Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegener's granulomatosis.

AIM: To describe the clinical characteristics of orbital socket contracture in patients with Wegener's granulomatosis (WG). METHODS: A retrospective cohort study The medical records of 256 patients with WG examined at the National Institutes of Health from 1967 to 2004 were reviewed to identify patients with orbital socket contracture. Details of the orbital disease including Hertel exophthalmometry readings, radiological findings, and results of eye examinations were recorded. Orbital socket contracture was defined as orbital inflammation with proptosis followed by the development of enophthalmos and radiographic evidence of residual fibrotic changes in the orbit. To examine for risk factors in the development of a contracted orbit, patients with orbital socket contracture were compared to patients without contracture with respect to multiple variables including history of orbital surgery, orbital disease severity, and major organ system involvement. The main outcome measures were the clinical characteristics of orbital socket contracture associated with inflammatory orbital disease in patients with WG. RESULTS: Inflammatory orbital disease occurred in 34 of 256 (13%) patients and detailed clinical data on 18 patients were available and examined. Orbital socket contracture occurred during the clinical course in six patients; the features included restrictive ophthalmopathy (five), chronic orbital pain (three), and ischaemic optic nerve disease (two) resulting in blindness (no light perception) in one patient. The orbital socket contracture occurred within 3 months of treatment with immunosuppressive medications for inflammatory orbital disease in five patients and was not responsive to immunosuppressive medications. The median degree of enophthalmos in the contracted orbit compared with the fellow eye was 2.8 mm (range 1.5-3.5 mm) by Hertel exophthalmometry. There were no risk factors that predicted development of orbital socket contracture. CONCLUSIONS: In six patients with WG and active inflammatory orbital disease, orbital socket contracture occurred during the treatment course with systemic immunosuppressive medications. The orbital socket contracture, presumably caused by orbital fibrosis, led to enophthalmos, restrictive ophthalmopathy, chronic orbital pain, and optic nerve disease and was not responsive to immunosuppressive therapy. Orbital socket contracture has not been previously reported as a complication of inflammatory orbital disease associated with WG and was an important cause of visual morbidity in our cohort of patients.

Testosterone-related exacerbation of a prolactin-producing macroadenoma: possible role for estrogen.

Men with PRL-producing macroadenomas often present with hypogonadism and impotence. This report documents exacerbation of a PRL-secreting tumor after two separate 200-mg testosterone enanthate (T) injections despite continued bromocriptine (BRC) therapy. A 37-yr-old man with a 60-mm invasive tumor and a serum PRL level of 13,969 +/- 332 ng/ml (mean +/- SD) responded to BRC therapy with rapid disappearance of visual field defect, headache, and facial pain as well as decrease in serum PRL to 5,103 +/- 1,446 ng/ml. T injection was followed by severe headache, facial pain, and increase in PRL to 13,471 ng/ml. Visual field deterioration and increased tumor size (height, 40-43 mm) by computed tomography were documented. A relationship between T injection and exacerbation of the prolactinoma was not recognized until after a second T injection 3 months later. After that therapy, baseline PRL increased from 6,900 to 12,995 ng/ml. The hypothesis that T was aromatized to estradiol, directly stimulating lactotrophs, was supported by an increase in serum estradiol from 24 to 51 pg/ml after the second T injection. Although T treatment is accepted as appropriate therapy for hypogonadism in men with prolactinomas, it may not only interfere with the response of the tumor to BRC therapy, but even stimulate tumor growth and secretion.

Primary aberrant oculomotor regeneration due to intracranial aneurysm.

Two elderly patients had unilateral ophthalmoparesis and retrobulbar pain. Both had subtle lid signs of aberrant regeneration of the third nerve without proceding acute oculomotor paralysis, and both were found to have intracranial aneurysms. Although primary aberrant oculomotor regeneration has been reported previously in patients with aneurysms and meningiomas, the diagnostic importance of this sign in the elderly has not been emphasized. The presence of lid elevation on downgaze associated with slowly progressive ophthalmoplegia in patients aged 65 or older suggests aneurysm in or near the cavernous sinus.

Craniopharyngioma simulating bilateral internal ophthalmoplegia.

A 15-year-old girl had rapid onset of an apparent bilateral internal ophthalmoplegia. Subsequent evaluation revealed a large craniopharyngioma. It is uncommon for a mass to cause such eye findings and unique for a craniopharyngioma to manifest in this fashion.

Delayed visual loss due to trauma of the internal carotid artery.

