Variable diplopia and blepharoptosis after orbital floor fracture repair.
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Biomedical subjects
Publications and source records attributed to T M Bosley.
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OBJECTIVE: To describe three patients with giant cell arteritis (GCA) who suffered profound, irreversible visual loss due to delayed initiation or discontinuation of corticosteroid therapy pending results of histopathologic examination of temporal artery biopsy specimens and to review the appropriate management of GCA once the clinical diagnosis is made. STUDY DESIGN: Case series. SETTING: University-affiliated eye hospital. PATIENTS: Three women with biopsy-proven GCA and bilateral visual loss due to delayed institution or discontinuation of corticosteroid therapy. MAIN OUTCOME MEASURES: Visual acuity and onset, and time course of corticosteroid therapy. RESULTS: Permanent visual loss resulted from delayed initiation of corticosteroid therapy in two patients and discontinuation of therapy in one patient with clinically suspected diagnosis of GCA. In two of these patients, visual loss was preceded by amaurosis fugax and other visual disturbances, although one patient did not volunteer this information to her physician. CONCLUSIONS: Giant cell arteritis may cause rapid and profound loss of vision bilaterally, often without warning. It is essential that systemic corticosteroid therapy be initiated as soon as the diagnosis of GCA is made clinically. Corticosteroid therapy should not be delayed until results of examination of the temporal artery biopsy specimen are obtained. Biopsy specimen interpretation will not be affected if the biopsy is performed within 7 to 10 days of starting therapy.
OBJECTIVES: To determine quantitative and qualitative hemodynamic alterations within the ophthalmic, central retinal, and short posterior ciliary arteries in patients with giant cell arteritis (GCA) proved by biopsy specimen. DESIGN, PATIENTS, AND SETTING: A consecutive case series of patients with GCA referred to an urban eye hospital who were evaluated with color Doppler imaging that was used to analyze orbital blood flow velocities and vascular resistance in 22 consecutive patients with GCA compared with age and sex-matched controls. RESULTS: Patients with GCA all demonstrated significantly reduced central retinal and short posterior ciliary arterial mean flow velocities as well as significantly increased vascular resistance compared with matched controls. Ophthalmic artery mean flow velocity demonstrated marked variation depending on the anatomic location studied. Other color Doppler imaging characteristics of GCA included the following: ophthalmic artery aliasing (high velocity and turbulent flow at presumed focal vasculitic stenoses), reversal of flow within the ophthalmic artery, reduced and truncated time-velocity waveforms of the central retinal and short posterior ciliary arteries, and absolute deficits of flow within the central retinal and short posterior ciliary arteries. Aliasing of flow velocity within the ophthalmic artery (two patients) was associated with clinical progression of GCA. CONCLUSIONS: These data support the concept that quantitative and qualitative alterations in blood flow or pathophysiologic mechanisms of visual loss in GCA. This technique may be useful in the diagnosis and management of GCA since some of the color Doppler waveforms observed in GCA have not been seen in non-arteritic optic neuropathy. Treatment with corticosteroids often appears to stop the progression of these hemodynamic abnormalities but generally does not improve preexisting vascular abnormalities.
PURPOSE: To determine whether the "gatekeeper physician system" for evaluating neuro-ophthalmologic problems is cost effective. METHODS: The authors retrospectively reviewed the records of 588 patients referred for neuro-ophthalmologic evaluation between July and December 1989 to determine the frequency and cost of unnecessary diagnostic testing ordered by "gatekeeper physicians." Pre-referral diagnostic testing costs were compared with the cost of neurophthalmologic consultation for four common problems: (1) optic neuropathy; (2) diplopia; (3) ptosis; and (4) proptosis. RESULTS: Between 16% and 26% of patients in the first three diagnostic categories were subjected to overtesting, resulting in $57,900 of excessive costs, a 724% overcharge. Although the evaluation of proptosis was performed correctly, the quality of 10 of the 18 neuro-imaging procedures was substandard. CONCLUSIONS: The gatekeeper system managed by primary care physicians for these four neuro-ophthalmologic problems not only did not conserve healthcare dollars but also had a negative impact on cost control. For neuro-ophthalmologic disorders, prompt subspecialty evaluation and examination appear to be a cost-effective strategy.
