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Biomedical subjects

R M Siatkowski

Publications and source records attributed to R M Siatkowski.

At least 37 records · Page 2Linked to original sources
PubMed1997

The clinical, neuroradiographic, and endocrinologic profile of patients with bilateral optic nerve hypoplasia.

PURPOSE: The purpose of the study was to expand on ophthalmologic and endocrinologic data and report the neuroimaging findings of 35 patients with bilateral optic nerve hypoplasia (BONH). METHODS: A retrospective chart review of 35 children with BONH was conducted. Data on visual acuity, refractive error, and presence of nystagmus and strabismus were collected. Twenty-six children underwent full-endocrinologic evaluation and magnetic resonance imaging or computed tomography scanning. RESULTS: The male:female ratio was 2:1. Ten percent of eyes had visual acuity of 20/60 or better, whereas 34% had no light perception. Eighty-six percent of eyes had acuity less than 20/200, and 80% of patients were legally blind. Most patients (86%) had nystagmus or strabismus or both. Forty-six percent had absence of the septum pellucidum and corpus callosum on neuroimaging. Twenty-seven percent of patients had endocrinologic abnormalities, with growth hormone deficiency being the most common. Panhypopituitarism occurred in 11.5% of children. CONCLUSIONS: Although the visual prognosis of children with BONH generally is poor, 10% of such patients have excellent acuity. In contrast to previous reports, endocrine abnormalities were seen in only one quarter of patients, and the full-blown deMorsier syndrome (septo-optic dysplasia with panhypopituitarism) was seen in only 11.5% of patients with BONH.

Abnormalities, Multiple↗
PubMed1997

Idiopathic orbital myositis.

Idiopathic orbital myositis is a subtype of nonspecific orbital inflammation primarily involving the extraocular muscles. It occurs most frequently in young to middle-aged adults with a 2 to 1 female predominance. The cardinal clinical feature is orbital pain exacerbated by eye movement. Other common findings include diplopia, proptosis (which is generally minimal), conjunctival injection and chemosis, and periorbital edema. Thyroid eye disease is commonly confused with orbital myositis, but the latter is characterized by a more acute onset, more severe pain, and a rapid response to systemic corticosteroid therapy. Echography and CT scanning reveal enlarged muscle bellies and thickened tendons, with low internal reflectivity echographically. Although the cause of orbital myositis is unknown, an immune-mediated pathophysiologic mechanism is likely. This review summarizes recent findings regarding the epidemiology, diagnosis, pathophysiology, and treatment of idiopathic orbital myositis.

Diagnosis, Differential↗
PubMed1997

The effect of edrophonium chloride on muscle balance in normal subjects and those with nonmyasthenic strabismus.

Because the Lancaster red-green test and the Hess screen are not widely used by most ophthalmologists, we used the alternate prism-cover test to study the effect of intravenous edrophonium chloride (Tensilon) on the ocular alignment of 30 normal subjects and 14 individuals with nonmyasthenic strabismus. After measurement of their baseline phorias and tropias, patients received an intravenous injection of Tensilon via the incremental dose technique until autonomic effects of the drug were noted or until 10 mg was administered. Another set of measurements of muscle balance was taken immediately postinjection and 2 and 5 min later. Apart from a small increase (mean, 2 prism dipoters; p = 0.004) in their exophoria at near, normal subjects exhibited no significant change in their phorias after Tensilon injection. One third to one half of the nonmyasthenic strabismics, however, showed a change in their vertical deviation after Tensilon (46% at distance and 38% at near), with the majority of them increasing their angle of squint. These changes were small (mean, 1.7 prism diopters; maximum, 5 prism diopters). In only one case did reversal of the direction of deviation occur. Tensilon produces a statistically significant increase in near exophorias of normal subjects and in vertical distance deviations of nonmyasthenic strabismics. These changes, however, are clinically insignificant and should not be considered to constitute a positive Tensilon test.

Adolescent↗
PubMed1997

Poor stereopsis can support size constancy in albinism.

