Treatment of acute comitant esotropia in Chiari I malformation.

Purpose: To explore the appropriate treatment of acute comitant esotropia in patients with Chiari I malformation.

Design: Interventional case reports and literature review.

Participants: Two patients with Chiari I malformation presenting with acute comitant esotropia are described.

Tonic ocular tilt reaction simulating a superior oblique palsy: diagnostic confusion with the 3-step test.

Background: The tonic ocular tilt reaction (OTR) consists of vertical divergence of the eyes (skew deviation), bilateral conjugate ocular torsion, and paradoxical head tilt. The head and superior pole of both eyes are rotated toward the hypotropic eye.

Objective: To describe ocular motility and torsion findings in 5 patients with OTRs that mimicked superior oblique palsies (SOPs).

Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression.

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.

High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome.

Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using 99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively.

"Joubert syndrome" revisited: key ocular motor signs with magnetic resonance imaging correlation.

Joubert syndrome is characterized by episodic hyperpnea and apnea, developmental delay, hypotonia, truncal ataxia, ophthalmologic abnormalities, and vermian dysgenesis. We studied 15 patients with the diagnosis of Joubert syndrome to (1) more fully define the syndrome's clinical features, and (2) correlate the clinical features with magnetic resonance imaging (MRI) findings. Eight of 15 patients had a history of episodic hyperpnea and apnea.

Neovascular glaucoma after carotid endarterectomy.

The authors examined a patient who had neovascular glaucoma (NVG) shortly after ipsilateral carotid endarterectomy and followed the patient's clinical course. The authors speculate that the sudden reperfusion after endarterectomy that occurs in patients with combined central retinal artery occlusion and carotid occlusive disease may enhance the circulation of preformed angiogenesis factors, leading to the development of neovascularization. The authors recommend frequent and careful surveillance of patients with central retinal artery occlusion associated with carotid occlusive disease if ipsilateral carotid endarterectomy is performed.

Intact binocular function and absent ocular torsion in children with alternating skew on lateral gaze.

Background: A form of skew deviation, called alternating skew on lateral gaze, resembles bilateral superior oblique overaction. Oblique muscle overaction has been recently speculated to result from loss of fusion with subsequent "free-wheeling" of the torsional control mechanisms of the eyes, causing sensory intorsion or extorsion with attendant superior or inferior oblique muscle overaction, respectively. We wanted to investigate whether loss of fusion plays a role in the pathogenesis of alternating skew on lateral gaze.

Venous stasis orbitopathy: a clinical and echographic study.

Aims: A variety of acute and chronic orbitopathies may be distinguished by standardised echography. Venous stasis orbitopathy (VSO) often presents with orbital signs when secondary to cavernous sinus and middle cranial fossa disorders. In this study, the aim was to assess whether differentiation between vascular and nonvascular causes of VSO could be made on the basis of clinical and echographic features at the time of presentation.

Use of scheimpflug photography to study iris configuration in patients with pigment dispersion syndrome and pigmentary glaucoma.

Purpose: To evaluate the use of Scheimpflug photography in the examination of iris configuration and anterior chamber depth in eyes with pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG).

Methods: The Nidek EAS 1000 Scheimpflug camera was used to photograph the anterior segment of 12 eyes with PDS/PG, as well as 12 age-matched controls and four eyes with primary open-angle glaucoma (POAG). Anterior chamber depth, iris insertion angle, and iris configuration were evaluated.

Fixation duress in the pathogenesis of upper eyelid retraction in thyroid orbitopathy. A prospective study.

Background: Upper eyelid retraction in thyroid eye disease may be caused by proptosis, levator and Müller's muscle infiltration with fibrosis or individual fiber enlargement, excessive sympathetic innervation, abnormal adhesions between levator palpebrae muscle and surrounding tissues, or fixation duress. Fixation duress refers to upper eyelid retraction while fixating with an eye with inferior rectus muscle restriction due to excessive simultaneous firing of the ipsilateral superior rectus and levator palpebrae muscles.

Methods: The authors prospectively examined six patients with strabismus and thyroid eye disease associated with inferior rectus restriction and upper eyelid retraction in whom the eyelid retraction was suspected clinically to be a result of fixation duress.

Spontaneous orbital hemorrhage during general anesthesia and arthroplasty.

