Cases reported "Pupil Disorders"

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1/8. Acute orbital compartment syndrome after lateral blow-out fracture effectively relieved by lateral cantholysis.

    PURPOSE: To report the observation of an acute traumatic orbital compartment syndrome in an 80-year-old man. methods: Lateral canthotomy and cantholysis. Computed x-ray tomography. RESULTS: Unilateral proptosis, blindness, a frozen globe and a dilated pupil developed within one hour after a blunt trauma to the left orbital region. Surgery two hours later resulted in normal orbital tension and near-complete recovery of functions. An orbital hematoma was found overlying a lateral blow-out fracture. CONCLUSION: Under favorable conditions, the orbital compartment syndrome can be effectively relieved by lateral canthotomy and cantholysis. The present and previous reports suggest that two hours of orbital ischemia is near the critical time limit for recovery of full visual function.
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2/8. pupil abnormality in amyloidosis with autonomic neuropathy.

    darkness pupil diameters, light reflexes, and redilatation times have been recorded with infrared TV pupillometry in 12 consecutive patients with systemic amyloidosis associated with sensory motor and autonomic neuropathy. Nine of the patients had AL amyloidosis, two had familial amyloidosis associated with a transthyretin abnormality, and one was untyped. The pupils were abnormal in all 12 patients. On the basis of redilatation lag without pupillotonia, six patients had bilateral Horner's syndrome and in one of them amyloid deposits were found in a sympathetic ganglion and in the attached sympathetic chain obtained at necropsy. Four patients had bilateral tonic pupils with light-near dissociation and two had abnormally small pupils with reduced light reactions which could not be characterised. It seems that in patients with systemic amyloidosis generalised autonomic neuropathy is strongly associated with pupil abnormality as shown by tonic reactions with light-near dissociation, by redilatation lag, or by reduced size in darkness.
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3/8. cocaine abuse, generalized myasthenia, complete external ophthalmoplegia, and pseudotonic pupil.

    We present the case of a 29-year-old woman with generalized myasthenia. Myasthenia with complete external ophthalmoplegia was unmasked by cocaine abuse. It was associated with changes of the pupillary motility, including light-near dissociation and positive 0.1% pilocarpine test. Treatment with acetylcholinesterase inhibitors improved the patient's condition rapidly, and led to complete normalization of extraocular movements and pupillary function. To our knowledge, this is the fourth case of cocaine-related myasthenia, and the first case of myasthenia with pseudotonic pupil.
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4/8. Two amino-acid substitutions in the myelin protein zero gene of a case of charcot-marie-tooth disease associated with light-near dissociation.

    charcot-marie-tooth disease caused by mutations of the myelin protein zero gene demonstrates considerable phenotypical variability. We describe a 45-year-old female with a peripheral neuropathy with demyelinating and axonal features, pes cavus and pupillary light-near dissociation. She was heterozygous for two mutations in the myelin protein zero gene (His81Tyr and Val113Phe), both present on the same allele. Our patient shows a less severe phenotype than previously described patients with a His81Arg mutation. Multiple mutations in the myelin protein zero gene, as well as Charcot-Marie-Tooth with pupillary abnormalities have previously been described in rare instances. However, concurrent occurrence of both phenomena is a novel finding.
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5/8. Visual side-effects from transdermal scopolamine (hyoscine).

    Transdermal scopolamine may be used to reduce drooling in children with disabilities. Side-effects include dilated pupils and a reduction in the near point of accommodation (the closest point at which clear vision is possible). Two male children with epilepsy, one with spinal dysraphism (aged 7y 6mo) and one with cerebral palsy (aged 5y 8mo), who have undergone treatment for drooling with transdermal scopolamine are described. Near visual acuity was reduced, and both children showed dilated pupils with reduced or no response to light. These responses became normal on cessation of the scopolamine patch. As the effect of this drug may be cumulative, and many patients are unable to communicate difficulties, clinicians need to be aware of these possible side-effects.
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6/8. spasm of the near reflex associated with cerebrovascular accident.

    spasm of the near reflex most often has a functional basis, but may be associated with organic disease. This case report describes a patient with a progressive 11-year history of spasm of the near reflex associated with cerebrovascular accidents and reviews the differential diagnosis, management and aetiology of this disease.
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7/8. Visual field loss following vitreous surgery.

    OBJECTIVE: To assess possible causes of visual field loss following vitreous surgery. DESIGN: charts of 8 patients prospectively identified, who developed visual field loss following vitreous surgery, were reviewed to characterize this newly recognized syndrome and assess possible causes. RESULTS: Two patients had preexisting chronic open-angle glaucoma and 1 had ocular hypertension. Indications for surgery included 4 eyes with macular holes, 1 eye with epiretinal membrane, 2 eyes with rhegmatogenous retinal detachment, and 1 eye with retinal detachment and giant retinal tear. All patients received retrobulbar anesthesia. Seven of 8 patients had fluid/gas exchange with installation of long-acting bubbles. In 1 patient with a macular hole, a small hemorrhage was noted along a vessel coming off the nerve superotemporally while attempting to engage the posterior cortical vitreous intraoperatively. This patient developed an inferior visual field defect. No intraocular pressure (IOP) measurements greater than 26 mm Hg were recorded in any eye perioperatively. Visual field defects included 4 eyes with inferotemporal defects, 2 eyes with inferior altitudinal defects, 1 eye with a cecocentral scotoma, and 1 eye with a superonasal defect. Only 1 patient had worsened visual acuity. A relative afferent pupillary defect was observed in 4 eyes and disc pallor in 5 eyes. CONCLUSIONS: Central or peripheral visual field loss can now be recognized as a possible complication of vitreous surgery. In some cases, a relative afferent pupillary defect and optic disc pallor are present, suggesting that the optic nerve is the site of injury. Possible mechanisms include ischemia due to elevated IOP or fluctuations in IOP, optic nerve damage from retrobulbar injection, direct intraoperative mechanical trauma to the optic nerve, indirect injury from vigorous suction near the optic nerve leading to shearing of peripapillary axons or vessels, or a combination of these. Certain optic nerves may be more susceptible to injury because of preexisting compromise from glaucoma or vascular disease.
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8/8. Congenital central hypoventilation syndrome: ocular findings in 37 children.

    BACKGROUND: Congenital central hypoventilation syndrome (CCHS) is a rare cause of central sleep apnea. Although ophthalmic abnormalities have been reported, the ocular findings have not been discussed in detail. methods: We examined or obtained the records of 37 children with CCHS. RESULTS: Twenty-seven patients were found to have abnormal pupils, most of which were miotic and reacted poorly to light. In 18 cases, the anterior surface of the iris was unusually smooth. Ten of the children with abnormal pupils also demonstrated light-near dissociation. Twenty had strabismus of various types, and 18 showed evidence of convergence insufficiency. CONCLUSIONS: The high incidence of strabismus, pupillary abnormalities, and convergence insufficiency may be a result of neurologic defects in the midbrain.
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