PURPOSE: The size of a retinal image is inversely related to the distance to the object that generates the image. Normal subjects therefore exhibit size constancy, in which the perceived size of an image is scaled according to its perceived distance. Albinos usually have such poor binocular vision that they perform very poorly on clinical tests for stereopsis. To investigate the functional consequences of this poor stereopsis, we investigated whether stereopsis in these subjects could support size constancy. METHODS: The stereothresholds of 10 albinos and 12 normal control subjects were measured. The presence of absence of size constancy was investigated by having subjects equate the subjective size of stereoscopically presented images whose image disparity indicated that they were at different distances. RESULTS: Laboratory results indicated that eight albinos (including five whose clinical tests indicated a lack of stereopsis) had measurable stereopsis of several thousand are seconds or better. Of these, four also exhibited size constancy. CONCLUSIONS: Albinos who do not demonstrate stereopsis on clinical tests can have stereoscopic perception that commonly used clinical tests do not detect. Moreover, some of these patients even use this poor stereopsis in judging the size of stereoscopically presented images.

Adolescent↗
PubMed1996

Bilateral lateral geniculitis associated with severe diarrhea.

PURPOSE: We examined a 28-year-old woman who developed incongruous binasal and bitemporal visual field defects one week after having a febrile gastroenteritis characterized by severe diarrhea while traveling in Mexico. METHODS: The patient was treated with intravenous methylprednisolone sodium succinate, 1 g daily for three days, followed by an eight-week course of oral prednisone. RESULTS: Neuroradiologic examination demonstrated bilaterally increased signal intensity within the lateral geniculate bodies. Cerebrospinal fluid analysis disclosed isolated protein increase. CONCLUSION: Severe diarrhea may be associated with an aseptic bilateral lateral geniculitis resulting in hourglass-shaped visual fields. Corticosteroid therapy may provide mild improvement.

Adult↗
PubMed1996

Vertical strabismus after cataract surgery.

PURPOSE: To compare anesthesia methods with resultant strabismus patterns in patients with vertical diplopia after cataract surgery. METHODS: The authors analyzed 28 consecutive patients with acquired vertical diplopia after cataract surgery to identify the strabismus pattern. The method of anesthesia administration was available in 21 patients. Three orbital dissections with simulated retrobulbar blocks were performed on cadavers to ascertain the possibility of injuring the vertical rectus muscles at the time of injection. RESULTS: Fifty percent of the involved muscles were overactive, 39 percent were restricted, and 11 percent were paretic. Eleven patients received retrobulbar, and ten received peribulbar anesthesia. The inferior rectus in 17 patients and the superior rectus muscle in 11 were involved. The odds of damaging the inferior rectus, as opposed to the superior rectus muscle, with peribulbar anesthesia was 4.8 times higher than with retrobulbar blocks. Cadaveric dissections showed the likelihood of direct needle injury to either vertical recti with retrobulbar blocks. CONCLUSIONS: In this patient population, permanent vertical strabismus after cataract surgery results more often from overacting or restricted muscles than from primary muscle paresis. Both the superior and inferior recti can be injured with retrobulbar anesthesia, but peribulbar injections affect the inferior rectus muscle more frequently.

Anesthesia, Local↗
PubMed1996

Automated suprathreshold static perimetry screening for detecting neuro-ophthalmologic disease.

PURPOSE: To devise and evaluate a rapid, accurate, and cost-effective method of detecting neuro-ophthalmologic visual field defects. METHODS: One hundred fifty-nine consecutive patients were evaluated with 76-point, central 30 degree automated static threshold perimetry on the Humphrey Visual Field Analyzer, as well as by a 76-point, central 30 degree suprathreshold examination with the central reference levels set at 2 or 4 dB lower than the estimated normal median central reference level adjusted for age. Six masked readers reviewed the fields. Their readings were compared with those of the other observers, as well as with the final diagnoses as determined from all available clinical information. RESULTS: In detecting abnormality, the full-threshold 30 degree test had a sensitivity (percent of eyes with true field defects identified by the field test) of 93 percent or 99 percent (depending on whether borderline results were counted as a positive or negative test) and a specificity (percent of cases without true field defects appropriately identified by the field test) of 71 percent or 91 percent. In comparison, the 4-dB offset suprathreshold test had a sensitivity (averaged over all reviewers) of 79 percent or 87 percent and a specificity of 81 percent or 89 percent, whereas the 2-dB test had a sensitivity of 87 percent or 94 percent and a specificity of 73 percent or 85 percent. The mean duration of the suprathreshold tests was 3.5 +/- 1.0 minute, compared with 14.8 +/- 2.8 minutes for the full-threshold technique. CONCLUSION: The central 30 degree, 76-point, 2-dB offset suprathreshold automated perimetry is more rapid and nearly as effective as the full-threshold test in detecting visual field abnormalities due to neuro-ophthalmologic disease. More quantitative, full-threshold perimetric strategies should be used in all equivocal cases and to follow progression of established disease.