A 72-year-old woman who had a history of arthritis and cerebral vascular accident 4 years earlier underwent hand surgery. Because regional anesthesia was not successful, general anesthesia was administered. Shortly before extubation, proptosis developed in her left eye, and she was unable to open it.

Temporary blindness in the TUR syndrome.

The TUR syndrome is a clinical disorder consisting of circulatory, gastrointestinal, and neurologic signs that are infrequently seen in some patients undergoing endoscopic surgery of the prostate gland with the use of nonconductive irrigating fluids. Several theories of its pathogenesis have been proposed. The condition may occasionally be associated with bilateral profound loss of vision.

Value of thallium-201 SPECT imaging in childhood brain tumors.

Thallium-201 chloride single photon emission computed tomography (201Tl SPECT) has been proposed as a diagnostic tool in the assessment of patients with brain tumors. We performed SPECT scans coupled with magnetic resonance imaging (MRI) in children with brain tumors to determine the sensitivity and potential value of SPECT in neuro-oncology. Each patient was injected with 2.

The prevalence of neurologic dysfunction in children with strabismus who have superior oblique overaction.

Background: Children with certain neurologic diseases (hydrocephalus, meningomyelocele, or cerebral palsy) have been reported to manifest a high frequency of A-pattern strabismus and superior oblique overaction. However, it is not generally recognized whether children with strabismus who have superior oblique overaction are more likely to have concurrent neurologic diseases than those without superior oblique overaction. In this study, the authors examine this issue.

Bilateral isolated retrobulbar optic neuropathy in limited Wegener's granulomatosis.

Wegener's granulomatosis causes a variety of ophthalmologic disorders, some of which occasionally constitute the initial presentation of the disease. We describe a patient who presented with bilateral, isolated, consecutive, posterior optic neuropathy with light perception and no light perception vision. The patient had no other symptoms or signs of orbital disease.

Juvenile xanthogranuloma of the corneoscleral limbus.

A 10-year-old boy had a slowly enlarging left limbal mass, extending into the corneal stroma, but not into the anterior chamber. The lesion was excised by lamellar sclerokeratectomy and subsequently confirmed histopathologically as a juvenile xanthogranuloma. Though rare, juvenile xanthogranuloma of the corneoscleral limbus should be considered in the differential diagnosis of limbal mass lesions extending into the cornea.

Magnetic resonance angiography of vascular lesions causing neuro-ophthalmic deficits.

Magnetic resonance angiography (MRA) is a noninvasive, rapidly evolving technique for imaging the intra- and extracranial carotid and vertebrobasilar circulations. It may in some circumstances obviate conventional angiography and the accompanying risks associated with catheterization and contrast injection. MRA exploits the different physical properties between moving protons and stationary tissue to yield flow sensitive data in the form of anatomic images or velocity and flow measurements.

Brainstem glioma: I. Pathology, clinical features, and therapy.

Gliomas that arise in the brain stem have been associated with a poor prognosis. Diagnostic neuroimaging readily identifies the tumor as it extends between normal brainstem structures. Histologic sampling of tumor with stereotactic methods is notoriously unreliable in establishing a definitive prognosis.

Parainfectious optic neuritis and encephalomyelitis. A report of two cases with thalamic involvement.

Two children developed mental status alteration and bilateral profound visual loss secondary to optic neuritis. The clinical picture was consistent with parainfectious encephalomyelitis. Magnetic resonance imaging showed bilateral involvement of the thalamus in both cases.

Alternating skew on lateral gaze. Neuroanatomic pathway and relationship to superior oblique overaction.

Background: Previous studies of patients with heterogeneous, often diffuse neurologic disorders concluded that the neurologic substrate for alternating skew on lateral gaze may be localized at the level of the brain stem tegmentum or the cervico-medullary junction, or both. The localized nature of brain tumors offers an opportunity to further investigate the anatomic localization for this as well as other conditions.

Methods: To test the hypothesis that cervico-medullary and cerebellar lesions are responsible for alternating skew on lateral gaze, the authors investigated a series of 50 children with brain tumors, 39 of whom showed neuro-ophthalmologic abnormalities on clinical testing.

Ocular syphilis.

There has been a steady increase in the number of cases of syphilis in the United States since the middle 1980s, with a dramatic rise in incidence among heterosexual men and women and of congenital syphilis. There also have been changes in geographic distribution of cases and an association with cocaine use. The ophthalmologic manifestations of syphilis are broad.