PURPOSE: The purpose of this study is to examine changes in color Doppler imaging parameters before and after optic nerve sheath decompression (ONSD) for chronic papilledema caused by pseudotumor cerebri (PTC). METHODS: Color Doppler imaging was performed within 48 hours before surgery and within 48 hours after the procedure using a color Doppler unit with a 7.5-MHz phased linear transducer. Pulsed Doppler spectrum analyses were recorded digitally on videotape from the ophthalmic, central retinal, and short posterior ciliary arteries, using a 0.4 x 0.6-mm sample volume. RESULTS: Blood flow velocities in the ophthalmic, short posterior ciliary, and central retinal arteries of 24 eyes were significantly decreased compared with a healthy age-matched group. Eyes with visual acuities worse than 20/30 before surgery had significantly decreased velocities in the ophthalmic, short posterior ciliary, and retinal arteries, whereas in eyes with visual acuities better than 20/30, only the short posterior ciliary and central retinal arteries demonstrated decreased velocities. In addition, Gosling's pulsatility index was increased for the central retinal artery but not the ophthalmic or short posterior ciliary arteries. Thirteen eyes improving in visual acuity and field after ONSD demonstrated significant improvement in all color Doppler imaging parameters for the short posterior ciliary arteries. The ophthalmic artery diastolic velocity also increased significantly but the central retinal artery parameters did not change. The eyes that remained stable or worsened did not demonstrate significant postoperative changes. CONCLUSION: These results suggest that some of the visual loss from chronic papilledema may be due to ischemia, and worsening visual acuity correlates with greater impairment of the retrobulbar circulation. One of the mechanisms by which ONSD improves visual function may be reversal of this ischemic process.
PURPOSE: The purposes of this study are to evaluate the retrobulbar circulation in progressive nonarteritic ischemic optic neuropathy (NAION) and to assess changes in blood flow after optic nerve sheath decompression (ONSD). METHODS: Twenty-five patients with progressive NAION were studied using color Doppler imaging (CDI) before and after ONSD. Blood flow velocities and vascular resistance were calculated for the ophthalmic artery, central retinal artery, and posterior ciliary arteries in each eye. Contralateral eyes served as the control group. RESULTS: Preoperatively, the study group demonstrated significantly lower blood flow velocities in the central retinal artery (P < 0.002) and posterior ciliary arteries (P < 0.02) when compared with the contralateral control group. Postoperatively, there was a significant increase in blood flow velocity in the ophthalmic artery (P < 0.04) and the central retinal artery (P < 0.05) as well as a significant decrease in vascular resistance in the posterior ciliary arteries (P < 0.02) in the study group. There were no significant changes in blood flow velocity or vascular resistance in the contralateral control group. Long-term follow-up on eight patients suggests a persistence of this trend. Seventeen of the 25 operated eyes demonstrated a postoperative improvement in visual function, defined as a gain of two lines or more in Snellen visual acuity or at least 20 degrees of visual field expansion. CONCLUSIONS: These data demonstrate that eyes with acute NAION have impaired blood flow when compared with the contralateral control group. Furthermore, they suggest that ONSD may improve blood flow to the ischemic optic nerve halting the progression of visual loss and in some cases improving visual function.
BACKGROUND AND PURPOSE: Ocular pulse amplitude, the amplitude of the ocular pneumoplethysmographic waveform, is altered in several ophthalmologic diseases that disturb ocular blood flow, implying that ocular pulse amplitude may provide an estimate of ocular blood flow. Because ocular blood flow currently cannot be quantified in humans, two experiments were undertaken to evaluate the association of ocular pulse amplitude with total body blood flow. METHODS: In experiment 1, cardiac output was determined by cardiac catheterization in 181 patients who underwent OPG-Gee testing during the same hospitalization. In experiment 2, 110 instances of atrial arrhythmia captured on ocular pneumoplethysmographic tracings were evaluated for transient changes in heart rate (R-R ratio) associated with transient changes in ocular pulse amplitude (ocular pulse amplitude ratio). RESULTS: In experiment 1, average ocular pulse amplitude in the two eyes (OPAAV) was significantly correlated with cardiac output/heart rate (r = 0.53; p < 0.0001) and cardiac index/heart rate (r = 0.43; p < 0.0001). In experiment 2, R-R ratio was significantly correlated with ocular pulse amplitude ratio (r = 0.85; p < 0.001). CONCLUSIONS: These results show that ocular pulse amplitude, a physiological measurement obtained from the globe, is correlated with cardiac output. They imply that ocular pulse amplitude may provide a clinically useful estimate of at least the pulsatile component of ocular blood flow.