Adult↗
PubMed1996

Color vision measured with pseudoisochromatic plates at five-and-a-half years in eyes of children from the CRYO-ROP study.

PURPOSE: To investigate the prevalence of color deficits at age 5 1/2 years in preterm children with birth weights of less than 1251 g who participated in the multicenter Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) study. METHODS: Two cohorts of CRYO-ROP participants served as subjects: 1055 children who participated in a long-term study of the natural history of ROP at 5 of the 23 CRYO-ROP centers, and 187 children (from all 23 study centers) who had threshold ROP in both eyes and who were randomized to receive cryotherapy in 1 eye. Monocular color vision was tested at age 5 1/2 years, using the Standard Pseudoisochromatic Plates, part 2 (SPP2) for acquired color vision defects. RESULTS: In the Natural History cohort, prevalence of red-green (R-G) color deficits was 6.6% for males and 1.0% for females, similar to that of the general adult population. Prevalence of blue-yellow (B-Y) color deficits was 2.8% for males and 2.2% for females, more than 200 times that in the general adult population. Prevalence of B-Y deficits was not related to birth weight, gestational age, acute-phase ROP, optic atrophy, or retinal residua of ROP, but was related to visual acuity. In the Threshold ROP cohort, color vision deficits were no more likely in eyes that had received cryotherapy than in control eyes. CONCLUSIONS: The results confirm an increased prevalence of B-Y deficits in children born before term, and provide evidence that the increased prevalence is not related to birth weight, gestational age, or severity of ROP within this group of preterm children. No evidence was found to indicate that cryotherapy increased the rate of color vision deficits in eyes with threshold ROP.

Birth Weight↗
PubMed1996

Neurosarcoidosis presenting as an intracranial mass in childhood.

A 13-year-old boy presented with bilateral panuveitis and a superior oblique palsy. Exhaustive laboratory workup was unremarkable, but magnetic resonance imaging (MRI) revealed an enhancing pontine mass. The mass was resected, and histopathology revealed a necrotizing granuloma. Although rare, particularly in the pediatric population, the combination of panuveitis and an intracranial mass likely represents sarcoidosis. Necrosis, although also rare, may similarly be seen in neurosarcoidosis.

Adolescent↗
PubMed1995

Simultaneous, multiple cranial neuropathies in diabetes mellitus.

Cranial mononeuropathies, particularly ophthalmoplegia and facial palsy, are common entities in the diabetic population. Simultaneous multiple cranial neuropathies due to diabetes are much less common, however. We present three patients with this entity.

Abducens Nerve↗
PubMed1994

Clinical and echographic findings in idiopathic orbital myositis.

We performed a retrospective chart review of 100 patients with idiopathic orbital myositis, who were of ages 9 to 84 years. Data from 75 patients gave the following results. Females were affected more than twice as often as males. Fifty-one patients (68%) had single muscle involvement, with the lateral and medial recti affected most frequently (38 cases [33%] and 33 cases [29%] of muscles, respectively). In 34 patients (45%), affected muscles functioned normally; the remaining 55% (63 muscles) were fairly equally distributed between paretic (20%), restrictive (20%), or combined paretic and restrictive (15%) myopathies. Analysis of muscle function, echographic findings, and duration of symptoms indicates that within days of onset of symptoms, the affected muscle is initially enlarged but retains normal function. Within the first two weeks, continued enlargement results in muscle paresis. The muscle may then enter a partially or completely restrictive phase, which may become permanent. Fifty-one patients (68%) responded well to systemic corticosteroids, although 11 patients (15%) had further recurrences of the disease. Seven patients (9%) later developed thyroid eye disease after initially having unimuscular orbital myositis. We advocate early institution of corticosteroids in order to avoid permanent restrictive myopathies.

Adolescent↗
PubMed1994

Ophthalmologic abnormalities in the pediatric cochlear implant population.

To determine the nature and prevalence of ophthalmologic abnormalities in children with congenital or prelinguistic sensorineural deafness, we performed complete ophthalmologic examinations on 54 children aged 2 to 14 years from the University of Miami Ear Institute's Cochlear Implant Program. Of 54 children, 33 (61.1%) had some form of ophthalmologic abnormality, with the majority (24 patients; 44.4%) being refractive errors. Of 54 patients, two (3.7%) had strabismus and two (3.7%) had external adnexal anomalies. One child (1.8%) had cataracts. The cause of the deafness, which had previously been unknown, was determined in three of 54 cases (5.6%) as a result of ocular examinations documenting rubella retinopathy, tapetoretinal degeneration (Usher's syndrome), and iris heterochromia (Waardenburg's syndrome). Routine ophthalmologic examination must be mandated for this population, and the ophthalmologist should be encouraged to play a broader role in the care of children with deficiencies of both vision and audition.

