S. Zonis

Hereditary Microphthalmia With Colobomatous Cyst

We examined five members of a highly inbred kinship who had isolated microphthalmia associated with colobomatous cysts and various other ocular lesions. They were all offspring of consanguineous (first cousins) and unaffected parents. Microphthalmia in this kindred was transmitted as an autosomal recessive trait. Ultrasonography was effective for prenatal diagnosis in two pregnancies at risk.

Pseudoexfoliation: Epidemiology, Clinical and Scanning Electron Microscopic Study

The prevalence of pseudoexfoliation among patients of Sephardic origin was more than twice the expected when compared with the ethnic distribution of the population consulting the Rambam Medical Center eye clinic. A strong association between brown irises and pseudoexfoliation was noted. No uniform scanning electron microscope pattern was seen of the pseudoexfoliation.

Light and Electron Microscopical Study of the Conjunctiva in Sicca Syndrome

Conjunctival biopsies of 11 patients with sicca syndrome were studied under light and transmission electron microscopy. The notable findings were vasculitis of the conjunctival vessels. There were severe changes in the microvilli, namely a loss of microvilli and decrease in their height, measuring approximately 1,000-2,000 A, in comparison with microvilli of the control conjunctiva, measuring 4,000 A in height. There was no branching yet or fusion of microvilli.

Microphthalmos with orbital cyst: a clinicopathological report.

The clinical and histopathological findings in a case of microphthalmos with orbital cyst is described. The clinical interesting feature of this case was the bilateral coloboma which represented interference in the development of the eye at different stages of embryonic life.

Myotonic Dystrophy: Pathological Study of the Eyes

A 56-year-old patient with histopathologically established myotonic dystrophy is described. Gross pathological examination of the eyes revealed cataracts and peripheral retinal dystrophy as described previously. Lacy vacuolization of the pigment epithelium of the iris and vacuolization of the nonpigmentary ciliary epithelium were also found. These latter findings have, to our knowledge, not been hereto described in the literature.

Hl-A 27 Antigen Associated by Uveitis and Ankylosing Spondylitis in a Family

Uveitis and ankylosing spondylitis coexisted in a family. Tissue typing of the family revealed the HL-A 27 antigen in five members: two had uveitis; two had ankylosing spondylitis; and one was asymptomatic. These diseases did not coexist in any one member of the family.

Möbius syndrome Electron microscopic study of the extraocular muscles

The medial rectus, external rectus and inferior oblique muscles were examined by electron microscopy in Möbius syndrome. The electron microscopic findings of the medial rectus and inferior oblique were of degenerative and inflammatory nature, whereas the lateral rectus showed complete fibrosis. The fibrotic lateral rectus could explain the absence of abduction in Möbius syndrome.

Influence of Bromhexine on Tear Lysozome Level in Keratoconjunctivitis Sicca

We tested tear lysozyme levels before and during bromhexine treatment in 18 patients with keratoconjunctivitis sicca. Bromhexine increased the lysozyme level in patients with keratoconjunctivitis sicca and systemic diseases and, to a lesser degree, in patients with keratoconjunctivitis sicca only. Bromhexine relieved the ocular symptoms in eight patients and improved Schirmer test findings in five.

Electron microscopic study of Duane's syndrome, endocrine ophthalmopathy and other myopathies with strabismus

The lateral and the medial rectus muscles from children with special forms of strabismus are described ultrastructurally. In Duanes retraction syndrome, the lateral rectus muscle was composed of mature and immature collagen fibrils and no nerve structures could be identified in the whole bulk of tissue. the medial rectus was partly fibrotic and partly composed of muscle fibers showing various stages of degeneration. Nerve axons were seen adiacent to the muscle cells. In endocrine ophthalmopathy and rod myopathy, the muscle fibers showed myofibrillar degeneration and abnormalities of the Z-band and also an increase in the amount of the interstitial collagen tissue. It is concluded that the absence of nerve structures in the area of the totally fibrotic lateral rectus muscle supports the view that Duanes syndrome is related to a neurogenic abnormality.

Collagen Fibril Abnormalities in the Extraocular Muscles in Ehlers-Danlos Syndrome

The dermal collagen fibrils and the fibrils of the extraocular muscles and the conjunctivas of both eyes of a child afflicted with Ehlers-Danlos syndrome (EDS) were studied ultrastructurally. The results were evaluated statistically. Biopsy material was used in all instances. The collagen fibrils from all involved tissues showed a high percentage of size variation. Abnormalities of shape were noted. In the reticular dermis, 48% of fibrils were of normal diameter, 23% were enlarged, and 29% were thinner than normal. In the extraocular muscles, 77% were of normal size, 14.5% were larger, and 8.5% were smaller. In the conjunctivas, 73% of fibrils were normal-sized, 22% were enlarged, and 5% were of small diameter. No small-sized fibrils were encountered in control extraocular muscles.