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Biomedical subjects

G Koniszewski

Publications and source records attributed to G Koniszewski.

At least 19 recordsLinked to original sources

Intraocular pressure in patients with Cushing's disease.

Externally applied corticosteroids increase the intraocular pressure in one-third of the general population. We evaluated the intraocular pressure in 62 patients with endogenously elevated serum cortisol levels due to hypothalamic pituitary-dependent Cushing's disease. All patients underwent transphenoidal microsurgical sella exploration within 4 years after the onset of subjective symptoms. The intraocular pressure (preoperative mean, 14.9 +/- 3.5 mm Hg) exceeded 21 mm Hg in four eyes before surgery and in one eye after surgery (postoperative mean, 14.3 +/- 2.8 mm Hg). In patients attaining postoperative remission and whose pressure readings were taken at least 3 months after surgery, the postoperative intraocular pressure was significantly (P = 0.04) lower than that measured preoperatively (14.2 +/- 2.7 vs 13.0 +/- 2.0 mm Hg). Biomorphometry of the optic disc topography and the retinal nerve-fiber layer in 19 patients revealed no glaucomatous changes. In contrast to topically applied corticosteroids, endogenously elevated serum cortisol levels in patients with hypothalamic pituitary-dependent Cushing's disease increased intraocular pressure only slightly, with no great risk of glaucomatous optic nerve damage.

Adolescent↗

[Glaucoma in central hypothalamic-hypophyseal Cushing syndrome].

One third of a general population reacts to exogenously applied corticosteroids with an increase of intraocular pressure. In this study we investigated the influence of endogeneously elevated serum cortisol levels on the intraocular pressure and the presence of glaucomatous changes in the morphology of the optic disc and retinal nerve fiber layer. 15 patients with hypothalamic-pituitary Cushing's disease due to an ACTH producing adenoma of the pituitary gland and undergoing transphenoidal microsurgical exploration of the sella turcica were ophthalmologically examined pre- and post-operatively. Subjective symptoms had been noticed not earlier than four years before operation. Intraocular pressure was preoperatively and postoperatively within the normal range for all but one patient with readings of 23/23 mm Hg preoperatively. In the subgroup with endocrinological remission the intraocular pressure readings decreased postoperatively. Photography of the retinal nerve fiber layer, morphometry of 15 degree color stereo optic disc diapositives, and perimetry revealed no changes typical for glaucoma. In 4 patients the retinal nerve fiber bundles were remarkably well visible. We conclude that patients suffering from hypothalamic-pituitary Cushing's disease with endogeneously elevated serum cortisol levels and a duration of subjective symptoms of less than 4 years run a lower risk of developing glaucomatous damage than patients with topical administration of corticosteroids.

Adolescent↗

[Acute hemorrhage and ischemic necroses in hypophyseal tumors: hypophyseal apoplexy].

Acute enlargement of pituitary adenomas due to haemorrhage or ischaemic necrosis in the tumour was described as "pituitary apoplexy" by Brougham et al. in 1950. Since then, more than 200 cases have been reported, but--especially in the German literature--the syndrome has caught only little attention. Therefore, in a series of 12 own patients, typical findings and clinical characteristics are demonstrated and the literature is discussed. 9 patients had a haemorrhage into the tumour, 3 an acute ischaemic necrosis. The guiding symptom was the acute onset with ophthalmoplegia (11 of 12 patients). Only in one case the adenoma was known before the apoplexy. Other symptoms were headache, blurred vision, drowsiness and, in severe cases, hemiparesis, coma, and hypothalamic disorder. Most important is the acute endocrinological substitution with hydrocortisone; this may be life-saving. Neuroophthalmological recovery depends on early operation: cases of oculomotor palsy require an operation within the first 2 weeks after the acute event. An emergency operation is required only by an acute amaurosis. In general there will be enough time for careful clinical endocrinological and radiological investigations.

Adenoma↗

["Morning glory syndrome" and "Handmann's anomaly" in congenital macropapilla. Extreme variants of "confluent optic pits"?].

Seventeen optic disks with morning glory syndrome were measured in absolute and relative size units (millimeters and parapapillary retinal vessel caliber) in 5 and 12 eyes, respectively. Their respective mean areas were 7.47 +/- 2.63 mm2 and 7.84 +/- 4.96 mm2. They were significantly (Mann-Whitney test) larger than 457 unselected normal optic nerve heads (2.69 +/- 0.70 mm2; p less than 0.0001) and 15 optic disks with pits (4.84 +/- 1.42 mm2; p less than 0.001). They are the largest optic nerve heads, probably with constant postnatal size, measured so far, and form part of a spectrum of optic disk anomalies and diseases whose frequency correlates with the size of the optic disk. This spectrum includes abnormally small optic nerve heads with increased prevalence of drusen, pseudopapilledema and nonarteritic anterior ischemic optic neuropathy, asymptomatic macrodisks with physiologic, "pseudoglaucomatous" macrocups, symptomatic macrodisks with pits and, finally, optic nerve heads with morning glory syndrome. The last-mentioned may be considered an extreme variation of optic disks with pits which have combined to form a single macropit covering the entire optic nerve head.

Fluorescein Angiography↗

[Recession of the inferior rectus muscle in superficial anesthesia in the treatment of thyroid orbitopathy].

