Biomedical subjects
Publications and source records attributed to F H Stefani.
An unusual unilateral fundus lesion in a 16-year-old boy is described consisting of hyperpigmentation and presumed hypertrophy of the retinal pigment epithelium combined with malformation and thickening of the overlying sensory retina. Based on its clinical and histological characteristics, this lesion is considered to be a congenital retinal pigment epithelial malformation.
Epibulbar osteomas are benign tumors which are usually present at birth. Up to now 39 cases have been described in the literature. In the last three years 4 further cases have been found in the Freiburg, Hamburg, Marburg, and Munich University Eye Clinics. Epibulbar osteomas probably occur more often than is generally believed. The clinical and histological findings in respect of these tumors are discussed.
Band keratopathy may be treated either surgically or by using chelating agents. If central corneal scars are present corneal grafting is indicated while in their absence dissolving therapy is preferable. Eight successful corneal grafts and three cases treated medically are reported. Details of treatment with sodium EDTA are described.
Paraffin-embedded sections of eyes enucleated because of hydrophthalmos (23 cases) or retinoblastoma (59 cases) were examined for vascular changes within the proximal optic nerve. Globes of adults, enucleated because of secondary glaucoma (146 cases), were studied for comparison as well as 19 myopic and 11 microphthalmic eyes enucleated for other reasons, and 19 autopsy eyes of newborns. Microscopic examination revealed an accumulation of dilated thin-walled blood vessels within the septae of the proximal optic nerve of 6 eyes with hydrophthalmos and 11 eyes with retinoblastoma; sometimes the vascular changes appeared somewhat similar to an angioma racemosum. A common finding in all 17 cases was an elevation of the intraocular pressure. There were only 2 cases among the eyes studied for comparison showing dilatations of the optic nerve capillaries: 1 was in a stillborn baby with general hyperemia, and the other was a case of secondary glaucoma in an adult. 9 similar cases have hitherto been published describing vascular dilatations in the optic nerve of children suffering either from glaucoma or retinoblastoma; none of the authors had related these changes to an increased intraocular pressure. It seems likely that hemodynamic changes play an important role in the pathogenesis of these vascular reactions in the proximal optic nerve of infants with flaucoma. A lower arterial blood pressure in infants as well as anatomical and morphological features may be further factors of significance.
The technique of Q-switched ruby laser induced mechanical destruction of the lens capsule was used to study the reactions of the adult rabbit lens. The defect produced in the anterior lens capsule was larger than the 40-60 micrometer focus, which was due to rolled-up edges of the capsule. Long-term observations revealed some decrease in size of developing localized opacification and a healing of the defect by outgrowing lens epithelium with the formation of a new capsule. The rupture of the posterior capsule led to a slowly progressive opacification with little intraocular macrophage reaction.
Two years after typical signs and symptoms of epidemic keratoconjunctivitis developed in a 67-year-old woman, in whom locally effective steroids were not used for treatment, a lamellar graft was transplanted because of marked visual disturbances that were due to corneal opacities. Histopathology of the cornea revealed notable changes in the superficial stroma, including Bowman's membrane, with some lymphocytic infiltration of the stroma and the overlying epithelium. Electron microscopy showed no virus-like particles.
A 22-year-old farmer with recurrent corneal erosions and keratoiritis underwent a penetrating keratoplasty. Investigations carried out on the corneal disc revealed an amoebic keratitis, which is considered to be a very rare condition.
Periarteritis nodosa and thrombotic thrombocytopenic purpura in siblings is reported. In both patients a localised serous retinal detachment and lesions of the retinal pigment epithelium had developed owing to choroidal vascular obstruction. These cases support the suggested possible relationship between the two conditions.
A high-risk pregnancy for X-linked recessive inherited Lowe's syndrome was terminated due to a male karyotype in the cultured amniotic fluid cells. The eyes of the male fetus showed specific cataracteous changes of the lens. A posterior lenticonus was due to a defect of the lens capsule. The lenses were of normal size. Loss of lens material through a lens capsule defect could account for the small discoid lens usually seen in Lowe's syndrome. Amino acids in amniotic fluid had normal concentrations except lysine and proline which were markedly elevated.
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Results on Q-switched ruby laser damage to lenses of Chinchilla grey rabbits are reported. Threshold irradiances of about 10(10)W/cm2 are shown to be necessary for lens damage. The minimal lesions consist of empty bubbles about 1 mm in diameter. Energy consideration as well as histologic investigation of the lesions indicate a mechanical damage process following a light-induced absorption in the focus. Retinal damage accompanied lenticular damage in all cases
In cases with persistent primary vitreous in full-term infants there were vitreoretinal reactions including vitreoretinal anastomoses of blood vessels, vitreoretinal strands, traction folds of the retina, avascular retinal areas, hypoplasia of retinal blood vessels, rarefaction of retinal ganglion cells, and ischaemic structural changes of the retina. New vessel formation on the surface of avascular retinal areas was supplied from persistent hyaloid blood vessels. This finding may be of some importance in explaining those cases histologically resembling classical retrolental fibroplasia with no history of prematurity or oxygen treatment.
Report of the typical clinical course and pathological findings in acute rhino-orbito-cerebral mucormycosis following diabetic coma. Invasion of orbital nerves by the fungus with neural and perineural changes may contribute to the neuroophthalmological symptoms in this disease besides the predilection for blood vessels with consequent thrombosis.
A report is given of the pathological changes in two eyes which were enucleated because of anterior luxation with rupture of the orbital optic nerve two hours after a traffic accident with frontal head injury. In the first case there was rupture of the trabecular meshwork and Schlemm's canal, the ciliary body and epithelium of the pars plana, a giant retinal tear at the ora serrata and a retinal avulsion at the optic disc, a partial rupture of the peripheral and a complete one of the central lamina cribrosa with a longitudinal tear in the optic nerve which lead to a complete rupture of the orbital optic nerve together with its sheats 22 mm behind the globe. The lesions produced arised probably from a slightly oblique course of the frontal trauma with a suggested additional rotating force followed by a sudden anterior movement of the eye with subsequent luxation. In the second case there was a giant tear at the ora serrata and at the pars plana with dislocation of unpigmented epithelium of the pars plana over the ciliary body, a rupture of the optic fascicle behind the lamina cribrosa, and a rupture of the optic dura mater 16.5 mm behind the globe.
In 14 out of 25 patients with retinoblastoma there was a family history of malignancies. Most of the malignant tumours had been carcinomas. In 2 families relatives died because of leukaemia; in these cases the tumour was a so-called diffuse retinoblastoma occurring in elder children. A third family with a retinoblastoma in one child and leukaemia in another is mentioned. This unusual coincidence of two tumourous conditions of which one might be of viral origin led to detailed electron microscopic examination of the retinoblastoma in one case; but there were no signs of reduplicating viruses.
Out of 22 patients with corneal dermoids, 9 showed other abnormalities consistent with the 1st branchial arch syndrome. In 4 cases there was hare lip, divided jaw and /or cleft palate along-side other abnormalities which made impossible a sure classification into one of the recognised syndromes. It is assumed that the anomalies arise in the 5th and 6th intrauterine week. The corneal dermoids were always unilateral and in only one case confined in position to the cornea.