Metabolic, pediatric, and systemic ophthalmology (New York, N.Y. : 1985)最新文献

We describe the ocular abnormalities seen in a new metabolic disease which is deficient in the transport of tryptophan. Besides the symptoms and signs reported previously, we have found microconea, hypoplasia of the optic disc and abnormal eye movements in our case--a ten-year-old-boy. The sibling involvement and consaguineous marriage of the parents suggest a recessive heredity.

Uveitis was studied in patients with Graves' Disease. Graves' disease was found in 2.3% of uveitis patients, but no uveitis was found in patients with Graves' disease who visited our clinic with Graves' ophthalmology. Most of the Graves' disease patients with uveitis had had uncontrolled thyroid function before the onset of uveitis. Generally speaking, uveitis was mild. Steroid hormone therapy was very effective. No specific HLA type was found in our patients with uveitis.

Maple Syrup Urine Disease is an autosomal recessive disorder of branched chain amino acid metabolism with an incidence in Ireland of one in 140,154 births. Ocular complications in untreated or late diagnosed patients includes optic atrophy, grey optic papilla, nystagmus, ophthalmoplegia, strabismus and cortical blindness. Seven patients with maple syrup urine disease were studied. All were diagnosed with the aid of newborn screening and commenced on early dietary treatment (mean age at diet introduction = 5 days). All remain physically well, with average intellectual performance, three having minor neurological defects and one strabismus. Early diagnosis, proper therapy and subsequent vigilant management may reduce substantially the risks of ophthalmic complications in this rare disease.

A retrospective study of 35 patients undergoing evisceration at Moorfields Eye Hospital, London between 1982 and 1987, revealed that 60% were known to be suffering from neovascular glaucoma, the majority as a result of a central retinal vein occlusion. 76% of these rubeotic eyes subsequently developed an endophthalmitis and required evisceration. 81% of this rubeotic, infected group were being treated with topical steroids prior to developing a suppurative keratitis and ensuing endophthalmitis. Every patient (9) with neovascular glaucoma that was debilitated (due to a variety of underlying medical conditions--4 were diabetic) developed an endophthalmitis and 78% of these were also using topical steroids. This study reveals that there may be a specific group of patients with absolute neovascular glaucoma, i.e. being debilitated and using topical steroids, who might appear to be susceptible to developing severe intraocular infection.

We report the case of a 41 year old lady with high hypermetropia and optic disc drusen (ODD) in both eyes. A recent study has shown that ODD has a greater tendency to form in eyes with a small scleral canal. It would therefore seem probable to find a higher rate of ODD in hypermetropic eyes. Some of the current literature denies this connection. In this article we discuss the pathogenesis of ODD and the possible connection between this entity and "high" hypermetropia.

An 8-year-old boy with congenital ptosis of the right upper eyelid due to plexiform neurofibroma was operated on because of a rapidly worsening of his ptosis. Only partial removal of the tumor was possible. A hypermetropic astigmatism of the right eye was caused by the condition of the upper lid, with secondary amblyopia. This finding suggests that in cases of congenital ptosis the presence of a lid tumor must be suspected. Such a tumor causes ptosis and this can lead to refraction error and amblyopia.

We conducted a nation-wide survey of the Prevalences of Myopia and other refractive errors in Israel, from data of medical examinations of an unselected population of 312,149 subjects ages 17 to 19 years. 80.47% of the population were emmetropic in both eyes. Myopia in both eyes was found in 16.27% of the population. The prevalence of monocular myopia was 1.69%. Manifest hyperopia in both eyes was found in 0.93% and astigmatism at least in one eye was found in 7.13%. The various errors of refraction (myopia, hyperopia and astigmatism) were more common among females.

Essential thrombocythemia is a myeloproliferative disorder with an increased amount of abnormal platelets, causing both hemorrhagic and thrombotic pathology. Some of its systemic complications include deep vein thrombosis, pulmonary emboli, myocardial infarcts, and renal vessel thrombosis among others. We present the rare case of a woman suffering from essential thrombocythemia with the sudden loss of vision in her right eye, caused by central retinal artery occlusion. A possible connection between these two disorders is discussed.

The advantages and limits of both CT and NMR imaging of optic nerve lesions are outlined and some examples are produced. Advantages of CT scanning are high resolution, multiplane reconstruction, quickness of examination, reliability, whereas the limits are the presence of artifacts from contiguous bone structures, the use of enhancement media and radiation damage. NMR is superior to CT scanning for detection of soft tissue lesions and possibility of multiplane scanning, but duration and high costs of the examination and some artifacts caused by ocular movements are limits to be taken into account. Both the techniques provide useful complementary information for the diagnosis of orbital lesions involving the optic nerve.