Recession of the inferior rectus in surface anaesthesia by 5% cocaine drops was performed in 8 patients with signs of hypotropia in the course of thyroid orbitopathia and with diplopia persisting after conservatory treatment and not corrected by prisms. This method enables us an accurate intraoperative settlement of the distance of recession and it may be recommended in cases metabolically normalized, with not too much advanced exophthalmos, with a stability of the motor disturbances for at least 6 months and without any signs of inflammation.

Adult↗

[Pseudo- "toxic lens" syndrome over the course of 4 years caused by staphylococcus epidermidis endophthalmitis].

A 79-year-old female patient presented with recurrent severe iridocyclitis after extracapsular cataract extraction and implantation of a PMMA posterior chamber lens in 1983: In April, June, July, September, October and December 1984 as well as April and August 1985 and January and April 1987, the uveitis appeared without a hypopyon and was diagnosed as a "toxic lens syndrome". Consequently, IOL explantation followed by anti-inflammatory and - intermittently - immunosuppressive therapy was performed in October 1984 with a resulting improvement in vision. In June 1987, the patient presented with a full-blown bacterial endophthalmitis, which required immediate pars plana vitrectomy. Lens cortex material and capsule residues were removed. Staphylococcus epidermidis was cultured from the vitreous aspirate using enrichment techniques. Antibiotic topical and systemic therapy was performed according to the antibiotic sensitivity test and resulted in a complete cure of the intraocular inflammation.

Aged↗

[Changes in the sensitivity of the cornea in patients with intracerebral tumors. Studies of patients with meningioma of the cranial base and neurinoma of the trigeminal nerve and the vestibulocochlear nerve using the Draeger esthesiometer].

The center of the cornea was measured preoperatively in 29 patients suffering from intracranial tumors (acoustic neuroma, trigeminus neuroma, basal meningioma). These measurements were made with the Draeger electronic-optical esthesiometer; the results were supplemented by an analysis of tumor diameters determined by computer tomography. In each case one-half of the patients with acoustic neuromas and medial meningiomas of the wing of the sphenoid bone manifested a reduction in sensitivity at the center of the ipsilateral cornea (normal: 0.8 to 1.7 x 10(-5) N). The diameters of the sensitivity-reducing acoustic neuromas ranged from 15 mm to 45 mm. It may be deduced both from the topographic conditions at the skull base in the vicinity of the porus acusticus internus and from the conditions associated with a pressure-induced lesion of a peripheral nerve that medial acoustic neuromas as small as 10.1 mm in diameter can lead to a reduction in the conductivity of the ipsilateral trigeminal nerve. Only when they attain a diameter of 28.4 mm and when the proportions of the skull base are equally spacious do the acoustic neuromas regularly cause an ipsilateral corneal hypesthesia.

Adult↗

[Ocular involvement in ectodermal dysplasia].

The authors describe the ophthalmological findings and clinical course in two patients with EEC syndrome and one patient with anhydrotic ectodermal dysplasia. A retinal complication was seen in a 43-year-old patient which seems to be associated with the EEC syndrome and has not yet been described. A review of the literature indicates the frequency of ocular findings. So far, primary ocular changes in anhydrotic ectodermal dysplasia have never been observed. The older the patient, the more severe the secondary complications affecting the lids and cornea can be; the eye may even be lost.

Adult↗

[Analogy of Blaschko lines in the eye].

In females random X-inactivation and subsequent embryonic development cause a specific distribution of cell clones. This aspect can be seen in carrier women for different X-linked diseases. In such dermopathies the carrier women show striated skin affections following a system of lines, which has been described by A. Blaschko in 1901. An analogous pattern can be seen in the retina of carrier women for the X-linked ocular albinism. The fundus shows a partial involvement with a striated pattern diverging from the papilla. In X-linked cataracts carrier women have lens opacities with an irregularly radiated pattern as well as segmental cataracts. This finding is demonstrated in the isolated X-linked cataract, the X-linked chondrodysplasia punctata, and in Lowe's syndrome.

Albinism↗

[The Lyon effect of the lens: findings in the carriers of X chromosome-linked cataract and in Lowe syndrome].

In carrier women for X-linked diseases the random X-inactivation and the subsequent embryonic process of development and differentiation induce characteristic patterns of tissue mosaicism. In the lens, the random mosaic pattern of equatorial epithelial cells and their proliferation oriented toward the posterior pole induce a radial or segmental distribution pattern of clonal cell populations. This can be shown in carrier women for X-linked cataracts. Findings in a carrier woman for X-linked cataract and for Lowe's syndrome respectively are reported.

Adult↗

[Necrotizing retinitis in subacute sclerosing panencephalitis].

Between 1978 and 1982 the authors saw 5 patients (13, 14, 15, 24 and 28 years old) with SSPE. The first symptom was a sudden loss of visual acuity (unilateral in 3 patients and bilateral in 2). Four of the patients treated were suffering from necrotizing, central, partly hemorrhagic retinitis (not affecting the vitreous or the choroid). In the area affected by retinitis there was a residual "depression", and in some cases alterations of the retinal pigment epithelium indicative of tissue loss. In recognizing the early symptoms of this neuro-ophthalmological disease the ophthalmologist makes an important contribution to early diagnosis. In all 5 patients the diagnosis of SSPE was substantiated by the neurological course, the elevated measles titer and the cytoplasmic and nuclear antibodies counteracting SSPE antigen in the plasma and cerebrospinal fluid.

Adolescent↗