The group of six patients in this study experienced delayed visual loss following head trauma. Visual loss occurred from 1 day to 13 years after the initial injury. All patients suffered indirect trauma to the internal carotid artery resulting in formation of either an aneurysm or pseudoaneurysm or a carotid-cavernous fistula. Review of the radiologic and clinical findings was performed in six patients. The diagnosis was established by computed tomography, magnetic resonance imaging, and angiography. All patients had follow-up clinical evaluation and imaging studies. Treatment by neurosurgical or interventional neuroradiologic procedures resulted in significant visual improvement in five patients. Different pathophysiologic mechanisms could be correlated with the delayed visual loss produced by the two types of lesions. The pathologic changes associated with the aneurysms/pseudoaneurysms included direct compression of optic nerves and/or chiasm and intracranial hematoma. A carotid-cavernous fistula caused delayed visual loss by either hematoma at the orbital apex or compression of the chiasm and/or optic nerves by saccular dilatation of the cavernous sinus. The delayed onset of decreased vision following head trauma should alert the physician to the possibility of a traumatic aneurysm/pseudoaneurysm or a carotid-cavernous fistula. Different neuro-ophthalmologic symptoms can usually be correlated with the pathologic changes demonstrated by neuroimaging procedures.

Pupillary escape.

I performed a pupillographic study of pupillary escape to determine its effectiveness in detecting optic nerve and retinal disease. Fourteen patients and 39 normal subjects were included in the study. Only one patient had abnormal pupillary escape, even though 13 patients had relative afferent pupillary defects. Testing for pupillary escape is not a reliable clinical technique.

Upbeat nystagmus changing to downbeat nystagmus with convergence.

A 35-year-old woman developed upbeating nystagmus while recovering from presumed Wernicke-Korsakoff syndrome. The upbeating nystagmus changed to downbeating nystagmus with convergence. The slow-phase velocity of the downbeating nystagmus varied with the convergence effort. We believe that this unusual nystagmus pattern implies convergence input to the supranuclear centers for vertical gaze.

Supranuclear disturbances of ocular motility in Lytico-Bodig.

We found abnormal supranuclear ocular or lid motility in all of 37 patients with Lytico-Bodig (amyotrophic lateral sclerosis/parkinsonism-dementia complex). Twenty-one patients had pursuit paresis, 18 abnormal vestibulo-ocular reflex (VOR) cancellation, 15 abnormal convergence, 13 abnormal optokinetic nystagmus (OKN), 12 conjugate gaze limitation, nine nystagmus, nine saccadic paresis, and six abnormal fixation. Lid abnormalities included glabellar hyperreflexia in 21, involuntary levator inhibition in three, and blepharospasm in two. Earlier reports have indicated infrequent ocular disturbances in Lytico-Bodig, but we now find supranuclear eye and lid deficits are universal and sometimes very prominent.

Pupillographic characteristics of simulated relative afferent pupillary defects.

Relative afferent pupillary defects were simulated in normal individuals by performing the alternating light test while dimming the light in front of one eye with neutral density filters. Pupillary responses were elicited using a binocular photostimulator and recorded using a binocular television pupillometer. In five subjects, four spatial variables of the pupillary response--contraction amplitude, minimum size, final size, and redilatation amplitude--were measured and compared. Contraction amplitude was found to be the best indicator of small pupil defects. In eight other subjects, the contraction amplitude elicited by using filters from 0.3 to 3.0 log units in density was plotted. Amplitude of consensual responses increased as direct responses decreased, and initial constrictions were visible in many subjects at the 1.8 log unit level. The best method for detecting relative afferent pupillary defects using the alternating light test is to compare contraction amplitudes, looking for consensual responses that are greater than direct responses.

Stunned left ventricular myocardium after exercise treadmill testing in coronary artery disease.

Myocardial stunning (postischemic ventricular dysfunction) occurs in dogs after coronary stenosis following treadmill exercise. Less data are available in humans regarding development of stunned myocardium after exercise. Regional wall motion changes were evaluated in 22 patients with known coronary artery disease using 2-dimensional echocardiography and exercise treadmill testing. Wall motion was scored as 1 = normal, 2 = hypokinetic, 3 = akinetic, 4 = dyskinetic. At least 1 left ventricular segment with normal resting function developed an increase in wall motion score at 15 or 30 minutes compared with values at rest. The wall motion score in the midportion of the ventricular septum increased from 1.0 at rest to 1.6 (p less than 0.004) at 30 minutes after exercise; the basal inferior wall score worsened from 1.0 at rest to 1.9 (p less than 0.01) at 30 minutes after exercise. Coronary angiographic data in these patients revealed that left anterior descending narrowing correlated best with left ventricular septal wall motion abnormalities, whereas right coronary artery and circumflex narrowing best correlated with inferior and posterior wall motion abnormalities. Eight normal adult volunteers with no history of myocardial ischemia also underwent 2-dimensional echocardiography and exercise testing. No wall motion abnormalities were observed at any time after exercise. The present study suggests that in patients with coronary artery disease, exercise treadmill testing may induce regional wall motion abnormalities of the left ventricle that persist greater than or equal to 30 minutes after exercise, an observation consistent with the phenomenon of stunned myocardium.