Because of the recent finding that a majority of clinically nonfunctioning pituitary macroadenomas in women could be identified as of gonadotroph origin by their LH beta-subunit responses to TRH, we evaluated in this study the value of the LH beta-subunit response to TRH in identifying gonadotroph adenomas in men with clinically nonfunctioning macroadenomas. Thirty-eight consecutively studied men with clinically nonfunctioning macroadenomas were given TRH iv, and intact FSH and LH and LH beta- and alpha-subunits were measured every 15 min for 90 min before and 90 min after. TRH tests were also performed on 15 healthy, age-matched control men and on 12 men with adenomas secreting GH or PRL. Of the 38 men with clinically nonfunctioning macroadenomas, basal values were supranormal in 10 for FSH and in 6 each for alpha- and LH beta-subunits. Responses to TRH were elevated, compared to the healthy, age-matched controls, in 14 for LH beta-subunit and in 5 each for intact FSH and LH. None of the men with adenomas secreting GH or PRL exhibited supranormal responses to TRH. Of the 38 clinically nonfunctioning adenomas, 35 were established in dispersed cell culture, and 29 secreted readily detectable amounts of intact FSH, LH, and LH beta-subunit, strongly suggesting that these adenomas were of gonadotroph cell origin. We conclude that the LH beta-subunit response to TRH can identify gonadotroph adenomas in men with clinically nonfunctioning adenomas better than can basal concentrations of intact FSH and alpha-subunit, alone or combined, but not as well as it can in women.
We evaluated 37 patients with hemifacial spasm and 16 age-matched control patients with other neurological disorders using magnetic resonance (MR) imaging, MR angiography, and MR tomographic angiography. MR tomographic angiography is a new technique using computer reconstruction of MR angiographic images to create coronal angiotomes that display tissue and arterial structures on the same image. Twenty-four of 37 (64.9%) patients with hemifacial spasm had ipsilateral vascular compression of cranial nerve VII or the pons noted by this technique, whereas only 1 of 16 (6.3%) control patients had compression. MR imaging and MR angiography were less sensitive and less specific in evaluating for vascular compression. This study supports vascular compression of cranial nerve VII or the pons as a cause of hemifacial spasm, and demonstrates MR tomographic angiography's value as an excellent, noninvasive technique to demonstrate the compression.
PURPOSE: This study describes hemodynamic characteristics of the ophthalmic, central retinal, and posterior ciliary arteries in 16 eyes of 11 patients with the ocular ischemic syndrome. Understanding the hemodynamic characteristics of the retrobulbar circulation may elucidate the natural history and pathophysiology of the ocular ischemic syndrome and perhaps form the basis for rational treatment of this condition. METHODS: Color Doppler imaging, a procedure that permits rapid noninvasive imaging of the ophthalmic, central retinal, and posterior ciliary arteries, was used to quantitate peak systolic blood flow velocities and vascular resistance (pulsatility index) within these vessels in study group eyes and in an age-matched control population. RESULTS: We demonstrated markedly reduced ocular ischemic syndrome central retinal and posterior ciliary artery peak systolic velocities compared with control group eyes. Central retinal and posterior ciliary artery vascular resistance (pulsatility index) was greater in ocular ischemic eyes versus control group eyes. Reversal of ophthalmic artery blood flow was detected in 12 of 16 ocular ischemic syndrome eyes. Study group eyes with poor vision had no detectable posterior ciliary arterial blood flow. CONCLUSION: Color Doppler imaging quantitates hemodynamic characteristics of the retrobulbar circulation in the ocular ischemic syndrome. There is markedly reduced peak systolic velocity and increased vascular resistance in ocular end arteries such as the central retinal and posterior ciliary arteries. Ophthalmic artery reversal of flow seems to represent collateral blood flow to lower resistance vascular beds. Posterior ciliary artery hypoperfusion may correlate with poor vision in the ocular ischemic syndrome.
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Six patients with thyroid ophthalmopathy presented with what appeared to be a unilateral superior oblique paresis by the three-step test, which was eventually followed by more typical findings of thyroid disease. This early motility defect in thyroid ophthalmopathy may be caused by a restrictive process due to involvement of the inferior rectus muscle. Clues to the proper diagnosis included an increase in vertical deviation in upgaze, elevation of intraocular tension in upgaze, and the lack of excyclodeviation. These features should be assessed in patients with isolated superior oblique paresis.
Congenital homonymous hemianopsia is an uncommon asymptomatic visual field defect discovered typically in young adult life that is caused by a diverse group of insults to the retrochiasmal afferent visual system occurring prenatally, at birth, or during early childhood. We treated eight patients with congenital homonymous hemianopsia; seven with damage involving the optic radiations and one with an abnormality of the optic tract. We performed positron emission tomography using 18F-fluoro-2-deoxyglucose on two patients with dense homonymous hemianopsias, lesions of the contralateral optic radiations, and largely intact occipital cortex. These studies showed minimal abnormalities in resting visual cortex glucose metabolism of the affected visual cortex.
