia but should be considered in the differential diagnosis of bilateral exophthalmos in adults.

Pseudophakic bullous keratopathy.

Pseudophakic bullous keratopathy (PBK) is a relatively new disease that is rapidly becoming the prime indication for penetrating keratoplasty. From 1977 thru 1981 we performed 81 corneal transplants on 66 eyes for this condition. In our experience, the incidence of PBK, with iris-supported lenses, is five times greater than aphakic bullous keratopathy (ABK). A series of 800 intracapsular cataract extractions (ICCE) with implantation of iris-supported lenses (1975-1979) were reviewed. Thirty-four patients (4.3%) developed PBK (average two years after surgery). In a series of 3,000 simple ICCEs (1955-1980), 24 patients (0.8%) developed ABK. Etiologic factors, methods of prevention, and the results of penetrating keratoplasty are considered. PBK following ICCE is a serious disease entity, usually of multifactorial origin. Though 88% of the patients have clear grafts, a high incidence of associated posterior segment disease tends to nullify the visual result. Planned extracapsular cataract extraction and posterior chamber lens insertion (1979-1982) has reduced our incidence of PBK to 0.3% (1/300). These results are promising but premature.

Four generations of arteriohepatic dysplasia.

Arteriohepatic dysplasia (AHD) is a multisystem disorder which is characterized by liver, heart, eye, and bony abnormalities. A characteristic facies is also present. The etiology is not clear, and some authors have favored an in utero toxin or infection as the cause. Other reports have shown parent-to-child transmission suggesting an autosomal dominant form of inheritance. This study documents the presence of AHD characteristics in four successive generations of a single kindred. Overall, 15 of 24 members had at least some characteristics of AHD. In addition to the usual findings, renal disease, a small flat face on lateral X-ray, and mild conductive hearing loss were frequently noted in this kindred. Physiological studies on one member revealed a normal bromosulfophthalein Tm and S but a late rise in plasma bromosulfophthalein and indocyanine green. This study strongly supports an autosomal dominant form of transmission for the AHD syndrome. The ease of determining the eye, bone, and heart abnormalities should simplify genetic studies of other patients' families. In addition, because these characteristics are recognizable at birth, documenting their presence in a jaundiced neonate should spare the infant unnecessary and potentially dangerous surgery to rule out extrahepatic obstruction, especially if one or more family members demonstrate the same abnormalities.

Fluorescein. Physiochemical factors affecting its fluorescence.

Fluorescein's property of fluorescence is reviewed. Of the many factors which affect its fluorescence, concentration is probably the most important and it best explains why leaking aqueous turns fluorescein bright green during Seidel's test. The intensity and pattern of fluorescein staining of corneal lesions is probably due to the concentration and distribution of fluorescein in the cornea. The concentration of fluorescein achieved in the retinal blood vessels during fluorescein angiography affects its fluorescence.

Keratophakia. Clinical evaluation.

The keratophakia and keratomileusis procedures of Barraquer for the correction of aphakia have not gained acceptance in the United States because of thier inherent complexity and degree of difficulty. To determine the practicality, feasibility, and visual rehabilitation potential of these procedures, 13 secondary keratophakia operations were performed at a 500-bed community hospital between March 1980 and April 1981. The first ten cases are reported to allow 6- to 12-month follow-up. All patients in the series were resistant to contact lenses or were poor candidates for secondary intraocular lens implantation. The anatomic results were excellent. Complications were minimal consisting of surface drying problems in three patients related to the sudden steepening of the cornea or pseudo keratoconus effect. Seven patients achieved 20/30 to 20/40 vision with minimal spectacle correction. The keratophakia procedure of Barraquer shows promise and can be duplicated by experienced corneal surgeons, but is not yet a viable alternative to the use of primary intraocular lenses and continuous wear lenses for the general ophthalmologist.

Ocular findings in arteriohepatic dysplasia (Alagille's syndrome).

The ocular findings in a father and two offspring with arteriohepatic dysplasia are reported in this paper. All three people had bilateral posterior embryotoxon, Axenfeld's anomaly and a pigmentary retinopathy. Other ocular findings were exotropia, an ectopic pupil, band keratopathy, choroidal folds, anomalous optic discs and infantile myopia. Persons with arteriohepatic dysplasia typically present with prolonged neonatal jaundice. Cognizance of the associated eye findings helps distinguish this syndrome from other types of familial intrahepatic cholestasis.