Epiphyseal dysplasia, microcephaly, nystagmus, and retinitis pigmentosa.

In 1975, we reported two brothers with epiphyseal dysplasia, microcephaly, and nystagmus; one of them also had mild mental retardation. Here we present information on the natural history, particularly with respect to the development of a new visual problem, namely, retinitis pigmentosa.

Visual evoked potential and pupillary signs. A comparison in optic nerve disease.

We measured the pupil cycle time, the relative afferent pupillary defect, and the pattern-reversal visual evoked potential (VEP) in 41 patients with unilateral anterior ischemic optic neuropathy (AION) and 24 patients with unilateral optic neuritis. We speculated that the relative afferent pupillary defect would match the VEP amplitude and that the pupil cycle time would correspond to the VEP latency. We found a correlation between the relative afferent pupillary defect and VEP amplitude in patients with AION, but not in patients with optic neuritis. We also found that the pupil cycle time and VEP latency were weakly correlated, but only in patients with optic neuritis. In these two groups of patients with unilateral optic neuropathy, the most sensitive objective indicator of disease was the relative afferent pupillary defect. The least sensitive indicator was the pupil cycle time.

Functional visual loss. Follow-up of 42 cases.

Forty-two patients with diagnosed functional visual loss were reexamined an average of four years after their initial visit. Twenty-three patients continued to have constricted or spiral visual fields at follow-up. Despite persistent evidence of functional visual defects, few patients were either socially or economically impaired by them. Patients who had other types of visual field loss or loss of visual acuity were more likely to have these signs resolve than were patients with constricted or spiral fields. Concurrent unrelated organic ocular disease was present in 11 patients. In 20 patients, it was not possible to identify psychiatric symptoms either related to the onset of functional eye signs or at follow-up. Treatment did not substantially alter the course.

Pupillography of a relative afferent pupillary defect.

The pupillary reactions during the alternating light test of Levatin consist of bilateral pupillary constriction followed by redilatation each time the light moves from one eye to the other. The pupils constrict less and redilate more in an eye with an optic nerve lesion than in the unaffected fellow eye. The amplitude of pupillary responses and the average pupil size decrease when the light is alternated more rapidly. Placing neutral density filters over the better eye has the effect of decreasing the amplitude of pupillary responses when that eye is stimulated while increasing responses of the diseased eye. Because of contraction anisocoria and physiologic anisocoria, the most reliable pupillographic and clinical criterion for diagnosing a relative afferent pupillary defect using the alternating light test is an initial pupillary constriction in the suspected eye that is smaller with direct than with consensual stimulation.

Contraction anisocoria resulting from half-field illumination.

We recorded pupillary responses to alternating half-field visual stimuli in 13 normal subjects (eight men and five women ranging in age from 23 to 28 years). We found that stimulation of the temporal visual field caused more pupillary constriction than did equivalent stimulation of the nasal field. Temporal stimuli also produced a direct pupillary response that was larger than the consensual response. Nasal stimulation, however, elicited a larger consensual pupillary response than a direct response. Our findings imply an asymmetric decussation of pupillomotor pathways in the midbrain in humans, as well as greater sensitivity of the nasal retina.

Pupillary constriction during forceful eyelid closure.

Pupillary constriction during forceful eyelid closure was investigated in 30 normal subjects. The right eye of each subject was filmed with an infrared-sensitive television camera in darkness before, during, and after eyelid closure. Clinically detectable constrictions were found in nine of the 30 subjects (30%). No significant correlation was found between amplitude of constriction and age of subject or baseline pupillary diameter. Forceful eyelid closure should be considered as a technique to elicit pupillary constriction in patients with a poor near response, but many subjects will have no detectable constriction using this maneuver.

Relative afferent pupillary defects in optic neuritis.

We measured relative afferent pupillary defects in 105 patients with various stages of optic neuritis. We detected pupillary defects in 96% of acute unilateral cases, 92% of recovered unilateral cases 91.7% of acute cases with evidence of optic neuropathy in the other eye, and 65.8% of recovered bilateral cases. With careful testing, one can find relative afferent pupillary defects in nearly all patients with unilateral optic neuritis and in most patients with bilateral disease. A patient without a pupillary defect after apparent unilateral optic neuritis frequently has evidence of disease in the other eye when visual-evoked potentials are tested.