Adolescent↗
PubMed1993

Optic nerve sheath fenestration for treatment of progressive ischemic optic neuropathy. Results in 26 patients.

Optic nerve sheath fenestration was performed in 26 eyes for treatment of the progressive type of common (nonarteritic) anterior ischemic optic neuropathy. During a mean follow-up period of 21 weeks (range, 6 to 52 weeks), results were as follows: visual acuity increased by two or more lines on the Snellen chart in 7 eyes; visual acuity decreased by two or more lines in four eyes; some regression of visual field defects occurred in six eyes, including two eyes in which acuity also improved. These results, attributed to surgical decompression, do not exceed the spontaneous recovery rates reported in the literature pertaining to nonarteritic anterior ischemic optic neuropathy and fail to substantiate the sanguine visual outcome in recently reported series of patients undergoing optic nerve sheath decompression.

Aged↗
PubMed1993

Fluorescein angiography in the diagnosis of giant cell arteritis.

Clinical data and fundus fluorescein angiograms were analyzed from 35 patients with acute (onset less than four weeks) anterior ischemic optic neuropathy. Nineteen of the 35 patients (54%) had nonarteritic disease, and 16 patients (46%) had giant cell arteritis confirmed by biopsy. Patients with arteritis had higher erythrocyte sedimentation rates, larger cup/disk ratios, and delayed fluorescein dye appearance and choroidal filling times. Three additional patients with cranial arteritis confirmed by biopsy, but without visual loss, had angiographic characteristics similar to patients with arteritic ischemic neuropathy. We consider fluorescein angiography a valuable diagnostic adjunct in identifying patients with giant cell arteritis.

Adrenal Cortex Hormones↗
PubMed1993

Serum antibody production to botulinum A toxin.

PURPOSE: Conflicting data have been reported regarding development of serum antibodies to botulinum A toxin. The purpose of this study is to determine conclusively whether antibody production to this toxin occurs in humans, and, if so, to determine its relationship, if any, to length of treatment, total cumulative dose, and clinical response to treatment. METHODS: Sixty-five sera samples from 42 adults treated with botulinum A toxin for essential blepharospasm, hemifacial spasm, or spasmodic torticollis were analyzed via a sphere-linked immunodiagnostic assay for antibody production. Results were plotted against length of treatment, number of injections, cumulative dose, and treatment effect produced. RESULTS: Twenty-four (57%) of the 42 patients produced antibodies in all three diagnostic groups. No significant differences were found between antibody producers and nonproducers with respect to age (P = 0.216), length of treatment (P = 0.586), number of injections (P = 0.619), or total cumulative dose (P = 0.286). Within the antibody-producing group, there was no significant correlation between amount of antibody and length of treatment (P = 0.081), number of injections (P = 0.134), or cumulative dose (P = 0.250). The presence of demonstrable antibodies in serum did not affect the clinical responsiveness to injection. CONCLUSION: Antibody production is present in a majority of patients treated with botulinum A toxin. The sphere-linked immunodiagnostic assay is a reliable and reproducible method for detecting and quantifying these antibodies. When antibody production occurs, it is likely due to variations in individual immune responsiveness and appears to have no direct effect on the patient's clinical response to treatment.

Adult↗
PubMed1993

Congruous quadrantanopia and optic radiation lesion.

Homonymous visual field defects are rare in patients with MS and are usually caused by large lesions in the white matter of the posterior optic radiations and visual cortex. The postulate of Holmes and others, early this century, that optic radiation lesions could produce quadrantic defects has recently been questioned. We report the full recovery of a patient with probable MS who presented with congruous quadrantanopia from a small white matter lesion in the contralateral trigone area.

Brain Diseases↗
PubMed1993

Do thalamic lesions really cause vertical gaze palsies?

Two patients with thalamic infarctions presented with vertical gaze palsies. Magnetic resonance imaging (MRI) demonstrated extension of the lesions into the upper midbrain. A common vascular supply to both areas, and the inability of computed tomography (CT) scans to adequately assess posterior fossa structures, necessitate MRI of the mesencephalon in the assessment of all patients with vertical gaze dysfunction. These cases shed further doubt regarding a specific role of the thalamus in control of vertical eye movements.

Adult↗