BACKGROUND: Pituitary adenomas that arise from the gonadotroph cells are being recognized with increasing frequency in men, but they are still rarely recognized in women. This rarity could be the result of an actual difference in occurrence or of greater difficulty in recognition. The tumors are usually recognized in men more than 50 years old, but elevated serum gonadotropin levels in women of that age could be produced by normal gonadotroph cells. METHODS: Because the stimulation of follicle-stimulating hormone (FSH), luteinizing hormone (LH), and the beta subunit of LH (LH beta) by thyrotropin-releasing hormone (TRH) is a characteristic of gonadotroph adenomas in men, we administered TRH to 16 women with apparently nonsecreting pituitary macroadenomas and measured serum FSH, LH, LH beta, and the glycoprotein hormone alpha subunit every 15 minutes for 90 minutes before and 90 minutes after. The results were compared with the responses in 16 healthy women matched for age and in 10 women with macroadenomas secreting prolactin, growth hormone, or corticotropin. The tumors from 12 of the women with nonsecreting adenomas were cultured, and the secretion of FSH, LH, and LH beta in culture was determined. RESULTS: Eleven of the 16 women with apparently nonsecreting adenomas had significant increases in serum LH beta in response to TRH, 3 had FSH responses, and 4 had LH responses. None of the 16 healthy women and none of the 10 women with secreting macroadenomas had LH beta, FSH, or LH responses to TRH. Ten of the 12 adenomas that were cultured secreted readily detectable amounts of FSH, LH, and LH beta, and their secretion in vitro correlated with the patients' responses to TRH in vivo. CONCLUSIONS: Most apparently nonsecreting pituitary macroadenomas in women arise from gonadotroph cells. The majority of these can be recognized, even in postmenopausal women, by the serum LH beta responses to TRH, and some can be recognized by the responses of serum FSH and LH.
Color Doppler imaging is a recent development in ultrasonography that allows for simultaneous two-dimensional structural imaging and Doppler evaluation of blood flow. With this technique, one patient with a traumatic carotid cavernous sinus fistula and two patients with spontaneous dural cavernous arteriovenous malformations were evaluated. Color Doppler imaging demonstrated a dilated superior ophthalmic vein with arterialized blood flow in all three patients. In two cases the diagnosis was confirmed by angiography, and in one of these cases the fistula was occluded with a detachable balloon catheter. Postembolization color Doppler imaging revealed return of normal venous flow in the superior ophthalmic vein. This technique offers a noninvasive means to confirm the clinical diagnosis and to track the hemodynamics of these arteriovenous fistulas. In certain cases, color Doppler imaging may eliminate the need for computed tomography and magnetic resonance imaging in the evaluation of suspected arteriovenous malformations of the orbit.
Chronic papilledema from pseudotumor cerebri can cause progressive visual loss despite functioning lumboperitoneal shunts. The authors performed modified optic nerve sheath decompression in 12 patients (16 operations) with functioning lumboperitoneal shunts and progressive visual loss. All of the patients demonstrated improvement in visual function. Optic nerve sheath decompression allows release of localized cerebrospinal fluid (CSF) pressure. By performing optic nerve sheath decompression, progressive visual loss secondary to chronic papilledema can be halted and reversed.
We reviewed retrospectively 114 prednisone dose reduction attempts in 63 myasthenic patients. Dose reduction was considered successful if a patient remained asymptomatic for more than 1 year on no prednisone or a stable low dose of prednisone. Successful dose reduction attempts were more common in patients taking azathioprine, but thymectomy did not influence taper outcome. Slower rate of dose reduction and higher ending dose of prednisone improved the chance of success.
Eight patients were evaluated with 18F-fluorodeoxyglucose positron emission tomography between 3 and 30 days after isolated stroke involving the middle cerebral artery territory that caused homonymous hemianopia. Diffuse hypometabolism was present throughout the damaged cerebral hemisphere, even in cortical areas not obviously ischemic by clinical examination or neuro-imaging. Glucose metabolism in primary and association visual cortex of the damaged hemisphere was decreased by more than 47% (p less than 0.01). Metabolism in the undamaged hemisphere was less profoundly affected, but significant decrements were found in calcarine (40%; p less than 0.01) and lateral occipital cortex (35%; p less than 0.05).