The Ahmed valve in refractory pediatric glaucoma.

PURPOSE: To evaluate the efficacy of the Ahmed Glaucoma Valve implant in pediatric patients with refractory glaucoma. METHODS: A retrospective chart review was conducted of 27 eyes from 23 consecutive pediatric patients (younger than 18 years) with refractory glaucoma treated with Ahmed Glaucoma Valve implant placement. The main outcome measure was time after surgery without failure. Success was defined as an intraocular pressure of 21 mm Hg or less without visually devastating complications or additional glaucoma surgery (exclusive of tube revision). Previous cycloablation, which was present in a subset of eyes, was examined as a possible influence on postoperative intraocular pressure control, glaucoma medication dependence, and surgical success. RESULTS: The median patient age was 4.8 years (range, 0.3 to 16.8 years), with a follow-up of 3 to 31 months (mean, 12.6 +/- 8.2 months). Intraocular pressure was reduced from a preoperative mean of 32.8 +/- 7.5 mm Hg to 16.7 +/- 5.4 mm Hg at 18 months postoperatively, with an overall success rate of 85.2% at last follow-up. Cumulative probabilities of success by Kaplan-Meier analysis were 90.6% at 12 months and 58.3% at 24 months. Compared with those that did not undergo previous cycloablation, eyes that had undergone previous cycloablation had a lower mean postoperative intraocular pressure at 3- and 6-month follow-up (P < .001 and P = .057, respectively) and required fewer glaucoma medications at postoperative examination time points up to 18 months (P < or = .01 at each time point). Fifteen eyes (56%) required glaucoma medications after surgery. The most common complication was corneal-tube contact, which occurred in five eyes (18.5%). One eye with a wound leak required anterior chamber reformation. Retinal detachment occurred in one aphakic eye 2 years postoperatively, resulting in loss of vision. One eye with neovascular glaucoma required enucleation after an enlarging (presumably preexisting) medulloepithelioma was discovered. CONCLUSIONS: Ahmed Glaucoma Valve implantation is a useful therapy for the treatment of refractory pediatric glaucoma, and it may be safely used in a subgroup of eyes with uncontrolled intraocular pressure despite previous cycloablation procedures.

Argon vs diode laser trabeculoplasty.

PURPOSE: To compare the efficacy of diode laser and argon laser trabeculoplasty in a randomized prospective study of 11 paired fellow eyes. METHODS: Fellow eyes of 11 patients, having had no prior laser trabeculoplasty and requiring laser trabeculoplasty to lower intraocular pressure, were randomly assigned prospectively to diode laser trabeculoplasty in one eye and argon laser trabeculoplasty in the other eye. RESULTS: In the diode laser group, the average baseline intraocular pressure was 21.6 +/- 2.0 mm Hg before trabeculoplasty and 19.6 +/- 2.1 mm Hg (or a 7.7% +/- 11.5% mean pressure reduction) at 1 month, 19.3 +/- 2.6 mm Hg (or a 6.9% +/- 13.5% mean reduction) at 2 months, and 19.0 +/- 3.3 mm Hg (or a 2.4% +/- 16.9% mean reduction) at 3 months postoperatively. In the argon laser group, the average intraocular pressure was 24.4 +/- 3.5 mm Hg before treatment and 17.6 +/- 1.7 mm Hg (or a 24.7% +/- 11.4% mean pressure reduction) at 1 month, 16.8 +/- 2.5 mm Hg (or a 26.7% +/- 15.3% mean reduction) at 2 months, and 15.5 +/- 1.2 mm Hg (or a 30.0% +/- 16.5% mean reduction) at 3 months after laser trabeculoplasty. The difference between argon and diode laser intraocular pressure reduction was statistically significant at 1 month (P < .01), 2 months (P < .01), and 3 months (P < .05) after treatment. CONCLUSION: Argon laser trabeculoplasty appears to be more effective than diode laser therapy in lowering intraocular pressure during the first 3 months after treatment.

Unilateral nasal hemianopia as a sign of intracranial optic nerve compression.

A 52-year-old woman complained of progressive loss of vision in the right eye. Although the visual acuity was normal, there was a relative afferent pupillary defect, and a mild decrease in color vision, mild optic disk pallor, and a nasal field defect along the central vertical meridian. The left eye was normal. Craniotomy disclosed a giant right-sided carotid-ophthalmic artery aneurysm.