Isaac Ben-Sira

Long-term Results of Cryotherapy for Active Stages of Retinopathy of Prematurity

Between 1976 and 1980, retinopathy of prematurity (ROP) stage III (moderate) (fibrovascular proliferation) developed in 29 eyes (confluent fibrovascular proliferation of 3 clock hours in extension) of 17 preterm babies. The eyes were treated by cryopexy to the avascular retina. Analysis of the visual function and anatomic results in these eyes, five to eight years postoperatively, revealed very good visual function and only a few anatomic abnormalities. Comparison of these eyes with those of two groups of age- and birth-weight-matched preterms (one with less severe ROP, not treated by cryopexy, and the other with no ROP) showed that the degree of myopia was significantly higher in the cryotreated group. Although not statistically significant, there was a higher incidence and a greater degree of astigmatism in this study group.

Autosomal Dominant Inheritance of Retinoschisis

Hereditary retinoschisis affected eight members of three generations of a family. The mode of transmission and the clinical features were not compatible with findings noted in either X-chromosome-linked or autosomal recessive forms of retinoschisis. The genetic and clinical features in this family strongly supported autosomal dominant inheritance, adding to the known genetic heterogeneity for the hereditary forms of retinoschisis. The expression of the condition varied in severity, but all affected members of the family had peripheral retinoschisis and peripheral retinal degeneration. Three had maculoschisis and five had macular pigmentary changes. Electroretinographic findings were normal in six of the eight.

Congenital Orbital Vascular Malformation Complicated by Massive Retrobulbar Hemorrhage

A 2-year-old female infant with a presumed diagnosis of infantile hemangioma of the left eyebrow developed acute proptosis of the left eye several days after a mild orbital trauma. Computerized axial tomography (CAT) and carotid angiography confirmed the presence of a vascularized mass in the retrobulbar space, spreading anteriorly to the orbital rim and the frontal area. Due to progression of the proptosis and the development of exposure keratitis, surgical intervention was necessary. A large vascular tumor and a massive hemorrhage occupying the orbit were removed. The pathological examination confirmed the diagnosis of a vascular malformation of the orbit, most probably orbital varices. A relatively good visual acuity was achieved after surgery by occlusion therapy.

Narrow-Band (540-nm) Green-Light Stereoscopic Photography of the Surface Details of the Peripapillary Retina

We used narrow-band (540-nm) green-light stereoscopic fundus photography to study the surface details of the peripapillary retina in 14 patients. Each photograph was divided into four areas; the papillomacular bundle, the upper arcuate bundle, the lower arcuate bundle, and the nasal sectorial fibers. We scored each area on a scale of 0 (normal) to 3 (total loss of nerve fiber layer), and compared these scores to those obtained by testing the corresponding visual fields. The scores were the same in 88 of 111 comparisons; the retinal scores were higher in 17 comparisons and the visual field scores were higher in six comparisons. This photographic technique makes the nerve fiber layer easier to see and evaluate and the detection of defects becomes less difficult.

Treatment of Macular Subretinal Neovascularization with the Red-Light Krypton Laser in Presumed Ocular Histoplasmosis Syndrome

A patient with presumed ocular histoplasmosis and decreased visual acuity in one eye had subretinal neovascularization and retinal hemorrhage in the macular area. Argon-laser treatment to eradicate the subretinal neovascular membrane was contraindicated because of the proximity of the hemorrhagic lesion to the fovea. We used a red-light krypton laser (647 nm) because the light is not absorbed by the hemoglobin in the retinal hemorrhage or by the macular luteal pigment (xanthophyll), and because it is transmitted into the choroid, allowing treatment within the macular zone with less damage to the internal retinal elements. After treatment, the subretinal neovascular membrane completely disappeared and the macular hemorrhage cleared. To the best of our knowledge, this is the first time that a histoplasmic macular subretinal neovascular membrane has been treated with the red-light krypton laser.

Intraocular involvement of Burkitt's lymphoma in a Bedouin child.

A child with a B-cell lymphoma with extensive abdominal disease responded to high doses of chemotherapy. One month later, eye and central nervous system recurrence developed. Intraocular involvement was documented by diagnostic vitrectomy. Differential diagnosis and therapy are discussed.

Retinal involvement in tuberous sclerosis.

In two infants hospitalized at the ages of three and one-half months and 11 months because of epileptic seizures, the diagnosis of tuberous sclerosis was reached upon the basis of retinal astrocytomas found in the fundus examination. It is recommended that indirect ophthalmoscopy be performed in all infants and young children referred with signs possibly related to this disease.

Corneal Metastatic Calcification in Werner's Syndrome

We examined twin sisters with a clinical picture typical of Werners syndrome. Both had undergone bilateral cataract extraction, one at 39 and one at 36 years of age, and had subsequently developed bilateral corneal metastatic calcification within a period of one to two years. In one twin, this keratopathy was associated with hypercalcemia. Each of the twins underwent penetrating keratoplasty in one eye, which was complicated by recurrence of metastatic calcification in a previously normal and clear corneal graft.

Combined Congenital Glaucoma, Pigmentary Glaucoma, and High Myopia in an Infant

A case of congenital glaucoma combined with pigmentary glaucoma and high myopia in a three-month-old infant is described. The pathophysiology, medical treatment, and review of the literature is discussed.

Keratopathy associated with hypoparathyroidism and Addison's disease.

Two siblings suffering from keratopathy, one with hypoparathyroidism associated with Addisons disease and the other with Addisons disease alone, are described. The hypothesis that there was an autoimmune etiology with variant manifestations in each subject is suggested and discussed.